Isolated hypoplasia of the right ventricle: diagnosis, treatment and observation experience of pediatric patients
DOI:
https://doi.org/10.15574/PS.2024.83.27Keywords:
congenital heart defects, prenatal diagnosis, cyanosis, increasing the pulmonary blood flow, stent, echocardiographyAbstract
Isolated hypoplasia of the right ventricle (IHRV) is a rare congenital heart defect characterized by a reduction of the right ventricular cavity due to hypoplasia of its trabecular part.
Aim - to analyze the anatomical variants of IHRV and their influence on the choice of treatment tactics based on the experience of observing pediatric patients.
Materials and methods. This retrospective study included 11 consecutive patients diagnosed with IHRV from 2012 to 2024. Patients were divided into 3 groups based on the ratio of RV to left ventricle (LV) length. The Group I (n=4): mild IHRV (RV/LV length ratio >0.8). The Group II (n=4): moderate IHRV (RV/LV length ratio = 0.7-0.8). The Group III (n=3): severe IHRV (RV/LV ventricle length ratio <0.67).
Results. From the Group I (n=4), one patient required medication therapy with beta-blockers and others did not require any treatment. From the Group II (n=4), one patient underwent Glenn shunt and patent foramen ovale (PFO) closure, another - Glenn shunt and PFO closure with fenestration. Two patients have stable clinical conditions. Patients from the Group III (n=3) had duct-dependent pulmonary blood flow in the neonatal period. One patient received treatment with beta-blockers; the second patient underwent patent ductus arteriosus stenting; and the third - systemic-to-pulmonary shunt.
Conclusions. Patients with severe IHRV may have critical hemodynamic disturbances requiring urgent cardiac surgical interventions, whereas patients with mild and moderate hypoplasia may require medical treatment with possible surgical intervention at a later age. Long-term follow-up in this defect reveals a slow progressive growth of the right heart chambers.
The research was carried out in accordance with the principles of the Declaration of Helsinki. Informed consent of the child and child's parents was obtained for the research.
The authors declare no conflict of interest.
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