CONGENITAL HEART DISEASE: CONGENITAL HEART DISEASE Dr.Kaushik


CASE #1: CASE #1 45 YEAR OLD FEMALE COMPLAINS OF DYSPNEA ON EXERTION AND EPISODES OF PALPITATION DENIES ANY CHEST PAIN, NO SYNCOPE HISTORY OF HEART MURMUR


PHYSICAL EXAM: PHYSICAL EXAM Jugular pulse reveals a prominent “A”wave Right parasternal lift First heart sound is normal.Second heart sound is widely split and does not vary with respiration Grade 2/6 systolic ejection murmur heard best at the 2nd ICS-LSB


Chest X-ray: Chest X-ray


ECG: ECG ECG


ECHOCARDIOGRAM: ECHOCARDIOGRAM ECHOCARDIOGRAM


Pathophysiology of ASD: Pathophysiology of ASD


TYPES OF ATRIAL SEPTAL DEFECTS: TYPES OF ATRIAL SEPTAL DEFECTS Ostium Secundum:75% Ostium Primum: 15% Sinus Venosus: 10% Unroofed coronary sinus


NATURAL HISTORY OF THE DISEASE: NATURAL HISTORY OF THE DISEASE Right ventricular dilatation and failure Pulmonary Hypertension Atrial flutter or fibrillation Paradoxical embolism


TREATMENT: TREATMENT Surgical closure either by direct stitch or use of pericardial patch Percutaneous atrial septal closure device


CASE #2: CASE #2 24 y/o male migrant worker was admitted because of chills and fever for several days No known past medical illness


Physical exan: Physical exan Poor dental hygiene Petechiae noted in the conjunctiva and fingers PMI is at the 5th ICS, 1 cm lateral to the MCL S1is soft,S2 is split but vary with respiration. Grade 4/6 holosystolic murmur at the 4th ICS,LSB with a thrill.


Chest X-ray: Chest X-ray


ECG: ECG Sinus rhythm Broad notched P wave,Tall R wave in V5 and V6


ECHOCARDIOGRAM: ECHOCARDIOGRAM


Pathophysiology of VSD: Pathophysiology of VSD


Types of VSD: Types of VSD Perimembranous: 70% Muscular: 20% Supracristal: 10% Inlet


Natural History Of VSD: Natural History Of VSD 25-40% of VSD close by age 2 and 90% of VSD that close do so by age 10 Small defects remain asymptomatic but are predisposed to endocarditis Large defects often develop LV failure, Pulmonary hypertension (Eisenmenger syndrome) and RV failure Aortic Regurgitation


Treatment: Treatment Endocarditis prophylaxis Surgical closure in the absence of pulmonary hypertension


Case #3: Case #3 18 y/o female was referred because of dyspnea on exertion and easy fatigue History of heart murmur Mother had rubella during pregnancy


Physical Exam: Physical Exam BP: 140/50 No cyanosis and no clubbing of fingers Pulse are bounding PMI is at the 6th ICS, 2 cm lateral to the MCL. Precordial heave is present. S1 is normal.Grade 3/6 continous murmur is heard below the left clavicle


ECG: ECG


Chest X-ray: Chest X-ray


Echocardiogram: Echocardiogram


Angiogram: Angiogram


Pathophysiology of PDA: Pathophysiology of PDA


Natural History of PDA: Natural History of PDA Small PDA usually remain asymptomatic Infective endocarditis LV volume overload and LV failure Pulmonary Hypertension (Eisenmenger Syndrome) Atrial arrhythmia


Treatment: Treatment Surgical ligation and interruption of the duct Percutaneous device closure Indomethacin


Eisenmenger Syndrome: Eisenmenger Syndrome Severe pulmonary vascular obstructive disease with increased in pulmonary artery resistance and severe pulmonary hypertension secondary to a large left to right shunt. Reversal of shunt occurs when the pulmonary arterial pressure exceeds the systemic arterial pressure


Eisenmenger Syndrome: Eisenmenger Syndrome Shunt reversal is accompanied by cyanosis & clubbing The original murmur disappear.P2 is loud. Pulmonic regurgitation murmur is heard Erythrocytosis develops with symptoms of hyperviscosity Risk of bleeding and thrombosis Risk of brain abscess


VSD with Eisenmenger Syndrome: VSD with Eisenmenger Syndrome


Treatment: Treatment Phlebothomy with volume replacement Intravenous epoprostenol maybe beneficial Lung transplantation with repair of cardiac defect Avoid volume depletion,high altitude, exertion and vasodilators


Case # 4: Case # 4 30 y/o male is referred for evaluation of hypertension Denies any headache,chest pain, dyspnea or syncope


Physical Examination: Physical Examination BP: RA=160/70;LA=156/70;RL=120/70 S1:normal,systolic ejection click is present. Grade 2/6 systolic ejection murmur at the LSB and also at the back LV heave is present Femoral pulse is delayed


Rib X-ray: Rib X-ray


Echocardiogram: Echocardiogram


Angiogram: Angiogram


Pathophysiology of Coarctation of the Aorta: Pathophysiology of Coarctation of the Aorta


Natural History of Coarctation of the Aorta: Natural History of Coarctation of the Aorta Persistent Hypertension LV failure Dissecting Aneurysm Premature coronary artery disease Rupture of cerebral aneurysms Infective endocarditis Bicuspid Aortic valve Turner’s syndrome


Treatment: Treatment Surgical repair Transcatheter balloon angioplasty and stenting


Case #5: Case #5 12 y/o male admitted because of dyspnea and cyanosis Patient has been cyanotic since few months after birth Has episodes of tachypnea and worsening cyanosis which improved with squatting Known history of heart disease but mother has refused surgery


Physical Exam: Physical Exam Bluish discoloration Clubbing of fingers and toes Parasternal lift S1:normal;S2:single Grade 3/6 systolic ejection murmur at the 2nd and 3rd ICS, LSB


ECG: ECG


Chest X-ray: Chest X-ray


Echocardiogram: Echocardiogram


Pathophysiology of Tetralogy of Fallot: Pathophysiology of Tetralogy of Fallot


Treatment: Treatment Complete surgical repair: VSD closure and relief of RVOT obstruction Blalock-Taussig operation: SCA-PA Potts operation:DA-LPA Waterston-Cooley:AA-RPA


Natural History: Natural History Prognosis is poor without surgical repair or palliation The survival rate of repaired TOF is worse compared to age-matched control Sudden cardiac death: VT Atrial arrhythmias Infective endocarditis Pulmonic Regurgitation Residual defects


Thank you: Thank you