Search for a rare disease
Other search option(s)
Hypoplastic right heart syndrome
Disease definition
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 (see this term). 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.
ORPHA:98723
Classification level: Group of disorders- Synonym(s): -
- Prevalence: -
- Inheritance: -
- Age of onset: Neonatal
- ICD-10: Q22.6
- OMIM: -
- UMLS: C0265856 C0344963
- MeSH: -
- GARD: 2922
- MedDRA: 10050053 10064962
Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Specialised Social Services
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.