Double Outlet Right Ventricle           Orient Journal of Medicine         Vol 25 [1-2] Jan-Jun, 2013
ORIGINAL ARTICLE

Double Outlet Right Ventricle with Anatomical Associations ofVentricular Septal Defect (VSD), Pulmonary Stenosis (PS) and Atrial SeptalDefect (ASD) Presenting as Inoperable Cardiac Disease: case series
Josephat M CHINAWA1
John C EZE 2
Herbert A OBU1
Bartholomew CHUKWU1



1
Department of Paediatrics
College of Medicine
University of Nigeria
Teaching Hospital
Ituku/Ozalla
Enugu State, NIGERIA
2Department of Surgery
College of Medicine
University of Nigeria
Teaching Hospital
Ituku/Ozalla, Enugu State NIGERIA


Author for Correspondence
Dr Josephat M Chinawa
Department of Paediatrics
UNTH PMB 01129 Enugu
Enugu State, NIGERIA
Postal Code 400001


E-mail:josephat.chinawa@unn.edu.ng
Phone: +234 806 398 1403


Received: October 15
th, 2012
Accepted: March 20
th, 2013
ABSTRACT
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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