DOI: https://doi.org/10.36347/sjmcr.2026.v14i06.025

Pages: 1425-1430

Background: Persistent ductus venosus (PDV) is a rare congenital intrahepatic portosystemic shunt with an estimated incidence of 1 in 30,000 live births. It can cause severe pulmonary arterial hypertension (PAH) through persistent shunting of portal blood rich in vasoactive mediators directly into the systemic circulation, bypassing hepatic first-pass metabolism. Case presentation: A four-month-old female infant with suspected trisomy 21 presented with acute respiratory distress and supra-systemic PAH. CT angiography revealed a patent ductus venosus with a 3-mm portocaval shunt and a markedly dilated umbilical vein (11 mm). Surgical closure was performed; however, the initial clamp test triggered severe hemodynamic instability requiring epinephrine and norepinephrine. The postoperative course was complicated by failed extubation, acute pulmonary edema, and reintubation. On postoperative days three to four, hospital-acquired pneumonia precipitated severe acute respiratory distress syndrome (ARDS). The patient died despite maximal supportive care. Conclusions: This case illustrates the diagnostic complexity of PDV presenting with evolving PAH initially supra-systemic in the neonatal period and iso-systemic at admission in infancy, the significant perioperative hemodynamic risk of surgical shunt closure, and the potentially fatal vulnerability of these patients to hospital-acquired infections and secondary ARDS. Multidisciplinary management and full preoperative hemodynamic characterization are essential. Earlier diagnosis and intervention, before the establishment of fixed pulmonary vascular disease, may offer the best prospect for survival.