Management outcomes of primary pulmonary vein stenosis

Rosenblum, Joshua M.; Altin, Hüsnü Fırat; Gillespie, Scott; Bauser‐Heaton, Holly; Kanter, Kirk; Sinha, Raina; Cory, Melinda; Alsoufi, Bahaaldin

doi:10.1016/j.jtcvs.2019.08.105

Cited by 34 publications

(22 citation statements)

References 35 publications

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“…La ECVP se asocia con frecuencia a defectos cardiacos, como comunicaciones atriales o ventriculares, persistencia del conducto arterioso y ventrículos únicos (30-80%) 1 , 3 , 4 , 6 , 7 , 17 . La mayoría son de afección unilateral 18 , 19 . Nosotros no encontramos casos asociados con secuestro pulmonar.…”

Section: Discussionunclassified

“…Los predictores para reintervención o incluso de mal pronóstico son diagnóstico tardío, antecedente de prematurez con distrés respiratorio, edad menor de 6 meses, peso inferior a 3 kg, hipertensión pulmonar, afección en la presión sistólica ventricular derecha o afección bilateral de las venas pulmonares, y cardiopatía congénita con cortocircuito de izquierda a derecha 2 , 11 , 14 , 19 , 22 , 23 .…”

Section: Discussionunclassified

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Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar

Rubens-Figueroa¹,

Bobadilla-Chávez²,

Solórzano-Morales³

2021

ACM

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El objetivo es mostrar el diagnóstico y la evolución de una paciente con estenosis de venas pulmonares y secuestro pulmonar. Se trata de una niña de 1 año de edad, con bronconeumonías de repetición, acrocianosis, 2R intenso, cardiomegalia, hipertensión venocapilar pulmonar, con diagnóstico clínico de comunicación interauricular. El ecocardiograma mostró estenosis de venas pulmonares izquierdas. El cateterismo cardiaco detectó fístulas arteriovenosas en la región apical del pulmón derecho. La imagen de resonancia magnética y la angiografía mostraron un vaso arterial aberrante paralelo a la aorta abdominal y con flujo dirigido al lóbulo pulmonar derecho. La angiotomografía reportó confluencia de las venas pulmonares del lado derecho. Se realizó lobectomía derecha. La paciente falleció en el posoperatorio debido a una hemorragia masiva pulmonar. Esta paciente es la primera descrita en la literatura con estenosis de venas pulmonares congénita asociada a secuestro pulmonar. La ecocardiografía es el estudio diagnóstico ideal inicial en los pacientes con estenosis congénita de venas pulmonares.

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Section: Discussionunclassified

Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar

Rubens-Figueroa¹,

Bobadilla-Chávez²,

Solórzano-Morales³

2021

ACM

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“…In spite of advances in medical and surgical management of the disease, patients are at risk for restenosis and may require repeated interventional catheterizations and surgeries [ 5 , 6 ]. Although outcomes are excellent for single-vessel pulmonary vein stenosis, mortality can be significantly higher when there is multivessel involvement [ 7 , 8 ]. Because of the high acuity of this disease, noninvasive techniques for assessment of clinical status and disease progression are needed to guide treatment and prognosis.…”

Section: Introductionmentioning

confidence: 99%

Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis

et al. 2021

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(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0–4 = no disease–atresia) and lung segments (0–3 = unaffected–severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.

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“…Even with aggressive, multimodal therapy, response to treatment varies among patients [ 6 , 12 , 13 , 14 , 15 ]. While some patients experience disease stabilization over time (evidenced by less frequent need for pulmonary vein re-interventions), a substantial proportion of patients continue to require frequent re-interventions despite maximal therapy.…”

Section: Introductionmentioning

confidence: 99%

Aspiration Is Associated with Poor Treatment Response in Pediatric Pulmonary Vein Stenosis

2021

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Intraluminal pulmonary vein stenosis is a disease with significant morbidity and mortality, though recent progress has been made using multimodal therapy with antiproliferative agents. The aim of this study was to evaluate the association between aspiration and poor treatment response in patients with intraluminal pulmonary vein stenosis. A retrospective, single-center cohort analysis was performed of patients treated with a combination of imatinib mesylate and multimodal anatomic relief between March 2009 and November 2019. Analysis focused on 2-ventricle patients due to small numbers and clinical heterogeneity of single ventricle patients. Among the 84 patients included, 15 had single ventricle physiology and 69 had 2-ventricle physiology. Among the 2-ventricle group, multivariable analysis revealed that patients with clinical aspiration had nearly five times higher odds of poor treatment response than patients without aspiration (OR 4.85, 95% CI [1.37, 17.2], p = 0.014). Furthermore, male patients had higher odds of poor treatment response than their female counterparts (OR 3.67, 95% CI [1.04, 12.9], p = 0.043). Aspiration is a novel, potentially modifiable risk factor for poor treatment response in pediatric multi-vessel intraluminal pulmonary vein stenosis in patients with 2-ventricle physiology.

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Management outcomes of primary pulmonary vein stenosis

Cited by 34 publications

References 35 publications

Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar

Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar

Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis

Aspiration Is Associated with Poor Treatment Response in Pediatric Pulmonary Vein Stenosis

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