AI Article Synopsis
- - Clinical outcomes for isolated right ventricular hypoplasia (IRVH) vary based on the severity of the condition, ranging from spontaneous improvement to the need for complex surgeries like Fontan circulation.
- - An 8-month-old infant with IRVH showed spontaneous recovery of right ventricular function after a modified Blalock-Taussig shunt was closed percutaneously, indicating positive progress in her case.
- - A newborn with severe right ventricular issues was effectively treated with a right-side MBTS, resulting in improved ventricular development and a successful balloon occlusion of the shunt at 8 months, maintaining normal oxygen saturation afterward.
Article Abstract
Clinical presentation, course, and treatment for patients with isolated right ventricular (RV) hypoplasia (IRVH) depends on the degree of hypoplasia that is present-this is a spectrum from spontaneous maturation to Fontan circulation over time. An 8-month-old infant presented with IRVH; in the patient, a modified Blalock-Taussig (MBTS) shunt was closed percutaneously after spontaneous RV function recovery. A female newborn was diagnosed with differential cyanosis at birth. The echocardiography showed a hypertrophic RV with a small cavity, a right-left shunt on the atrial septal defect, an almost closed ductus arteriosus (DA), and a small tricuspid valve ring (Z-score-2) with mild regurgitation (pressure gradient 30 mmHg). On the 4th day of life, the patient showed deepened cyanosis and hyperlactatemia was registered. The echocardiography examination revealed a closed DA. Right ventriculography performed on the 5th day of life evidenced the presence of a small hypertrabeculated RV. The pressure in the RV increased. A right-side MBTS was created on the 6th day of life. Further echocardiographic findings indicated a gradual development of the RV and a decrease in RV pressure. MBTS occlusion was performed when the patient was 8 months old. Vital parameters were monitored invasively and noninvasively after the balloon occlusion of MBTS. Percutaneous MBTS occlusion was successfully performed using an Amplatzer vascular plug 2 (AVP2). During the follow-up period, the patient was found to have maintained a normal percutaneous oxyhaemoglobin blood saturation.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10672504 | PMC |
| http://dx.doi.org/10.3390/jcdd10110460 | DOI Listing |
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