Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays
Background Isolated cardiac sarcoid (iCS) is reported to have more severe clinical presentation and greater risk of adverse events compared with cardiac sarcoid (CS) with extracardiac involvement (nonisolated CS). Delays in diagnosing specific organ involvement may play a role in these described dif...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-10-01
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| Series: | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| Subjects: | |
| Online Access: | https://www.ahajournals.org/doi/10.1161/JAHA.122.028342 |
| _version_ | 1827580735921324032 |
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| author | Joshua Sink Cara Joyce Max J. Liebo David J. Wilber |
| author_facet | Joshua Sink Cara Joyce Max J. Liebo David J. Wilber |
| author_sort | Joshua Sink |
| collection | DOAJ |
| description | Background Isolated cardiac sarcoid (iCS) is reported to have more severe clinical presentation and greater risk of adverse events compared with cardiac sarcoid (CS) with extracardiac involvement (nonisolated CS). Delays in diagnosing specific organ involvement may play a role in these described differences. Methods and Results A retrospective observational study of patients with CS over a 20‐year period was conducted. Objective evidence of organ involvement and time of onset based on consensus criteria were identified. CS was confirmed by histology in all patients from myocardium only (iCS) or extracardiac tissue (nonisolated CS). The primary end point was a composite of mortality, orthotopic heart transplant, and durable left ventricular assist device implantation. CS was isolated in 9 of 50 patients (18%). Among baseline characteristics, iCS and nonisolated CS differed significantly only in the frequency of sustained ventricular tachycardia at presentation (78% versus 37%; P=0.03) and delay in CS diagnosis >6 months (67% versus 5%; P<0.01). A nonsignificant trend toward lower left ventricular ejection fraction and more frequent heart failure in iCS was observed. Over a median follow‐up of 9.7 years (95% CI, 6.8–10.8), 18 patients reached the primary end point (13 deaths, 2 orthotopic heart transplants, and 3 durable left ventricular assist device implantations). The 1‐, 5‐, and 10‐year event‐free survival rates were 96% (95% CI, 85%–99%), 79% (95% CI, 64%–88%), and 58% (95% CI, 40%–73%), respectively, without differences between groups. There were no significant predictors of the primary end point, including delayed CS diagnosis. Conclusions Long‐term outcomes were similar between iCS and nonisolated CS in patients with histologically documented sarcoid. Diagnostic delays may contribute to differences in the dominant clinical presentation, despite similar outcomes. |
| first_indexed | 2024-03-08T22:23:29Z |
| format | Article |
| id | doaj.art-6f4abcefddb94ce187dfb845ff6dde12 |
| institution | Directory Open Access Journal |
| issn | 2047-9980 |
| language | English |
| last_indexed | 2024-03-08T22:23:29Z |
| publishDate | 2023-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| spelling | doaj.art-6f4abcefddb94ce187dfb845ff6dde122023-12-18T11:20:28ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802023-10-01121910.1161/JAHA.122.028342Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic DelaysJoshua Sink0Cara Joyce1Max J. Liebo2David J. Wilber3Loyola University Chicago Stritch School of Medicine Maywood IL USADepartment of Medicine Loyola University of Chicago Stritch School of Medicine Maywood IL USASection of Advanced Heart Failure, Division of Cardiology, Department of Medicine Loyola University Chicago Stritch School of Medicine Maywood IL USADepartment of Medicine Loyola University of Chicago Stritch School of Medicine Maywood IL USABackground Isolated cardiac sarcoid (iCS) is reported to have more severe clinical presentation and greater risk of adverse events compared with cardiac sarcoid (CS) with extracardiac involvement (nonisolated CS). Delays in diagnosing specific organ involvement may play a role in these described differences. Methods and Results A retrospective observational study of patients with CS over a 20‐year period was conducted. Objective evidence of organ involvement and time of onset based on consensus criteria were identified. CS was confirmed by histology in all patients from myocardium only (iCS) or extracardiac tissue (nonisolated CS). The primary end point was a composite of mortality, orthotopic heart transplant, and durable left ventricular assist device implantation. CS was isolated in 9 of 50 patients (18%). Among baseline characteristics, iCS and nonisolated CS differed significantly only in the frequency of sustained ventricular tachycardia at presentation (78% versus 37%; P=0.03) and delay in CS diagnosis >6 months (67% versus 5%; P<0.01). A nonsignificant trend toward lower left ventricular ejection fraction and more frequent heart failure in iCS was observed. Over a median follow‐up of 9.7 years (95% CI, 6.8–10.8), 18 patients reached the primary end point (13 deaths, 2 orthotopic heart transplants, and 3 durable left ventricular assist device implantations). The 1‐, 5‐, and 10‐year event‐free survival rates were 96% (95% CI, 85%–99%), 79% (95% CI, 64%–88%), and 58% (95% CI, 40%–73%), respectively, without differences between groups. There were no significant predictors of the primary end point, including delayed CS diagnosis. Conclusions Long‐term outcomes were similar between iCS and nonisolated CS in patients with histologically documented sarcoid. Diagnostic delays may contribute to differences in the dominant clinical presentation, despite similar outcomes.https://www.ahajournals.org/doi/10.1161/JAHA.122.028342cardiac sarcoidcardiomyopathyheart failuremortalitysarcoidosis |
| spellingShingle | Joshua Sink Cara Joyce Max J. Liebo David J. Wilber Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease cardiac sarcoid cardiomyopathy heart failure mortality sarcoidosis |
| title | Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays |
| title_full | Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays |
| title_fullStr | Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays |
| title_full_unstemmed | Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays |
| title_short | Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays |
| title_sort | long term outcomes of cardiac sarcoid prognostic implications of isolated cardiac involvement and impact of diagnostic delays |
| topic | cardiac sarcoid cardiomyopathy heart failure mortality sarcoidosis |
| url | https://www.ahajournals.org/doi/10.1161/JAHA.122.028342 |
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