To summarize T.O.F. To summarize T.G.A. To summarize tricuspid atrsia. To summarize Ebstein’s anomaly To outline the non cardiac complications of cynotic C.H.D.

Cyanotic Congenital Heart Disease

Lesions Associated with Decreased Pulmonary Blood Flow Tetralogy of Fallot

Epidemiology and pathology Tetralogy of Fallot is the most common cyanotic congenital heart defect, representing about 10% of all congenital heart defects . Anatomically, there are four structural defects: VSD, pulmonary stenosis, overriding aorta and right ventricular hypertrophy. The VSD is large and the pulmonary stenosis is most commonly subvalvularor (infundibular).

Clinical features A- Symptoms 1.  Most patients are symptomatic with cyanosis at birth or shortly thereafter. Dyspnea on exertion, squatting, or hypoxic spells develop later, even in mildly cyanotic infants . 2. Occasional infants with acyanotic TOF may be asymptomatic or may show signs of CHF from a large left-to-right ventricular shunt. 3.Immediately after birth, severe cyanosis is seen in patients with TOF and pulmonary atresia.

4-Hypoxic ("Tet") spells occur during the 1st 2 yr of life, they are usually progressive. During a spell, the child typically becomes restless and agitated, crying inconsolably, hyperpneic with gradually increasing cyanosis and of the murmur. In severe spells, they develope unconsciousness , convulsions, hemiparesis, or death may occur. Independent of hypoxic spells, patients with tetralogy are at increased risk for cerebral thromboembolism and cerebral abscesses resulting in part from their right-to-left intracardiac shunt.

Placement of the infant on the abdomen in the knee-chest position while making certain that the infant's clothing is not constrictive. Premature attempts to obtain blood samples may cause further agitation and –ve effect. O2 adminstration S.c morphine not > 0.2mg/kg. B-adrenergic blockers e.g propranolol (0.1-0.2) mg /kg i.v and slowly. Treatment of spells

Physical examination 1-Growth and developmental delay in patients with severe untreated tetralogy of Fallot, particularly when oxygen saturation is chronically <70%. Puberty may also be delayed in patients who do not undergo Surgery. 2-Varying degrees of cyanosis, tachypnea, and clubbing (in older infants and children) are present. 3-The pulse is usually normal, as is venous and arterial pressure.

4-The left anterior hemithorax may bulge anteriorly because of RVH. 5-In about half the cases, a systolic thrill is felt along the left sternal border . 6-The murmur is usually loud, systolic, most intense at the left sternal border. It is due to PS and generally ejection in quality , but it may sound more holosystolic toward the lower sternal border.

1-The classic chest x-ray finding is a boot-shaped heart created by the small main pulmonary artery and upturned apex secondary to right ventricular hypertrophy with olegaemic lungs.

2-The ECG usually has right axis deviation and right ventricular hypertrophy.

VSD

4-Cardiac catheterisation and right ventriculography may be required to show the detailed anatomy of the pulmonary arteries, which may be small or stenosed. Lateral view of a selective right ventriculogram in a patient with the tetralogy of Fallot. I- is the infundibular stenosis that is below the infundibular chamber (C). V-is the narrowed pulmonary valve orifice is seen at the distal end of the infundibular chamber. V I

Natrural history and complications 1. Worsening of cyanosis with time. 2. Physicians need to watch for the development of relative iron-deficiency state (i.e., hypochromia). 3. Polycythemia develops secondary to cyanosis. 4. Growth retardation may be present if cyanosis is severe. 5. Hypoxic spells may develop in infants. 6. Brain abscess and cerebrovascular accident rarely occur 7. SBE is occasionally a complication. 8.Coagulopathy is a late complication of a long-standing cyanosis.

Management Initial management is medical, with corrective surgery at around 6 months of age. It involves closing the VSD and relieving right ventricular outflow tract obstruction with an artificial patch, which sometimes extends across the pulmonary valve.

Infants who are very cyanosed in the neonatal period require a shunt to increase pulmonary blood flow. This is usually done by surgical placement of an artificial tube between the subclavian artery and the pulmonary artery (a modified Blalock-Taussig shunt) or sometimes by balloon dilatation of the right ventricular outflow tract

*Frequently there is no significant murmur. TRICUSPID ATRESIA Etiology, Epidemiology and pathophysiology Tricuspid atresia accounts for approximately 2% of all congenital heart defects . The absence of the tricuspidvalve results in a hypoplastic right ventricle. All systemic venous return must cross the atrial septum Into the left atrium. A PDA or VSD is necessary for pulmonary blood flow andsurvival. Clinical Manifestations Severe cyanosis If a VSD is present, there may be a murmur. A diastolic murmur across the mitral valve may be audible. *Frequently there is no significant murmur.

TRICUSPID ATRESIA Diagnosis ECG shows LVH and Lt axis deviation. CXR shows cardiomegaly with decreased pulmonary blood flow Echo is diagnostic and shows the abnormalities. Treatment If there is no VSD PGE1 is infused to maintain ductal patency and pulmonary flow. Surgical correction involve (Blalock-Taussig procedure) then Fontan opertation. SVC RPA IVC

Ebstein Anomaly Pathophysiology Ebstein anomaly consists of downward displacement of an abnormal tricuspid valve into the right ventricle. Pathophysiology The right ventricle is divided into 2 parts by the abnormal tricuspid valve: the 1st, a thin-walled “atrialized” portion, is continuous with the cavity of the right atrium; the 2nd, often smaller portion consists of normal ventricular myocardium. RV output is decreased due to a combination of the poorly functioning small right ventricle and tricuspid valve regurgitation, “atrialized” portion

*Neonates with severe form present with severe cyanosis, Ebstein Anomaly Clinical Manifestations Symptoms Severity is variable depending on the severity of pathology. Mild cases present at teenage or adulthood with fatigue and palpitation as a result of cardiac dysrhythmias. There may be atrial right-to-left shunt through an ASD causing cyanosis and polycythemia. Physical signs 1- High JVP. 2- A holosystolic murmur caused by tricuspid regurgitation is audible over most of the anterior left side of the chest. *Neonates with severe form present with severe cyanosis, heart failure and pulmonary hypoplasia and may die.

Ebstein Anomaly Diagnosis ECG shows RBB, large tall p wave, prolonged PR interval and SVT due to Wolf-Parkinson white syndrome. CXR shows cardiomegaly Echo is diagnostic and shows the abnormalities. Treatment In non severe cases by surgical repair of TV In severe cases by single ventricle repair ( Fontan opertation) SVC RPA IVC

D-Transposition of the Great Arteries Lesions Associated with Increased Pulmonary Blood Flow D-Transposition of the Great Arteries

Etiology and Epidemiology T.G.A represents about 5% of congenital heart defects, and it is the most common cyanotic lesion to present in the Newborn period . T.G.A is ventriculoarterial discordance secondary to abnormalities of septation of the truncus arteriosus, the aorta arises from the right ventricle, anterior and to the right of the pulmonary artery, which arises from the left ventricle. This transposition results in desaturated blood returning to the right heart and being pumped back out to the body, while well-oxygenated blood returning from the lungs enters the left heart and is pumped back to the lungs. Without mixing of the two circulations, death occurs quickly. Mixing can occur at the atrial (patent foramen ovale/ASD), ventricular (VSD), or great vessel (PDA) level

CLINICAL MANIFESTATIONS 1.    History of cyanosis from birth is always present. 2.    Symptoms of congestive heart failure (CHF) with dyspnea and feeding difficulties may develop during the newborn period On examination:- 1.Moderate to severe cyanosis and tachypnea. 2. No VSD no murmur, VSD holosystolic murmur, and there may be murmur of RVOT or LVOT obstruction. 3. There may be features of heart failure.

1. Cardiomegaly with increased pulmonary vascularity .    Investigations X-ray Studies 1. Cardiomegaly with increased pulmonary vascularity . 2. An egg-shaped cardiac silhouette . The electrocardiogram shows the normal neonatal right-sided dominant pattern.

Echocardiography. Two-dimensional echo and color flow Doppler studies usually provide all the anatomic and functional information needed for the management of infants with D-TGA.

Treatment Initial medical management includes prostaglandin E1 to maintain ductal patency. If the infant remains significantly hypoxic on prostaglandin therapy, a balloon atrial septostomy is performed to improve mixing between the two circulations.

Complete surgical repair is most often an arterial switch; the atrial switch is rarely done. The arterial switch usually is performed within the first 2 weeks of life, when the left ventricle still can maintain a systemic pressure

Extracardiac Complications of Cyanotic Congenital Heart Disease and Eisenmenger Physiology

CNS, central nervous system; DIC, disseminated intravascular coagulation; RSV, respiratory syncytial virus.