Bilateral sequential lung transplantation in Kartagener syndrome.
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ABSTRACT:
SUBMITTER: Lucius Lee KF
PROVIDER: S-EPMC8302999 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
Publications
Bilateral sequential lung transplantation in Kartagener syndrome.
Lucius Lee Kwok Fai KF Michael Hsin Kuan Yew KY Alva Sit Ko Yung KY Cally Ho Ka Lai KL Timmy Au Wing Kuk WK
JTCVS techniques 20200624
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Project description:The surgical technique for lung transplantation has evolved dramatically over the last three decades. Significant improvements in short term outcomes in the early years of lung transplantation were due, in large part, to techniques developed to reduce airway anastomotic complications in single lung transplantation. Following development of the technique of en bloc double lung transplantation, evolution to the bilateral sequential technique further reduced airway complications for double lung transplantation. More recently, some programs have utilized the en bloc double lung transplant technique with bronchial artery revascularization to aid airway healing and potentially improve short- and long-term outcomes. The experience with bronchial artery revascularization remains limited to a few series, with the technique having not been widely adopted by most lung transplant programs. With the implementation of priority allocations schemes in many countries, patients with higher risk profiles are being prioritized for transplantation which results in more complex procedures in fragile recipients with multiple comorbidities. This includes the increased need for concomitant cardiac procedures as well as performing lung transplantation after prior cardiothoracic surgery. Different surgical approaches have been described for bilateral sequential lung transplantation with or without intra-operative mechanical circulatory support (MCS), such as sternotomy, clamshell (bilateral anterior thoracotomies with transverse sternotomy), and bilateral thoracotomy incisions. Herein, we aim, not only to describe the various surgical approaches for double lung transplantation, but to provide a comprehensive review of other aspects related to the recipient pathology and different anatomical variants as well as handling technical challenges that might be encountered during the procedure.
| S-EPMC8662466 | biostudies-literature
Project description: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.
| S-EPMC9970992 | biostudies-literature
Project description:Kartagener syndrome (KS) is an autosomal recessive disorder characterized by situs inversus, paranasal sinusitis and bronchiectasis. We report the successful use of double lung transplant (DLTx) to treat end-stage KS. A 49-year-old Han woman was admitted to Renmin Hospital (Wuhan University, China) in September 2017 with a ?15 year history of chronic productive cough that had worsened during the past year. Clinical examination and imaging investigations revealed respiratory failure and situs inversus consistent with KS. The patient was successfully treated with DLTx involving bilateral bronchial anastomoses. DLTx is a feasible treatment option for end-stage KS.
| S-EPMC7212169 | biostudies-literature
Project description:BackgroundSize mismatch between donor lungs and a recipient thorax could affect the major determinants of maximal expiratory airflow: airway resistance, propensity of airways to collapse, and lung elastic recoil.MethodsA retrospective review of 159 adults who received bilateral lung transplants was performed. The predicted total lung capacity (pTLC) for donors and recipients was calculated based on sex and height. Size matching was represented using the following formula: pTLC ratio = donor pTLC / recipient pTLC. Patients were grouped according to those with a pTLC ratio > 1.0 (oversized) or those with a pTLC ratio ≤ 1.0 (undersized). Allograft function was analyzed in relation to the pTLC ratio and to recipient and donor predicted function.ResultsThe 96 patients in the oversized cohort had a mean pTLC ratio of 1.16 ± 0.13 vs 0.89 ± 0.09 in the 63 patients of the undersized group. At 1 to 6 months posttransplant, the patients in the oversized cohort had higher FEV(1)/FVC ratios (0.895 ± 0.13 vs 0.821 ± 0.13, P < .01) and lower time constant estimates of lung emptying (0.38 ± 0.2 vs 0.64 ± 0.4, P < .01) than patients in the undersized cohort. Although the FVCs expressed as % predicted for the recipient were not different between cohorts, the FVCs expressed as % predicted for the donor organ were lower in the oversized cohort compared with the undersized cohort (at 1-6 months, 52.4% ± 17.1% vs 65.3% ± 18.3%, P < .001). Kaplan-Meier estimates for the occurrence of bronchiolitis obliterans syndrome (BOS) showed that patients in the oversized cohort had a lower probability of BOS (P < .001).ConclusionsA pTLC ratio > 1.0, suggestive of an oversized allograft, is associated with higher expiratory airflow capacity and a less frequent occurrence of BOS.
| S-EPMC5991546 | biostudies-literature
Project description:PurposeWe present the first case in the literature of a patient with Kartagener syndrome and ocular findings of nonexudative age-related macular degeneration.ObservationsA 55-year-old woman with Kartagener syndrome and chronic angle closure glaucoma presented for evaluation of the retina. Optos ultra-widefield imaging of the fundus showed glaucomatous cupping, drusen, and retinal pigment epithelium changes within the macular region. Humphrey visual field testing confirmed glaucomatous changes. Drusenoid pigment epithelial detachments were observed bilaterally with optical coherence tomography.Conclusions and importanceWe hypothesize that in addition to the lungs, spermatozoa and the Fallopian tubes, the retinal pigment epithelium may also be affected by ciliary dysfunction in individuals with Kartagener syndrome. Given recent advances in our knowledge of retinal ciliopathies, further studies are needed to understand how ciliary dysfunction affects the retina in Kartagener syndrome.
| S-EPMC5834646 | biostudies-literature
Project description:Situs inversus totalis is a congenital syndrome characterized by a total left-right transposition of all abdominal and thoracic organs. It may be associated with chronic respiratory conditions such as sinusitis and bronchiectasis, composing the Kartagener syndrome. If not detected, this condition may compromise the early diagnosis of surgical emergencies such as cholecystitis and appendicitis. A rare case of appendicitis in a patient with Kartagener syndrome is here reported.
| S-EPMC7063213 | biostudies-literature
Project description:BackgroundDonor-to-recipient lung size matching at lung transplantation (LTx) can be estimated by the predicted total lung capacity (pTLC) ratio (donor pTLC/recipient pTLC). We aimed to determine whether the pTLC ratio is associated with the risk of primary graft dysfunction (PGD) after bilateral LTx (BLT).MethodsWe calculated the pTLC ratio for 812 adult BLTs from the Lung Transplant Outcomes Group between March 2002 to December 2010. Patients were stratified by pTLC ratio >1.0 ("oversized") and pTLC ratio ≤1.0 ("undersized"). PGD was defined as any ISHLT Grade 3 PGD (PGD3) within 72 hours of reperfusion. We analyzed the association between risk factors and PGD using multivariable conditional logistic regression. As transplant diagnoses can influence the size-matching decisions and also modulate the risk for PGD, we performed pre-specified analyses by assessing the impact of lung size mismatch within diagnostic categories.ResultsIn univariate analyses oversizing was associated with a 39% lower odds of PGD3 (OR 0.61, 95% CI, 0.45-0.85, p = 0.003). In a multivariate model accounting for center-effects and known PGD risks, oversizing remained independently associated with a decreased odds of PGD3 (OR 0.58, 95% CI 0.38 to 0.88, p = 0.01). The risk-adjusted point estimate was similar for the non-COPD diagnosis groups (OR 0.52, 95% CI 0.32 to 0.86, p = 0.01); however, there was no detected association within the COPD group (OR 0.72, 95% CI 0.29 to 1.78, p = 0.5).ConclusionOversized allografts are associated with a decreased risk of PGD3 after BLT; this effect appears most apparent in non-COPD patients.
| S-EPMC4329253 | biostudies-literature
Project description: Not available
| S-EPMC9037598 | biostudies-literature
Project description:PurposeEnd-stage lung diseases result in anatomical changes of the thoracic cavity. However, very few studies have assessed changes in the thoracic cavity after lung transplantation (LTx). This study aimed to evaluate the relationships between thoracic cavity volume (TCV) changes after LTx and underlying lung disease.MethodsWe reviewed 89 patients who underwent a pre-LTx pulmonary function test (PFT), chest computed tomography (CT) scan, and 1-year follow-up CT after LTx. These patients were classified into two groups according to pre-LTx PFT as follows: obstructive group [forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio < 70%] and restrictive group (FEV1/FVC ratio > 70%). We measured TCV using CT scan before and at 1 year after LTx and compared the TCV change in the two groups.ResultsIn the restrictive group, TCV increased after LTx (preop: 2,347.8 ± 709.5 mL, 1-year postop: 3,224.4 ± 919.0 mL, p < 0.001). In contrast, in the obstructive group, it decreased after LTx (preop: 4,662.9 ± 1,296.3 mL, 1-year postop: 3,711.1 ± 891.7 mL, p < 0.001). We observed that restrictive lung disease, taller stature, lower body mass index, and larger donor lung were independently associated with increased TCV after LTx.ConclusionThe disease-specific chest remodeling caused by restriction and hyperinflation is at least, in part, reversible. After LTx, the chest remodeling appears to occur in the opposite direction to the disease-specific remodeling caused by the underlying lung disease in recipients.
| S-EPMC9204381 | biostudies-literature
Project description: Not available
| S-EPMC8302984 | biostudies-literature