“Pathfinder-CHD Registry”: A prospective, comprehensive heart failure database of adults with Congenital Heart Disease

Ann-Sophie Kaemmerer-Suleiman (Erlangen)1, O. Dewald (Erlangen)2, P. Ewert (München)3, A. Freiberger (München)3, F. Harig (Erlangen)1, J. Hörer (München)4, M. Huntgeburth (München)3, N. Kohls (Coburg)5, N. Nagdyman (München)3, M. Suleiman (Erlangen)6, F. von Scheidt (München)3, H. Kaemmerer (München)3, S. Freilinger (München)3

1Universitäklinikum Erlangen Erlangen, Deutschland; 2Universitätsklinikum Erlangen Kinderherzchirurgische Abteilung Erlangen, Deutschland; 3Deutsches Herzzentrum München Klinik für angeborene Herzfehler und Kinderkardiologie München, Deutschland; 4Europäisches Kinderherzzentrum der Klinik für Chirurgie angeborener Herzfehler und Kinderherzchirurgie München, Deutschland; 5Hochschule Coburg Bereich Integrative Gesundheitsförderung Coburg, Deutschland; 6Universitätsklinikum Erlangen Klinik für Herzchirurgie Erlangen, Deutschland


Introduction: Heart failure is a common and substantial challenge in the management of adults with congenital heart defects (CHD). Managing heart failure in this particular population is complicated by the complex nature of the underlying CHD’s, as well as residua from previous surgical or interventional treatment. It often differs markedly from therapeutic approaches utilized in acquired heart diseases. The goal of the registry is to document clinical and epidemiological data, treatment approaches, and clinical outcome of adults with CHD and heart failure based on real-world data.

Methods: The Pathfinder-CHD Registry is a prospective, observational, web-based heart failure registry, established 2022 in Germany. Adults with all forms of manifest heart failure, with a history of previous heart failure, or at risk for the future development of heart failure from abnormal ventricular function or anatomy are included.

Results: Since November 2022, 1,377 patients (37.4 ± 17.3 years; 47.5% female) meeting the inclusion criteria have given consent to participate in a longitudinal study. A total of 554 (40.2%) patients had “complex heart anomalies”, including univenticular hearts, AV-valve atresia, congenitally corrected transposition, transposition of the great arteries, truncus arteriosus or Ebstein´s anomaly. 432 patients (31.7%) had “right sided heart anomalies”, including tetralogy of Fallot, pulmonary atresia, double outlet right ventricle, and pulmonary valve disease. “Left sided heart anomalies” (n=143, 10.4%), encompassed aortic valve disease, aortic coarctation CoA, and interrupted aortic arch. “Left to right shunt lesions” were present in 129 (9.3%), and “others” in 119 (8,6%). The mean age of the patients was 37.4 ± 17.3 years with 47.5% being female. 870 (63.2%) patients had cyanotic congenital heart disease as their primary diagnosis. The registry provides comprehensive documentation of the various heart failure types, as well as medical, surgical, and/or interventional treatments, comorbidities, and outcomes.    
Conclusion: The study contains comprehensive documentation of various heart failure types, as well as medical, surgical, and/or interventional treatments, comorbidities, and outcomes. The database enables the evaluation of the disease progression, risk factors, and the efficacy of different treatment strategies in the long term. The registry facilitates the creation of evidence-based, personalized treatment and predictive models to effectively address the increasing cardiovascular problems of adults suffering from CHD and heart failure.

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