MRCPCH 1: Essential Questions in Paediatrics - PasTest

12CARDIOLOGY – ANSWERS3. F: Blalock–Taussig shuntA Blalock–Taussig systemic-to-pulmonary shunt will increase pulmonaryblood flow in the severely cyanosed baby with tetralogy of Fallot and arecent history of severe spells of cyanosis. Most of these children go on tohave elective surgical repair at 6–9 months to close the ventricular septaldefect and widen the right ventricular outflow tract.1.15 The sick newborn infant1. G: Obstructed total anomalous pulmonary venous connectionIn total anomalous pulmonary venous connection, the pulmonary veins donot make the normal connection with the left atrium. Instead, they candrain upwards to the innominate vein, to the liver or to the coronary sinus.If the connection becomes obstructed, the baby will present at 1–7 days oflife with cyanosis, acidosis, breathlessness, collapse, and signs of rightheart failure, and will require emergency resuscitation, ventilation andsurgery.2. F: Interrupted aortic archInterrupted aortic arch can occur at any site from the innominate artery asfar as distal to the left subclavian artery. It is a duct-dependent conditionand is associated with DiGeorge syndrome. In DiGeorge syndrome, there iscardiac disease, 22q11.2 gene deletion, absent thymus, absentparathyroids, abnormal facies, and cleft palate. Affected babies usuallypresent with absent left brachial and femoral pulses, and signs of heartfailure when the ductus arteriosus closes.3. I: Ebstein anomalyIn Ebstein anomaly, the tricuspid valve is abnormal: the posterior leaflet ofthe tricuspid valve originates within the right ventricular cavity, which isthus ‘atrialised’. A huge right atrium results from the inability of the rightventricle to pump blood forward into the pulmonary artery and thereforesometimes a palliative Blalock–Taussig shunt has to be inserted. Affectedbabies present with a loud heart murmur (tricuspid regurgitation), asuperior axis on the ECG, and reduced pulmonary vascular markings onchest X-ray, with an enlarged cardiac silhouette.