cardiovascular · cv 042 valvular heart disease ii t1 cv dr. j tam cv 044 development of the heart...

Notes compiled for Pediatrics

Cardiovascular

(Med I, Block 3, CV)

Contents

CV 036 Valvular Heart Disease A CV Dr. J Tam

CV 042 Valvular Heart Disease II T1 CV Dr. J Tam

CV 044 Development of the Heart and Lung L AN Dr. M Torchia

CV 045 Valvular Heart Disease III T5 CV Dr. J Tam

CV 046 Congenital Heart Disease I A PD Dr. R Soni

CV 048 Development of the Heart and Lung A AN Dr. M Torchia

CV 049 Congenital Heart Disease II L PD Dr. R Soni

CV 050 Congenital Heart Disease III T1 PD Dr. R Soni

CV 075 Genetic Aspects of Cardiomyopathy L GN Dr. A Chudley

CV 076 Acquired Pediatric Heart Disease L PD Dr. R Soni

CARDIOVASCULAR COURSE: MED I BLOCK III

Valvular Heart Disease I & II (CV036)

OBJECTIVES: Assigned Reading to be completed prior to CV042

University of Manitoba –Faculty of Medicine

Instructor: Dr. J. Tam

1

Objectives:

At the completion of these sessions, the student will be able to:

1. Describe the common etiology, pathology and pathophysiology of:

a) aortic stenosis

b) aortic regurgitation

c) mitral stenosis

d) mitral regurgitation

e) tricuspid regurgitation

2. Apply the above knowledge to describe the clinical manifestations and physical findings of the

above valvular abnormalities.

3. Describe the laboratory findings (chest x-ray, ECG, echocardiogram) that assist in the diagnosis of

the above entities.

4. Relate the pathophysiology to therapeutic approaches (both medical and surgical).

5. Briefly discuss the indications for surgical intervention of valvular heart disease.

6. List the various surgical options – repair, mechanical replacement, bioprosthetic replacement,

homograft replacement, autograft replacement.

7. Describe the acute and chronic pathologic changes with rheumatic heart disease.

8. Describe the common features of acute rheumatic fever.

Knowledge from these sessions will be integrated with the Clinical Skills Program to allow students to:

1. Differentiate between innocent and pathologic murmurs.

2. Distinguish between regurgitate murmurs, flow murmurs and obstructive murmurs.

3. Assess the severity of valvular heart disease based on physical examination findings alone.

References:

Please feel free to find your own page references within Lilly, Cecil‟s and Harrison‟s textbook and any

other sources of information that you feel are appropriate to allow you to understand the subject

material. Also see notes for Valvular Heart Disease I.

1. Kirklin and Barratt-Boyes, Cardiac Surgery, 1993.

2. Glenn‟s Thoracic and Cardiovascular Surgery, 1996.

3. L. Henry Edmunds, Jr. Cardiac Surgery in the Adult, 1997.

4. Pascoe, Teskey, Bhattacharya. Surgical Treatment of Valvular Heart Disease. Manitoba Medicine;

1989: 59(2).


Valvular Heart Disease I & II – CV042

OBJECTIVES


Instructor: Dr. J. Tam (2006)

2

Objectives:

NB: See assigned study CV036

At the completion of the sessions, the student will be able to:

1. Describe the common etiology, pathology and pathophysiology of:

f) aortic stenosis

g) aortic regurgitation

h) mitral stenosis

i) mitral regurgitation

j) tricuspid regurgitation

2. Apply the above knowledge to describe the clinical manifestations and physical findings of the

above valvular abnormalities.

3. Describe the laboratory findings (chest x-ray, ECG, echocardiogram) that assist in the diagnosis of

the above entities.

4. Relate the pathophysiology to therapeutic approaches (both medical and surgical).

5. Briefly discuss the indications for surgical intervention of valvular heart disease.

6. List the various surgical options – repair, mechanical replacement, bioprosthetic replacement,

homograft replacement, autograft replacement.

7. Describe the acute and chronic pathologic changes with rheumatic heart disease.

8. Describe the common features of acute rheumatic fever.

Knowledge from these sessions will be integrated with the Clinical Skills Program to allow students to:

1. Differentiate between innocent and pathologic murmurs.

2. Distinguish between regurgitate murmurs, flow murmurs and obstructive murmurs.

3. Assess the severity of valvular heart disease based on physical examination findings alone.

References:

Please feel free to find your own page references within Lilly, Cecil‟s and Harrison‟s textbook and any

other sources of information that you feel are appropriate to allow you to understand the subject

material. Also see notes for Valvular Heart Disease I.

1. Kirklin and Barratt-Boyes, Cardiac Surgery, 1993.

2. Glenn‟s Thoracic and Cardiovascular Surgery, 1996.

3. L. Henry Edmunds, Jr. Cardiac Surgery in the Adult, 1997.

4. Pascoe, Teskey, Bhattacharya. Surgical Treatment of Valvular Heart Disease. Manitoba Medicine;

1989: 59(2).

1

VALVULAR HEART DISEASE

James W. Tam, MD, FRCP(C), FACCAssociate Professor of Medicine

[email protected]

Objectives

• 1) Describe the common etiology, pathologyand pathophysiology of :– aortic stenosis (we will cover today)– aortic regurgitation (we will cover today)– mitral stenosis (this is in your notes!!)– mitral regurgitation (we will cover today)– tricuspid regurgitation (do this on your own)

• 2) Apply the above knowledge to describe theclinical manifestations and physical findings ofthe above valvular abnormalities. (today andon own)

Objectives

• 3) Describe the laboratory findings (chest X-ray, ECG, echocardiogram) that assist in the diagnosis of the above entities. (today and PR sessions)

• 4) Relate the pathophysiology to therapeutic approaches (both medical and surgical). (today)

• 5) Briefly discuss the indications for surgical intervention of valvular heart disease. (later today)

• 6) List various surgical options: repair, mechanical replacement, bioprosthetic replacement, homograft replacement, autograft replacement. (read this on your own)

Objectives

• 7) Describe acute and chronic changes with rheumatic heart disease

• 8) Describe the common features of acute rheumatic fever (read on your own)

References

• Lilly, Pathophysiology of Heart Disease, 3rd Edition, Chapter 8, pages 185 - 209; Chapter 2 pages 29 - 43

• Cecil’s Essentials of Medicine, 4th editionpages 10 - 19; 45 - 52

• Berne and Levy, Physiology, 2nd edition, pages 249 - 255

Supplemental Sources:Harrison’s Principles of MedicineBraunwald’s Heart Disease

Supino PG et al, Am J Cardiol 2007; 100: 1274-81.

History

• 54 year old man

• Shortness of breath and chest pain on exertion.

• Began 2 years ago symptoms after running 2k

• Progressed to the symptoms now at about 400m

• WHAT IS THE DIFFERENTIAL DIAGNOSIS?

• WHAT ADDITIONAL INFORMATION WOULD YOU NEED?

2

Differential Diagnosis

• Heart Disease

– Coronary artery disease

– Myocardial disease

– Valvular heart disease

– Pericardial disease

• Pulmonary Disease

– Obstructive Airways disease

– Pulmonary interstitial disease

– Pulmonary arterial disease

• Other

– Gastroesphageal spasm

– Acid Reflux

– Peptic Ulcer Disease

More History

• Central tightness without radiation

• Duration is short, min after slowing or stopping

• Increases with further walking or running

• Not worse with breathing, eating or a change in body position

• Shortness of breath felt as an inability to “catch his breath”

More History

• No orthopnea, PND, fainting, wheezing, cough or bitter taste in the mouth (pyrosis)

• Still able to carry out his usual activities but is unable to participate in weekend sporting events such as basketball or tennis.

• CAN WE REFINE THE DIFFERENTIAL Dx?

Refined Differential Diagnosis

• Heart Disease– Coronary artery disease

– Myocardial disease

– Valvular heart disease

– Pericardial disease

• Pulmonary Disease– Obstructive Airways disease

– Pulmonary interstitial disease

– Pulmonary arterial disease

More History

• Previously healthy, without known hypertension, diabetes or abnormal cholesterol

• Never smoked

• No known history of asthma, previous lung infections or allergies to medications or environmental elements

• No history of rheumatic fever

• Brother with a heart condition in his 50’s, which required open heart surgery; ?? details

Take a Poll of Likely Diagnosis

• A) Coronary artery disease

• B) Obstructive valvular heart disease

• C) Regurgitant valvular heart disease

• D) Primary pulmonary hypertension (primary pulmonary arterial disease)

• E) Pulmonary fibrosis

3

Physical Examination

• Average build, normal appearance

• No apparent distress

• HR 72 and regular. BP 150 / 90 both arms

• Jugular venous pressure is normal and does not increase with abdominal compression

• Carotid pulse is delayed and diminished

Physical Examination

• Apex beat is prominent and sustained

• S1 normal. S2 is single. S4

• Loud late peaking systolic ejection murmur hearteverywhere, radiating well to the base of the neck

• No increase with handgrip or with standing

• No diastolic murmur is heard

• Chest exam is normal

• Remainder of physical exam also normal

http://www.blaufuss.org/tutorial/#

I just found this tutorial site!

Author won educational award for this

Murmurs

• “The noise of bloodflow in motion”• May or may not be related to valvular disease

(eg. “flow” murmur)• May or may not represent PATHOLOGY• May or may not represent ABNORMAL VALVE• Characteristics of murmur and association with

other cardiac findings ESSENTIAL in diagnosis• Essential to have an “approach” to murmurs

Murmurs

Systolic murmur

"Flow"

ValvarAortic

Stenosis

Supravalvar

or membranous

subvalvar

Aortic Stenosis

Fixed

Muscular

Hypertrophy

(HOCM or

IHSS)

Dynamic

Obstructive

Left sided Right sided

(? valvar PS

subvalvar PS

supravalvar PS)

Ejectionor flow

TricuspidRegurgitation

Right sided

MitralRegurgitation

VentricularSeptal Defect

Left sided

Regurgitant

Re-order Differential Diagnosis

??

4

Time for Another Poll

• A) Valvular aortic stenosis

• B) Subvalvular aortic stenosis(Hypertrophic cardiomyopathy)

• C) Supravalvular aortic stenosis

• D) Mitral regurgitation

• E) Coronary artery disease

Pathology of the Obstruction

• Anatomy of aortic valve and possible location of obstruction

• SUBVALVULAR structures

• Valve leaflets (VALVULAR obstruction)

• SUPRAVALVULAR structures

VALVULAR Obstruction

• Calcific aortic stenosis

• Congenital defect (bicuspid or unicuspid)

• Rheumatic aortic stenosis

• SHOW EXAMPLES from CD ROM

Pathophysiology of Aortic Stenosis

• Valvular thickening produces fixed obstruction

• Obstruction to forward flow diminishes oxygen supply

• Compensatory hypertrophy of LV

• Increases oxygen demand - - - -> angina

• Elevation in filling pressures transmitted back to the lungs - - - > SOB and eventually pulmonary edema

Investigations

• Of consequences of AS: ECG, CXR (show)

• Of structure of AS: echocardiogram (show)

• Of severity of AS: Doppler echocardiogram (show)

Natural History of AS

• Asymptomatic phase long but variable

• Symptomatic phase

– Median survival with angina = 5 years

– Median survival with syncope = 3 years

– Median survival with CHF = 2 years

– Median survival with Afib = 6 months

5

Treatment

• Antibiotic prophylaxis no longer required

• Avoidance of situations producing hypotension

• Indications for surgery: SYMPTOMS in proper clinical setting

• Surgical options:

– Percutaneous balloon valvuloplasty (POOR)

– Surgical repair and decalcification

– Valve replacement (mechanical, bioprosthetic, homograft, autograft)

Take a break

Other Valve Lesions

• Mitral Stenosis

• Mitral Regurgitation

• Aortic Regurgitation

• Tricuspid Stenosis

• Tricuspid Regurgitation

• Pulmonic Stenosis

• Pulmonic Regurgitation

• Any combination of above

Mitral Stenosis

• Etiology: Rheumatic Heart Disease (see previous lecture), rarely CONGENITAL

• Pathology: thickening of leaflets, cords, fusion of commissures

• Pathophysiology:

– obstruction to LA emptying

– increased LA pressure and LA size

– reduced filling of LV and forward output

– increased pulmonary pressure and RV strain

Mitral Stenosis

• Symptoms: (can be predicted from previous slide)

– shortness of breath

– fatigue

– pulmonary congestion and hemoptysis

– peripheral swelling

– chest pain

– palpitations if atrial fibrillation

– severity depends on how much stenosis

Mitral Stenosis

• Signs: (can also be predicted)– JVP has prominent A wave

– RV enlargement (parasternal lift)

– LOUD S1 (thick mitral leaflets close at higher pressure)

– OPENING SNAP (heard in early DIASTOLE)

• as severity of stenosis incr, snap CLOSER to S2

• if valve is heavily calcified and IMMOBILE, then S1 and SNAP not heard

– DIASTOLIC murmur

6

Mitral Stenosis

• Laboratory Findings:– CXR (LA enlargement)

– ECG (LA enlargement or maybe Atrial Fib)

– Echo Doppler (upcoming lecture)

– Cardiac Catheterization (direct pressure measurements)

• Prognosis (Table 8.2 of Lilly)– Asymptomatic = 84% ten year survival

– Mild symptoms = 42%, Moderate = 15%

Mitral Stenosis

• Treatment:– Antibiotic prophylaxis no longer required

– Reduce heart rate if fast (esp in Atrial Fib)

– Diuretics maybe

– 10 y survival 64%

– Balloon valvuloplasty (stretch it open)

– Surgical considerations generally when symptomatic (NYHA functional class 2 or more)

Mitral Regurgitation

• Etiology and Pathology:– Valvular problem (eg. rheumatic, myxomatous or

prolapse, endocarditis)

– Annular (LAE, LVE, pap muscle, calcium)

• Pathophysiology: (if chronic)– progressive LA enlargement

– progressive LV enlargement

– eventual pulmonary hypertension

– eventual LV muscle dysfunction (late)


• Symptoms (of chronic regurgitation):– maybe NONE (especially if early on)

– shortness of breath, fatigue, etc.

• Signs: (extent depends on severity of regurgitation)– enlarged LV (apex dilated and displaced)

– soft S1

– REGURGITANT systolic murmur

• (holosystolic, blowing quality)

– S3 or even diastolic FLOW murmur


• Laboratory:

– CXR (LA and LV enlargement)

– ECG (LA enlargement and LVH)

• recall that LVH denotes “more muscle” which may represent increased wall thickness diffusely as with AS or due to increased LV size/volume as with MR



• Treatment:

– Antibiotic prophylaxis no longer required

– Diuretics

– ?? Medications to reduce blood pressure and to reduce the degree of regurgitation

– Surgery (repair or replace) if symptomatic or even if asymptomatic if there is any hint of ventricular dysfunction (as this is usually irreversible even with surgery)

7

ACUTE Mitral Regurgitation

• NO TIME for the LA and LV to adapt

• SUDDEN increase in LA pressure leading to ACUTE Pulmonary Edema

• SUDDEN loss of forward output leading to SHOCK

• soft S1

• the murmur is still systolic but is NOT loud

• listen for S3

Aortic Regurgitation

• Etiology and Pathology:– Valvular problem (congenital bicuspid, rheumatic,

endocarditis)

– Supporting Structure problem (big aortic root such as aneurysm, ectasia, Marfan)

• Pathophysiology (if CHRONIC):– progressive LV enlargement

– eventual LV muscle dysfunction (late)

• Pathophysiology (if ACUTE):– sudden cardiovascular collapse (SHOCK)


• Symptoms (if chronic regurgitation):– often NONE

– progressive shortness of breath, fatigue, angina

• Signs: (extent depends on severity of regurgitation)– increased pulse pressure, bifid carotid pulse

– enlarged LV (apex dilated, displaced, hyperdynamic)

– soft S2, maybe S3

– REGURGITANT diastolic m (blowing quality)

– Systolic FLOW murmur


• Laboratory:

– CXR (LV enlargement ± dilated aorta)

– ECG (LVH)



• Treatment:

– Antibiotic Prophylaxis no longer required

– Medications to reduce blood pressure and to reduce the degree of regurgitation CONTROVERSIAL

• eg. ACE inhibitors, Calcium channel blockers, hydralazine

– Surgical intervention (similar indications to mitral regurgitation)

Infective Endocarditis

• Infection of the lining (endocardium) of the heart especially the heart valves

– endocardial surface injury (chronic turbulence)

– thrombus formation at site of injury

– bacterial entry into the circulation

– bacterial deposition to injured endocardium

• For most people, the risks of taking prophylaxis antibiotics for certain procedures outweigh the benefits

• Recognize the heart conditions that lead to injury (cyanotic heart disease, prosthetic material including artificial valves, prior endocarditis, heart transplants with valve disease)

• Recognize the situations leading to bacterial entry (dental, GI, GU)

• Recognize the bugs (you will learn treatment later)

8

Valvular Heart Disease

• Questions ??

• Hand over to Dr. Manji

7/21/2009

1

Surgery for Valvular Heart Disease

Rizwan A. Manji

Outline• To Operate or Not to Operate

– Calculation of Risk

– Outcomes post operatively

• Prosthetic valve choices – mechanical, bioprosthetic, homografts, autografts

– Advantages and disadvantages of the types

– When to chose which type

• Anatomy of aortic and mitral valves

Outline (cont’d)• Operative indications and techniques aortic valve

disease– Aortic stenosis – aortic valve replacement, percutaneous aortic

valve replacement

– Aortic insufficiency – aortic valve replacement, aortic valve repair

• Operative indications and techniques for mitral valve disease– Mitral regurgitation – mitral valve repair, mitral valve

replacement

– Mitral stenosis – mitral balloon valvuloplasty, mitral valve replacement

BEFORE WE START…..

Acute vs. Chronic

“Acute” almost always requires surgery or

palliation.

Aortic Valve and Mitral Valve

• Aortic valve – important and generally easy

• Mitral valve – important and much more complicated – “Mitral valve apparatus”

7/21/2009

2

Mitral valve anatomy

The mitral valve consists of the mitral annulus, anterior and posterior leaflets, chordae tendineae, and the papillary muscles. Mitral regurgitation may be due to a disease that primarily affects the valve leaflets, such as mitral-valve prolapse or rheumatic mitral-valve disease, or may result from alterations in the function or structure of the left ventricle, such as those induced by ischemic disease or dilated cardiomyopathy. Reproduced with permission from: Otto, CM. Clinical practice. Evaluation and management of chronic mitral

regurgitation. N Engl J Med 2001; 345:740. Copyright © 2001 Massachusetts Medical Society.

Investigations in most patients

• Echo:

• Angiogram:

NOW WE CAN GET STARTED?

• Does this patient need an operation?

Calculation of Risk and Outcomes Post Surgery

Calculation of Risk• Severity systems used (most look at in-hospital events or events

within 30 days of operation):

– APACHE III8

– Pennsylvania

– New York

– Society for Thoracic Surgeons

–EuroSCORE– Veterans Administration

– Parsonnet

– Canadian

– Northern New England

– Cleveland Clinic

Cardiac Surgery in Adult – 3rd edition

Calculation of Risk (cont’d)• Need to consider pre-operative “frailty” , level of functioning and

independence, cognition as most will have some degree of “hit” post

cardiac surgery (eg. cognitive).

• Need to also consider morbidity that may cause patient – eg. stroke, and renal failure. (Cardiac Surgery in Adult – 3rd edition)

• For many diseases and patient population, cardiac surgery is not curative, it is palliative (exceptions – aortic stenosis, left main, 3VD with decreased ventricular function, others).

• Sometimes conservative medical management may be the best for the patient and the health care system.

http://cardiacsurgery.ctsnetbooks.org/content/vol3/issue2008/images/large/315fig5.jpeg?ck=nck


7/21/2009

3

Calculation of Risk (cont’d)


Calculation of Risk (cont’d)Relative risk of outcome

Variable Serious morbidity (95% CI) Mortality (95% CI) Decreased cost (95% CI) Decreased LOS (95% CI)

Congestive heart failure 4.81 (2.16–5.98) 9.20 (6.02–14.0) 0.56 (0.51–0.63) 0.79 (0.73–0.85)

NYS predicted mortality risk 1.28 (1.16–1.41) 0.93 (0.89–0.97) 0.78 (0.76–0.80)

Type of operation 6.04 (3.48–10.5) 0.43 (0.40–0.47) 0.53 (0.48–0.59)

Creatinine > 2.5 mg/100 mL 0.40 (0.33–0.49) 0.47 (0.38–0.58)

Priority 18.6 (7.42–46.6) 0.53 (0.50–0.56)

Age/RBC volume (per 0.01 unit increase) 6.93 (3.21–11.5) 0.61 (0.55–0.67) 0.32 (0.30–0.36)

Reoperative procedure 0.68 (0.62–0.76)

Preoperative IABP 0.65 (0.56–0.75)

Hypertension 5.62 (2.11–15.2) 0.86 (0.81–0.92) 0.83 (0.78–0.89)

More than one prior Ml 0.83 (0.75–0.91)

Dialysis-dependent renal failure 0.61 (0.47–0.78)

Peripheral vascular disease 0.85 (0.71–0.94)

Prior CNS disease 3.41 (2.99–4.91) 0.81 (0.72–0.92)

COPD 0.87 (0.79–0.94)


Outcomes - Aortic Stenosis Post OROutcomes – Chronic Aortic

Insufficiency Post OR

Long-term postoperative survival in aortic regurgitation

Patients with aortic regurgitation who are in functional class I or II before surgery have a survival after surgery that is similar to that expected in an age and gender matched normal population. In contrast, the outcome among patients who are in functional class III or IV before surgery is worse than for those with functional class I and II (p<0.0001) and worse than expected for a matched control group. Data from Klodas, E, Enriquez-Sarano, M, Tajik, AJ, et al, J Am Coll Cardiol 1997; 30:746.

Outcomes – Chronic Aortic Insufficiency Post OR (cont’d)

Survival in severe chronic aortic regurgitation varies with left ventricular volume

Among 246 patients with severe aortic regurgitation managed conservatively, the 10-year survival of those with a baseline left ventricular end systolic volume (LVESV) 25 mm/m2 was significantly lower than that of patients with an LVESV <25 mm/m2 (34 versus 81 percent). Survival in the patients without left ventricular dilatation was not different from expected. Data from Dujardin, KS, Enriquez-Sarano, M, Schaff, HV, et al, Circulation 1999; 99:1851.

Survival after mitral valve surgery is better for patients in NYHA class I/IIindependent of procedure

Long-term patient survival after mitral valve surgery for chronic mitral regurgitation is better in those with NYHA class I/II compared to those in class III/IV at the time of surgery. This benefit is independent of the type of procedure performed (replacement versus repair), although the outcomes are better in those undergoing repair. Data from Tribouilloy, CM, Enriquez-Sarano, M, Schaff, HV, et al, Circulation 1999; 99:400.

7/21/2009

4

What type of valve to use? Options for Valve Replacement

How to choose a valve?

• Mechanical – durability but 1%/yr risk of bleeding and 1%/yr risk of thromboembolism related to coumadin

• Bioprosthetic – shorter durability (age, renal failure) but less issues with bleeding and thromboembolsim – risks of repeat OR

• Homograft – endocarditis, young age, ROSS procedure

Complications of Mechanical Valve

Anatomical Considerations for the Cardiac Surgeon

7/21/2009

5

Relationships of Valves, Coronaries and Conduction Tissue


Important Structures around Aortic Valve

Edmunds et al. Cardiac Surgery in the Adult 2003

Location of Important Structures for Mitral Valve


General Operative Points

• Median sternotomy

• Cardiopulmonary bypass (post heparinization ~ 35000 units &

start to cool)

General Operative Points (cont’d)

• Cross clamp aorta and inject cardioplegia

• Proceed with specifics of operation

Aortic Valve Surgery


7/21/2009

6

AORTIC INSUFFICIENCY AORTIC STENOSIS

NOTE: Wall thickness

Asymptomatic patients with normal left ventricular (LV) systolic

function

Progression to symptoms and/or LV

dysfunction< 6 percent/year

Progression to symptoms, LV dysfunction, or death

According to LV end-systolic dimension*

>50 mm 19 percent/year

40 to 50 mm 6 percent/year

<40 mm 0 percent/year

Progression to asymptomatic LV dysfunction Less than 3.5 percent/year

Sudden death Less than 0.2 percent/year

Asymptomatic patients with LV systolic dysfunction

Progression to cardiac symptoms More than 25 percent/year

Symptomatic patients

Mortality rate More than 10 percent/year

Natural history of chronic aortic regurgitation mostly based upon data from nine series with a total of 593 patients followed for a mean of 6.6 years Data from Bonow, RO, Lakatos, E, Maron, BJ, et al. Serial long-term assessment of the natural history of asymptomatic patients with chronic aortic regurgitation and normal left ventricular systolic function. Circulation 1991; 84:1625.

ACC/AHA guideline summary: Criteria for selection of an aortic

valve in patients undergoing aortic valve replacement (AVR)

Class I - There is evidence and/or general agreement for the choice of a

mechanical or bioprosthetic valve in patients undergoing AVR in the

following settings

A mechanical valve in patients who already have a mechanical valve in the mitral or

tricuspid position.

A bioprosthetic valve in patients who will not take or are incapable of taking warfarin or

have a major contraindication to warfarin therapy.

Class IIa - The weight of evidence or opinion is in favor of the choice of a

mechanical or bioprosthetic valve in patients undergoing AVR in the

following settings

A bioprosthesis in patients 65 years of age who do not have risk factors for

thromboembolism.

Patient preference can be considered in patients less than 65 years of age:

1. A mechanical valve is reasonable in patients who do not have a contraindication to warfarin therapy.

2. A bioprosthetic valve may be chosen after a detailed discussion of the risks of warfarin therapy compared to the likelihood of repeat valve replacement in the future.

A homograft when aortic valve re-replacement is performed for active prosthetic valve

endocarditis.

Class IIb - The weight of evidence or opinion is less well established for the

choice of a mechanical or bioprosthetic valve in patients undergoing AVR in

the following setting

A bioprosthesis in women of child-bearing age to avoid the problems associated

anticoagulation during pregnancy.

Data from Bonow, RO, Carabello, BA, Chatterjee, K, et al. ACC/AHA

2006 guidelines for the management of patients with valvular heart disease. A report of the American College of Cardiology/American

Heart Association Task Force on Practice Guidelines (Writing

committee to revise the 1998 guidelines for the management of patients with valvular heart disease). J Am Coll Cardiol 2006; 48:e1.

Steps in AVR


http://images.google.ca/imgres?imgurl=http://www.pathology.vcu.edu/education/cardio/images/1f.c.jpg&imgrefurl=http://www.pathology.vcu.edu/education/cardio/lab2.e.html&h=434&w=400&sz=71&hl=en&start=10&tbnid=AOzsOQJO4UYO3M:&tbnh=126&tbnw=116&prev=/images%3Fq%3Daortic%2Bvalve%26gbv%3D2%26ndsp%3D20%26hl%3Den%26sa%3DN

http://cardiacsurgery.ctsnetbooks.org/cgi/content/full/3/2008/857/F3?ck=nck




7/21/2009

7

Various Aortic Valve Repairs

Repair of cusp prolapse

Repair for stress fenestration

Repair for incompetent bicuspid valveRepair for dilated sinotubular junction


LONGNECK FREESTYLE

LONGNECK FREESTYLE LONGNECK FREESTYLE


7/21/2009

8


Pre op Post op

Bentall Procedure

Mitral Valve Surgery

7/21/2009

9

MITRAL REGURITATION MITRAL STENOSIS

Note: Ventricular Geometry

Mitral Regurgitation (cont’d)

• Chronic MR:

– Etiology:

• Primary (problem with valve apparatus)- myxomatous mitral valve (most common in developed world, floppy valves), rheumatic (developing world), infective endocarditis, trauma, anorectic drugs, congenital

• Secondary (functional) – ischemic heart disease, left ventricular systolic dysfunction, hypertrophic cardiomyopathy (papilary muscles don’t work or ventrical is dilated)

Mitral Regurgitation (cont’d)

ACC/AHA Guidelines: Management strategy for asymptomatic patients with chronic severe mitral regurgitation (MR)

LVEF: left ventricular ejection fraction; LVESD: left vendricular end-systolic dimension; AF: atrial fibrillation.•Mitral valve repair may be performed in asymptomatic patients with normal left ventricular function if performed by an experienced surgical team and the likelihood of •successful MV repair is greater than 90 percent. Adapted from Bonow, RO, Carabello, BA, Chatterjee, K, et al. ACC/AHA 2006 Guidelines for the management of patients •with valvular heart disease. A report of the American College of Cardiology / American Heart Association Task Force on Practice Guidelines (Writing committee to revise the •1998 guidelines for the management of patients with valvular heart disease). J Am Coll Cardiol 2006; 48:e1.

ACC/AHA guidelines: Management strategy for symptomatic patients withchronic severe mitral regurgitation (MR)

LVEF: left ventricular ejection fraction; LVESD: left ventricular end-systolic dimension.Adapted from Bonow, RO, Carabello, BA, Chatterjee, K, et al. ACC/AHA 2006 Guidelines for the management of patients with valvular heart disease. A report of the American College of Cardiology / American Heart Association Task Force on Practice Guidelines (Writing committee to revise the 1998 guidelines for the management of patients with valvular heart disease). J Am Coll Cardiol 2006; 48:e1.

7/21/2009

10

Surgical ApproachLateral Atriotomy

Most common approach

Effective in virtually all cases

Interatrial (or Sondergaard’s) groove

Surgical ApproachLateral Atriotomy

Superior extension

+/- Division of SVC

Transseptal extension

Intraoperative Assessmentof Mitral Valve

Direct inspection

–Cold saline injection

–Nerve hook inspection

Transesophageal echo (TEE)

Mitral Valve RepairQuadrangular Resection

Posterior leaflet

–(usually middle scallop)

Pledgeted annular suture

Mitral Valve RepairSliding Leaflet Repair

Reduces height of posterior leaflet

Used in combination with

quadrangular resection

Prophylactic / therapeutic for SAM

in some centers

Mitral Valve RepairTriangular Resection

Less stress on suture line andless distortion of annulus (than quadrangular resection)

7/21/2009

11

Mitral Valve RepairPlication

Most effective for

small prolapsing

segments of

posterior leaflet

Mitral Valve RepairArtificial Chordae

Simple and durable

ePTFE suture from PM to the free edge of prolapsing segment

Gaining popularity

Mitral Valve RepairChordal Transfer

Transpose well-supported segment of posterior leaflet to prolapsing anterior leaflet

Mitral Valve RepairAnterior Leaflet Prolapse

Edge-to-edge (Alfieri) Repair, 1991

Annuloplasty

Purposes

• Reinforces leaflet repair

• Increases coaptation of leaflets

• Restores annular circumference

• Prevents further annular dilatation

Types of Annuloplasties

Pericardium Cosgrove-Edwards

Carbomedics Annuloflex Carpentier-Edwards (C-E) Physio

C-E Classic

Medtronic

7/21/2009

12

AnnuloplastyComplete vs. Partial Ring

Only posterior annulus dilates

Advantages of partial ring

–Reduce risk of injury to AoV, conduction system, & anterior leaflet MV

–Reduce crossclamp & CPB time

Prolapsing middle scallop of posterior leaflet

Annuloplasty sutures

7/21/2009

13

• Post-operative management of valve surgery patients

– Bleeding issues

– Hemodynamics issues

– Rhythm issues

– Anticoagulation issues

– Endocarditis, paravalvular leak, hemolysis

– Patient-prosthesis mismatch

7/21/2009

14

Acknowledgments

1. Dr. Alan Menkis slides – Cardiac Sciences Program – St. Boniface Hospital

2. Dr. Luis Quinonez – Mayo Clinic slides

3. Cardiac Surgery in the Adult – 3rd Edition - Editor: Lawrence H. Cohn

4. Pathophysiology and clinical features of valvular aortic stenosis in adultsCatherine M Otto, MD - www.uptodate.com

5. Pathophysiology, clinical features, and management of acute mitral regurgitation Catherine M Otto, MD – www. uptodate.com

6. Pathophysiology and stages of chronic mitral regurgitation William H Gaasch, MD – www. uptodate.com

7. Etiology, clinical features, and evaluation of chronic mitral regurgitation Catherine M Otto, MD -- www. uptodate.com

8. Course and management of chronic aortic regurgitation in adults William H Gaasch, MD

9. Surgical management of mitral stenosis Matthew J Sorrentino, MD, FACC

10. Various Google Image sites – Mayo Clinic, Yale Medical Center, Ohio University

http://www.utdol.com/utd/content/author.do?authorId=624331







Development of the Heart [CV044]


Instructor: Dr. M. Torchia

3

Objectives

At the end of the study session on the development of the heart, the student should be able to:

1. Describe the developmental events occurring between the third and sixth week, which change the

simple heart tube into the shape that characterizes the adult heart.

2. Explain how the major septa of the heart are formed and discuss the clinically significant atrial and

ventricular septal defects.

3. Discuss the fetal circulation and the changes in the vascular system at birth.

4. Discuss the embryological basis of important congenital defects of the heart and great vessels.

Reference:

1. Moore and Persaud, The Developing Human: clinically oriented embryology 7e. pp. 330-380

Notes:

1. Primitive Heart Tube (Fig. 14-7)

a. Cardiogenic region (mesoderm) is located at the most rostral part of unfolded embryo

b. A pair of endocardial heart tubes develop in cardiogenic region by process of

angiogenesis and neovascularization (note that the myocardium and epicardium form

from surrounding mesoderm)

c. As the embryo folds, endocardial heart tubes approach each other and fuse to form

primitive heart tube i. Generates endothelium, myocardium, visceral pericardium and cardiac jelly

d. Over time, this heart tube elongates and also forms a series of dilations termed:

i. Sinus venosus – becomes coronary sinus

ii. Primitive atrium – becomes atria

iii. Primitive ventricle – become trabeculated parts of ventricles

iv. Bulbus cordis – becomes smooth parts of both ventricles

v. Truncus arteriosus – becomes aorta/pulmonary trunk

e. Elongation of heart tube within confined space of developing thoracic cavity results in

bending and twisting of heart tube.





4

2. Septation of Heart

a. AV Septum (Fig 14-11)

i. Dorsal and Ventral Endocardial (AV) cushions bulge into lumen of heart tube

and fuse at the level of separation between atria and ventricles

1. DEFINITLY EXAM QUESTION

2. Neural crest cells from developing neural tube contribute to

endocardial cushions, thought to control the process

3. Dorsal and ventral aspects, grow toward each other

4. Fuse

5. FIRST PARTITIONING OF THE HEART

6. Act as anchors for partitions, and believed to be the start of AV

valve formation

b. Atrial Septum (Fig 14-13)

i. A crescentic membrane grows from superior to inferior aspect of atria (septum

primum). The ever decreasing foramen primum (window resulting from

formation of septum primum) is obliterated when septum primum contacts AV

cushions.

ii. As the foramen primum is finally obliterated, the cells in the central portion of

the septum primum undergo apoptosis resulting in the formation of the foramen

secundum iii. A second partial septum – the septum secundum, develops (to the right of the

septum primum)

iv. In combination, the septae and foramen act as a “flap valve” (ovale foramen)

allowing blood to shunt R-to-L side of heart in fetus

v. At birth, the pressure changes cause this „valve‟ to close

c. Interventricular Septum (Fig 14-18)

i. Composed of two distinct parts

1. Muscular component (majority of IV septum) grows from floor of

ventricle towards endocardial cushions

2. Membranous component forms from AV cushions and R and L Bulbar

ridges and fuses to the muscular IV septum

d. Aorticopulmonary (AP) Septum (Fig 14-21) – divides truncus arteriosus into aorta

and pulmonary trunk

i. Truncal and bulbar ridges grow in a spiral fashion along length of truncus

arteriosus. Results from neural crest cell migration into the truncus.





5

3. Fetal circulation (Fig 14-46)

a. Shunts

i. Ductus arteriosus – blood shunts from pulmonary trunk to aorta (bypassing the

lungs)

ii. Ductus venosus – blood shunts from umbilical vein to inferior vena cava

(bypassing the liver)

iii. Oval foramen – blood shunts from R to L atrium (bypassing the pulmonary

4. Clinical Correlations a. Cyanotic and non-cyanotic defects

b. Septal defects (p.354-356) – non cyanotic

i. VSD – usually in membranous aspect

c. Persistent truncus arteriosus (p.357) - cyanotic

i. Membrane splitting aorta and pulmonary doesn‟ t form

d. Coarctation of aorta (p.366) – non cyanotic

i. Constriction can be removed

e. Tetralogy of Fallot (p.360)

i. Misaligned truncal ridges

1. Pulmonary stenosis

2. Overriding aorta – overrides the ventricular septum

3. Ventricular septal defect

4. Right ventricular hypertrophy


Valvular Heart Disease III [CV045]

OBJECTIVES



6

Objectives:

Please refer to CV36 and CV42 Objectives.



OBJECTIVES



Question #1: A forty year old female presents to hospital with marked dyspnea. She denies any past cardiac disease. She had

been well until one hour prior to presentation when she noted a thumping in her chest and a sudden onset of

dyspnea. She denies any previous shortness-of-breath on exertion, chest pain, ankle edema nor orthopnea. She

had noticed previous bouts of chest fluttering which lasted a few minutes. These attacks were not associated

with chest pain. She complains of mild chronic fatigue. She also complains of vague bouts of left

inframammary chest discomfort. These discomforts do not seem to be specific on exertion.

On exam, blood pressure was 110/90. Pulse was irregular at 139 beats per minute (see attached). She was in

obvious respiratory distress breathing at 25 breaths per minute. The pertinent points of her physical exam

include prominent crackles throughout both lung fields, a 6 cm jugular venous distention (above the sternal

angle), a normal carotid upstroke and a palpable S1 which was quite loud on auscultation. No murmurs, no extra

heart sounds were otherwise audible. Chest x-ray revealed pulmonary edema and a straight left heart border.

1. What is the likely underlying valvular disease?

2. What has precipitated this bout of pulmonary edema? What is the rhythm?

3. Would you expect her stroke volume to be low, normal or high?

4. After appropriate therapy her heart rate became regular at 70 beats per minute. The following physical

findings were noted:

- a prominent A-wave in the jugular venous pulsations

- a palpable and loud S1

- a loud single S2

- a Grade III/6 diastolic rumble with pre-systolic accentuation

- a prominent opening snap

a) Explain each of the above findings.

b) How does the severity of the mitral stenosis relate to the murmur and the timing of the opening snap?

Question 2:

A fifty year old male presents to the Emergency Room complaining of headache, backache, fever, chills and dyspnea. He had these complaints for about one week following a tooth extraction. He saw a general

practitioner at the onset of the illness who diagnosed "bronchitis" and prescribed oral Ampicillin. Over the last

few days his dyspnea has become quite severe at rest. He now requires four pillows in bed. He denies past

symptoms of dyspnea, chest pain nor palpitations. He has been troubled with psoriasis for many years. He was

told five years ago that he has a "heart murmur" and that no therapy was necessary for this.

On examination, he was in obvious respiratory distress breathing at 30 breaths per minute. His blood pressure

was 160/60. His pulse was 120 and regular. He had palpable radial, ulnar and digital pulses. Capillary pulsations

could be detected in his fingertips. Apex beat was hyperdynamic and felt in the fifth intercostal space just

lateral to the mid-clavicular line. Carotid artery pulsations were quite prominent. A soft S1, a soft A2 with low

P2 was audible. An S3 was present. Grade II/VI high pitch murmur was heard following A2. The murmur

appeared to extend throughout diastole. The diastole murmur was heard best along the sternal border. The

physical examination was unremarkable apart from a few petechiae evident under both eyelids.

../../../JTAM/Local%20Settings/Temporary%20Internet%20Files/OLKF/39c.gif



OBJECTIVES



Explain the abnormal physical findings.

a) What is the underlying valvular heart disease? Is it acute, chronic or both?

b) What investigations are most important in the initial management of this patient?

c) What medications would be of use in stabilizing the patient?

Question 3: A seventy-three year old male collapsed at home and was brought to the Emergency Room by ambulance. On

arrival he was in severe respiratory distress and was promptly intubated for respiratory failure. The past history

obtained from the wife indicates the patient had been unwell for the past six months complaining of exertional

fatigue, dyspnea and chest discomfort. There were two episodes of pre-syncope in the last month. These

episodes were both while the patient was climbing stairs. The patient refused to seek medical advice prior to

today.

On examination the blood pressure was 130/80 and the pulse was 120 beats per minute and regular. The chest

had diffuse inspiratory crackles. The carotid upstroke did not seem slow. The apex beat was in the fifth

intercostal space and was sustained. S1 was normal, S2 was soft and single. A Grade IV/6 mid-pitch systolic

ejection murmur was heard at the aortic area and radiated to both carotids. A higher pitch mid-systolic Grade

II/6 murmur was present at the apex. The EKG revealed left ventricular hypertrophy. The chest x-ray revealed

pulmonary edema, a mild cardiomegaly and a prominent ascending aorta. Some calcification appeared to be

present in the heart shadow on the lateral chest x-ray. An echocardiogram revealed severe calcific aortic stenosis

with severe left ventricular hypertrophy and mildly globally reduced systolic function.

a) What are the mechanisms of dyspnea, chest discomfort and syncope that were present in this patient prior to

presentation?

b) Why is S2 soft?

c) Would you expect the carotid upstroke to be slow? Why is it not slow in this case?

d) What drugs should be used to stabilize the patient?

e) What other investigations are required prior to definitive therapy?

f) What therapeutic options are there beyond medical therapy?

Question 4:

A fifty-four year old male was admitted for elective cholecystectomy. He was found to have cardiomegaly on

chest x-ray. He denies past medical problems or illness. He complains of easy fatigability though he attributes

this to being "out of shape". He denies chest pain on exertion, orthopnea, ankle edema nor palpitations.

On examination, his blood pressure was 120/80 and his heart rate was 70 and regular. He was in no respiratory

distress. His carotid upstroke was normal. The apex beat was hyperdynamic and was felt in the fifth intercostal

space lateral to the mid-clavicular line. A systolic thrill was present in the vicinity of the apex beat. No jugular

venous distension was evident. The S1 was soft, S2 was difficult to hear and a Grade IV/6 pansystolic murmur

was audible throughout the precordium. This appeared to radiate to the axilla though was also faintly heard at

the clavicles and lower neck. A Grade II/6 low pitch diastolic murmur was audible at the apex. An S3 was

present.

a) Explain the physical findings

b) Do you think the systolic murmur is aortic stenosis or mitral regurgitation? Why?

c) Do you think that mitral stenosis is also present?

d) What are possible underlying etiologies of this valvular disease? How could you best determine etiology?

e) Contrast the risks and benefits of surgical correction of the valvular abnormality.

../../../JTAM/Local%20Settings/Temporary%20Internet%20Files/OLKF/39d.gif

7/24/2009

1

7/24/2009

2


Congenital Heart Disease I [CV046]

OBJECTIVES: Assigned Reading


Instructor: Dr. Reeni Soni

7

Objectives:

1. Discuss the transition from fetal physiology to postnatal physiology

2. Discuss classification of types of congenital heart disease

3. Discuss acyanotic heart lesions (left to right shunts vs. obstruction)

4. Discuss features of CHF in children

5. Discuss Eisenmenger Syndrome (etiology, prognosis)

6. Discuss cyanotic heart lesions

7. Discuss diagnostic modalities utilized in pediatrics

8. Discuss common forms of surgical intervention of CHD

9. Discuss complications associated with CHD

Reference:

Cecil. Essentials of Medicine, Ch. 5 pp. 40-44.

The following presentation will be covered in CV049

1

Introduction toCongenital Heart Disease

Dr. Reeni Soni

Section of Pediatric CardiologyDepartment of Pediatrics and Child Health

FE241 (Community Services Bldg.)

Objectives

• Fetal Physiology Review

• Adaptation to Postnatal Circulation

• Basic Pathophysiology

• Investigations

• Congenital Heart Defects

• Management

Fetal Physiology

• Fetal lungs not expanded or utilized for oxygenation of fetal blood (pulmonary circuit has “high resistance”

• maternal placenta responsible for oxygenation (low resistance)

• deoxygenated blood from body mixes with oxygenated blood from placenta in right atrium

Fetal Physiology continued

• Right heart blood is diverted away from the lungs at two levels:

• foramen ovale

• ductus arteriosus

• right sided blood mixes with left atrial blood and descending aortic blood

• development of cardiac structures is completed by week 5 of gestation

Fetal Physiology

PULMONARY

VASCULAR

RESISTANCE

SYSTEMIC

VASCULAR

RESISTANCE

Fetal Hemodynamics

2

Transition of Circulation at Birth

• First breaths result in expansion of lungs and beginning of drop in pulmonary vascular resistance

• disconnection of low resistance placenta results in sudden increase in pulmonary vascular resistance

• increased arterial oxygen level promotes beginning of spontaneous closure of ductus arteriosus

Transition continued

• Ductal closure initially due to smooth muscle contraction followed by permanent fibrosis (usually complete by Day 3)

• increased left atrial pressure results in functional closure of foramen ovale “trap door” followed by permanent sealing

• right ventricular blood now directed to pulmonary vascular bed

Transition continued

• Factors which alter normal transition:

– prematurity (delayed ductal closure)

– neonatal stresses (hypoxia, acidosis, sepsis) result in delayed fall in pulmonary vascular resistance

• majority of fall in PVR occurs in first 48 hours but full drop occurs by 2 months of age

Postnatal Hemodynamics

PULMONARY

VASCULAR

RESISTANCE

SYSTEMIC

VASCULAR

RESISTANCE

Normal Heart Congenital Heart Disease

• Involve alterations in fetal cardiac embryology

• affects 1% of all live births

• defects range from insignificant to life threatening

• may present as murmur, congestive heart failure or cyanosis or combination of above

• timing of presentation depends on type of lesion

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/normal.wav

3

Common Terms in CHD

Stenosis: narrowing of valve, artery

Coarctation: narrowing of blood vessel

Atresia: complete blockage

Regurgitation: valve leakage

Acyanotic Heart Defects

• Most common group

• Subdivided into:

• lesions associated with a left to right shunt

• lesions associated with obstruction

• presentation depends on type of lesion and severity

Lesions with Left to Right Shunt

4 most common types:

• Atrial Septal Defect

• Ventricular Septal Defect

• Patent Ductus Arteriosus

• AV Canal Defect

ASD

VSD PDA

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/asd.wav

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/vsd.wav

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/pda.wav

4

AV Canal Defect Clinical Features

• All of these lesions do not present immediately as they require fall in neonatal pulmonary vascular resistance in order to manifest left to right shunt

• large shunts will usually result in cardiac failure by 2-4 months of age

• may be no clinical signs initially because of equal right and left ventricular pressures

Clinical Features

• All of these lesions do not present immediately as they require fall in neonatal pulmonary vascular resistance in order to manifest left to right shunt

• large shunts will usually result in cardiac failure by 2-4 months of age

• may be no clinical signs initially because of equal right and left ventricular pressures

Eisenmenger Syndrome

• Refers to the development of irreversible pulmonary hypertension in someone who has long standing significant left to right shunt (VSD, AV Canal etc)

• due to permanent hypertrophy of pulmonary arterioles in response to chronically excessive blood flow

• leads to progressive cyanosis and death

Manifestations of L-R Shunt Lesions

• No symptoms if shunt is small

• signs and symptoms of cardiac failure if shunt is large

• frequent respiratory infections

• poor growth

Cardiac Failure in Childhood

• Cardinal Signs:

• tachycardia

• tachypnea

• hepatomegaly

• failure to thrive

• peripheral edema, lung crackles not commonly seen, JVP difficult to assess

5

Acyanotic Lesions with Obstruction

• Usually obstruction of left side of circulation

• most common types:

• aortic stenosis

• coarctation of the aorta

Aortic Stenosis

Coarctation of the Aorta

Interrupted Aortic Arch Cyanotic Heart Lesions

• Usually present in newborn period when ductus arteriosus closes

• suspected when infant presents with cyanosis which fails to correct with supplemental oxygen

Hyperoxic Test: administration of 100% oxygen with failure to raise pO2 above 150 mmHg

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/aosten.wav

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/coarct.wav

6

Pulmonary Stenosis Transposition of the Great Arteries

• Commonest cause of cyanotic CHD in the newborn period

• typically presents with cyanosis without a murmur

• CXR typically shows “egg on a string” cardiac silhouette

Transposition of theGreat Arteries

Pulmonary Atresia Tetralogy of Fallot

4 Hallmark Features:

large VSD

pulmonary stenosis

overriding aorta

right ventricular hypertrophy

commonest cause of cyanotic CHD past neonatal period

https://uofm.medcis.net/http:/red40.medcis.net:8082/dspace/retrieve/7201/Presenatation/pulsten.wav

7

Tetralogy of Fallot Truncus Arteriosus

Tricuspid AtresiaTotal Anomalous Pulmonary Venous

Return (TAPVD)

Hypoplastic Left Heart Syndrome Cyanotic Heart Disease

Stabilization:

• most crucial aspect is to maintain patency of patent ductus arteriosus in ductal dependent lesions…life saving

• this is accomplished by administration of Prostaglandin E1 which acts on ductal smooth muscle…should be started as soon as cyanotic CHD is suspected while awaiting definitive diagnosis

8

Diagnostic Tools

• Echocardiography is mainstay of diagnosis…provides detailed hemodynamic and anatomical information in this age group

• Cardiac catheterization:• hemodynamic measurements, oximetry

• angiography

• CXR, EKG

Fetal Echocardiography

• Current echocardiographic capabilities enable detailed imaging of fetal cardiac structures

• ideal time for assessment is 18-24 weeks

• early diagnosis allows for early parental counselling, surgical planning, termination

• performed by pediatric cardiology

Cardiac Catheterization

• Catheter passed from femoral artery and vein into cardiac chambers…pressures measured in each chamber and oxygen saturation measured at each point

• measurement of intracardiac shunts (I.e.movement of blood between systemic and pulmonary circulations)

Hemodynamic Measurements

• RA pressure usually less than LA pressure

• RV/PA systolic pressure usually < 30% of LV/aortic systolic pressure

• usually no pressure gradient between RV and MPA or LV and aorta

Oximetry

• Refers to measurement of oxygen saturations in various chambers

• right sided saturations usually all the same (60-70%)

• left sided saturations usually all the same (95-100%)

• left to right shunts…acyanotic

• right to left shunts…cyanotic

Important Calculations

• Systemic Blood Flow: amount of blood pumped out to body per minute

• Pulmonary Blood Flow: amount of blood pumped to lungs per minute

• Systemic to Pulmonary blood flow ratio: ratio of flow of one circuit to the other (increased in left to right lesions, decreased in right to left lesions)

9

Fick Principle

• Enables calculations of pulmonary and systemic blood flows

Oxygen Consumption

O2 Content Difference X Oxygen Capacity

Pulmonary to Systemic Flow Ratio (Qp:Qs Ratio)

Aortic Sat - SVC Sat

LA sat - PA sat

should be 1:1 in absence of intracardiac shunt

Management

• Medical management (e.g. diuretics for cardiac failure, prostaglandin infusion)

• interventional cardiac catheterization

• cardiac surgery• definitive repair vs. palliative surgery

• cardiac transplantation

Interventional Catheterization

Interventional Catheterization

10

Cardiac Surgery for CHD

• Complete Repair when possible • eg. VSD, AV Canal, Transposition

• Palliative Surgery when complete repair not feasible (single ventricle circulation)

• all surgery performed out of Manitoba…infants require medical stabilization prior to transport

Definitive Repair Palliative Surgery

• PA Banding

• Systemic to PA Shunts

• Fontan Circulation

Palliative Surgery - Systemic to PA shunt

Bidirectional Glenn Anastamosis

11

Fontan Procedure Fontan Procedure

• Allows deoxygenated blood from SVC and IVC to bypass heart and go straight to lungs

• oxygenated blood returns to heart and is pumped by single ventricle to rest of body

• relies on passive blood flow to lungs (no pump within that portion of the circuit)

• used in any lesion where there are not two viable ventricles

Complications of CHD

• Endocarditis common because of turbulent flow…SBE prophylaxis for dental work, surgical interventions

• chronic cyanosis can cause polycythemia, thrombosis (CVA)

• increased recurrence risk in first degree relatives

Summary

• Congenital heart disease includes a wide spectrum of anatomical problems

• presentation can be predicted based on anatomy and hemodynamics

• each case is unique…essential to reason out the above variables each time

• therapeutic options available for almost all lesions in the present era


Development of the Heart & Lungs [CV048]

OBJECTIVES


Instructor: Dr. M Torchia

8

Objectives:

At the end of the study session on the development of the heart, the student should be able to:

1. Describe the developmental events occurring between the third and sixth week, which change the

simple heart tube into the shape that characterizes the adult heart.

2. Explain how the major septa of the heart are formed and discuss the clinically significant atrial and

ventricular septal defects.

3. Discuss the fetal circulation and the changes in the vascular system at birth.

4. Discuss the embryological basis of important congenital defects of the heart and great vessels.

Reference:

1. Moore and Persaud, The Developing Human: clinically oriented embryology 7e. pp. 330-380.

TAKE THESE NOTES TO THE LAB THEY ARE NOT PROVIDED IN THE LAB



OBJECTIVES



9

Case 1 – Patent Ductus Arteriosus (PDA) Radiological Findings: On the Frontal radiograph, there is cardiomegaly and increased pulmonary vascularity associated with prominence of the pulmonary trunk. The Aortic arch and descending aorta are both prominent. On the lateral view, the left mainstem bronchus is displaced posteriorly by the dilated left atrium. Discussion:

Third most common congential heart lesion (15% of total)

In the fetus the PDA shunts blood from main pulmonary artery to ascending aorta

Postnatal, increased arterial oxygenation leads to closure

Functional closure early, anatomic closure within one week

Patent ductus may be lifesaving in neonates with obstructed LV outflow or severely diminished pulmonary blood flow

In the neonate the systemic pressure>pulmonary artery pressure, leading to a L to R shunt across the PDA from aorta to pulmonary artery

Increased flow in pulmonary artery leads to increased pulmonary shunt vascularity and dilation of the LA, LV, and aorta proximal to ductus

PDA Clinical picture: 1. Classic form without severe pulmonary tension seen in older kids and associated with a continuous murmur

(machinery murmur) 2. That seen in infant with large shunt and cardiac failure 3. PDA with severe pulmonary changes 4. PDA complicating respiratory distress in premature infants 5. PDA coexisting with other cardiac malformation

Other Possible radiological findings:

In a small PDA – may be normal chest X-ray

Large PDA – shunt vascularity and L sided cardiac enlargement

Thoracic aortography can define PDA

Ductus may at times be same caliber as aorta and pulmonary artery



OBJECTIVES



10

Case 2 – Atrial Septal Defect Radiological Findings: Chest X-ray: The chest X-ray reveals minimal cardiomegaly (cardiothoracic ratio of 54%) with prominence of the R heart border. The lateral view shows encroachment on the retrosternal superior mediastinum by the enlarged RV. Pulmonary vascularity is increased and of the “shunt type”. The superior vena cava which normally forms the R lateral margin of the superior mediastinum is not seen. The aorta is normal. Right ventriculography: Film 3 – trabeculated RV with unimpeded flow across the pulmonary valve into a dilated main pulmonary artery and branches Film 4 – the pulmonary veins drain into a normal sized LA Film 5 – loss of normal sharp R margin of the LA with contrast extending to the margin of the RA. Progressive opacification of the dilated RA and simultaneous opacification of the AO and PA Film 6 – retrograde injection into the R upper lobe vein shows pulmonary drainage into the LA. The superior atrial septum is intact with L-to-R shunting through a secundum type atrial septal defect in to the RA Discussion:

Accounts for ~8% of congential heart defects

Most common congential defect presenting in adults

One of the most benign form of congential heart disease

Most common ASD – ostium secundum (60%) 1-4 cm hole in region of fossa ovalis

Hemodynamics – L-to-R shunting of blood through ASD into R heart and the pulmonary vascular bed with concomitant dilation to accommodate toe blood volume overload

L atrium does not enlarge since the increased pulmonary return is immediately shunted across the ASD Other Possible radiological findings:

Normal chest radiograph with small shunts

Moderate size ASD – heart enlarged and increased pulmonary vascularity



OBJECTIVES



11

Case 3 – Aortic Valve Stenosis Radiological Findings: Chest X-ray: The chest radiograph reveals normal heart size: the left ventricle is rounded with the apex pointed downward. The prominent ascending aorta projects to the right of the spine, forming the R superior mediastinal boarder. The aortic arch and descending aorta are normal. On the lateral view, the LV projects posterior to the inferior vena cava, and the retrosternal space is filled in posteriorly by the enlarged descending aorta. Discussion:

Comprises ~6% of congenital cardiovascular lesions

Obstruction of LV outflow occurs at level of aortic valve in 60-75% of cases

Associated with a bicuspid valve in >50% of cases

Supravalvular stenosis is rare

Hemodynamic sequelae – related to degree of obstruction and pressure gradient across the stenotic valve and cardiac output o LV hypertrophy develops in response to increased V end-systolic pressure o Dilation does not occur until stenosis is complicated by ventricular decompensation

Most patients are asymptomatic although may have chest pain and syncope (in severe stenosis)

About 10-15% of infants with aortic stenosis develop CHF in the first 12 months of life; subsequently, CHF rarely occurs during childhood

Other Possible radiological findings:

Normal CXR in mild cases

V hypertrophy combined with dilation results in LV enlargement that is associated with downward projection of cardiac apex (often to a point below level of left hemidiaphragm)

Most consistent finding – post-stenotic dilation of the ascending aorta which is a result of the jet of blood through the stenotic valve striking the lateral aortic walls

Normal AO arch and descending AO are important in differentiating isolated AO valve stenosis from coarctation of AO (aortic valve stenosis and bicuspid aortic valve are also seen in coarctation).


Congenital Heart Disease II [CV049]

OBJECTIVES


Instructor: Dr. Reeni. Soni

12

Objectives:

See objectives and notes from Congenital Heart Disease I CV 46


Congenital Heart Disease III [CV050]

CLINICAL PROBLEMS


Instructor: Dr. R. Soni

13

Objectives:

See Objectives for Congenital Heart Disease I. & II (CV 46 and CV49).

Problem #1

A “pink” 10-week old female infant presents for assessment of a murmur and failure to thrive.

Unremarkable prenatal history and delivery. Normal neonatal cardiac exam at the time of discharge from

hospital on Day 2 of life.

1. What other questions should you ask the parent?

2. What features are important to assess on physical examination?

3. What is the relevance of the presence of the diastolic murmur?

4. What are the features of cardiac failure in an infant?

5. What congenital heart defects can present in this manner?

6. Did the pediatrician make a mistake during his neonatal examination?

7. What is the most important diagnostic tool in this situation?

8. What are the medical/nutritional treatment modalities available?

9. What are indications for surgical intervention?

Problem #2

You are asked to assess a newborn infant at six hours of age because of dusky colour in Thompson. The

baby is fullterm with an uncomplicated prenatal course. The baby‟s vital signs are as follows: heart rate

is 150 beats per minute, respiratory rate is 40-45 beats per minute, BP 70/40 right arm, oxygen saturation

70% in the right hand.

1. What other information do you want?

2. What broad categories of conditions can cause cyanosis in a newborn?

3. Describe your initial approach to the management of this infant?

4. What other test/s available in Thompson may be helpful?

5. What management will you institute while waiting for the baby to be transferred to Winnipeg for a

cardiology assessment?

6. What is the most common cause of cyanosis from CHD in a newborn?

7. What else can be done to stabilize the infant prior to surgery?

Problem #3

A five year old child known to have a large ventricular septal defect since two months of age, and who

has been lost to follow-up, comes to your office. At present here are mild symptoms of exercise

intolerance, the child is mildly cyanosed and has the following cardiac catheterization date.

1. Explain the significance of the various saturations recorded. What is the systemic to pulmonary

blood flow ratio? Is this unusual in the setting of a large VSD?

2. What is the cause of the shunting in both directions across the VSD?

3. What is the status of this child‟s pulmonary vascular resistance?

4. What do you tell the family about this child‟s prognosis?

5. What long-term complications may this child experience?


Congenital Heart Disease III [CV050]

CLINICAL PROBLEMS



14

Problem #4

Two five year old boys are admitted with fever for seven days. The first one has conjunctivitis, rash,

palmar erythema and lymphadenopathy. The second one has pain and swelling of his ankles and knees,

loud murmur and a rash.

1. Describe the difference in your differential diagnosis for both patients.

2. What criteria are required to make a diagnosis of Kawasaki disease?

3. What is the treatment during the acute phase of this condition? How quickly should treatment be

started and why? What other investigation is important?

4. What are the Jones criteria?

5. What other investigations are warranted for the second patient?

6. What treatment does patient B require in the short-term and long-term?


Genetic Aspects of Cardiomyopathy [CV075]

LECTURE NOTES


Instructor: Dr. A. Chudley

15

LEARNING OBJECTIVES

Review classification.

Review prevalence.

Discuss genetic causes and issues of variable expression, penetrance, and heterogeneity

(genetic & molecular ).

Issues of Utility of Molecular testing

Ethics, Costs, Practicality

CARDIOMYOPATHY CLASSIFICATION Cardiomyopathies (from the Greek: kardia, heart; mys, muscle; pathos, suffering) are defined by the

World Health Organization as diseases of the myocardium associated with ventricular dysfunction.

Dilated Cardiomyopathy (DCM): Characterized by dilatation and impaired contractility of the left (or

right) ventricle. Presentation is usually with heart failure. Arrhythmia, thromboembolism, and sudden

death are common.

Hypertrophic Cardiomyopathy (HCM): Characterized by left (or right) ventricular hypertrophy, which is

usually asymmetric and involves the interventricular septum. Typically, left ventricular volume is

reduced. Systolic gradients are sometimes present. Typical presentations include dyspnea, arrhythmia,

and sudden death.

Restrictive Cardiomyopathy (RCM): Characterized by restrictive filling of the left (or right) ventricle

with normal or near normal ventricular contractility and wall thickness. Presentations are usually with

heart failure.

The cardiomyopathies are not the only causes of heart failure syndrome. In western countries, coronary

artery disease with resultant ischemic cardiomyopathy remains the primary cause of the heart failure

syndrome.

Other components to aid in the classification of CM: Age, appearance, etiology (toxic esp. alcohol,

ischemic, metabolic, infectious), genetic

PREVALENCE of CM

HCM: 1 in 500

DCM 1 in 2500

Approximately 30% are confirmed inherited — many more probably are genetic!



LECTURE NOTES



16

FAMILIAL DILATED CARDIOMYOPATHY (DCM)

Several cytoskeletal protein genes are associated with mutations that lead to DCM (e.g. dystrophin,

desmin, tafazzin, lamin A/C, and cardiac actin genes). Most are part of syndromes: Skeletal myopathy or

specific conduction system defects.

DCM as non-syndromic disorders have been linked to 5 different chromosome regions and 2 disease

causing mutations in the cardiac actin gene. All are dominantly inherited. Recently, sarcomeric protein

defects have been confirmed to cause DCM: Cardiac myosin heavy chain gene mutations and troponin T-

mutations which make up about 10% of the familial cases.

FAMILIAL HYPERTROPHIC CARDIOMYOPATHY (HCM)

1. Previously known as IHSS (idiopathic hypertrophic subaortic stenosis)

2. >13 mm LV wall thickness without dilation of the ventricular cavity on cardiac echo

3. Histology shows myofibrillar and myocyte disarray

4. Inherited as an autosomal dominant trait

5. Most with the gene are asymptomatic until there is obstruction or syncope or sudden death in young

people

6. Sudden death in a subset-most due to arrhythmias and massive LVH (left ventricular hypertrophy).

Mortality is 1% per year

There are 10 sarcomeric contractile proteins; all have been associated with mutations that cause HCM:

(Troponin T; β- cardiac myosin heavy chain; cardiac myosin-binding protein C account for most- (Figure

1 genotype-phenotype correlation between the mutations). Over 150 disease causing mutations have been

described, and cardiac myosin binding protein C accounts for 15% (Figure 2).

Treatment:

Β-blockers and calcium channel blocking agents, (promise of ACE inhibitors is emerging), dual chamber

pacing with defibrillator, septal myomotomy-myomectomy, alcohol septal ablation.

Detection

DNA testing for disease causing mutations in these 10 genes is only available to families in Manitoba of

Mennonite background (where one mutation in the myosin-binding protein C gene identifies most

affected due to a founder effect in this population) Testing in families with more than 4 affected

individuals in at least 2 generations is possible but less practical. This recommendation may change in

time as more reliable, inexpensive DNA microchip technology becomes commercially available. Harvard

Laboratories offers a kit that will test a panel of the more common mutations in 7 of these genes. Costs

are very high.

Benefits of DNA testing:

1. Identify those at highest risk

2. Risk stratification for treatment/ genotype-phenotype correlation (See Figure 1)

3. Excludes those not at risk, cost benefit by stopping surveillance



LECTURE NOTES



17

Dilemmas of DNA Testing:

1. Limitations of tests

2. Should we test children?

3. Risk psychological harm

4. Discrimination/ stigmatization (insurance, employer)

5. Needs to be voluntary

Genetic counseling

Counseling is recommended before DNA testing is offered to families. The future is bright for more

targeted therapy as we learn more about the function of these altered proteins and develop more rational

protein specific therapy or through transcriptional or translational inhibition.

Figure 1. Age-Related Penetrance of Familial Hypertrophic Cardiomyopathy Caused by Mutations in the Genes for

Cardiac Myosin-Binding Protein C, Cardiac Troponin T, and Cardiac -Myosin Heavy Chain. Solid bars

denote the percentage of persons with both cardiac myosin-binding protein C mutations and cardiac

hypertrophy. Significant differences in the penetrance of familial hypertrophic cardiomyopathy caused

by cardiac myosin-binding protein C mutations and hypertrophic cardiomyopathy caused by mutations in

cardiac troponin T or cardiac -myosin heavy chain are indicated as follows: asterisks denote P<0.05, the

dagger P<0.005, and double daggers P<0.001. From Niimura et al, N Engl J Med. 1998 Apr

30;338(18):1248-57.



LECTURE NOTES



18

Figure 2.

The Human Cardiac Myosin-Binding Protein C Polypeptide and Gene and 12 Mutations Causing Familial

Hypertrophic Cardiomyopathy. FN denotes fibronectin-like motif, Int intron, DS donor splice site, AS

acceptor splice site A, Del deletion, and Ins insertion. Positive numbers indicate residues following

exons; negative numbers indicate residues preceding exons. The Manitoba Mennonite mutation is

InsG791 in exon 25. From Niimura et al, N Engl J Med. 1998 Apr 30;338(18):1248-57.


Acquired Pediatric Heart Disease [CV076]

OBJECTIVES



19

Objectives:

Discuss the more common forms of acquired pediatric heart disease with respect to:

1. Diagnostic features

2. Management

3. Prognosis

The specific conditions to be discussed will be:

1. Kawasaki Disease

2. Acute Rheumatic Fever

3. Endocarditis

4. Myocarditis

1

Acquired Pediatric Heart Disease

• Reeni Soni, MD FRCPC

• Associate Professor and Head

• Section of Pediatric Cardiology

• Director, Variety Children’s Heart Center

Acquired pediatric heart disease

Acute rheumatic fever (ARF)

Kawasaki disease (KD)

Infective endocarditis (IE)

Myocarditis

Acute rheumatic fever

ARF is an inflammatory disease

sequel to pharyngeal infection with group A beta-hemolytic streptococci – usually follows untreated “strep throat”most likely immune mediatedoccurs after time lagcharacteristic tendency to recur without prophylaxis

Incidence and epidemiology

Typical age of presentation 5 and 15 years

Overcrowding

Genetic predisposition (especially 1st

Nations)

Incidence and epidemiology

❖Primary attack rate 3 % (after Strep throat)

❖Secondary attack rate as high as 5-65 % (hence need for prophylaxis)

❖Recurrences decline over years (determines duration of chemoprophylaxis) only 4-8% of recurrences occur >10 years after last attack

Pathophysiology

Throat infection with rheumatogenic strain

One or more putative streptococcal antigen initiates molecular mimicry

M-like protein -myocardial damage Group A polysaccharide - heart valves Cell membrane antigens - chorea

2

Pathophysiology

Verrucous vegetations on

the leaflets

chordae

subchordal apparatus

Healing with fibrosis and thickening

8

ARF : Jones criteria

Dr. T Duckett Jones - 1944

Revised in 1965 and 1984 and most recently 1987 by the WHO Rheumatic Fever Study Group

Currently used guidelines are referred to as the “Modified Jones criteria”

Acute rheumatic fever : Jones criteria

Major manifestations

Carditis

Polyarthritis - migratory

Chorea

Erythema marginatum

Subcutaneous nodules

Minor manifestations

Clinical

Fever

Arthralgia

Previous rheumatic fever or

rheumatic heart disease

Lab

Elevated acute phase

reactions – ESR, CRP

Prolonged PR interval

ARF : Jones criteria

2 major criteria or 1 major and 2 minor criteria PLUS:

supporting evidence of preceding Strep infection

history of scarlet fever

positive throat culture

increased ASO titer or other antibodies

ARF carditis

pancarditis Valvulitis (usually mitral and aortic)MyocarditisPericarditis

• occurs within first 2 to 3 weeks of ARF episode

3

Carditis Endocardial disease –valvulitis - heart murmurs

Mitral regurgitationAortic regurgitation Carey Coombs murmur - mid diastolic - relative

mitral stenosis [mitral valvulitis and volume related to MR]

Myocarditis – S/S of congestive heart failure

Tachycardia out of proportion to fever, gallop (S3,S4)HepatomegalyTachypnea, crepitationsMuffled heart soundsCardiomegaly – x-rayEKG prolonged PR interval – nonspecific

Carditis

Pericarditis

Chest pain relieved by bending forwards

Pericardial rub

14

ARF – migratory polyarthritis

“migratory polyarthritis” – may move from joint to joint within hours

Occurs early in the disease

Exquisitely painful compared to the degree of swelling

Usually involves the large joints (knees, elbows, ankles)

Responds dramatically to ASA

ARF – migratory polyarthritis

Dramatic response to aspirin

one should reconsider the diagnosis of ARF if inadequate

response

Arthralgia – joint pain without objective

signs of inflammation

ARF – chorea - long latent period

Sydenham’s chorea or St Vitus’s dance

Incidence as high as 30 percent

Female preponderance F:M - 2:1

Only clinical feature which can definitely make a diagnosis of ARF (after excluding other causes of Chorea – Wilson’s)

ARF - chorea

Movement disorder and muscle weaknessAbrupt, purposeless, small amplitude involving the distal muscle groups

Handwriting becomes clumsy

Jack in the box, lizard tongue

Milkmaids grip

Pronator drift - dinner fork deformity

Emotional lability

4

ARF – subcutaneous nodulesOccur after the first few weeks of illness

Almost always associated with carditis

Pea sized

Painless nodules over the bony prominences

without involvement of the skin or the

underlying tissues

Characteristic distribution over the extensor

aspect

ARF – erythema marginatum

ARF: laboratory tests

ASO titer - > 250 units in an adult and > 330 units in a child

Additional evidence of streptococcal infection includes anti-DNase B

ESR, CRP

ARF - Differential diagnosis

Musculoskeletal infectious arthritis, Lyme disease, rheumatoid arthritis, reactive arthritis, allergic reactions, viral infections, hematological disorders

Movement disorders ticks or habit spasms, Huntington’s chorea, encephalitis, attention deficit hyperactivity disorder

Treatment of ARF

Primary prophylaxis - identification and treatment of streptococcal throat infection

IM benzathine penicillin - 0.6 -1.2 million units

Pen V K - oral 10 days

Erythromycin 25 mg/kg/day - 10 days

Reduces attack rate from 3 to 0.3 %

Socioeconomic and sanitary improvement

Treatment of ARF

Secondary prophylaxis IM benzathine penicillin 1.2 million units q 1 mo

Pen V K 500 -1000 mg PO BID

Erythromycin 250 mg PO BID

Duration is controversial Lifetime vs until age of 18 years

5 years if no evidence of carditis

24

5

ARF - Treatment

REMEMBER there is no specific cure for ARF

General supportive treatment

Control of fever, Bed rest - controversial

Eradication of streptococcus - even if throat

culture negative

penicillin, erythromycin, sulfa, cephalosporins,

macrolides

ARF -Treatment

Suppressive therapy using anti-inflammatory agents

Aspirin - 90-120 mg/kg/day, monitor serum levels

Corticosteroids - 2 mg/kg/day (if significant carditis)

Management of congestive heart failure

Diuretics, Afterload reduction, Digoxin, Steroids, ICU

measures - ventilation, inotropic support

Treatment of ARF

As corticosteroids are weaned, aspirin is added to

prevent rebound

Resting heart rate, erythrocyte sedimentation rate,

and CRP used to monitor “rheumatic activity”

Treatment of chorea

Self - limiting - 3 months (6-12 mo)

Rest

Injury prevention

Chlorpromazine, diazepam, haloperidol

Kawasaki disease

• The most common acquired pediatric heart disease in North America

Introduction

Kawasaki disease is an acute, self-limited vasculitis of unknown etiology -predominantly in infants and young children

First described in Japan in 1967 by Tomisaku Kawasaki

6

Introduction

Occurs both endemic and community wide epidemic forms in - all races (but especially Japanese and Filipino populations)

Coronary artery aneurysms or ectasia develop in 15-25 percent of untreated children

Epidemiology

More common during winter and early spring months

Male: female 1.5-1.7: 1

More than 76 % < 5 years old

Etiology and pathogenesis

Remains unknown

Strong suggestion of transmissible infectious etiology

Ubiquitous infectious agent - clinical disease in genetically predisposed

Typical Kawasaki DiseaseClinical diagnosis (no definitive lab test)

> 5 days of fever and > 4 of 5 principal clinical features

Changes in the extremityAcute: erythema of palms, soles; edema of hands, feet

Subacute: periungual peeling of fingers, toes in second and third week

Polymorphous exanthem

Bilateral bulbar conjunctival injection without exudate

Changes in the lips and oral cavity: erythema, lip cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae

Cervical lymphadenopathy (> 1.5 cm), usually unilateral

Diagnosis

Diagnosis suspected - fever of unknown origin

associated with any of the principal clinical features

Diagnosis can be made on the fourth day or earlier

if > 4 principal criteria are present

Patients with fever > 5 days and < 4 principal

features can be diagnosed if coronary artery

disease is noted by echocardiography

Clinical Features

Cardiovascular

Congestive heart failure, myocarditis, pericarditis, valvular regurgitation

Coronary artery abnormalities

Aneurysms of medium-size non-coronary

arteries

7

Clinical pearls - laboratory investigationsKawasaki vs Viral?

Moderately to markedly elevated CRP and ESR is uncommon in viral infections

Platelet counts > 450,000/mm3

Low WBC count , lymphocyte predominance, low platelet count in the absence of DIC suggests viral etiology

Differential diagnosis

Viral infections (measles, adenovirus, enterovirus, Epstein -Barr virus)Scarlet feverStaphylococcal scalded skin syndrome, Toxic shock syndrome, Mercury hypersensitivity reactionBacterial cervical lymphadenitisDrug hypersensitivity reaction, Steven - Johnson syndromeJuvenile rheumatoid arthritisRocky Mountain spotted feverLeptospirosis

Incomplete (atypical) Kawasaki disease

More common in young infants

Consider in all children with unexplained fever > 5 days associated with two or three of the principal clinical features

Laboratory findings similar to classic cases

Coronary, myocardial findings may be helpful surrogates

Incomplete (atypical) Kawasaki disease

Echocardiography in infants less than 6

months with fever > 7 days duration and

laboratory evidence of systemic

inflammation

Young infants and adolescents are

particularly at risk for missed diagnoses

Echocardiography

Echocardiography is the ideal imaging

modality

noninvasive

high sensitivity and specificity

Echocardiography

Initial - at time of diagnosisinitiation of treatment should not be delayed

FU - 2 weeks and 6-8 weeks after onset of

disease

Final - 1 year after initial diagnosis

More frequent - in children at high risk -

persistent fever, positive coronary findings

or myocardial involvement

8

High risk for coronary aneurysms

Age < 12 months

Male sex

WBC count > 12,000

Platelet count < 350,000

CRP > 3+

Hematocrit < 35%

Albumin < 3.5 g/dl

Other tests for coronary abnormalities

Cardiac catheterization and angiography

MRI and MRA

Nuclear stress testing for reversible ischemia, stress echocardiography

AspirinDoes not lower frequency of coronary abnormalitiesDuring the acute febrile phase of illness

80 to 100 mg/kg per day in 4 doses additive anti-inflammatory effect with IVIG

Once afebrilelow-dose aspirin (3–5 mg/kg per day) maintain until no evidence of coronary changes at 3 monthsfor children who develop coronary abnormalities, continued indefinitely

Theoretical risk of Reyes syndromeAnnual influenza vaccine+/- discontinue ASA 6 weeks for varicella immunizationsSubstitute other antiplatelet agent during influenza or varicella

IVIG

Standard of careNeeds to be given within first 10 days of fever onsetReduces risk of coronary aneurysms from 20% to 1%Generalized anti-inflammatory effect

IVIG

IVIG, 2 g/kg in a single infusion (evidence level A)instituted within the first 10 days of illness and, if possible, within 7 days of illness. Treatment before day 5 appears no more likely to prevent cardiac sequelae

may be associated with an increased need for retreatment

IVIG also should be administered to children presenting after the 10th day of illness if

persistent fever aneurysms and ongoing systemic inflammation manifested by elevated ESR or CRP

Gamma globulin is a biological product

IVIG

With IVIG - 5% develop transient coronary

artery dilation and 1% develop giant

aneurysms

Measles and varicella immunizations should

be deferred for 11 months

9

Natural History of Coronary Lesions

Angiographic resolution in 1 to 2 years observed in 50% to 67% of vessels with coronary aneurysms

Risk of rupture or distal coronary stenosis

Contact sports are contraindicated

Stenotic lesions progressive

Coronary Interventions

Bypass grafting - CABBAGE

Percutaneous coronary intervention

Angioplasty

Stents - ? all drug eluting

Myocarditis

Inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent myocytes not typical of ischemic damage associated with coronary artery disease

Incidence

Unknown

Clinical presentation infrequent

Asymptomatic - likely higher incidence

? Leads to dilated cardiomyopathy

Etiology

Viral

Enteroviruses, CB3

Bacterial

Diphtheria

Rickettsial, Protozoal, Parasitic, Fungal

Toxins and drugs

Hypersensitivity/Autoimmune

Others

Epidemiology

Sporadic

Epidemic

Enteroviruses

Rubella

10

Pathophysiology

55

Clinical presentation - spectrum

57

Differential diagnosis

Sepsis

Metabolic disorders

Structural heart disease

Supraventricular tachycardia

Anomalous left coronary artery from the pulmonary artery

Pericarditis

Treatment - supportive

Inotropic support

Digoxin

Dobutamine/dopamine/epinephrine

Milrinone

Diuretics

Afterload reduction

ACE inhibitors

Milrinone/nitroprusside

Positive pressure ventilation

Beta blocker - counterintuitive

11

Treatment - supportive

Mechanical support

Bridge to transplant

Bridge to recovery

ECMO/ECLS/VAD (Berlin Heart)

Controversial Therapy

Steroids

Intravenous immunoglobulin

Immunosuppressive agents

Investigations

EKG

Echocardiogram

Cardiac Enzymes CKMB/Troponin

Cardiac Catheterization

Endomyocardial biopsy (routine +/- PCR +/-immunohistochemistry)

Viral titers and cultures

MRI

Infective endocarditis

colonization or invasion of the heart

valves or the mural endocardium by a

microbe.

The aorta, aneurysmal sacs, other blood

vessels, and prosthetic devices can also

become infected.

Acute and sub-acute IE

Acute endocarditis - destructive,

tumultuous infection with a highly virulent

organism

Sub-acute endocarditis - low virulence,

insidiously protracted course of weeks to

months

12

Treatment

• Longterm IV antibiotics (6-8 weeks)

• May require cardiac surgical intervention for severe valve destruction

67

cardiovascular · cv 042 valvular heart disease ii t1 cv dr. j tam cv 044 development of the heart...

Documents