Rare presentation and management of patent large vertical vein in adulthood

A 32-year-old female presented to our Adult Congenital Heart Disease unit with progressive shortness of breath on exertion and lethargy. As a neonate she had been diagnosed with infra-cardiac TAPVC which was repaired in the conventional way with anastomosis of the pulmonary venous confluence to the left atrium, together with repair of an atrial septal defect, on the twelfth day of life. Apart from an episode of severe pneumonia at the age of 27, the patient had no other significant medical events. A triple-phase computed tomography (CT) scan demonstrated a persistent VV from the pulmonary venous confluence to the portal vein, as shown in (Fig. 1). Cardiac magnetic resonance imaging established that there was a haemodynamically significant shunt with a Qp:Qs of 2:1. Subsequent cardiac catheterisation confirmed normal pulmonary arterial pressure and no obstruction to the pulmonary venous confluence as it entered the left atrium (Fig. 2). During multi-disciplinary (MDT) team meetings, it was concluded that the shunt should be abolished by occlusion of the VV. Sternotomy and ligation of the VV in the chest was considered an excessively invasive approach and transcatheter intervention was rejected on the basis that it would necessitate high risk transeptal puncture and that there would be uncertainty about the stability of a vascular occlusion device placed in such a large VV. The preferred option, on the basis of safety and simplicity, was to perform a limited laparotomy with a trial of clamping of the VV before proceeding to ligation. At laparotomy, the VV was identified and with intraoperative ultrasound demonstrated to have an extremely high flow, the vein was fully mobilized from the diaphragm to its confluence with the portal vein (Fig. 3). A trial of clamping was performed which produced no major cardiovascular changes over a 30-min period. The maximum length of vein possible was resected to prevent recannalation by the use of a stapling device at the diaphragm superiorly and flush ligation and oversewing inferiorly at the junction with the portal vein. Post-operatively, the patient was admitted to ITU and subsequently made an uneventful recovery. She was discharged home on day 5 with 28 days of prophylactic-dose Dalteparin sodium. During the 4 month post-operative follow-up, echocardiography demonstrated good systolic function of both the left and right ventricles. The right ventricle remained mildly dilated but with a reduced end diastolic size compared with the pre-operative study. At 1 year follow up, the patient was asymptomatic and had returned to normal exercise, including strength training. It has often been assumed that in the case of neonatal TAPVC repair, the veins connecting the pulmonary and systemic veins will close spontaneously once the pulmonary venous confluence has been anastomosed to the left atrium. Here we demonstrate that this is not always the case. Our patient presented late in adulthood with a large persistent VV, which resulted in presenting symptoms. A persistent patent VV is found more commonly in cases of supra-cardiac TAPVC, as it provides less resistance when compared with the VV of an infra-diaphragmatic TAPVC.1 The literature concerning VV repair predominately describes treatment of the supra-cardiac type where the VV drains into the superior vena cava or brachiocephalic vein, while in the less common infra-cardiac variety it drains into the portal vein or inferior vena cava. Where VVs are obstructed, the left heart chambers are underdeveloped and smaller compared with non-obstructive cases and pulmonary arterial hypertension is present.2-5 The reduced cardiac output is a significant problem and treatment options include surgical attempts to increase the dimension of the left atrial cavity and pharmacological strategies to overcome pulmonary hypertensive crises. This results in haemodynamic instability and morbidity after surgery.4-7 These observations lead to the hypothesis that a patent VV may function as a temporary reservoir for pulmonary venous blood after TAPVC repair, volume unloading the small, noncompliant, left-sided cardiac chambers until they are able to grow and adapt to the increased flow demands.8, 9 Ligation of the VV can result in elevated left atrial pressure affecting cardiac output as a result of a poorly compliant left heart.1 The additional benefit of preserving the VV in infra-cardiac TAPVC is to prevent liver necrosis. Once the pulmonary pressures reduce satisfactorily and the left ventricle compliance has increased adequately, the VV can be safely closed.10 However, not all physicians and surgeons actively pursue this approach and there remains a paucity of evidence from the long-term follow-up data of patients after TAPVC repair with regard to persistent venous–venous connections. Prathima Gogineni: Data curation; formal analysis; project administration; writing – original draft; writing – review and editing. Muhammad Fahad Ullah: Conceptualization; methodology; resources; writing – original draft. Trisha Kanani: Writing – review and editing. John Isherwood: Supervision; validation; writing – review and editing. Aidan Bolger: Investigation; supervision; writing – review and editing. Ashley Dennison: Investigation; project administration; resources; supervision; validation; writing – review and editing.