title: Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature.
abstract: 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.
authors.affiliation: Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Lalita
authors.initials: L
authors.lastname: Nemani
authors.affiliation: Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Ramachandra
authors.initials: R
authors.lastname: Barik
authors.affiliation: Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Amar Narayana
authors.initials: AN
authors.lastname: Patnaik
authors.affiliation: Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Ramesh C
authors.initials: RC
authors.lastname: Mishra
authors.affiliation: Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Amaresh M
authors.initials: AM
authors.lastname: Rao
authors.affiliation: Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: Pragati
authors.initials: P
authors.lastname: Kapur
keywords: Adden Brooks Cognitive Examination (ACE); Atrial septal defect (ASD); Coffin-Siris syndrome (CSS); Infundibular stenosis; Persistent left superior venacava (LSVC); arteria lusoria; subaortic membrane (subAS); ventricular septal defect (VSD)