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Yazicioğlu, Alkın; Alıcı, İbrahim Onur; Karaoğlanoğlu, Nurettin; Yekeler, Erdal

doi:10.6002/ect.2015.0190

Cited by 6 publications

(3 citation statements)

References 6 publications

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“… 1 Failed graft implantation was even reported in 1 patient with concomitant scoliosis. 2 With adequate PA length and mobilization, we were able to perform an end-to-end anastomosis by swapping the donor PA between the epibronchial and prebronchial location. For airway anastomosis, 2 methods have been proposed.…”

Section: Discussionmentioning

confidence: 99%

Bilateral sequential lung transplantation in Kartagener syndrome

et al. 2020

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Video clip is available online. Kartagener syndrome (KS) is a rare congenital disease characterized by a triad of sinusitis, bronchiectasis, and situs inversus. Bronchiectasis can progress to end-stage lung failure, and lung transplantation (LTx) might be the only effective treatment. LTx in patients with KS has been technically challenging; however, available reported data are scarce. We have described 2 cases of bilateral sequential LTx in patients with KS and highlighted the pitfalls encountered. The institutional review board of the University of Hong Kong approved the study protocol, and the included patients provided written informed consent. CASE REPORT Patient 1Our first patient was 40-year-old nonsmoking woman with KS and advanced bronchiectasis requiring repeated hospital admissions for infective exacerbation. She had been listed for LTx in 2013 and had undergone bilateral sequential LTx in 2015. The donor and recipient characteristics are listed in Table 1. During the recipient procedure, a clamshell incision was used, and chest entry was performed through the fourth intercostal space. We inserted a Medtronic EOPA 18F arterial cannula and Medtronic Carmeda venous cannula (Medtronic, Dublin, Ireland) into the ascending aorta and right atrium, respectively, and venoarterial extracorporeal membrane oxygenation (ECMO) support using the Cardiohelp system (Maquet, Rastatt, Germany) was begun. The recipient's morphologically right (anatomically left) lung was explanted. The right main bronchus was anastomosed, followed by left atrial anastomosis. The donor pulmonary artery (PA) had to be transposed in a posterosuperior direction to anastomose it to the superiorly located recipient PA. On the left side, the donor PA was displaced anteriorly, circumventing the left main bronchus (Figure 1, A). The remainder of the left lung implant was performed in accordance with standard practice. The duration of ECMO support was 5 hours, 44 minutes. Because of the significant size mismatch, nonanatomic lung volume reduction over the right middle lobe

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Section: Discussionmentioning

confidence: 99%

Bilateral sequential lung transplantation in Kartagener syndrome

et al. 2020

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“…[ 1 ] It is characterized by the reversal of the organs in the mirror image of the normal arrangement. [ 2 ] Situs inversus totalis reported in current literature is often associated with congenital heart disease, [ 3 ] kidney malformation, [ 1 ] umbilical cord dysplasia, [ 4 ] chronic sinusitis/nasal polyps, and bronchiectasis (Kartagener syndrome, KS). [ 5 ] Some researchers suggested that the physical and chemical factors and mutant genes leading to situs inversus totalis may be also the reason for the tethered spinal cord, split cord malformation and Klippel Feil syndrome.…”

Section: Introductionmentioning

confidence: 99%

Congenital hemivertebrae combined with situs inversus totalis: A rare case report

Guo,

Li,

Zang

2024

Medicine

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Rationale: Situs inversus totalis is a rare malposition of organs that typically involves lesions in the respiratory, circulatory, or urinary systems. Cases of congenital hemivertebrae combined with situs inversus totalis are extremely rare and have limited reports. Patient concerns: We report a 2.5 years old girl with 2 congenital hemipyramids and complete visceral inversion who ultimately underwent hemilaminectomy. Diagnosis: Congenital hemivertebrae combined with situs inversus totalis. Intervention: The patient underwent hemilaminectomy. Outcomes: The spinal deformity was corrected. Lessons: For patient with spinal deformities combined with situs inversus totalis, surgery can be an effective treatment method. But we also need to be vigilant about the dysfunction of various systems.

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“…Since normal ciliary function is necessary to control the cardiac laterality during embryologic development, a spectrum of organ laterality defects occur with PCD including situs inversus totalis and situs ambiguus. The triad of situs inversus, chronic sinusitis, and bronchiectasis has historically been referred to as Kartagener syndrome (KS), a subgroup today identified as PCD with situs abnormalities ( 4 , 5 ).…”

Section: Introductionmentioning

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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Cited by 6 publications

References 6 publications

Bilateral sequential lung transplantation in Kartagener syndrome

Bilateral sequential lung transplantation in Kartagener syndrome

Congenital hemivertebrae combined with situs inversus totalis: A rare case report

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

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