Recanalization and Stenting of the Occluded Vertical Vein as Rescue and Bridging Strategy in a Critically Ill Neonate with Supracardiac Total Anomalous Pulmonary Venous Connection

Objectives: TAPVC of the supracardiac type involves drainage of all pulmonary veins (PVs) into the superior vena cava (SVC), typically via a vertical vein (VV). Severe obstruction or occlusion of the VV rapidly leads to postnatal decompensation with pulmonary edema, which may be indicative against immediate surgical correction. Moreover, VV occlusion makes diagnosis harder by echocardiography alone. Although catheterization usually exacerbates the condition, it may also provide the palliative option of VV stenting.

Methods: After a prenatal diagnosis of congenital heart disease, a term female newborn was born at our center weighing 3.1 kg. At birth, she was intubated, and prostaglandin E1 infusion started. Via mechanical ventilation (with FiO₂ 1.0), we achieved a maximum 70% oxygen saturation. Chest X-ray showed pulmonary edema. Postnatal echocardiography revealed situs ambiguous with a left-sided inferior vena cava and right-sided SVC, dysbalanced AV-septal defect, ventriculoarterial discordance, pulmonary atresia, vertical tortuous duct (PDA) to confluent branch pulmonary arteries (PAs), and PVs converging to a horizontally oriented confluence behind the left atrium but with no reliable evidence of a draining VV. To clarify this, we opted for a PA angiography via the PDA. Moreover, the internal jugular vein was considered bailout access for venovenous extracorporal membrane oxygenation.

Result: Catheterization was urgently done via transfemoral venous access. Following PDA stenting, however, selective PA angiography still failed to identify a draining vein for the PVs: unfortunately only a diffuse, shadowy distribution of contrast agent was visible in the venous phase (video). Actually, we detected the VV’s blind stump via the V. Anonyma and the left vein angle: its caudal segment was completely occluded, but probable with coronary wires, followed by dilation and recanalization with conventional and cutting balloons, as well as 2 stents. As a result, pressure in the confluence dropped from initially 31 to 8 mm Hg. Through all these measures, we succeeded in stabilizing the child with saturation levels around 85% (with FiO₂ 0.21), and successful extubation after 11 days. We are now planning to postpone surgery for another 3 to 6 weeks.

Conclusion: Here, we report on a critically ill neonate with pulmonary atresia and obstructive supracardiac TAPVC successfully palliated by transfemoral stenting both the PDA and VV. In this setting, VV stenting may be considered a short-term rescue option to bridge to surgical intervention.