2015-06-03

1

UWMAgnieszka Banach

Systolic Aortic stenosis and pulmonary stenosis Mitral valve prolapse Mitral and tricuspid regurgitation VSD, ASD HCM

Diastolic Aortic and pulmonary regurgitation Mitral and tricuspid stenosis

Continuous PDA

http://depts.washington.edu/physdx/heart/demo.htmlhttp://www.wilkes.med.ucla.edu/inex.htm

Definition - blue color of skin and mucous membranes caused by reduced oxygen content.

Absolute increase of amount of reduced hemoglobin in blood, >5g/dl (capillary), >5% deoxygenated blood

Cyanosis usually noted when Sat’s <86% Cyanosis more easily seen in polycythemia Cyanosis more difficult to see in anemia

Central cyanosis

noted in the trunk, tongue, mucous membranes

due to reduced oxygen saturation

Causes:

▪ Upper airway obstruction

▪ Respiratory distres syndrome

▪ Pulmonary hypoplasia

▪ Asthma

Peripheral cyanosis noted in the hands and feet, around mouth

due to reduced local blood flow

Causes:▪ Vasoconstriction (exposure to cold)

▪ Polycythemia

▪ Low cardiac output

2015-06-03

2

All babies blue at birth By 10 minutes, should have pink

bodies, But hands and feet still blue Crying may cause cyanosis Examine baby in white light while

quiet

Congenital heart disease: 5 to 8 cases per 1,000 live births

Child with congenital anomaly usually does not show cardiovascular problems in utero.

Changes at birth place great stress on infant’s cardiovascular system.

Some cyanotic heart conditions are highly dependent on shunting through ductusarteriosus. Closure can be terminal event.

Congentialheart diseaes

(CHD)

CYANOTIC: 22%

Decreasedpulmonaryblood flow

Tricuspidatresia

Tetralogy of Fallot

Mixed bloodflow

Transposition of thegreat arteries

Total anamalouspulmonary venous return

Truncus arteriosus

Hypoplastic left heartsyndrome

ACYANOTIC: 68%

Increased inpulmonaryblood flow

ASD

VSD

AVSD

PDA

Obstruction of blood flow

from ventricle

Pulmonarystenosis

Aortic stenosis

Coarctation of the aorta

Defect in the septum separating the left and right atria

Blood flow from left to right atrium Accounts for 5-10% of congenital heart

disease Twice as frequent in girls versus boys

Ostium secundum most common In the middle of the septum in the region of the foramen

ovale Ostium primum

Low position Form of AV septal defect

Sinus venosus Least common Positioned high in the atrial septum Frequently associated with partial anomalous venous

return

2015-06-03

3

Secundum ASD • Sinus VenosusASD

Most are asymptomatic but may have easy fatigability or mild growth failure.

Rarely presents with signs of CHF or other cardiovascular symptoms.

Cyanosis does not occur unless pulmonary HTN is present.

II-III/VI systolic ejection murmur A large ASD causes right ventricular

enlargement Arrhythmias

If defect is small (less than 2mm), will usually resolves spontaneously

If defect is large, surgical correction is needed Closure generally recommended when ratio of

pulmonary to systemic blood flow (Qp/Qs) is > 2:1

Closure is performed electively between ages 2 and 5 yrs to avoid late complications.

Minimally invasive procedures available Transcatheter devices, such as a Amplatzer

septal occluder may be used

VSD – is an abnormal opening in the ventricular septum, which allows free communication between the right and left ventricles.

The most common congenital heart malformation, accounting for almost 30% of all CHD

Can occur singly or in multiples anywhere along the ventricular septum

Small defects often close spontaneously in the first 2 years of life while large defects require surgical repair

Perimembranous (or membranous) – Most common.

Infundibular (subpulmonary or supracristalVSD) – involves the RV outflow tract.

• Muscular VSD – can be single or multiple.

2015-06-03

4

The left to right shunt occurs as long as pulmonary vascular resistance is lower than systemic resistance

This leads to elevated RV and pulmonary pressures

With the increased pulmonary blood flow, pulmonary hypertension can occur with large defects

This causes volume hypertrophy of the LA and LV

Hemodynamically insignificant Between 80% and 85% of all VSDs < 3 mm in diameter All close spontanously

50% by 2 years

90% by 6 years

10% during school years

Muscular close sooner than membranous

3-5 mm in diameter Least common group of children (3-5%) Without evidence of congestive heart failure

(CHF) or pulmonary hypertension, may be followed until spontaneous closure occurs

6-10 mm in diameter Usually requires surgery Unrepaired the large defects may lead to

pulmonary hypertension and Eisenmenger’ssyndrome.

And will develop congestive heart failure(CHF)

Rapid breathing Irritability Excessive Sweating Poor weight gain Respiratory infections, Exercise intolerance Congestive Heart Failure, usually within 6 to 8

weeks of life if defect is large Pulmonary Hypertension if defect is large II-III/VI harsh holosystolic murmur heard along

the left sternal border, more prominent with small VSD, maybe absent with a very Large VSD

Natural history : Small VSDs close spontaneously depending on the site.

Unrepaired the large defects may lead to Eisenmenger’s syndrome.

Large VSDs are closed surgically by 6 months of age.

Diuretics,digoxin and afterload reducing agents are used prior to surgery - if needed

2015-06-03

5

It’s results from incomplete fusion the theendocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves.

1/3rd of babies with this have Down syndrome

Low primum ASD continuous with a posterior VSD.

Cleft in both septal leaflets of tricuspid valveand mitral valve.

Results in a large left to right shunt at both levels.

Tricuspid regurgitation and mitral regurgitation

Pulmonary hypertension.

Any one of the components may be present. Most common is primum ASD, cleft in the

mitral valve and small VSD. Hemodynamics are dependent on the lesions.

Incomplete AVSD maybe indistinguishable from ASD - usually asymptomatic.

Congestive heart failure in infancy. Recurrent pulmonary infections. Exercise intolerance, easy fatigability. Failure to thrive. Late cyanosis from pulmonary vascular

disease. Holosystolic murmur

Surgery is always required. Treat congestive symptoms. Pulmonary banding maybe required in

premature infants or infants < 5 kg. Correction is done during infancy to avoid

irreversible pulmonary vascular disease. Mortality is low - incomplete 1-2% and as

high as 5% with complete AVSD.

2015-06-03

6

The ductus arteriosus connects the pulmonary artery to the descending aorta during fetal life.

Accounts for about 10% of all cases of CHD PDA results when the ductus fails to close

after birth. Normally closes in the 1st wk of life. More common in girls than boys. Very common in preterm babies.

Blood flows from aorta to the pulmonary artery, creating a left to right shunt, resulting in left atrium and ventricle overload.

Increased pulmonary blood flow can result in pulmonary hypertension and reversal of the shunt, which is known as Eisenmenger’sSyndrome.

This results in flow of desaturated blood to the lower extremities.

Children with small patent ductus are usually asymptomatic.

Large left to right shunts develop symptoms of congestive heart failure such as tachypnea, tachycardia, poor feeding and slow growth

A large PDA causes LA and LV enlargement Classic continuous machinary systolic

murmur heard best at the left sternal border.

Indomethacin, inhibitor of prostaglandin synthesis can be used in premature infants.

Indomethacin - help to close the ductusarteriosus

PDA requires surgical or catheter closure. Closure is required treatment heart failure

and to prevent pulmonary vascular disease. Transcatheter closure of small defects has

become standard therapy Mortality is < 1%

Pulmonary Stenosis

Aortic Stenosis

Coarctation of the Aorta

2015-06-03

7

Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract.

There are three types:

Valve stenosis

Supravalve stenosis

Subvalve stenosis

Accounts for 7-10% of all CHD. Noonan’s Syndrome is associated with PS Most cases are isolated lesions Maybe biscuspid or fusion of 2 or more

leaflets Hemodynamics:

RV pressure hypertrophy which leads to RV failure.

Post-stenotic dilation of main PA.

Cyanosis is indicative of Critical PS.

Depends on the severity of obstruction.

None in mild or moderate stenosis.

Heart failure and cyanosis seen in severecases.

Ejection click, followed by systolic murmur

If Critical PS - Prostaglandins to keep the PDA open.

Mild PS no intervention required, close follow-up.

Mod-severe – require relieve of stenosis. Balloon valvuloplasty is treatment of choice. Surgical valvotomy is also a consideration.

2015-06-03

8

Aortic Stenosis is an obstruction to the outflow from the left ventricle at or near the aortic valve

Accounts for 7% of CHD. Williams Syndrome is associated with AS More common in males. This is a more significant and a dangerous

lesion compared to PS.

3 Types

Valvular – Most common.

Subvalvular(subaortic) – involves the left outflow tract.

Supravalvular – involves the ascending aorta is the least common.

Valvar AS is usually associated with a bicuspid aortic valve.

Pressure hypertrophy of the LV and LA with obstruction to flow from the LV.

Harsh systolic murmur and thrill along left sternal border, systolic ejection click.

A type of subvalvar AS is also called HCM which is the commonest cause of sudden death in children

SEVERITY OF AORTIC STENOSIS

Degree of aortic stenosisMean gradient

(mmHg)

Aortic valve

area

(cm2)

Mild aortic stenosis <25 >1.5

Moderate aortic stenosis 25 - 40 1.0 - 1.5

Severe aortic stenosis >40 < 1.0

Critical aortic stenosis >70 < 0.6

2015-06-03

9

Mild AS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic.

Moderate AS – Chest pain, dypsnea on exertion, dizziness and syncope.

Severe AS – Weak pulses, left sided heart failure, Sudden Death.

May cause severe heart failure in infants.

Treatment is generally not necessary in asymptomatic patients

Beta-blocker or Ca channel blocker to decreased hypertrophy

Aortic valve replacement Balloon valvuloplasty is the standard of

treatment. For infants and children, balloon valvuloplasty where

a balloon is inflated to stretch the valve and allow greater flow, may be effective.

In adults, however, it is generally ineffective, as the valve tends to return to a stenosed state.

Coarctation is a congenital narrowing of upper descending thoracic aorta adjacent to the site of attachment of ductus arteriosus

8-10% of CHD More common in males Associated with Turner’s Syndrome CoA is also associated with bicuspid aortic

valve

There are 3 types:

Preductal type –proximal to ductusarteriosus - infantile (B)

Juxtaductal (A)

Postductal - distal to ductusarteriosus/ligamentumarteriosum – adulttype(C)

Preductal type very high load on left ventricle

elevation of both systolic and diastolic pressure

absence of collaterals

almost immediately symptomatic with CHF from birth Postductal type

development of collaterals connecting branches of subclavian artery to arteries arising from aorta fromin-utero life

distant level of coarctation spares infant from leftventricular failure

2015-06-03

10

Narrowed aorta produces increased leftventricular afterload and wall stress, leftventricular hypertrophy, and congestive heartfailure.

Systemic perfusion is dependent on theductal flow and collateralization in severecoarctation

Higher BP in the upper extremities as compared to the lower extremities.

Decreased femoral pulses are an important sign especially in neonates

90% have systolic hypertension of the upper extremities.

Pulse discrepancy between right and left arms. Leg pains, fatigue Nosebleeds Headache II/VI systolic ejection murmur

Preductal – presents in infancy as Congestive heart failure

Selective cyanosis of lower extremities

Femoral pulses are weaker than those of the upperextremities

Postductal – presents in older chlildren and adults No selective cyanosis is seen

Hypertension of the upper extremities

Blood pressure is low and pulses are week in lowerextremities

Notching of ribs due to collaterals

Intermittent claudication – arterial insufficiency

Goals of management are to improve ventricular function and restore blood flow to the lower body.

A continuous intravenous medication, prostaglandin (PGE-1), is used to open the ductus arteriosus (and maintain it in an open state) allowing blood flow to areas beyond the coarctation.

Angioplasty is used by some centers. Re-coarctation can occur, balloon angioplasty is

the procedure of choice. Surgical intervention, to prevent LV dysfunction

Congentialheart diseaes

(CHD)

CYANOTIC: 22%

Decreasedpulmonaryblood flow

Tricuspidatresia

Tetralogy of Fallot

Mixed bloodflow

Transposition of thegreat arteries

Total anamalouspulmonary venous return

Truncus arteriosus

Hypoplastic left heartsyndrome

ACYANOTIC: 68%

Increased inpulmonaryblood flow

ASD

VSD

AVSD

PDA

Obstruction of blood flow

from ventricle

Pulmonarystenosis

Aortic stenosis

Coarctation of the aorta

2015-06-03

11

There has to be a RIGHT to LEFT shunt to cause cyanosis.

Tetralogy of Fallot (TOF) Tricuspid atresia (TA) Total anomalous pulmonary venous return

(TAPVR) Truncus arteriosus Transposition of the great arteries (TGA) Hypoplastic left heart syndrome (HLHS)

Most common cyanotic lesion (7 to 10% of all CHD)

The four abnormalities include:

Pulmonary stenosis

Right ventricular hypertrophy (RVH)

VSD

Overriding Aorta (dextra position )

Increased resistance by the pulmonary stenosis causes deoxygenated systemic venous return to be diverted from RV, through VSD to the overriding aorta and systemic circulation this causes systemic hypoxemia and cyanosis

The degree of cyanosis depends on the severity of pulmonary stenosis

Most patients are cyanotic by 4 months and it is usually progressive

Hypoxemic spells (“tet spells”) are one of the hallmarks of severe tetralogy

A “tet” spell consists of rapid breathing and increased cyanosis. Any event like crying or increased physical activity can initiate the spell.

Tet spells most commonly start around 4 to 6 months of age and are charcterized by Sudden onset or deepening of cyanosis Sudden onset of dyspnea Alterations of consciousness Decrease in intensity of systolic murmur Limpness, convulsion, cerebrovascular accident, even

death.• Patients learn to alleviate symptoms by squatting which

increases systemic resistance and decreases the right-to-left shunt and directs more blood to the pulmonary circulation.

2015-06-03

12

Oxygen administration, Placing the child in the knee-chest position

(to increase venous return), Giving morfine sulfate (to relax the

pulmonary infundibulum and for sedation), If spells are frequent – propranolol

(decreases muscular spasm)

• Dyspnea on exertion or when crying

• Tet spells and Squatting

• Failure to thrive

• Lack of energy

• Infections

• Polycythemia

• Clubbing of fingers and toes

• Cardiomegaly

• Systolic ejection murmur heard at the upper left sternal border created by turbulent blood flow through stenotic RV outflow tract

Sergical: closure of the VSD

removal of PS if indicated – paliative procedures:

the Blalock-Taussig procedure - increases blood flow to the lungs.

Post surgical: Endocarditis prophylaxis

Arrythmias monitoring Overall incidence of sudden death in TOF

patients after surgical repair is about 0.3%.

1-2% of CHD. Slightly more common in males. Definition - congenital absence or agenesis of

the tricuspid valve with no direct communication between the right atrium and the right ventricle.

TA results is a number of problems:

The tricuspid valve is absence or closed

Tre right ventricle is hypoplastic or absence

The pulmonary artery is narrow

The pulmonary valve may be blocked

2015-06-03

13

To survive your baby will need either: ASD or VSD

PDA Types:

Muscular - dimpling or fibrous thickening – 89%

Membranous – septal form the floor of RA – 6,6%

Valvular - fused cusps – 1%

Ebstein’s anomaly – septal or posterior liflets, or bothare displaced downward into RV, and they aredeformed

Clinical features of TA depend on the quantity of pulmonary blood flow (PBF)

If the PBF is decreased Severe cyanosis

Hypoxemia

Acidosis

Tachypnea If the PBF is increased

No cyanosis but may present wit signs of CHF

Fatigue, difficulty feeding

Holosystolic type of murmur at the lowersternal border

Policytemia Stroke Coagulation abnormalities Clubbed fingers Left ventricular hypertrophy

Medical: Intravenous infusion of PGE1

0,03 – 0,1 mcg/kg/min to open the ductusarteriosus

Anticongestive therapy with dogoxin and diuretics

Rashkind balloon atrial septostomy It open the septal wall

This procedure allows to mix oxygen – rich and oxygen- poor blood and improves circulation

First stage: Blalock-Taussig shunt placed to form a conduit

between pulmonary artery and aorta to maintain oxygenation

Pulmonary artery banding in cases of increasedPBF

Second stage – Glenn Anastomosis (superior vena cava to pulmonary artery)

Third stage – Fontan Anastomosis ( inferiorvena cava to pulmonary artery)

2015-06-03

14

Is a serious heart defect in which the twomain arteries leaving the heart are reversed(transposed). Aorta arises from the rightventricle and pulmonary artery arises fromleft ventricle.

Accounts for 5-7% of all CHD More common in boys TGA is more common in infants of diabetic

mothers

Survival is depending on the presence of mixing between the pulmonary and systemiccirculation

Atrial septal defect is essential for survival 50% of patients have a VSD Usually presents in the first day of life with

profound cyanosis

Blueness of the skin Shortness of breath Poor feeding Tachypnea Tachycardia Systolic ejection murmur may be present

(with VSD or PS)

Cardiomegaly - an egg-shaped cardiac silhouette with a narrow superior mediastinumis characteristic

Medical treatment includes starting the infant on PGE1.

At the time of cardiac catheterization, a baloon atrialseptostomy is performed to enlarge an ASD and improve systemic and venous mixing (Rashkindprocedure).

A supravalvular arterial switch with coronary artery relocation may be performed in the first 2 weeks of life

Definitive surgeries may consist of switching the right- and left-sided structures at the atrial level (Senning or Mustard operation) and at the ventricular level (Rastelli operation)

2015-06-03

15

Is a cardiac malforamtion in which there is no direct connection between any pulmonaryvein and left atrium.

All the pulmonary veins connect to rightatrium or one of it’s tributaries.

To survive after birth must be present PFO orASD.

1-2% of CHD

Supracardiac – pulmonary veins drain into theright atrium through the superior vena cava(50 % of cases)

Cardiac – there are two types

pulmonary veins can directly enter into rightatrium

pulmonary veins can drain into the coronary sinus (30%)

Infracardiac – pulmonary veins drain into theright atrium through the liver (hepatic) veinsand the inferior vena cava (15%)

Mixed - pulmonary veins split up and drainpartially to more than one of these options(5%)

A- Supracardiac; B – Infracardiac; C – Cardiac; D - Mixed

Clinical manifestaion depends on thepresence of obstruction to pulmonary venousdrainage and size of ASD

From mild cyanosis through pulmonaryhypertension and CHF

Tachypnea Tachycardia Metabolic acidosis

2015-06-03

16

Diagnosis by cardiac catheterization or echocardiography.

Medical treatment includes starting the infant on PGE1.

Open-heart surgery as soon as possible

Single large vessel overrides the ventricular septum and distributes all the blood ejected from the heart.

Almost always associated with VSD

1-2,5 % of CHD

Type 1

Single pulmonary trunk from the left lateralaspect of the common trunc

With branching of the left and right pulmonaryarteries from the pulmonary trunk

Type 2

Separate but proximate origins of the left and right pulmonary arterial branches from theposterolateral aspect of the common trunk

2015-06-03

17

Type 3

Branch pulmonary arteries originateindependently from the common trunk

Type 4

Pseudo trunkus

Absent pulmonary arteries

Collaterals originate from the systemiccirculation, most frequently from the descendingaorta

Cyanosis Faigue Pale skin Rapid and heavy breathing Tachycardia Poor weight gain Systolic ejection murmur at the left sternal border Cardiomegally Hepatomegally Hypocalcemia if associated with DiGeorge Syndrome Respiratory tract infections CHF

Treat CHF – diuretics, digoxin Prostaglandin Hypocalcemia correction in syndrome Corrective operation with a valved conduit

between right ventricle and pulmonary vessels.

Conduit will need to be changed as child grows but likelihood to develop pulmonary vascular disease is greatly reduced.

HLHS occurs when part of the left side of theheart (mitral valve, left venticle, aortic valve, aorta) do not develope completely.

The left ventricle is nonfunctional. The left side of the heart is completely unable

to supply blood to the systemic circulation. Large PDA supplies blood to the systemic

circulation.

2015-06-03

18

4% of all CHD The left side of heart is unable The right side must maintain the circulation

for both the lung and the body. The right ventricle pump the blood to the

lungs and also to the systemic circulationthrough the PDA.

The only possibility of survival is a connectionbetween the right and left side of heart.

The children are born healthy, no cyanosis, no murmur.

After a few hours or one or two days theinfant becomes critically ill.

Cyanosis, irritability, low volume pulse withhypotension

Tachycardia, tachypnea, hypotermia Soft systolic ejection or holosystolic murmur

Correct acidosis and hypoglycemia

Support systemic blood flow: PGE1 for PDA

A ventilation may be needed to help the baby

breathe 3 stages:1. The Norwood procedure (first 2 wks)

--reconstruction of Aorta

2. Hemi-Fontan (4-6 months)3. Fontan (18-36 months)

--separate pulmonary and systemic flow

Norwood procedure Atrial septectomy

Ductus arteriosus ligation

Form a neoaorta

Aortopulmonary shunt Hemi-Fontan:

Connect VCS to pulmonary arteries Fontan:

Connect VCI to pulmonary arteries

Bleeding, low cardiac output syndrome, and arrhythmia

Aortic arch obstruction at the site of surgical anastomosis

Progressive cyanosis caused by limited blood flow through the shunt

Success rate (survival to discharge):

Stage1:75%

Stage2:95%

Stage3:70%

5-year survival: 70%

2015-06-03

19

UWMAgnieszka Banach

It is syndrome in which heart is unable to provide the output required to meet themetabolic demands of the body (systolicfailure) and/or inability to receive blood in to the ventricular cavities at low pressure duringdiastole (diastolic failure).

When the heart fails, cardiac output is diminished. Heart rate, preload, contractitility, and afterload are affected.

Congenital

Cyanotic

Acyanotic

Acquired

At birth – HLHS, large A-V fistula, pulmonaryatresia

1st week – TGA, TAPVR, preterm PDA, criticalAS or PS

1-4 week – CoA, critical AS 6 weeks -4 month – large VSD, large PDA

Cardiomyopathy

Dilated

Hypertrophy

Restrictive

Viral myocarditis Acute rheumatic carditis Post-op rapaired cyanotic CHD Myocarditis in Kawasaki’s disease

2015-06-03

20

In the infant

Feeding difficulties

Failure to thrive

Sweating

Tachycardia

Tachypnea

In the child

Breathlessness

Tachycardia

Tachypnea

Peripheral edema

Cardiomegaly

Fatigue

Exercise intolerance

Right side failure Hepatomegaly

Face and peripheral edema Left side failure

Tachycardia

Tachypnea

Cough and wheezing Either side failure

Cardiomegaly

Gallop rhythm

Cyanosis

Class I – asymptomatic Class II – mild tachypnea or diaphoresis with

feeding in infants; dyspnea on exertion inolder children

Class III – marked tachypnea or diaphoresiswith feeding in infants; marked dyspnea on exertion in older children; prolonged feedingtimes with growth failure

Class IV – symptoms such as tachypnea, grunting, retractions or diaphoresis at rest

NYHA I – no sign NYHA II – RR>50, with or without

hepatomegaly NYHA III – all above with rib retraction NYHA IV – RR>60, HR>160, with

hepatomegaly, rib retraction, with or withoutpoor perfusion

General measures

Propped – up position

Oxygen

Adequate calories

Salt restriction

Bed rest

2015-06-03

21

Hypertension Anemia Arrhythmia Hyperthyroidism Infection Fever

Diuretics:

First line of drugs

Act by decrease venous return end diastolicvolume

Reduces edema

Furosemide - is diuretic of choise; 0.5-1.0 mg/kg/dose

Spironolactone - may be used as add on drug; 2-4 mg/kg/day

Digitalis - digoxin:

increases contractility and decreases heart rate.

Stimulates vagus nerve

Levels are helpful only in cases of suspected toxicity, not for management

Symptoms : nausea, vomiting, anorexia Most common sign of cardiac toxicity is

arrhythmia: bradycardia, AV block, Treatment includes holding doses for 1-2 half

lives, atropine for sinus bradycardia, and “FAB” fragments in cases of significant toxicity

Dopamine peripheral vasodilatation

Increase renal blood flow

5-10mcg/kg/min Dobutamine

2,5-40mcg/kg/min

Dose is gradually increased Milrinone

After open heart surgery

ACE-inhibitors - arterial vasodilator / afterload reducing agent

Captopril 0,5-6mg/kg

Used as an add on with diuretics and digoxin

Beta Blocker –

increases contractility

Improve left ventricle function and exercisetolerance

Carvedilol – initial dose 0,08 -0,46mg/kg

2015-06-03

22