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Submitted: 10 June 2008
HERDIN Record #: PCHRD061208020602

Univentricular heart: The chamber of secrets (A case report on double outlet right ventricle with restrictive vsd, mitral valve atresia with hypoplastic left ventricle, pulmonic stenosis with normally related great arteries).

Rachelle Angeli R. NiÃÆââ‚

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OBJECTIVE: To present a rare case of complex congenital heart disease of different embryologic etiology and describe its epidemiology, pathology, hemodynamics, clinical features, diagnostic features and management.

DESIGN: Case Report

ABSTRACT: A three-month old male was admitted at the PHC in June 2005 due to cyanosis. He was born acyanotic with unremarkable maternal, birth and neonatal history. He presented with an incidental finding of murmur at 1 month, interrupted feedings and episodes of diaphoresis at 2 months, and incessant crying with cyanotic lips and nailbeds at 3 months. He was referred by another institution for intervention. Clinical findings showed a well-nourished and well developed infant with cyanotic lips
and nailbeds, RVheave, SI normal, S2 single and loud, grade 3/6 SEM LUSB, grade 3/6 HSM LLSB and peripheral O2sats - 50-60 percent. Chest radiograph showed decreased vascularity, straight MPA, RAE and RVE. Electrocardiogram showed RAD, LAE and RVH. 2DEchocardiogram revealed CHD, double outlet right ventricle with restrictive VSD, mitral valve atresia with hypoplastic left ventricle, severe pulmonic stenosis, infundibulovalvar with normally related great arteries, PFO and pulmonary arterial hypertension. Balloon Atrial Septostomy was done with subsequent Hemodynamic Study and adequate post-BAS defect. DORV with intact ventricular septum is an extremely rare malformation first described by MacMahon and Lipa in 1964. Its association with MVA, hypoplastic LV and PS make it even more rare and only seven cases have been identified thus far. Regular medical follow-up and serial 2DEcho was advised. An option to do Glenn Shunt is recommended if the RVOT obstruction progresses resulting to significant hypoxemia. Univentricular repair can be done at later age. (Author)

Publication Type
Journal
Publication Sub Type
Case report
Title
Philippine Journal of Cardiology
Frequency
Semi-Annual
Publication Date
July-December 2006
Volume
34
Issue
2
Page(s)
89-92

Objectives

This case report aims to present a rare case of complex congenital heart disease of different embryologic etiology and describe its epidemiology, pathology, hemodynamics, clinical features, diagnostic features and management.

LocationLocation CodeAvailable FormatAvailability
Philippine Council for Health Research and Development Library Abstract Print Format (Request Document)