Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study

Background Cardiac involvement can be an initial manifestation in sarcoidosis. However, little is known about the association between various clinical phenotypes of cardiac sarcoidosis (CS) and outcomes. We aimed to analyze the relation of different clinical manifestations with outcomes of CS and to...

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Main Authors: Emanuele Bobbio, Per Eldhagen, Christian L. Polte, Clara Hjalmarsson, Kristjan Karason, Araz Rawshani, Pernilla Darlington, Susanna Kullberg, Peder Sörensson, Niklas Bergh, Entela Bollano
Format: Article
Language:English
Published: Wiley 2023-08-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.123.029481
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author Emanuele Bobbio
Per Eldhagen
Christian L. Polte
Clara Hjalmarsson
Kristjan Karason
Araz Rawshani
Pernilla Darlington
Susanna Kullberg
Peder Sörensson
Niklas Bergh
Entela Bollano
author_facet Emanuele Bobbio
Per Eldhagen
Christian L. Polte
Clara Hjalmarsson
Kristjan Karason
Araz Rawshani
Pernilla Darlington
Susanna Kullberg
Peder Sörensson
Niklas Bergh
Entela Bollano
author_sort Emanuele Bobbio
collection DOAJ
description Background Cardiac involvement can be an initial manifestation in sarcoidosis. However, little is known about the association between various clinical phenotypes of cardiac sarcoidosis (CS) and outcomes. We aimed to analyze the relation of different clinical manifestations with outcomes of CS and to investigate the relative importance of clinical features influencing overall survival. Methods and Results A retrospective cohort of 141 patients with CS enrolled at 2 Swedish university hospitals was studied. Presentation, imaging studies, and outcomes of de novo CS and previously known extracardiac sarcoidosis were compared. Survival free of primary composite outcome (ventricular arrhythmias, heart transplantation, or death) was assessed. Machine learning algorithm was used to study the relative importance of clinical features in predicting outcome. Sixty‐two patients with de novo CS and 79 with previously known extracardiac sarcoidosis were included. De novo CS showed more advanced New York Heart Association class (P=0.02), higher circulating levels of NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) (P<0.001), and troponins (P<0.001), as well as a higher prevalence of right ventricular dysfunction (P<0.001). During a median (interquartile range) follow‐up of 61 (44–77) months, event‐free survival was shorter in patients with de novo CS (P<0.001). The top 5 features predicting worse event‐free survival in order of importance were as follows: impaired tricuspid annular plane systolic excursion, de novo CS, reduced right ventricular ejection fraction, absence of β‐blockers, and lower left ventricular ejection fraction. Conclusions Patients with de novo CS displayed more severe disease and worse outcomes compared with patients with previously known extracardiac sarcoidosis. Using machine learning, right ventricular dysfunction and de novo CS stand out as strong overall predictors of impaired survival.
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spelling doaj.art-527424d7c5e04e8ca6937b2c1952aa282023-08-23T10:41:23ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802023-08-01121510.1161/JAHA.123.029481Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter StudyEmanuele Bobbio0Per Eldhagen1Christian L. Polte2Clara Hjalmarsson3Kristjan Karason4Araz Rawshani5Pernilla Darlington6Susanna Kullberg7Peder Sörensson8Niklas Bergh9Entela Bollano10Department of Cardiology Sahlgrenska University Hospital Gothenburg SwedenDepartment of Medicine Solna Karolinska Institutet Stockholm SwedenInstitute of Medicine at Sahlgrenska Academy University of Gothenburg Gothenburg SwedenDepartment of Cardiology Sahlgrenska University Hospital Gothenburg SwedenInstitute of Medicine at Sahlgrenska Academy University of Gothenburg Gothenburg SwedenDepartment of Cardiology Sahlgrenska University Hospital Gothenburg SwedenDepartment of Internal Medicine Södersjukhuset Stockholm SwedenDepartment of Respiratory Medicine, Theme Inflammation and Ageing Karolinska University Hospital Stockholm SwedenDepartment of Respiratory Medicine, Theme Inflammation and Ageing Karolinska University Hospital Stockholm SwedenDepartment of Cardiology Sahlgrenska University Hospital Gothenburg SwedenDepartment of Cardiology Sahlgrenska University Hospital Gothenburg SwedenBackground Cardiac involvement can be an initial manifestation in sarcoidosis. However, little is known about the association between various clinical phenotypes of cardiac sarcoidosis (CS) and outcomes. We aimed to analyze the relation of different clinical manifestations with outcomes of CS and to investigate the relative importance of clinical features influencing overall survival. Methods and Results A retrospective cohort of 141 patients with CS enrolled at 2 Swedish university hospitals was studied. Presentation, imaging studies, and outcomes of de novo CS and previously known extracardiac sarcoidosis were compared. Survival free of primary composite outcome (ventricular arrhythmias, heart transplantation, or death) was assessed. Machine learning algorithm was used to study the relative importance of clinical features in predicting outcome. Sixty‐two patients with de novo CS and 79 with previously known extracardiac sarcoidosis were included. De novo CS showed more advanced New York Heart Association class (P=0.02), higher circulating levels of NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) (P<0.001), and troponins (P<0.001), as well as a higher prevalence of right ventricular dysfunction (P<0.001). During a median (interquartile range) follow‐up of 61 (44–77) months, event‐free survival was shorter in patients with de novo CS (P<0.001). The top 5 features predicting worse event‐free survival in order of importance were as follows: impaired tricuspid annular plane systolic excursion, de novo CS, reduced right ventricular ejection fraction, absence of β‐blockers, and lower left ventricular ejection fraction. Conclusions Patients with de novo CS displayed more severe disease and worse outcomes compared with patients with previously known extracardiac sarcoidosis. Using machine learning, right ventricular dysfunction and de novo CS stand out as strong overall predictors of impaired survival.https://www.ahajournals.org/doi/10.1161/JAHA.123.029481cardiac sarcoidosisendomyocardial biopsyheart failureinflammatory heart diseasemachine learningright ventricular function
spellingShingle Emanuele Bobbio
Per Eldhagen
Christian L. Polte
Clara Hjalmarsson
Kristjan Karason
Araz Rawshani
Pernilla Darlington
Susanna Kullberg
Peder Sörensson
Niklas Bergh
Entela Bollano
Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
cardiac sarcoidosis
endomyocardial biopsy
heart failure
inflammatory heart disease
machine learning
right ventricular function
title Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
title_full Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
title_fullStr Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
title_full_unstemmed Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
title_short Clinical Outcomes and Predictors of Long‐Term Survival in Patients With and Without Previously Known Extracardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study
title_sort clinical outcomes and predictors of long term survival in patients with and without previously known extracardiac sarcoidosis using machine learning a swedish multicenter study
topic cardiac sarcoidosis
endomyocardial biopsy
heart failure
inflammatory heart disease
machine learning
right ventricular function
url https://www.ahajournals.org/doi/10.1161/JAHA.123.029481
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