A RARE CASE OF DORV IN ADULTHOOD

Abstract

Background: Double outlet right ventricle (DORV) is a rare cardiac malformation in children and even more rare when it comes to adulthood. Clinical Presentation: We report a boy with DORV with Mitral atresia and hypoplastic Left Ventricle who presented to us with breathlessness on climbing 2 flight of stairs at the age of 22 years.He was not seriously limited when he came to our hospital with complaint of breathlessness on exertion. The mother first noticed him getting breathless on climbing 2 flight of stairs at 5 years of age.The patient had history of recurrent infections in the past with failure to gain weight since childhood.On Examination the Pulse was 90 beats per minute,Spo2 was 85% on Room Air in all four limbs, BP was 90/60 mmhg, no tachypnea.There was Grade 2 clubbing present with cyanosis on the nails of all four limbs. Patient had cleft lip and cleft palate with pes cavus in left foot. On inspection, precordial bulge seen on left side with bony cage deformity and apex impulse in left 5th intercostal space lateral to midclavicular line and visible pulsations in 3rd intercostal space. On palpation, Diastolic thrill present in 2nd left intercostal space with grade 3 parasternal heave. On Auscultation, Grade 3/6 loud harsh diastolic murmur heard over the left precordium and interscapular and infrascapular area with diaphragm of stethoscope in supine position.Soft high pitched early diastolic murmur heard best at left upper sternal border and increased during inspiration. 2D echo suggestive of Double outlet Right ventricle with mitral atresia with hypoplastic left ventricle with pulmonary arterial hypertension and severe pulmonary regurgitation. Discussion: To our knowledge very few cases of DORV survives upto this age without any serious limitation that have been reported.DORV if diagnosed early and operated between the age of 1 year to 3 years have satisfactory results. Some cases that remain asymptomatic or are diagnosed late leads to development of pulmonary hypertension which makes them poor candidates of surgery.It was surprising as this patient have survived till now without any serious limitation or symptoms.