Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

Autor:	Constantine A; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Ferrero P; ACHD Unit, IRCCS-Policlinico San Donato, San Donato Milanese, Milan, Italy., Gribaudo E; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK., Mitropoulou P; Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Krishnathasan K; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Costola G; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK., Lwin MT; Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Fitzsimmons S; Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Brida M; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK.; Department of Medical Rehabilitation, Medical Faculty University of Rijeka, Rijeka, Croatia., Montanaro C; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Kempny A; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Heng EL; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Chessa M; ACHD Unit, IRCCS-Policlinico San Donato, San Donato Milanese, Milan, Italy.; UniSR - Vita Salute Sal Raffaele University, Milan, Italy., Dimopoulos K; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK., Rafiq I; Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.; National Heart and Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY London, UK.
Jazyk:	angličtina
Zdroj:	European journal of preventive cardiology [Eur J Prev Cardiol] 2024 Aug 22; Vol. 31 (11), pp. 1316-1323.
DOI:	10.1093/eurjpc/zwae031
Abstrakt:	Aims: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. Methods and Results: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. Conclusion: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment. Competing Interests: Conflict of interest: A.C. has received educational grants, personal fees, and non-financial support from Janssen-Cilag Limited. K.D. has received non-financial support from Janssen-Cilag Limited and has been a consultant to and received grants and personal fees from Janssen-Cilag Limited, Pfizer, GlaxoSmithKline, and Bayer/MSD. All the remaining authors have no relationships with industry to declare. (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)
Databáze:	MEDLINE
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