Transcatheter Recanalization of Atretic Pulmonary Veins in Infants and Children

Abstract

Background: Pulmonary vein stenosis is a progressive disease associated with a high rate of mortality in children. If left untreated, myofibroblastic proliferation can lead to pulmonary vein atresia (PVA). In our experience, transcatheter recanalization has emerged as a favorable interventional option. We sought to determine the acute success rate of recanalization of atretic pulmonary veins and mid-term outcomes of individual veins after recanalization. Methods: We reviewed all patients with PVA at our institution between 2008 and 2020 diagnosed by either catheterization or cardiac computed tomography. All veins with successful recanalization were reviewed and procedural success rate and patency rate were noted. Competing risk analysis was performed to demonstrate outcomes of individual atretic veins longitudinally. Results: Between 2008 and 2020, our institution diagnosed and treated 131 patients with pulmonary vein stenosis. Of these, 61 patients developed atresia of at least one pulmonary vein. In total, there were 97 atretic pulmonary veins within this group. Successful recanalization was accomplished in 47/97 (48.5%) atretic veins. No atretic pulmonary veins were successfully recanalized before 2012. The majority of veins were recanalized between 2017 and 2020—39/56 (70%). The most common intervention after recanalization was drug-eluting stent placement. At 2-year follow-up 42.6% of recanalized veins (20.6% of all atretic veins) remained patent with a median of 4 reinterventions per person. Conclusions: Transcatheter recanalization of PVA can result in successful reestablishment of flow to affected pulmonary veins in many cases. Drug-eluting stent implantation was the most common intervention performed immediately post-recanalization. Vein patency was maintained in 42.6% of patients at 2-year follow-up from recanalization with appropriate surveillance and reintervention. Overall, only a small portion of atretic pulmonary veins underwent successful recanalization with maintained vessel patency at follow-up. Irrespective of successful recanalization, there was no detectable survival difference between the more recently treated PVA cohort and non-PVA cohort.

Utility of simultaneous triple balloon inflation technique through a single 6-French sheath in treating pulmonary vein stenosis

“…Pessimism has given way to optimism in the management of this condition. [1][2][3][4] While trifurcation pulmonary vein lesions are frequently encountered in clinical practice, there is a lack of literature describing their treatment. Most published research primarily focuses on individual pulmonary veins, without addressing lobar or segmental vein involvement.…”

“…Most published research primarily focuses on individual pulmonary veins, without addressing lobar or segmental vein involvement. [1][2][3][4] Treating trifurcation lesions presents greater challenges in preserving the patency of each segmental or lobar vein's ostium. Interventional cardiologists often face the dilemma of sacrificing patency in one segment to maintain it in the larger ones.…”

“…Infants with pulmonary vein disease often undergo multiple cardiac catheterisation procedures, including stent placement and balloon angioplasty. [1][2][3][4] In practice, one or more pulmonary veins become compressed when adjacent veins are dilated. Interventional cardiologists frequently accept some degree of narrowing in one vein to maintain the patency of others.…”

Pulmonary Vein Atresia: Is There Hope for a Good Outcome?

“…7 In other diseases, these mTOR (mechanistic target of rapamycin) inhibitors are used to prevent the upregulation of myofibroblasts, making it understandable to extrapolate that they could be helpful in pulmonary vein disease management as well whether delivered locally or systemically. [8][9][10][11] In this issue of Circulation: Cardiovascular Interventions, Patel et al 12 present the largest cohort of pediatric patients treated for pulmonary vein atresia, the most difficult to manage subtype of disease. While the study was not focused on analyzing overall survival benefits with reintervention, it was able to demonstrate that percutaneous intervention for the treatment of pulmonary vein atresia was safe and feasible with vessel patency at 2-year follow-up (42.6%).…”

“…In this issue of Circulation: Cardiovascular Interventions , Patel et al 12 present the largest cohort of pediatric patients treated for pulmonary vein atresia, the most difficult to manage subtype of disease. While the study was not focused on analyzing overall survival benefits with reintervention, it was able to demonstrate that percutaneous intervention for the treatment of pulmonary vein atresia was safe and feasible with vessel patency at 2-year follow-up (42.6%).…”