It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management. Thus, a registry of MCS in this population would be beneficial to the field. A conference was convened in January 2012 of pediatric and adult cardiologists, pediatric cardiac intensivists, congenital heart surgeons, and adult cardiothoracic surgeons to discuss the current state of MCS, ventricular assist device, and total artificial heart therapy for patients who have undergone cavopulmonary connection, either superior cavopulmonary connection or total cavopulmonary connection. Specifically, individual experience and challenges with VAD therapy in this population was reviewed and creation of a multiinstitutional registry of MCS/ventricular assist device in this population was proposed. This document reflects the consensus from the meeting and provides a descriptive overview of the registry referred to as Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts.
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http://dx.doi.org/10.1111/chd.12053 | DOI Listing |
Diagnostics (Basel)
November 2024
Department of Pediatric Cardiac Surgery and Congenital Heart Disease, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico.
Background: Fontan circulation maintains an elevated venous pressure; this promotes venous and lymphatic congestion and may lead to late circuit failure. Our objective was to determine the association between thoracic lymphatic perfusion patterns assessed by magnetic resonance imaging and late Fontan failure.
Methodology: A retrospective study was performed.
Eur J Cardiothorac Surg
November 2024
Adult Congenital Heart Diseases Unit, Royal Brompton and Harefield Hospitals, Part of Guy's and St Thomas's NHS Foundation Trust, London, UK.
Objectives: There is dearth of data on long-term outcomes of systemic semilunar (SS) or atrioventricular (AV) valve operation in adult patients with a Fontan circulation. We describe a single-centre experience of adults who underwent valve surgery late after a Fontan procedure.
Methods: We retrospectively reviewed all patients with a Fontan circulation who had a reoperation for severe valve disease during adulthood in our centre.
Sci Rep
November 2024
Department of Pediatric Cardiology, University Children's Hospital, University of Tübingen, Tübingen, Germany.
Recent research in patients with functionally univentricular hearts (UVH) is focusing on pathologies of the lymphatic vessels. Morphology of the abdominal lymphatic vessels was analyzed by MRI in patients with UVH following total cavopulmonary connection (TCPC) and it was examined, if clinical and laboratory parameters correlate with changes after TCPC. We prospectively examined 33 patients at the age of 19.
View Article and Find Full Text PDFJ Am Heart Assoc
December 2024
Montreal Heart Institute, Université de Montréal Quebec Canada.
Background: The univentricular heart with a predominant right ventricle morphology (uRV) has been associated with a higher rate of adverse cardiovascular events. It remains to be determined whether the specific type of uRV influences outcomes.
Methods And Results: A North American multicenter retrospective cohort study was conducted by the Alliance for Adult Research in Congenital Cardiology on individuals with a uRV and total cavopulmonary connection Fontan.
Interdiscip Cardiovasc Thorac Surg
November 2024
Department of Cardiac Surgery, University Hospitals Leuven, Leuven, Belgium.
Objectives: This study aims to identify the causes of failure in Fontan patients with a total cavopulmonary connection.
Methods: We conducted a comprehensive review of all patients who underwent a total cavopulmonary connection procedure at our centre between 1988 and 2023, aiming to identify and analyse the factors contributing to Fontan failure (defined as mortality, heart transplantation, Fontan takedown, protein-losing enteropathy, plastic bronchitis or New York Heart Association Functional Classification class III or IV).
Results: The study included 217 patients (median age at time of Fontan completion 3.
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