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Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult
oleh: Jagdish C. Mohan, Vishwas Mohan, Madhu Shukla, Arvind Sethi
Format: | Article |
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Diterbitkan: | Elsevier 2016-09-01 |
Deskripsi
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.