Living-donor single-lobe lung transplantation for pulmonary hypertension due to alveolar capillary dysplasia with misalignment of pulmonary veins

Abstract

Abbreviations: ACD/MPV, alveolar capillary dysplasia with misalignment of pulmonary veins; ASD, atrial septal defect; CLAD, chronic lung allograft dysfunction; CPB, cardiopulmonary bypass; CT, computed tomography; EBER, Epstein-Barr virus encoded small RNAs; EBV, Epstein-Barr virus; ECMO, extracorporeal membrane oxygenation; FDG, fluorodeoxyglucose; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; ICU, intensive care unit; LDLLT, living donor lobar lung transplant; MMF, mycophenolate mofetil; PET, positron emission tomography; POD, postoperative day; PTLD, posttransplant lymphoproliferative disorder.

Meticulous closure of collateral vessels in the perihilar mediastinal pleura to control intraoperative bleeding during lung transplantation for pulmonary hypertension

“…Our results lend support to the notion that in patients with primary PH, hypoxia and endothelin-1 serve as potent stimuli for the growth of new vessels, resulting in neovascularization of the vasa vasorum and remodeling of collateral vessels (10)(11)(12)(13)(14)(15). Although we mainly use monopolar electrocautery during LT, division of the collateral vessels by electrocautery alone is associated with the risk of after-bleeding, especially under the condition of anticoagulation used for CPB or veno-arterial ECMO, which is generally required for LT in patients with primary PH (26)(27)(28)(29).…”

“…In the patients with primary PH, the elevated pulmonary pressure has been shown to cause neovascularization of the vasa vasorum and remodeling of the collateral vessels in the perihilar mediastinal pleura (10)(11)(12)(13)(14)(15). Unintentional injury of the collateral vessels in the perihilar mediastinal pleura, consequent to their becoming less visible due to the decreased blood flow during cardiopulmonary support, could result in uncontrollable bleeding during LT in patients with PH.…”

Alveolo-kapilläre Dysplasie – Fallbesprechung eines Neugeborenen mit typischem klinischen Verlauf und neuer genetischer Variante

“…dessen Expression beeinflussen. Heute werden einige Fälle auch genetisch identifiziert, die auch mildere Verläufe haben können 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 .…”

A Morphomolecular Approach to Alveolar Capillary Dysplasia

“…In most cases, ACD is a fatal developmental disorder of the pulmonary vasculature, and the only option for survival is lung transplantation, which again is extremely challenging in newborns. 9,10 To date, >200 ACD cases have been documented. Approximately 60% of all reported cases have been associated with distinct heterozygous variants of forkhead box F1 (FOXF1) gene (located at 16q24.1), its transcriptional enhancer (located 257 kb 5 0 to FOXF1), or other genetic alterations.…”