Technical Challenges in Lung Transplantation of Kartagener Syndrome Recipients: A Unique Team Experience With 12 Patients

Castro, Caio César Bianchi de; Reis, Flávio Pola dos; Carvalho, Guilherme Vieira Soares de; Fernandes, Lucas Matos; Abdalla, Luís Gustavo; Samano, Marcos Naoyuki; Júnior, José Eduardo Afonso; Pěgo‐Fernandes, Paulo M.

doi:10.1016/j.transproceed.2020.02.031

Cited by 3 publications

(3 citation statements)

References 13 publications

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“…Another approach described by Mentzer facilitated the anastomosis of the left pulmonary artery by a termino-lateral anastomosis between the donor left pulmonary artery and the recipient origin of the truncus anterior artery, preventing possible distortion and obstruction of the pulmonary vessel ( 25 ). Furthermore De Castro suggested, to prevent a left pulmonary artery kinking, to leave a shorter pulmonary artery stump during the graft back table preparation ( 26 ).…”

Section: Discussionmentioning

confidence: 99%

Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study

Marro¹,

Leiva‐Juárez²,

D’Ovidio³

et al. 2023

Transpl Int

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Primary ciliary dyskinesia, with or without situs abnormalities, is a rare lung disease that can lead to an irreversible lung damage that may progress to respiratory failure. Lung transplant can be considered in end-stage disease. This study describes the outcomes of the largest lung transplant population for PCD and for PCD with situs abnormalities, also identified as Kartagener’s syndrome. Retrospectively collected data of 36 patients who underwent lung transplantation for PCD from 1995 to 2020 with or without SA as part of the European Society of Thoracic Surgeons Lung Transplantation Working Group on rare diseases. Primary outcomes of interest included survival and freedom from chronic lung allograft dysfunction. Secondary outcomes included primary graft dysfunction within 72 h and the rate of rejection ≥A2 within the first year. Among PCD recipients with and without SA, the mean overall and CLAD-free survival were 5.9 and 5.2 years with no significant differences between groups in terms of time to CLAD (HR: 0.92, 95% CI: 0.27–3.14, p = 0.894) or mortality (HR: 0.45, 95% CI: 0.14–1.43, p = 0.178). Postoperative rates of PGD were comparable between groups; rejection grades ≥A2 on first biopsy or within the first year was more common in patients with SA. This study provides a valuable insight on international practices of lung transplantation in patients with PCD. Lung transplantation is an acceptable treatment option in this population.

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Section: Discussionmentioning

confidence: 99%

Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study

Marro¹,

Leiva‐Juárez²,

D’Ovidio³

et al. 2023

Transpl Int

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“…Lung transplantation in patients with Kartagener's syndrome and situs inversus challenges include dextrocardia with discrepancies between donor and recipient vessels and airways. [5][6][7][8] In addition, the chest cavity mismatch between the donor right lung between and the recipient's dextrocardia can result in right lower lobe atelectasis and kinks of the pulmonary artery. In the present report, we did not experience any anastomotic or postoperative challenges.…”

Section: Discussionmentioning

confidence: 99%

Bridge to second double lung transplant with an extracorporeal carbon dioxide removal system in situs inversus patient

Furukawa

Chan

Sánchez

2022

Journal of Cardiac Surgery

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Background: Extracorporeal life support use in redo-lung transplant is limited due to poor outcomes. Extracorporeal circulation with a single duo-lumen cannula provides the advantage of more comfortable mobilization particularly in patients in which we expect a longer bridge to transplant.Case: A 29-year-old female with Kartagener syndrome and complete situs inversus underwent a double lung transplant for end stage lung disease. Within one year after transplant the patient had primarily hypercapnic respiratory failure with radiographic signs of chronic lung allograft dysfunction. To optimize her nutritional status and muscle strength before re-do lung transplantation, we decided to bridge her with an extracorporeal carbon dioxide removal system due to anatomical difficulty. She was listed and underwent an uneventful re-do double lung transplant with cardiopulmonary support. Conclusions:We report a first case with the use of extracorporeal carbon dioxide removal system as a bridge to re-do lung transplant in complete situs inversus patient.

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“…A discinesia ciliar primária ainda é uma doença pouco estudada na população brasileira. A maioria dos artigos de grupos brasileiros apresentam relatos ou series de casos e revisões da literatura (77)(78)(79)(80)(81) (47). Esses pacientes apresentavam mediana de idade de 41 anos, e mais da metade apresentou deterioração da função pulmonar grave (47).…”

Section: Realidade Brasileiraunclassified

Avaliação da microscopia eletrônica, do teste da sacarina, características clínicas e genéticas em pacientes com diagnóstico clínico de discinesia ciliar primária

Dalbo Contrera Toro

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Technical Challenges in Lung Transplantation of Kartagener Syndrome Recipients: A Unique Team Experience With 12 Patients

Cited by 3 publications

References 13 publications

Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study

Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study

Bridge to second double lung transplant with an extracorporeal carbon dioxide removal system in situs inversus patient

Avaliação da microscopia eletrônica, do teste da sacarina, características clínicas e genéticas em pacientes com diagnóstico clínico de discinesia ciliar primária

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