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
Background: Postcongenital heart surgery pulmonary regurgitation requires subsequent pulmonary valve replacement. We sought to compare the outcomes of pulmonary valve replacement after using bioprosthetic valves, porcine versus pericardial bioprosthesis. Method: Retrospective single-center study of consecutive pulmonary valve replacement in patients with pulmonary regurgitation following initial congenital cardiac surgery. From 2004 to 2016, 82 adult patients (53 males, 29 females) underwent pulmonary valve replacement at a mean age of 28.7 + 8 years (range 18-52 years) with a mean time to pulmonary valve replacement of 24 + 7 years (range 13-43 years). Porcine bioprosthetic valves (group 1, n ¼ 32) and pericardial valves (group 2, n ¼ 50) were used. Cardiac magnetic resonance imaging was performed (n ¼ 54) at a mean of 18 + 13 months before and 24 + 21 months after pulmonary valve replacement. Results: No significant difference was seen between the groups except that the mean follow-up was longer for group 1 (5.02 + 2.06 vs 4.08 + 3.21 years). In-hospital mortality was 1.1%. Follow-up completeness was 100% with no late death. Mean right ventricular end-systolic and end-diastolic volumes reduced significantly in both the groups (P < .001), whereas right ventricular ejection fraction remained unchanged (group 1, P ¼ .129; group 2, P ¼ .675). Only the left ventricular end-diastolic volume increased in both the groups, but the increase was significant for group 2 only (group 1, P ¼ .070; group 2, P ¼ .015), whereas the left ventricular end-systolic and ejection fraction remained unchanged in both the groups. There was no reoperation for pulmonary valve replacement. Freedom from intervention was 93.8% (group 1) and 100% (group 2) at eight years after pulmonary valve replacement (P ¼ .407). Conclusion: Midterm outcomes of pulmonary valve replacement in our adult cohort were satisfactory. Both types of bioprosthetic valves performed comparably for eight years and were a good option in adults.
Background Total arch replacement with the frozen elephant trunk (FET) procedure has changed the landscape of therapy for aortic arch diseases. The optimal landing zone for a FET is controversial. We sought to share our early and midterm results of the FET procedure as well as compare the clinical outcomes of proximal and distal FET anastomosis. Methods A total of 100 patients who underwent total arch replacement using the FET technique were identified between November 2014 and August 2021. According to the FET anastomosis over the aortic arch, patients were classified into two groups (zone 0/1 vs. zone 2/3). In-hospital mortality, complications, and midterm outcomes were assessed based on patient characteristics. Results The overall in-hospital mortality was 8%. Major complications occurred in 32% of patients, including spinal cord injury (5%), stroke (7%), and acute kidney injury requiring dialysis (7%). Zone 2/3 FET (odds ratio: 6.491, 95% confidence interval: 1.930–21.835, p = 0.003) was an independent predictor of the composite endpoint of major complications. The rate of complete false lumen thrombosis was comparable (64.3% vs. 71.4%, p = 0.567). All patients, patients with zone 0/1 FET, and patients with zone 2/3 FET had 3-year freedom from aorta-related events of 73.0, 70.2, and 75.0%, respectively. There were no significant differences (log-rank test, p = 0.500). Conclusion Compared with zone 2/3, proximalization of FET using zone 0/1 for anastomosis was associated with better early outcomes and comparable rates of midterm aorta-related events. To substantiate its use, more research on this approach is required.
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