Percutaneous Modified Blalock–Taussig Shunt Closure in a Patient with Isolated Right Ventricular Hypoplasia

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.

Keywords

• isolated right ventricular hypoplasia
• MBTS
• MBTS percutaneous closure
• AVP2