title: Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult.
abstract: Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation. The patient died owing to progressive heart failure 4 years after the diagnosis was made.
authors.affiliation: Department of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, Delhi 88, India. Electronic address: a51hauzkhas@gmail.com.
authors.forename: Jagdish C
authors.initials: JC
authors.lastname: Mohan
authors.affiliation: Department of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, Delhi 88, India.
authors.forename: Vishwas
authors.initials: V
authors.lastname: Mohan
authors.affiliation: Department of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, Delhi 88, India.
authors.forename: Madhu
authors.initials: M
authors.lastname: Shukla
authors.affiliation: Department of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, Delhi 88, India.
authors.forename: Arvind
authors.initials: A
authors.lastname: Sethi
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D002972:Cleft Palate / Q000175:diagnosis*; D004431:Ear, External; D004452:Echocardiography; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis*; D006349:Heart Valve Diseases / Q000151:congenital / Q000175:diagnosis*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D008831:Microcephaly / Q000175:diagnosis*; D008844:Micrognathism / Q000175:diagnosis*; D011664:Pulmonary Valve / Q000002:abnormalities*
keywords: Absent pulmonary valve; Bicuspid aortic valve; Hypoplastic right heart syndrome; Non-compaction of the left ventricle