Double orifice mitral valve (DOMV) is an unusual finding, most commonly seen with atrioventricular canal defect (AVCD). Due to the varied anatomical types, hemodynamic effects and co existence of other malformations, the surgical management of this anomaly can be challenging. Achieving a compromise between residual regurgitation and new stenosis, should be the aim of surgical intent. We present a case report of DOMV detected incidentally during surgery in a case of AVCD with severe MR.
Congenital superior vena cava (SVC) stenosis is a very rare anomaly especially in pediatric population. Co-existence with obstructed supracardiac total anomalous pulmonary venous connection (TAPVC) has never been reported. Clinical examination should prompt detailed and focused evaluation for this treatable etiology. SVC stenosis, although causing SVC syndrome, may decrease the severity of pulmonary venous hypertension by limiting the amount of blood in obstructed common chamber. Pericardial patch augmentation can cure SVC stenosis, and may allow for growth potential as well. We describe a case of congenital SVC stenosis in a case of obstructed supra-cardiac TAPVC in a 3 month old infant, managed successfully.
Pulmonary artery (PA) band is done in a variety of congenital heart diseases (CHDs) with the primary goal of reducing pulmonary overcirculation. Its use has declined during the last two decades, however, its role still exists in classic univentricular heart with unrestricted pulmonary blood flow. PA banding in univentricular morphology with transposition of great arteries (TGA) can be extremely morbid. Our patient had ST segment elevation in inferior leads after banding, indicating acute ischemia in right coronary artery territory. We hypothesize that, the cause for this was sudden shift of volume to the rudimentary camber through the bulboventricular foramen after PA banding, causing subendocardial ischemia. Interpretation of electrocardiogram (ECG) and subsequent management is not straightforward in this scenario .We hereby describe successful management of sudden ST segment changes observed in immediate post-operative period after PA banding for univentricular, TGA heart in an 8 month old infant.
Tetralogy Of Fallot (TOF) with absent pulmonary valve (APV) and unilateral absence of pulmonary artery (UAPA) is an extremely rare congenital cardiac anomaly. “Complete” unilateral absence implies absence of both intrapericardial and hilar segments. Co- existence of both free pulmonary regurgitation (PR) and decrease in the cross sectional area for right ventricular outflow tract (RVOT) in this substrate, are detrimental for right ventricular (RV) function. Early intervention to preserve RV function is imperative. Durable and competent valve in RVOT can be extremely helpful in improving the overall prognosis. We present a case of a two year old child, TOF with APV, absent left pulmonary artery (ALPA) who underwent RVOT reconstruction, with a pulmonary valve (PV) made from polytetrafluorethylene (PTFE) membrane. To our knowledge construction of PV using Graham Nunn technique, has not been reported with this morphology.