Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Elfeky M, Hacking C, et al. Hypoplastic right heart syndrome. Reference article, Radiopaedia.org (Accessed on 28 Mar 2024) https://doi.org/10.53347/rID-62223
Hypoplastic right heart syndrome is a congenital cardiac anomaly. It is characterized by an underdeveloped right side of the heart, including the right ventricle, tricuspid valve, pulmonary valve, and pulmonary arteries.
It may be present in around 1.1% of stillbirths and is rarer than hypoplastic left heart syndrome.
Neonates are cyanotic at birth.
It is caused by decreased blood flow in and out of the right ventricle during development. The most common right-sided defect to cause this syndrome is pulmonary atresia with an intact ventricular septum. Coronary arterial supply may be also abnormal.
Fetal echocardiography
Prenatal ultrasound may show a small right ventricle with hypertrophy and a small or absent pulmonary artery, with decreased or absent flow through the tricuspid valve and pulmonary valve 1.
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1. Rajiah P, Mak C, Dubinksy TJ, Dighe M. Ultrasound of fetal cardiac anomalies. (2011) AJR. American journal of roentgenology. 197 (4): W747-60. doi:10.2214/AJR.10.7287 - Pubmed
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2. Mohan JC, Mohan V, Shukla M, Sethi A. Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult. (2016) Indian heart journal. 68 Suppl 2: S229-S232. doi:10.1016/j.ihj.2016.03.030 - Pubmed
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3. Kadam PG, Shah VR, Saikia K, Patel RR. Resuscitation of a Newborn with Right Hypoplastic Heart Syndrome: A Rare Confrontation for an Anesthesiologist. (2017) Anesthesia, essays and researches. 11 (4): 1124-1125. doi:10.4103/0259-1162.194592 - Pubmed
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4. Jacobstein MD, Fletcher BD, Goldstein S, Riemenschneider TA. Magnetic resonance imaging in patients with hypoplastic right heart syndrome. (1985) American heart journal. 110 (1 Pt 1): 154-8. Pubmed
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5. Dib C, Araoz PA, Davies NP, Dearani JA, Ammash NM. Hypoplastic right-heart syndrome presenting as multiple miscarriages. (2012) Texas Heart Institute journal. 39 (2): 249-54. Pubmed
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