cyanotic heart lesions

8/3/2019 Cyanotic Heart Lesions

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Cyanotic Heart Lesions

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Dr. Kalpana MallaMBBS MD (Pediatrics)

Manipal Teaching Hospital


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The 5 Ts

Tetralogy of Fallot

Transposition of the Great Arteries Truncus Arteriosus (Persistent)

Tricuspid Atresia

Total Anomalous Pulmonary Venous Return(TAPVR)


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Hypoplastic left heart syndrome (HLH)

Pulmonary atresia (PA) / critical PS Double outlet right ventricle (DORV)

Ebstein anomaly

Single ventricle


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RpL

RpL with pulmonary stenosis

TOF

Tricuspid atresia Ebsteins anomaly


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Evaluation possible congenital heart

Exam: rate, rhythm, impulse, murmur, pulses

(brachial and femoral)

Oxygen saturation - Hyperoxia test ABG

Chest X- ray

Echocardiogram

Cardiac catheterization


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Tetralogy of Fallot (TOF):

Most common (75% )cyanotic CHD in >2yrs

~ 10% of all CHD


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Tetralogy of Fallot TOF = consists

Ventricular septal defect

Rt ventricular outflow obstruction infundibular or

infundibular + pulmonary valve stenosis

Aorta position is shifted to the right and over-rides the VSD

Hypertrophy of the right ventricle


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Essential components:

VSD

Pulmonary stenosis

Other components :overriding of Aorta

RVH

Pentalogy of Fallot: all above + ASD


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Hemodynamics:

Large, non-restrictive VSD, perimembranous type,

extending upto right ventricular outlet allows

equalisation of pressures in two ventricles VSD is

silent

Pulmonary stenosis Shunting of blood from RpL

ventriclemixing of oxygenated & deoxygenated

blood in left ventricle circulated to whole body


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Severity depends upon degree of pulmonary stenosis

Pulmonary stenosis causes concentric rt ventricularhypertrophy without cardiac enlargement & rt vent

pressure

Flow from Rt vent to pul artery across pul stenosis produce ejection systolic murmur

I

f obstruction small, R

L shunt minimal or absent(pink or acyanotic TOF)


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P2 Delayed & reduced in intensity due to rt

vent outflow obstruction reduced PA pressure

S2

single and A2 audible Severity of cyanosis directly proportional to

severity of pul stenosis but intensity of systolic

murmur inversely related to severity of

pulmonic stenosis


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Clinical features:

May become symptomatic any time after birth usually 2nd half of 1st yr

Anoxic spells (synonyms- hypoxic,hypercyanotic, blue, tet ) paroxysmal attackof dyspnea

Common symptoms dyspnea on

exertion,exercise intolerance Cyanosis

H/O squatting during dyspneaic episodes


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Anoxic spells

Occur predominantly after waking up or

following exertion

Most commonly start around 4 to 6 months ofage and are charcterized by

1.Sudden crying

2.Sudden onset or deepening of cyanosis3.Sudden onset of dyspnea


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Anoxic spells

4. Alterations of consciousness

5. Convulsions

6.Decrease in intensity of systolic murmurFrequency varies from once in a few days

to numerous attacks every day


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Mild outflow obstruction: cyanosis in later part of 1st year

Severe outflow obstruction: cyanosis immediately afterbirth (as ductus starts to close)

CCF unusual in children with TOF except in:

Severe anemia

Valvular regurgitation

Infective endocarditisSystemic hypertension

Coincidental myocardial diseases


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Physical examination:

Cyanosis

Clubbing

Polycythemia

Normal sized heart

Mild parastrnal impulse


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Auscultation

S1 normal

S2 Single, (A2 heard,P2 soft &delayed

Murmur

Shunt absent

Flow - Loud short pulmonary ejection systolic

murmur grade 3-5/6 at 3rd

ICS in left side


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Diagnosis: Blood: polycythemia CXR:

1. Upturned apex (RVH)- Small boot shaped

heart2. Oligemic lung fields

3. Absence or concavity of pulmonary arterysegment gives the shape described as cor-

en sabot

4. Right aortic arch ~25 -30%


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Diagnosis:

ECG:

RAD, RVH with tall peaked p waves

Echo: overriding aorta,RVH,outflow

obstruction

Cardiac catheterisation


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Complications:

Cerebral thrombosis

Brain abscess

Bleeding diathesis

I

nfective endocarditis

CCF-in acyanotic or pink TOF


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Complications:

CNS - Embolism to CNS - sluggish circulation

from polycythemia

Hemiplegia - infarction in CNS during anoxicspell


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Management:

Management of Tet spells:- knee-chest position

- humidified O2 inhalation

- morphine 0.1 mg/kg s/c/ iv


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Management of Tet spells:

- IV fluids

- Correct metabolic acidosis- Na-bicarbonate- Propanolol 0.1mg/kg/iv during spell (0.5-1

mg/kg PO 6 hrly


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Management:

Vasopressors- methoxamine IM or IV drip

penylephrine Correct anemia

Consider surgery


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General measures:

- Correction of iron deficiency anemia

- Adequate hydration

- Antibiotics for infection

- Prophylaxis with propranolol


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Surgery

Palliative surgery:

- Blalock-Taussig shunt subclavian artery to

pulm artery- Potts shunt-descending aorta to PA

- Waterson operation ascending aorta to

Rt pulm artery-Modified Blalock-Taussig shunt


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Corrective surgery: open heart surgery for

closer of VSD

- resecting the infundibular obstruction PSSurgery can be performed at any age

Success 85-90%


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Complications of surgery

Complete heart block

RBBB

Residual VSD & Pulm stenosis Pulm regurgitation


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Tricuspid atresia

Cong absence of

Tricuspid valve

Rt ven hypoplastic

Absent inflow portion

2% of CHD


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Hemodynamics

No communication between Rt atrium rt

ventricle (hypoplastic)

Blood from Rt atrium

lt atrium throughpatent foramen ovale or ASD.mixing of

oxygenated + deoxygenated blood to lt

ventricle aorta

Lt vent rt vent there is VSD pul artery ( lt

.ventricle maintains both systemic &

pulmonary circulation saturation of blood is

identicle in pulm artery and aorta


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Clinical features

Depends on state of pulmonary flow

90 % are with diminished blood flow

Features :As TOF

Differentiating points :

1.Cyanosis from birth

2.More sicker than TOF

3.Lt ventricular type of apical impulse

4.Enlarged liver with presystolic pulsations

5.ECG- LAD,LVH


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Diagnosis

Blood: polycythemia CXR:1.Oligemic lung fields

2.Left ventricular configuration3.Prominent SVC shadow ECG:

Rt & Lt atrial hypertrophy, LAD,LVH

Echo: large single ventricular cavity


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Tricuspid Atresia

Repair not usually performed in neonatal

period- over a series of procedures

Systemic to PA shunt SVC to PA shunt (followed by ligation of first

shunt) Glenn Shunt

IVC to PA shunt completion Fontan


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Ebsteins Anomaly

Rare CCHD

Post and septal leaflet of TV displaced downwards

The upper part of the right ventricle is part of theright atrium - atrialized rt ventricle

Rt ventricle is too small and Rt. atrium is too large.

Leaflets malformed and fused obstruction offlow to rt ventricle


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Ebsteins Anomaly

Often Associated with other heart lesions

ASD

Pulmonary Stenosis Pulmonary Atresia


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Hemodynamics :

Abnormal leaflets obstruction to forward

flow & regurgitation from Rt ven to Rt

atrium atrium dilates Patent FO / ASD

allows R L shunt( cyanosis) Lt atrium

(enlarged) Lt ventricle (enlarged &

hypertrophied)


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Clinical picture

Cyanosis

Effort intolerance

Fatigue Paroxysmal attack of tachycardia

Clubbing

Lt ventricular apical impulse Systolic thrill may be palpable LSB


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Auscultation

S1- normal

S2 widely split but variable

Rt ventricular 3rd

soundor rt atrial 4th

soundaudible triple/quadruple sound usually

heard

Murmur-midsystolic ejection or pansystolic

Short tricuspid delayed diastolic M


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Investigations

CXR cardiomegaly square shaped

Lung oligemic

ECG- p pulmonale pmitrale,RBBBWolff Parkinson white type conducton defect

maybe seen

ECHO- displaced tricuspid valve


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Treatment

Surgical obliteration of atrialised portion of

rt.ventricle and repairof tricuspid valve


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Fallots physiology

Presence of large VSD with PS

Useful for bedside identification of group of

condition with similar clinical findings

Defects with Fallots physiology:

1. Complete TGA with VSD & PS

2. DORV with PS & large VSD (subaortic)3. Tricuspid atresia with diminished pul flow

4.Single ventricle with PS

5. corrected TGA with VSD & PS


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Transposition of the Great Arteries

Most common cyanotic condition thatrequires hospitalization in first 2 weeks oflife

Aorta arises from RV

Pulmonary artery originates in the leftventricle


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Oxygenated pulmonary venous blood

recirculates in lungs and systemic venous

blood recirculates in systemic circulation


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Transposition of the Great Vessels

A PDA,ASD,VSD, is necessary for these infants to

survive until they can have corrective surgery

More common in infants of diabetic mothers


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Classification

1. Complete variety

2. Physiologically corrected type


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Complete variety

Rt atrium Rt ventricle aorta

Lt atrium Lt ventricle pulmonary artery

Systemic & pulmonary circulation separate

survival possible only if there isASD,VSD,PDA

Classification

A) With intact ventricular septum mixing site is

atrial communication PFOB) with VSD with/without pul stenosis


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Physiologically corrected type

Rt atrium morphologically inverted left

ventricle pulmonary artery

Lt atrium morphologically inverted Rt

ventricle aorta

Route of blood flow is normal


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C /F with intact VS

Cyanotic at birth

Interatrial mixing poor (PFO) rapid breathing,congestive failure due to hypoxia within 1st wk of life

CCF

S1 normal

S2- single

Ejection systolic murmur grade 1-2/6

CXR egg on side appearance,plethoric lung field


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With VSD

Good mixing at ventricular level, large

pulmonary blood flow cyanosis milder

CCF at 4-10 wks

Exam cyanosis,CCF

S1- Normal

S2 single Murmur ejection systolic grade 2-4/6


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Diagnosis

CXR- egg on side appearance ,cardiomegaly

with narrow base, plethoric lung field

ECG without VSD RAD,RVH

ECG with VSD RAD, biventricular

hypertrophy

Cardiac catheterization

Angiocardiography


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Medical management

Control CCF

Balloon atrial septotomy by cardiac catheterization -

Inter-atrial septum opened

Definitive repair Jatenes switch operation -

removal of aorta and pulmonary artery from their

origins and re-attached to the correct ventricles

Less preferred atrial switch operation mustard or

senning


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Corrected TGA

Normal route of blood flow

Commonly associated with other anomalies

98% - symtoms are due associated anomalies:

1.VSD with/without PS

2.Lt sided Ebsteins anomaly of tricuspid valve

3.Atrioventricular conduction abnormalities


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Truncus Arteriosus

Truncus fails to divide completely during fetal life,leaving a connection between the aorta andpulmonary arteries

Mixed oxygenated and de-oxygenated blood exits theheart and enters the systemic circulation


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Truncus Arteriosus

Single artery arises from the heart, supplying both aortaand pulmonary artery.

VSD below the truncal valve allows mixing of right and leftventricular blood

Degree of cyanosis is variable Presents with progressive heart failure


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Truncus Arteriosus

Medical Management

Digoxin and Diuretics

Surgical Repair

Usually required by 2-3 months of age

VSD is closed

PA trunk is separated from truncus

Conduit created between RV and PA using a

valved graft


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TAPVR

The pulmonary veins, instead of being connected to

the left atrium , are connected to the right atrium

or superior vena cava, and return oxygenated blood

to the right side of the heart.


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Total Anomalous Venous Return

Uncommon CCHD

Cyanosis

CCF at age 4-10 wks Murmur : pul ejection systolic + tricuspid flow

murmur

Continuous venous hum audible at upper leftor rt sternal border or in suprasdternal notch


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Diagnosis

CXR- snowman or figure of 8 configuration

ECG RAD,RVH,

ECHO- demonstrate abnormal course of pulveins


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Total Anomalous Venous Return

Control of CCF, pul infections

The only accepted treatment is surgery

Surgical connection is made between pulmonaryvenous confluence and the LA

Connection to systemic venous circulation is

ligated.


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Hypoplastic Left Heart

Fatal without early surgical

intervention


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Treatment- continued

General procedure for cyanotic heart lesionsinvolves a systemic to PA shunt.

Procedure known as the Blalock-Taussig shunt

Uses a small Gore-Tex shunt to connect eitherleft or right subclavian to left or right branch PA.

Allows partially desaturated blood to enter PA,increasing pulmonary blood flow and oxygenation


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CCHD with PA HTN

This group is named Eisenmenger syndrome

severe PA HTN resulting in RL shunt at

atrial, ventricular or pulmonary arterial level

Eisenmenger complex severe PA HTN with

VSD resulting in RL shunt


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Eisenmenger's syndrome named by Dr. Paul

Wood after Dr. Victor Eisenmenger, who first

described the condition in 1897.


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Hemodynamics

LR shunt in the heart causes:-

- increased flow through PA

- High O2 saturation in PA

- Hyperreactive pul vasculature Pul

vascular obstructive Ds PA HTN

PA HTN causes increased pressures in the right

side of the heart and reversal of the shunt into R Lshunt

R L shunt with VSD & PDA decompresses rt

ventricle RV has only concentric hypertrophy with

no increase in size ( no heave)


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R L shunt with ASD RVH +dilatation rtven failure

R L with ASD or VSD mixing of blood

reaches ascending aorta distributed towhole systemic circulation equal cyanosis

R L with PDA mixed blood directeddownwards to descending aorta (junction isdistal to lt Subclavian artery cyanosis +clubbing of toes only (differential cyanosis)


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Examination

Cyanosis

Clubbing

Fatigue Effort intolerance

Dyspnea

h/o recurrent chest infection


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Sounds

S1- normal

S2- ASD- wide fixed split

VSD- single PDA- normally split

Murmurs

Pulmonary regurgitation ( graham steel) Ejection systolic


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Investigations

CXR- prominance of pul artery,heart size

normal to large

ECG RVH ECHO-

Cardiac catherization-bi-directional shunt


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Treatment

Heart-lung transplant is required to fully treat

the syndrome

If this option is not available - treatment is

palliative-

Anticoagulants

Pulmonary vasodilators

Antibiotic prophylaxis to prevent endocarditis

Phlebotomy to treat polycythemia

Maintaining proper fluid balance


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