Systemic Sirolimus Therapy for Infants and Children With Pulmonary Vein Stenosis

Patel, Jay; Briones, Michael; Mandhani, Mansi; Jones, Shannon; Suthar, Divya; Gray, Rosemary; Pettus, Joelle; McCracken, Courtney; Thomas, Amanda S.; Petit, Christopher J.

doi:10.1016/j.jacc.2021.04.013

Cited by 42 publications

(31 citation statements)

References 43 publications

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“…The establishment of this program, with the attendant enhanced surveillance of PVS patients, likely accounts for the higher rate of PVA in recent years. 19…”

Section: Discussionmentioning

confidence: 99%

Transcatheter Recanalization of Atretic Pulmonary Veins in Infants and Children

Patel

Mandhani

Gray

et al. 2022

Circ: Cardiovascular Interventions

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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.

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“…The establishment of this program, with the attendant enhanced surveillance of PVS patients, likely accounts for the higher rate of PVA in recent years. 19…”

Section: Discussionmentioning

confidence: 99%

Transcatheter Recanalization of Atretic Pulmonary Veins in Infants and Children

Patel

Mandhani

Gray

et al. 2022

Circ: Cardiovascular Interventions

Self Cite

View full text Add to dashboard Cite

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“…Furthermore, in addition to using the pressure gradient measured by echocardiography to assess the efficacy of BD, fluoroscopic measurement of the post-PD lumen size can be used to determine whether WSS has been restored to normal levels. Persistently elevated WSS following surgical or transcatheter intervention may lead to decisions in clinical management, including repeat interventions or possible use of targeted antiproliferative therapy [19,20]. The analysis method used in this study is relatively simple and is based on assumptions that might not strictly hold in some cases.…”

Section: Discussionmentioning

confidence: 99%

The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies

et al. 2021

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Pulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease progression include surgery and catheter-based balloon dilation and stent implantation. Its cause and mechanism of progression are unknown. In this pilot study, we propose and explore the hypothesis that elevated wall shear stress at discrete pulmonary venous sites triggers stenosis. To assess this theory, we retrospectively analyzed cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. Results are consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. The analysis also suggests the possibility of predicting the target lumen size necessary in a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression.

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“…We recently completed a review of the outcomes following SST therapy in 15 patients with severe PVS [25]. We found a positive response in the cohort treated with SST.…”

Section: Therapy For Pulmonary Vein Stenosis 41 Primary Therapymentioning

confidence: 96%

Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy

Kuo

Petit

2021

Children

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Pulmonary vein stenosis (PVS) is a difficult condition to treat due to recurrence and progression. In 2017, we developed a comprehensive PVS Program at our center to address the multidisciplinary needs of these patients. We discuss the components of our program and our approach to these patients, using a combination of primary (medical) therapy in addition to anatomic therapy to preserve vessel patency. A multidisciplinary approach to treating these challenging patients is critical.

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Systemic Sirolimus Therapy for Infants and Children With Pulmonary Vein Stenosis

Cited by 42 publications

References 43 publications

Transcatheter Recanalization of Atretic Pulmonary Veins in Infants and Children

Transcatheter Recanalization of Atretic Pulmonary Veins in Infants and Children

The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies

Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy

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