A RARE NEONATAL CARDIAC CASE OF DOUBLE OUTLET RIGHT VENTRICLE WITH ASD AND PAH.

Objective: Congenital Heart Diseases (CHD) are the most common structural birth abnormalities. A Neonate presenting in the rst month of life without cyanosis, but with clinical features of congestive cardiac failure (CCF) and murmur on auscultation needs to be further investigated for lesions like a large VSD with PDA, unobstructed TAPVC, Double Outlet Right Ventricle (DORV) and Single Ventricle. The foetal heart beg Background: ins to form on 18th day of pregnancy, with formation of a heart tube that elongates and develops dilation and contractions ability as a ventricle and outow tract. Both arterial trunks arise from right ventricle. Persistence of left ventriculo-infundibular fold (VIF) between aorta and mitral valve, which was supposed to be regressed is associated with DORV. By fourth and fth week of foetal life atrial septum and ventricular septum develop. These structural developments are affected by idiopathic, genetic and environmental factors; resulting into CHD in-situ. But the presentations vary with lesion, age and clinical representation. The incidence being 6-8 per 1000 live births. It is most common direct cause of death in paediatric age group due to congenital heart anomalies, if not investigated and treated within time. DORV is a complex CHD where aorta and pulmo Discussion: nary artery arise predominantly from morphological right ventricle along with presences of Atrial Septal Defect and Ventricular Septal defect (VSD) which requires a denitive surgical management. If DORV is present with cyanosis, there is pulmonary stenosis (PS) which resembles a Tetralogy of Fallot. In contrast, our case has pulmonary hypertension without cyanosis, but with features of CCF, as seen in DORV without PS. With advancements in foetal and paediatric cardiology, Take Home Message: early diagnosis and treatment of CHDs is now possible, and is of utmost importance for survival, prevention of complications, genetic counselling and improving the quality of life.