Cyanotic Congenital Heart Diseases MCQs

Tetralogy of Fallot Most common cyanotic congenital heart disease Components: pulmonary stenosis, VSD, overriding of aorta, RV hypertrophy Characterised by cyanotic / tet spells during childhood while exertion Squatting increases peripheral vascular resistance thereby reducing the magnitude of right to left shunt across VSD Boot shaped heart or 'coeur en sabot' is the characteristic xray appearance Reference : Harrison's Principles of Internal Principles, 20th edition, ch:264 pg:1833, 1834

Eponymous chest X ray appearances in congenital heart disease Snowman sign : Total anomalous pulmonary venous connection Egg on side appearance: Transposition of great arteries Boot shaped heart : Fallot's tetralogy Scimitar sign : Partial anomalous pulmonary venous connection Figure of 3 sign : Coarctation of aorta Waterbottle sign : Ebstein anomaly (commonly described in pericardial effusion) Reference: Harrison's Principles of Internal Principles, 20 th edition, ch:264 pg:1831,1832

Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly open into the right atrium. O2 saturation of blood in the pulmonary artery is often identical to that in aorta due to complete mixing of systemic and pulmonary venous returns in the right atrium.

Patients with prosthetic material used during palliative or corrective surgery are at the highest risk for bacterial endocarditis . In the current guidelines, patients with aorto-pulmonary shunts continue to be in the small subset of patients for whom antibiotic prophylaxis is recommended. Other Indications for prophylaxis of infective endocarditis Child with transposition and transplant for the first 6 months following the operation. (Option b, c) The child with unrepaired cardiac defects (option d) Tags: Topic: ACQUIRED CARDIAC DISEASE Difficulty level: GOOD TO KNOW PYQ: INICET MAY 2021 Reference: Park’s Pediatric cardiology, 6 th ed., pg. 342

As per Nelson 21 st Ed, the most common heart defects in Turner syndrome patients are bicuspid aortic valves, coarctation of the aorta, aortic stenosis, and mitral valve prolapse. TOF is not described in most textbooks and is thus the answer by exclusion. Reference: Nelson 21 st Ed, Chapter 98.4, OP Ghai 10 th Ed, Page 669

The VSD of TOF is always large enough to allow free exit to the right-to-left shunt into overriding aorta. Since the right ventricle is effectively decompressed by the VSD, CCF seldom occurs in TOF. The exceptions to this rule include Anemia Infective Endocarditis Systemic HTN RV Dysfunction from long-standing hypoxia AR or TR Reference: OP Ghai 10 th Edition, Page 445

TGA with VSD is the most common cause of cyanosis in the first week of life. About other options: Although TOF is a cyanotic disease, there must be atleast 5 g/dl of reduced Hb to produce clinical cyanosis. Hence despite having TOF, in the presence of severe anemia, child will not show cyanosis PDA and supracristal VSDs are acyanotic diseases. Ref: Perloff's Cardiology, Nelson 21 st Edition

Turner’s syndrome is associated with coarctation of the aorta. 49XXXXY and 49XXXXX are associated with patent ductus arteriosus. Holt-Oram syndrome is associated with ASD. Ellis-van Creveld syndrome is associated with ASD, PDA, hypoplastic left heart syndrome, TAPVD and TGA. Tags: Topic: EPIDEMIOLOGY Difficulty level: MUST KNOW PYQ: INICET NOV 2021 Reference: Park’s Pediatric cardiology, 6th ed., pg. 237