Lalita Nemani1, Ramachandra Barik1, Amar Narayana Patnaik1, Ramesh C Mishra2, Amaresh M Rao2, Pragati Kapur2
1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
2 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderbad - 500 082, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
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