Congenital heart disease

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Information about Congenital heart disease

Published on March 4, 2008

Author: ksbarot


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

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