Transcatheter recanalisation for absent intra-pericardial pulmonary arteries.

Unilateral absence of intra-pericardial pulmonary artery is a rare congenital malformation. If untreated, it can lead to morbidity and mortality in adulthood. Early intervention and restoration of physiologic pulmonary blood flow is necessary. Transcatheter stenting as initial intervention has been rarely reported. We present transcatheter recanalisation and stenting of the obliterated ductus in two newborns with unilateral absence of intra-pericardial pulmonary artery with cross-sectional imaging, procedural details, angiography, and follow up to surgical repair. We believe that such procedure promotes ipsilateral pulmonary vasculature growth to facilitate unifocalization surgery at a later age.

Prenatal diagnosis of ductal origin of distal pulmonary artery: presentation of three cases and literature review.

Ductal origin of distal pulmonary artery (DODPA) is a rare congenital cardiovascular anomaly, with an incidence of approximately 1 in 200 000 people. It involves the proximal interruption of one pulmonary artery branch, with the distal part arising from the base of the brachiocephalic artery, via the ipsilateral ductus arteriosus (DA) and the intrapulmonary branch of the pulmonary artery is usually intact. If timely treatment is not provided after birth, it is very likely that, due to DA closure, severe hypoplasia will occur in the lung supplied by the abnormal pulmonary artery. Hence, prenatal diagnosis of DODPA is important to enable initiation of prostaglandin treatment and early rehabilitation of the affected lung. Herein, we report three cases of fetal DODPA diagnosed via two-dimensional (2D) ultrasonography combined with four-dimensional (4D) spatiotemporal image correlation. We also present a literature review, and explore the ultrasonographic findings and the importance of 2D and 4D ultrasonography in obtaining an accurate prenatal diagnosis of DODPA. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

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

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.

Case report: the 'vanished' left pulmonary artery.

Background: We report a case of isolated ductal origin of pulmonary artery (DOPA) diagnosed in an asymptomatic newborn. The primary aim of this case is to highlight the need to investigate for DOPA in patients diagnosed with an 'absent branch pulmonary artery'. Case summary: Our patient was an asymptomatic newborn infant, with normal intracardiac anatomy. He was initially diagnosed post-natally with 'absent left pulmonary artery' (LPA), though the LPA was seen in antenatal scans. He underwent angiography and was re-diagnosed with bilateral arterial ducts, with ductal origin of the LPA from the left arterial duct. The LPA was salvaged by first stenting the left arterial duct on Day 11 of life, with subsequent surgery to connect the LPA to the main pulmonary artery at 4.5 months old. The patient had an uneventful recovery after the surgery. Discussion: Ductal origin of pulmonary artery is a rare vascular anomaly characterized by continuity of the left or right pulmonary artery (PA) with the distal end of the arterial duct, and discontinuity with the main PA. It is commonly misdiagnosed as pulmonary artery agenesis when the patent arterial duct constricts, with cessation of blood flow into the affected pulmonary artery. A high index of suspicion is necessary for diagnosis of DOPA. Once diagnosed, this lesion is clearly amenable to intervention, with benefits from unifocalization, to prevent late onset pulmonary hypertension or cardiac failure.