APPROACH TO A CHILD WITH CYANOTIC CONGENITAL HEART DISEASE

PRESENTED BY : DR. ADIL LANKER MODERATED BY : DR.GULAM RASOOL

INTRODUCTION
y The initial evaluation for suspected congenital heart disease involves a

systematic approach with three major components. 1. Congenital cardiac defects can be divided into two major groups based on the presence or absence of cyanosis, which can be determined by physical examination aided by pulse oximetry . 2. These two groups can be further subdivided according to whether the chest radiograph shows evidence of increased, normal, or decreased pulmonary vascular markings. 3. The electrocardiogram can be used to determine whether right, left, or biventricular hypertrophy exists.
y The character of the heart sounds and the presence and character of any

murmurs further narrow the differential diagnosis. y The final diagnosis is then confirmed by echocardiography or cardiac catheterization, or by both.

Cyanotic Congenital Heart Lesions

y This group of congenital heart lesions can also be further divided according to

pathophysiology: whether pulmonary blood flow is decreased or increased.

The CXR is a valuable tool for initial differentiation between these two categories.

Cyanosis
y Cyanosis in children happens when there is clinically apparent amount of desaturated

hemoglobin y Usually requires 3-4 grams/ dL of reduced hemoglobin y This usually correspond to oxygen saturation of 70-80% y Therefore mild desaturation may clinically be missed

Mechanisms of Cardiac Cyanosis

Right to left shunt TOF

Mixing Single ventricle Chest radiography Electrocardiogram Hyperoxia test Echocardiogram

Recirculation TGA

Testing

TETRALOGY OF FALLOT
y Commonest cause of cyanotic heart disease after one yr of age. y Cl presentation depends on severity of RV outflow obstruction. y Most are cyanotic since birth. y May presents with exertional dyspnoea. y Hypoxic spell y squatting

SIGNS a) RV type impulse b) Systolic thrill at left 3rd ics c) S2single(A2) d) Ejection systolic murmur at left 3rd ics e) Continuous murmur faintly audible over the ant & post chest

y y

y y y

CHEST X RAY normal-sized, boot-shaped heart (coeur en sabot) prominence of the right ventricle and a concavity in the region of the underdeveloped right ventricular outflow tract and main pulmonary artery. The pulmonary vascular markings are typically diminished, the aortic arch may be on the right side (25 percent). Dilatation of the ascending aorta

y Electrocardiography 1. Right axis deviation 2. Right ventricular and right atrial 3.

y ECHOCARDIOGRAPHY
1.

2. 3.

hypertrophy Adults with repaired tetralogy of Fallot, a complete right bundle branch block following repair has been the rule.

malaligned and nonrestrictive VSD overriding aorta (<50 percent override) presence and degree of right ventricular outflow tract obstruction (infundibular, valvular, and/or pulmonary arterial stenosis).

y Cardiac catheterization

y Left ventriculography demonstrates the

demonstrates a systolic pressure in the right ventricle equal to systemic pressure.

y Selective right ventriculography

size of the left ventricle, the position of the VSD, and the overriding aorta; it also confirms mitral-aortic continuity, thereby ruling out a double-outlet right ventricle.
y Aortography or coronary

best demonstrates the anatomy of the tetralogy of Fallot. Contrast medium outlines the heavily trabeculated right ventricle.

arteriographyoutlines the course of the coronary arteries.

Medical Management
y Polycythemia
y HCT should be 45-50% y HCT > 60% dramatically increases risk y > 65% serious hyperviscosity risk y Neurologic sequelae y Clotting abnormalities y Consider phlebotomy pre-operatively

y Infection
y R->L shunt, direct route to body y Bacterial endocarditis y Brain abscess

y TET spells
y Knee-chest y O2, volume, MSO4, vasoconstrictors y surgery

Surgical Management
y VSD closure
y transatrial access if possible y Infundibular resection for visualization y Patch closure

y Relief of RVOT obstruction

y Infundibular resection vs.

transannular patch
y Results
y y y y

Surgical Mortality is quite low, 1.6% Residual obstruction is common Residual VSDs may occur PI - initially well tolerated, but significant late consequences

1. Primary repair during infancy has become the treatment of choice of many centers with surgery scheduled at 2-4 months of age for asymptomatic infants. This is to normalize the physiology sooner and promote normal growth of the pulmonary arteries. It requires CP bypass. 2. Modified Blalock Taussig Procedure the most commonly performed in some symptomatic NBs that are poor candidates for primary repair. This is a lower risk surgical procedure by creating a systemic pulmonary artery shunt to increase the pulmonary blood flow. This is usually not done with the child in CP bypass.

Pulmonary Atresia with Ventricular Septal Defect

y Pulmonary atresia with VSD is an extreme form of the tetralogy of y

y y y

Fallot. The pulmonary valve is atretic, rudimentary, or absent, and the pulmonary trunk is atretic or hypoplastic. The entire right ventricular output is ejected into the aorta. Pulmonary blood flow is then dependent on a PDA or on bronchial collateral vessels. Most patients are severely cyanotic and require urgent prostaglandin E1 infusion and palliative surgical intervention Some patients have heart failure caused by increased pulmonary blood flow via bronchial collateral vessels (MAPCAs),and Some infants have adequate pulmonary blood flow and can be managed like patients with uncomplicated tetralogy of Fallot.

y The surgical procedure of choice depends on whether the main

pulmonary artery segment is adequate and on the size of the branch pulmonary arteries. y In patients with small branch pulmonary arteries, surgical intervention is directed toward increasing pulmonary blood flow in the hope that pulmonary artery growth will be stimulated. y Two options are currently considered: 1. an aortopulmonary (Blalock-Taussig or central) shunt or 2. the establishment of a connection from the right ventricle directly to the pulmonary artery, a. either by patch unroofing of the outflow tract or b. by implanting a homograft conduit. y To be a candidate for full repair, the pulmonary arteries must be of adequate size to accept the full volume of right ventricular output. y Complete repair includes closure of the VSD and placement of a homograft conduit from the right ventricle to the pulmonary artery

Pulmonary Atresia with Intact Ventricular Septum

y In pulmonary atresia with an intact ventricular septum, the

pulmonary valve leaflets are completely fused to form a membrane and the right ventricular outflow tract is atretic. y Because no VSD is present, no egress of blood from the right ventricle occurs. Right atrial pressure increases, and blood shunts via the foramen ovale into the left atrium, where it mixes with pulmonary venous blood and enters the left ventricle. The combined left and right ventricular output is pumped solely by the left ventricle into the aorta. y In a newborn with pulmonary atresia, the only source of pulmonary blood flow occurs via a PDA. y The right ventricle is usually hypoplastic, although the degree of hypoplasia varies considerably.

Clinical Manifestations
y As the ductus arteriosus closes in the 1st hr or days of life, infants with pulmonary atresia y y y y

and an intact ventricular septum become markedly cyanotic. Untreated, most patients die within the 1st wk of life. Physical examination reveals severe cyanosis and respiratory distress. The 2nd heart sound is single and loud. Often, no murmurs are audible.

Treatment
y Infusion of prostaglandin E1 is usually effective before surgery. y A surgical pulmonary y

valvotomy is carried out to relieve outflow obstruction. To preserve adequate pulmonary blood flow, an aortopulmonary shunt is often
performed during the same procedure.

y Surgical unroofing of the right ventricular outflow tract and patch grafting. y Interventional catheterization, in which the imperforate pulmonary valve is first punctured with a wire or radiofrequency ablation catheter, followed by balloon

valvuloplasty.

Tricuspid Atresia
y Complete absence of communication y y y y y

between the right atrium and right ventricle About 3 % of congenital heart disease There is an obligate interatrial communication Usually associated with VSD The pulmonary blood flow is dependent on the size of the VSD Pulmonary blood flow can be increased or decreased causing variable presenting symptoms If there is no VSD ( also called Hypoplastic right ventricle) the pulmonary blood flow is dependent on the PDA

Clinical presentation
y The presentation will depend on the amount of pulmonary blood flow y If the PBF is decreased, the main presenting symptom is cyanosis y If the PBF is increased the presentation is that of congestive heart failure

y y y y y y y

Severe cyanosis since birth Hypoxic spell Prominent a wave in jvp LV type impulse Tender hepatomegaly with presystolic pulsation S2 ---single loud( A2) A holosystolic murmur at left mid & lower sternal border

y CXR will also reflect the amount of pulmonary blood flow y Two types----1.TA with normal related great art(70%)

2.TA with TGA ( 30%)

ECG 1. Left axis deviation, 2. Tall peaked rt atrial P wave

ECHO 1. Atretic tricuspid valve in Apical subxiphoid view 2. Hypoplastic RV

3. Left ventricular hypertrophy. 3. Large LV 4. Left atrial enlargement may be present if pulmonary flow is high. 4. ASD ,VSD,Aortic arch anomaly

CHEST RADIOGRAPHY 1. Left ventricular configuration 2. Oligaemic lung field 3. The main pulmonary trunk is inapparent. 4. A right aortic arch exists in 25 percent of patients

Therapeutic Management:
a. Medical Management PGE1 infusion is initiated for infants who depend on the PDA for pulmonary blood flow; and the infant is stabilized and readied for surgery. b. Interventional Cardiac catheterization Balloon atrial septostomy during cardiac catheterization to allow blood to flow from the RA to the LA.

c.Surgical Management Single ventricle paliation y First stage : to establish a reliable source of PBF y Aorta to pulmonary artery shunt ( BT shunt) y Pulmonary arterial banding in cases of increased PBF y Second stage: Glenn Anastomosis ( superior vena cava to pulmonary artery y Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery

DoubleDouble-Outlet Right Ventricle with Pulmonary Stenosis

y Both the aorta and pulmonary artery arising from the right ventricle characterize

y y y y y y

double-outlet right ventricle with pulmonary stenosis; the outlet from the left ventricle is a VSD into the right ventricle. The aortic and mitral valves are separated by a smooth muscular conus, similar to that seen under the normal pulmonary valve. The aorta may override the VSD by a variable amount but is at least 50% committed to the right ventricle. This defect may be viewed as part of a continuum with the tetralogy of Fallot, depending on the degree of aortic override. The physiology as well as the history, physical examination, electrocardiogram, and roentgenograms are similar to that in the tetralogy of Fallot, Surgical correction consists of creating an intraventricular tunnel so that the left ventricle ejects blood through the VSD, through the tunnel, and into the aorta. The pulmonary obstruction is relieved either with an outflow patch or with a pulmonary or aortic homograft conduit (Rastelli operation).

EBSTEIN ANOMALY
y Septal & post leaflet of tricuspid valve displaced downward ----atrialisation of RV y h/o maternal use of lithium y h/o cyanosis,effort intolerance & y y y y y

fatigue,arrythmia LV impulse S1 widely split loud T1(the sail sound) S2 widely split S3,S4---triple & quardruple rhythm Systolic TR murmur not with resp

y Cyanosis with normal or reduced pulmonary blood flow and a dominant left

ventricle with type B WPW is diagnostic

Electrocardiography
1. 2. 3. 4. 5. 6.

CXR
1.

Himalayan p wave Prolonged PR RBBB WPW AF Deep q wave in inf & V1-V4

RT BORER OF HEART PROMINENT WITH LEFTWARD CONVEXITY

2. 3. 4.

WATER BOTTLE APPEARANCE OLIGEMIC LUNG FIELD AORTA & PUL ART INCONSPICOUS

ECHOCARDIOGRAPHY

1. 2.

Apical displacement of the septal leaflet of the tricuspid valve by 8 mm/m2 or more & An elongated sail-like appearance of the anterior leaflet, confirms the diagnosis

Treatment
In neonates:
an aortopulmonary shunt alone or by surgical patch closure of the tricuspid valve, atrial septectomy, and placement of an aortopulmonary shunt (Starnes procedure). y This operation creates a functional tricuspid atresia, which can then be further repaired with first a Glenn and then a Fontan.

In older children:
y with mild or moderate disease,

control of supraventricular dysrhythmias is of primary importance; surgical treatment may not be necessary until adolescence or young adulthood. y with severe tricuspid regurgitation, repair or replacement of the abnormal tricuspid valve along with closure of the atrial septal defect is then carried out. In some patients, a bidirectional Glenn shunt is performed, with the superior vena cava anastomosed to the pulmonary arteries. This procedure reduces the volume of blood that the dysfunctional right side of the heart has to pump, thus creating a one-and-one-half ventricle repair.

Transposition of Great Areries (TGA)

y Aorta originating from the right

y y

y y y y

ventricle, and pulmonary artery originating from the left ventricle Accounts for 5-7% of all congenital heart disease Survival is dependent on the presence of mixing between the pulmonary and systemic circulation Atrial septal defect is essential for survival 50% of patients have a VSD Usually presents in the first day of life with profound cyanosis More common in boys

TRANSPOSITION OF GREAT ARTERIES

( TGA ) COMPLETE TGA --------------- CONGENITALY CORRECTED TGA
WITHOUT VSD ------------------- WITH VSD (ASD,PDA) WITH PS--------------------------- WITHOUT PS

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES

y d-TGA with an intact ventricular septum is y y y

y Signs :
Pulsefull volume JVP---N or increased in CHF RV type impulse Palpable S2 at left base----originate in aortic valve early or holosystolic murmur of VSD may be audible in less cyanotic infants with associated VSD. A soft midsystolic murmur of pulmonary stenosis (PS or LVOT obstruction) may be audible.

also referred to as simple TGA or isolated TGA 1. Male : female 4:1 2. Average birth weight and size are greater than 3. normal. 4. Dyspnea and cyanosis from birth, progressive hypoxemia, and congestive heart failure usual 5. presentation Sever cyanosis & hypoxemia in infants who have only a small patent foramen ovale or ductus arteriosus and an intact ventricular 6. septum; or left ventricular outflow tract obstruction. cyanosis can be minimal, and heart failure is the dominant after the first few weeks of life if large VSD or PDA present

CXR
Cardiomegaly with increased pulmonary vascularity is typically present. 2. An egg-shaped cardiac silhouette with a narrow, superior mediastinum is characteristic
1.

Electrocardiography 1. rightward QRS axis 2. RVH is usually present after the first few days of life 3. Biventricular hypertrophy (BVH)

ECHOCARDIOGRAPHY
In the parasternal short-axis view, 1. The great arteries appear asdouble circles 2. The PA is in the centre of the heart 3. The aorta is usually anterior and slightly to the right of the PA.

Congenitally Corrected Transposition of the Great Arteries

y In L-transposition, the atrioventricular relationships are discordant, with

y y y y y y y

the right atrium connected to the left ventricle and the left atrium to the right ventricle (ventricular inversion). Rare less than 1% Asymptomatic when L-TGA is not associated with other defects. Most patients with associated defects become symptomatic with cyanosis resulting from VSD and PS or CHF resulting from a large VSD. Exertional dyspnea and easy fatigability may develop with regurgitation of the systemic AV valve Hyperactive precordium occurs in the presence of a large VSD The S2 is loud and single at the upper left or right sternal border. A grade 2 to 4/6 harsh, holosystolic murmur along the lower left sternal border indicates the presence of VSD or systemic AV valve regurgitation.

y CXR
1. A straight, left upper cardiac border,

formed by the ascending aorta 2. Cardiomegaly and increased pulmonary vascular markings are present with VSD. 3.RT pulmonary hilum often prominent & elevated------rt sided water fall appearance
y ECHOCARDIOGRAPHY

1.The morphological left ventricle is <characterized by its smooth endocardial surface and is guarded by a bileaflet AV (mitral) valve with no direct septal attachment. 2.The morphological right ventricle is recognized by its apical trabeculation and moderator band and is guarded by a trileaflet apically displaced AV valve (tricuspid valve) with direct attachment to the septum. 3.The AV valves show reversed offsetting, a strong clue to the diagnosis. 4.Ebstein-like malformation of the left (tricuspid) AV valve may be present

TGA .. Acute Management :

y PGE-1 with no supplemental O2

Maintain ductus arteriosus patency, this will increase the effective pulmonary blood flow, and thence increase the left atrial pressure, therefore inhance the left to right shunt at the atrial level
y Rashkind balloon atrial septostomy

Life saving procedure in the presence of inadequate atrial septal defect

y The arterial switch (Jantene) procedure y with re-implantation of the coronary artery to the new aortic site. y Atrial switch (Mustard or Senning operation) : y the old style surgery y Redirecting the pulmonary and systemic venous return to result in a physiologically normal state y The right ventricle remains the systemic ventricle y Rarely needed

DOUBLE-OUTLET RIGHT VENTRICLE

y 50 percent of each semilunar valve arises from the morphological right ventricle. y Clinical physiological responds depends on size & location of VSD and presence or absence

of PS

Major clinical patterns

1. with a subaortic VSD with PS PS---------- commonest clinical scenario and management algorithm are similar or identical to that of tetralogy of Fallot with a subaortic VSD ,no PS with low pulmonary vascular resistance ----resembles : no non restrictive perimembranousVSD with a subaortic VSD ,no PS with high pulmonary vascularresistance ----no resembles: Eisenmenger syndrome with a subpulmonary VSD with no PS(Taussig-Bing anomaly) ----resembles : PS(TaussigTGA with non restrictive VSD.

2.

3.

4.

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

y All of the systemic and pulmonary venous return drains to the right atrium y Most have symptoms during the first year of life,80 percent die before 1

year of age if not treated y Infradiaphragmaticcyanotic since birth y Supradiaphragmaticcyanosis & CHF at 4-6wk y Feature similar to ASD with increased rt sided flow.  RV type impulse  S1-loud(T1)  Wide fixed splitting of s2  Accentuated P2  Midsystolic murmur at left 2nd ics

y CXR figure-of-8 or snowman heart due to enlargement of the heart and the presence of a dilated right superior vena cava, innominate vein, and left vertical vein.

ECG

ECHOCARDIOGRAPHY

1. Rt axis deviation 1. Marked enlargement of the right ventricle and a small left atrium. 2. RAH & 2. An echo-free space representing the pulmonary 3. RVH venous confluence can usually be seen behind the left atrium.

Treatment.
y Surgical correction of TAPVR is indicated during infancy. y Before surgery, infants may be stabilized with prostaglandin E1 to dilate

the ductus venosus and the ductus arteriosus. y Some may require balloon atrial septostomy, but it is of little or no benefit in the presence of pulmonary venous obstruction. y Surgically, the commonpulmonary venous trunk is anastomosed directly to the left atrium, the ASD is closed, and the connection to the systemic venous circuit is interrupted. y Results have been generally good, even for critically ill neonates.

Truncus Arteriosus
y In truncus arteriosus, a single arterial trunk (truncus arteriosus) arises from the y

y y y

heart and supplies the systemic, pulmonary, and coronary circulations. A VSD is always present, with the truncus overriding the defect and receiving blood from both the right and left ventricles . Generally patients have increased pulmonary blood flow Degree of cyanosis is mild and may not be evident clinically until late stage with pulmonary vascular disease Presenting feature is congestive heart failure (tachypnia, hepatomegally) Exam is significant for y Single S2 y Ejection click of the abnormal truncal valve y Systolic murmur of truncal valve stenosis if present y Diaastolic murmur of truncal valve insufficiency y Gallop CXR : Cardiomegally , increased pulmonary circulation

Types:
y pulmonary arteries(P.A) may arise together

from the posterior left side of the persistent truncus arteriosus and then divide into left and right P.A(type I truncus arteriosus). y In types II and III truncus arteriosus, no main P.A is present, and the right and left P.As arise from separate orifices in the posterior (type II) or lateral (type III) aspects of the truncus arteriosus. y Type IV truncus has no identifiable connection between the heart and P.A, and pulmonary blood flow is derived from major aortopulmonary collateral arteries arising from the transverse or descending aorta; also been called pseudotruncus but is essentially a form of pulmonary atresia with a VSD

y Therapeutic Management: a.

Medical Management: aimed at reducing the effects of CHF and preventing polycythemia CHF is treated with digoxin and diuretics. Surgical Management: Pulmonary artery banding for NBs who do not respond to early medical management. Rastelli repair- Total corrective repair includes closing the VSD and placement of a conduit from the RV to the pulmonary artery. Valvuloplasty of the truncal valve which is the new aortic valve may be performed to improve valvular competence. Blood flow postop is normal. Conduit replacement is necessary as the child grows and a future truncal valve repair or replacement may be needed.

b.
1. 2. 3.

4.

Single Ventricle (Double-Inlet Ventricle, Univentricular Heart)

y With a single ventricle, both atria empty through a common atrioventricular

y y y y y

valve or via two separate valves into a single ventricular chamber, with total mixing ofsystemic and pulmonary venous return. The aorta and pulmonary artery both arise from this single chamber. Variable Cl. features and depends on the associated intracardiac anomalies. If pulmonary outflow is obstructed, the findings may be similar to those of TOF: marked cyanosis without heart failure. If pulmonary outflow is unobstructed, the findings are similar to those of transposition with VSD: minimal cyanosis with marked heart failure. The eventual development of pulmonary vascular disease reduces pulmonary blood flow so that the cyanosis increases and signs of cardiac failure appear to improve (Eisenmenger physiology

y Findings on the ECG are nonspecific. y CXR confirms the degree of cardiomegaly. In the absence of pulmonary stenosis,

y y y y

pulmonary vasculature is increased, whereas in the presence of pulmonary stenosis, pulmonary vasculature is diminished. Absence or near absence of the ventricular septum is the principal echocardiographic sign. Treatment : If pulmonary stenosis is severe, an aortopulmonary shunt is indicated. If pulmonary blood flow is unrestricted, pulmonary arterial banding is used to control heart failure and prevent progressive pulmonary vascular disease. The Glenn shunt followed by a modified Fontan operation (cavopulmonary isolation procedure, is the ultimate treatment of choice. If subaortic stenosis is present because of a restrictive connection to a rudimentary outflow chamber, surgical relief can be provided by anastomosing the proximal pulmonary artery to the side of the ascending aorta (Damus-StansylKaye operation).

Hypoplastic Left Heart Syndrome

y Accounts for 1% of all CHDs. It is seen more frequently in males than in females.

Approximately 95% of all affected infants who are untreated will die within the 1st months of life. y Inadequate development of the left side of the heart results in only one effective ventricle. The syndrome may include aortic valve atresia, hypoplasia of the LV, atresia or hypoplasia of the ascending aorta, and mitral valve stenosis or atresia. Most infants have intact ventricular septum.

y Manifestations: y Most infants present (within the first few days of life) with tachypnea and early CHF from

increased pulmonary blood flow and as the ductus arteriosus begins to close, systemic hypoperfusion and shock.
y The infant appears grayish blue in color with dyspnea and hypotension. y Therapeutic Management:

Die within the first month of life without surgical intervention. a. Medical Management: correction of the acid-base and electrolyte imbalances and reestablishment of ductal patency with PGE1. b. Surgical Management y Norwood Procedure a three-step palliative repair. y An alternative therapy is cardiac transplantation, either in the immediate neonatal period, thereby obviating stage I of the Norwood procedure, or after a successful stage I Norwood procedure is performed as a bridge to transplantation. Cardiac transplantation as a single, definitive correction has been successful with 85% operative survival rate and 81% 5-year survival rate. The scarcity of neonatal donor heart, however, greatly limits the number of infants who may receive transplant.

Eisenmenger syndrome
it is a pulmonary vascular obstructive disease that develops as a consequence of a large preexisting left-to-right shunt such that pulmonary artery pressures approach systemic levels and the direction of the flow becomes bidirectional or right to left. y Causes 1.Simple--ASD,VSD, and PDA 2.Complex-- AV septal defect, T.arteriosus, aortopulmonary window, and univentricular heart. y The high pulmonary vascular resistance is usually established in infancy (by age 2 years, except in ASD) and is sometimes present from birth. SYMPTOMS y Cyanosis --- central 1st decadeVSD 2nd decade---PDA 3rd decadeASD y Exercise intolerance (dyspnea and fatigue) is proportional to the degree of hypoxemia or cyanosis. y Palpitations in nearly half the patients (atrial fibrillation/flutter in 35 percent, Ventricular tachycardia in up to 10 percent); y Hemoptysis in about 20 percent; due to bleeding bronchial vessels or pulmonary infarction. y Pulmonary thromboembolism, angina, syncope, and endocarditis in about 10 percent each; and y congestive heart failure after 40 year
y DEFINITION:

Physical examination
Central cyanosis and clubbing of the nail beds. Pink nail beds on the right (>left) hand and cyanosis and clubbing of both feet, so-called differential cyanosis. JVP normal or elevated. Signs of pulmonary hypertension 1. a right ventricular heave, palpable and loud P2, and a right-sided S4 2. pulmonary ejection click and a soft and scratchy systolic ejection murmur, attributable to dilation of the pulmonary trunk, and 3. high-pitched decrescendo diastolic murmur of pulmonary regurgitation (Graham Steell) are audible. Peripheral edema is absent until right-sided heart failure ensues.

CXR
1. Dilated central pulmonary arteries with rapid tapering of the peripheral pulmonary vasculature are the radiographic hallmarks of Eisenmenger syndrome(E.S). 2. Pulmonary artery calcification in long-standing PAH 3. E.S due to VSD or PDA usually has a normal or slightly increased cardiothoracic ratio. 4. E.S due to an ASD typically has a large cardiothoracic ratio due to right atrial and ventricular dilation, along with an inconspicuous aorta. 5. Calcification of the duct & prominent aortic knuckle -----Eisenmenger PDA.

ECG.

ECHOCARDIOGRAPHY. 1. The intracardiac defect should be seen readily 1. Peaked P waves consistent with right atrial along with bidirectional shunting. overload 2. A pulmonary hypertensive PDA is not easily 2. right ventricular hypertrophy with right axis seen. deviation 3. Evidence of pulmonary hypertension is found. 3. Atrial arrhythmias can be present. 4. Assessment of pulmonary right ventricular function adds prognostic value.

CARDIAC CATHETERIZATION. 1. provides direct measurement of the pulmonary artery pressure, documenting the existence of severe pulmonary hypertension, 2. Administration of pulmonary arterial vasodilators (O2, nitric oxide, prostaglandin I2 [epoprostenol]) can discriminate among patients in whom surgical repair is contraindicated and those with reversible pulmonary hypertension who may benefit from surgical repair. OPEN-LUNG BIOPSY. when reversibility of the pulmonary hypertension is uncertain from the hemodynamic data.

Summary
Cyanotic heart disease commonly presents in the neonatal period. Rapid diagnosis and referral are mandatory because patients can become unstable very quickly. Prostaglandin E1 promotes blood flow through the ductus arteriosus and is a useful stabilizing maneuver in many, but not all, of these conditions. Echocardiography and cardiac catheterization are the diagnostic tools of choice Early surgical intervention is often required, either for palliation or for definitive correction. Current surgical therapy for most lesions has evolved from early palliation and delayed repair to complete correction in early infancy with improved morbidity and mortality.

Thankyou