Congenitally corrected transposition of great arteries: a case series of five unoperated African children
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(2020) 4:8
Journal of Congenital Cardiology
CASE REPORT
Open Access
Congenitally corrected transposition of great arteries: a case series of five unoperated African children Bernard Obongonyinge* , Judith Namuyonga, Hilda Tumwebaze, Twalib Aliku, Peter Lwabi and Sulaiman Lubega
Abstract Background: Congenitally corrected transposition of great arteries (ccTGA) is rare. It is commonly associated with ventricular septal defect (VSD), pulmonary stenosis and heart block. Early anatomic repair is recommended between 3 and 6 months of age to prevent development of tricuspid valve regurgitation and systemic right ventricular failure. Case presentation: We retrospectively identified five cases of ccTGA. Cases were between one and 13 years of age. All the cases were unoperated. Four of the five cases had associated intracardiac defects/complications. These included: VSD, pulmonary stenosis, tricuspid valve regurgitation, right ventricular systolic dysfunction and heart block. Conclusion: These cases demonstrate the challenges of access to early diagnosis and surgery in a low resource setting. This delay in anatomic repair leads to complications of tricuspid valve regurgitation and systemic right ventricular failure. Keywords: Case series, Heart block, ccTGA, Systemic right ventricle, Tricuspid regurgitation
Background Congenitally corrected transposition of great arteries (ccTGA) is a rare congenital cardiac malformation. It accounts for approximately 0.05% of congenital cardiac anomalies [1]. In a 7-year review of congenital heart disease (CHD) at the Uganda Heart institute, ccTGA accounted for 0.14%, while D-TGA (dextro-transposition of the great arteries) accounted for 0.7% of all the CHD [2]. This anomaly arises from Left (Levo) looping of the developing heart tube [3]. In situs solitus, Levo-looping brings the morphological left ventricle (LV) to lie on the right connected to pulmonary artery and the morphological right ventricle (RV) to lie on the left connected to the aorta [3]. ccTGA is associated with conduction, physiological and anatomical abnormalities. Ninety percent of patients will have a cardiac abnormality [1]. The commonest anatomic cardiac abnormalities include VSD, pulmonary stenosis and abnormalities of the tricuspid valve [4]. Conduction defects
include heart block and supraventricular tachyarrhythmias [5]. Physiologically, absence of early corrective surgery leaves the right ventricle as the systemic ventricle leading to progressive RV systolic dysfunction. Progressive RV fibrosis, presence of tricuspid valve regurgitation and univentricular pacing of the morphological left ventricle lead to RV systolic failure [1, 6, 7]. Clinical presentation depends on the concomitant cardiac defects. In patients with nonrestrictive VSD, early anatomic repair is recommended between 3 and 6 months of age to prevent the deleterious effect of a systemic RV [8]. However, in our low resource setting, cost and lack of access to corrective surgery delays corrective surgical repair as seen in these cases.
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