Contralateral Pulmonary Hypertension Following Resuscitation of Unilateral Ductal Origin of a Pulmonary Artery: A Multi-institutional Review

Abstract

In isolated unilateral ductal origin of a pulmonary artery (DOPA), intervention to establish reperfusion of the affected lung without direct re-anastomosis may lead to pulmonary hypertension (PH) in the contralateral lung. Multicenter retrospective review of patients with unilateral DOPA, who underwent palliation with a ductal stent (DS) or Blalock-Taussig (BT) shunt with subsequent development of PH in the contralateral lung, was conducted. Ten patients (4 females; median weight 3.2 kg, range 2.1-5.2) who underwent DS or BT shunt and developed contralateral PH were identified. Eight infants had right-sided DOPA. Stents/shunt used had a median diameter of 3.5 mm (range 2.5-5.2). After DS in 9 patients, 7 patients were exclusively treated with medical therapies, whereas 2 patients received intermediate procedures: one received an additional contralateral DS and other underwent surgical banding of the DS prior to PA reimplantation. Seven patients who underwent DS and one patient with BT shunt underwent PA reimplantation at median of 3.3 (0.6-18) months. PA pressure was documented to be normal in 5 patients immediately following PA reimplantation, 1 year later in 2 patients, and 1 patient is on Tadalafil with elevated PVR of 5.5 indexed Wood units. One patient died and one patient is awaiting surgery with normal PA pressure. We describe the development of severe contralateral PH following DS or BT shunt as the initial intervention for unilateral DOPA. Pulmonary hypertension resolved in 7/8 patients who underwent surgical PA reimplantation. The cause of PH in the normally connected lung in these cases remains unclear.

Definition and Management of Segmental Pulmonary Hypertension

“…Several authors have used the terms “isolated PA of ductal origin” or “unilateral ductal origin of a PA” to more accurately describe this condition. Strategies to rehabilitate the isolated PA have been reported 12, 13, 14, 15, 16, 17…”

Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery

“…Although unifocalization subsequent to systemic-pulmonary shunt is often recommended for UAPA, primary unifocalization was selected in this case, assuming that the LPA had been fed adequately until birth. This strategy is further supported by the fact that a systemic-pulmonary shunt can be a risk factor for later pulmonary hypertension [7] and by the report that intervention in younger age is a determinant of neither increasing complications nor subsequent interventions [2,6]. Indeed, as compared with primary repair, patients with staged repair had significantly low pulmonary perfusion in the affected lung and underwent more surgical and total interventions despite older age (2.4 months vs 35 months).…”

The many faces and outcomes of pulmonary vein stenosis in early childhood

“…Although survival was better in drug eluting stent group, this was not statistically different. A limitation of this therapy is still the small size of initial stent implant, necessitating re‐dilation with intentional stent fracture in the catheterization laboratory over time 29,35 . It is thus important to recognize that no matter what therapy is utilized, strict surveillance therapy and frequent interventions in the catheterization laboratory are necessary to improve clinical outcomes over time 36 .…”