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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.

SUBMITTER: Mohan JC

PROVIDER: S-EPMC5067759 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature

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Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult.

Mohan Jagdish C JC Mohan Vishwas V Shukla Madhu M Sethi Arvind A

Indian heart journal 20160415

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, ...[more]

PMID: 27751299

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