CONGENITAL HEART DISEASE Uptofate Study Summary Acyanotic Atrial septal defect Ventricular septal defect Patent foramen ovale Patent ductus arteriosus Aortic coartation Pulmonary stenosis Cyanotic Tetralogy of Fallot Atrial Septal Defect Location of defect a) Ostium secumdum (1) 75% b) Ostium primum (2) 15% c) Sinus venous (3) 10% Physical examination Cardiac catheterisation Treatment Fixed splitting of 2 nd heart sound R ventricular impulse R atrial enlargment Incomplete RBBB R ventricular enlargment Prominent pulmonary artery Increased pulmonary markings Diagnostic test of choice See secondary complications pulmonary hypertension R heart enlargement Determine magnitude and direction of shunting Severity of pulmonary hypertension ASD should be closed if evidence of L to R shunt with pulmonary flow a) Pulmonary to systemic flow ratio of > 1.5 b) Volume overload c) Sx related to defect
Patent Foramen Ovale In the fetus, it allows oxygenated placental blood to be transferred to the fetal systemic circulation Investigation Agitated N/Saline contrast is injected into peripheral IV catheter to demonstrate the R to L intracardiac shunt Enhanced by Valsalva that reduces preload Complications 1) Cryptogenic stroke paradoxical embolism through the patent foramen ovale higher risk if atrial septal aneurysm present device or surgical closure suggested benefit is controversial o early studies suggest device closure is safe and may be beneficial o not established if pt best treated with asprin, warfarin, device closure or combination 1) Other associations Cyanosis in setting of TR or R sided failure Ventricular Septal Defect Most common, females = males Spontaneous close in 90% by 10yo Location Membranous (70%) Muscular (20%) Below aortic valve- cause aortic regurg (5%) Physical findings Small defect Loud systolic murmur Displaced apical L ventricular pulse Large defect AR present Holosystolic murmur Decrescendo diastolic murmur Pulmonary HT R ventricular heave present Holosystolic murmur and thrill will diminish and eventually disappear as flow through defect decreases Murmur of pulmonary regurgitaion (Graham Steel s) Cyanosis and clubbing Small defect Normal Large defect L atrial and ventricular enlargement
Doppler Catheterization Closure indications Pulmonary HT R axis deviation present Enlargement of pulmonary arteries LV enlargement To locate location of VSD Determine direction and magnitude of shunt Magnitude of shunting Pulmonary vascular resistance 2) Aortic regurgitation 3) Pulmonary regurgitation 4) LV overload 5) Recurrent endocarditis Need Antibiotic prophylaxis small or large defect Coarctation of Aorta Males > females Physical findings Surgical repair Post surgery Hypertension in upper arms Radial femoral delay Early systolic click and aortic systolic murmur due to bicuspid valve S4 is common Figure 3 sign - dilation of aorta above and below area of coarctation. LVH Confirms coarctaion Associated features such as bicuspid aortic valve Indicated if gradients are > 30mmHg Age at time of initial repair is the most important predictor of long term survival and persistent HT. Still at increased risk of cardiovascular mortality and morbidity from : 1) HT 2) Coronary artery disease 3) Recoartation 4) Aortic dissection 5) endocarditis Patent ductus arteriosus Communication between pulmonary artery and aorta Associated with maternal rubella
Tiny one Continuous murmur heard around S2 around L clavicle No need for intervention Endocarditis prophylaxis If previous endocarditis, closure recommended Moderate sized one Presents with symptoms of heart failure LV hypertrophy Percutaneous device closure recommended to prevent progressive heart failure. Pulmonary Stenosis Associated with Noonan syndrome Short stature, intellectual impairment, neck webbing, congenital heart defects Physical signs Intervention Required if: Tx Prominent a waves R ventricular heave Ejection systolic click If severe, pulmonary component of S2 disappears R ventricular hypertrophy R axis deviation gradient is > 50mmHg R ventricular hypertrophy Pulmonary balloon valvuloplasty Increased risk of CV events post surgery Cyanotic Congentical heart disease Causes 1) Eisenmenger syndrome Common cause of cyanosis in adults Irreversible pulmonary vascular disease due to longstanding cardiac shunt 2) Tetralogy of Fallot Defects: a. Last Ventricular septal defect b. Pulmonary stenosis c. Overiding of aorta over VSD d. RVH At childhood, all get surgically repaired Problems later in adulthood : Pulmonary regurgitation (Diastolic murmur) Right ventricular hypertrophy (parasternal heave) Tricuspid regurgitation (holosystolic murmur) Risk of sudden death due to ventricular arrhythmias
2001 P1 Question 36 A young man presents with breathlessness and ankle swelling. He had a ventricular septal defect (VSD) repaired at the age of three years. He is centrally cyanosed and has a prominent right ventricular heave on palpation of the chest. The most likely finding is: A. pulmonary hypertension. B. blood oxygen saturation of 95%. C. left ventricular hypertrophy. D. left to right shunt. E. normal haemoglobin. Ans A