Double Outlet Right Ventricle with Anatomical Associations of Ventricular Septal Defect (VSD), Pulmonary Stenosis (PS) and Atrial Septal Defect (ASD) Presenting as Inoperable Cardiac Disease: case series.

Abstract

Background: Complex congenital cardiac abnormalities involving double outlet right ventricle (DORV) are uncommon diseases. They contribute to mortality and morbidity among children in Nigeria and the exact etiology is unknown. However, infective, genetic and environmental factors among others are commonly implicated. We present three rare cases of DORV with various associations which were inoperable.
Objectives: To present these congenital cardiac conditions which are rather rare and to highlight that early surgical correction improves the outcome in patients with these conditions.
Case Series
Case 1: OU was a 2-year old male who presented with a history of poor weight gain, bluish discoloration of the body of two years’ duration. Echocardiography (2-D) result showed double outlet right ventricle (DORV) with pulmonary stenosis and membranous ventricular septal defect (VSD).
Case 2: CA was a 1-year old female who presented with a history recurrent infection and cyanotic spells from birth. Echo (2-D) findings showed double outlet right ventricle (DORV), severe pulmonary stenosis, membranous ventricular septal defect (VSD) and atrial septal defect (ASD).
Case 3: EA was a 5-month old female who presented with cough and deformities in the feet and upper limbs and poor feeding. Clinical examination revealed a small for age child with grade IV pansystolic murmur maximal at the left sternal border.
Conclusion: Double outlet right ventricle (DORV), with pulmonary stenosis and ventricular-septal defect (VSD), is a rare anomaly. Surgical correction offers good and long term benefits if the patient presents early.

Keywords: Children, complex cardiac anomaly, Nigeria