syndromic disease univentricular cardiopathy hypoplastic right heart syndrome https://rarediseases.info.nih.gov/diseases/2922/hypoplastic-right-heart-syndrome https://www.malacards.org/card/hypoplastic_right_heart_syndrome ICD9:746.89 MONDO:0020291 Orphanet:98723 DOID:0070315 UMLS:C0344963 GARD:0002922 right hypoplastic heart syndrome MedDRA:10050053 ICD10CM:Q22.6 Hypoplastic right-heart syndrome (HRHS) is a rare, cyanotic congenital heart malformation caused by underdevelopment of the right-sided heart structures (tricuspid valve, RV, pulmonary valve, and pulmonary artery) commonly associated with an atrial septal defect, ostium secundum type. Pulmonary blood flow is diminished and right-to-left shunting occurs at the atrial level, leading to dyspnea, fatigue, atrial arrhythmias, right-sided heart failure, hypoxemia, repeated miscarriages that were mostly due to hypoxemia and cyanosis. Two subtypes of HRHS have been characterized: pulmonary atresia-intact ventricular septum and right ventricular hypoplasia. NCIT:C99053 MedDRA:10064962 SCTID:268180007 MEDGEN:83376