Hypoplastic right heart syndrome

Case contributed by Tudonyang Kibet Denis , 10 Jan 2023

Diagnosis certain

Changed by Mostafa Elfeky, 18 Jan 2023

Disclosures - updated 11 May 2022: Nothing to disclose

Updates to Case Attributes

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Hypoplastic right heart syndrome (HRHS) is a rare congenital cardiac anomaly that presents with underdevelopment of the right ventricle.

itIt is usually associated with pulmonary atresia, tricuspid atresia and septal defects.

-<a href="/articles/hypoplastic-right-heart-syndrome" title="Hypoplastic right heart syndrome (HRHS) ">Hypoplastic right heart syndrome (HRHS)</a> is a rare <a href="/articles/congenital-cardiovascular-anomalies" title="Congenital cardiovascular anomalies">congenital cardiac anomaly</a> that presents with underdevelopment of the right ventricle.it is usually associated with pulmonary atresia, tricuspid atresia and septal defects.
+<a href="/articles/hypoplastic-right-heart-syndrome" title="Hypoplastic right heart syndrome (HRHS) ">Hypoplastic right heart syndrome (HRHS)</a> is a rare <a href="/articles/congenital-cardiovascular-anomalies" title="Congenital cardiovascular anomalies">congenital cardiac anomaly</a> that presents with underdevelopment of the right ventricle.It is usually associated with pulmonary atresia, tricuspid atresia and septal defects.

References changed:

2. Mohan J, Mohan V, Shukla M, Sethi A. Hypoplastic Right Heart Syndrome, Absent Pulmonary Valve, and Non-Compacted Left Ventricle in an Adult. Indian Heart J. 2016;68 Suppl 2(Suppl 2):S229-32. <a href="https://doi.org/10.1016/j.ihj.2016.03.030">doi:10.1016/j.ihj.2016.03.030</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27751299">Pubmed</a>
2.Mohan J, Mohan V, Shukla M, Sethi A. Hypoplastic Right Heart Syndrome, Absent Pulmonary Valve, and Non-Compacted Left Ventricle in an Adult. Indian Heart J. 2016;68 Suppl 2(Suppl 2):S229-32. <a href="https://doi.org/10.1016/j.ihj.2016.03.030">doi:10.1016/j.ihj.2016.03.030</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27751299">Pubmed</a>

Systems changed:

Findings was changed:

Atretic right ventricle with intact interventricular septum.

Absent right atrioventricular flow on color Doppler.

Additional A wave reversal was demonstrated in the ductus venosus spetral waveform.