Objectives: Elevated pulmonary vascular resistance may contribute to late Fontan circulation failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contributed to the failure.
Methods: Fifteen patients (14 Fontan circulations, 1 Kawashima circulation) underwent transplantation. The most common indication was ventricular dysfunction (mean ventricular end-diastolic pressure 12.5 mm Hg). Patients with early failures (n = 4) required transplantation less than 1 year after the Fontan operation. Those with late failures (n = 11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fontan-transplantation interval was 7.4 years. Mean pulmonary arterial pressure, transpulmonary gradient, and pulmonary vascular resistance before and after transplantation were assessed. Paired t tests of variable differences were used to compare variables. Survival was estimated by the Kaplan-Meier method.
Results: In-hospital mortality was 7%. There were 2 late events (1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82% at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistance was elevated (>2.0 Wood units . m 2 ) in 11 of 14 survivors past initial hospitalization (mean 3.3 +/- 1.7 Wood units . m 2 ). Only patients with early Fontan failures (3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired comparisons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg ( P < .0001) relative to pretransplantation value.
Conclusions: Outcomes after heart transplantation for failed Fontan circulation were good. Mild-to-moderate pulmonary vascular disease was evident after heart transplantation for late failure. Elevated pulmonary vascular resistance is a likely contributor to Fontan circulation failure.
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http://dx.doi.org/10.1016/j.jtcvs.2004.07.013 | DOI Listing |
Andes Pediatr
August 2023
Departamento de Pediatría, Hospital Clínico Universitario San Cecilio, Granada, España.
Unlabelled: Unilateral absence of pulmonary artery (UAPA) is a rare and underdiagnosed entity. Due to its varied clinical expression, especially respiratory and most frequently associated with congenital heart disease, it can also present in isolation and remain asymptomatic for a long time. There is no consensus on its treatment, which is generally reserved for the presence of complications, mainly pulmonary hypertension, hemoptysis, or recurrent respiratory infections.
View Article and Find Full Text PDFClin Exp Rheumatol
January 2025
Department of Oncology and Vascular Interventional Radiology, Zhongshan Hospital Xiamen University, School of Medicine, Xiamen University, Xiamen, Fujian, China.
Objectives: Dermatomyositis (DM) is frequently associated with interstitial lung disease (ILD); however, the molecular mechanisms underlying this association remain unclear. This study aimed to employ bioinformatics approaches to identify potential molecular mechanisms linking DM and ILD.
Methods: GSE46239 and GSE47162 were analysed to identify common differentially expressed genes (DEGs).
Clin Exp Rheumatol
January 2025
Department of Medicine, Division of Rheumatology, University of California, Los Angeles, USA.
Objectives: This structured, targeted literature review aimed to assess the mortality, humanistic and economic burden of eight organ manifestations which are commonly experienced by systemic sclerosis patients.
Methods: Identification of relevant literature was carried out by searching in Ovid MEDLINE and EMBASE, PubMed, and NHS Economic Evaluation Database in August 2023. Studies reporting original data on patients with systemic sclerosis with at least one of eight organ manifestations (interstitial lung disease and/or pulmonary hypertension, skin, peripheral vascular, musculoskeletal, gastrointestinal, cardiac or renal involvement) published within the last 15 years were included.
J Thorac Cardiovasc Surg
January 2025
Coronary Center, Department of Thoracic and Cardiovascular Surgery, Miller Family Heart, Vascular, & Thoracic Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address:
Ann Thorac Surg
January 2025
Coronary Center, Department of Thoracic and Cardiovascular Surgery, Miller Family Heart, Vascular & Thoracic Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address:
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