Letter by Al-Kindi and Oliveira Regarding Article “Cardiac Sarcoidosis: Epidemiology, Characteristics, and Outcome Over 25 Years in a Nationwide Study”
To the Editor:
We read with interest the article by Kandolin and colleagues1 published in Circulation. In a nationwide study in Finland, they report that the annual detection rate of cardiac sarcoidosis (CS) has increased >20-fold over a 25-year period. Interestingly, these findings are in alignment with our own data regarding the increased incidence of heart transplantation (HT) for CS in the United States.
Using the United Network for Organ Sharing registry, we identified 150 adult patients with CS listed for HT over a 21-year period (1994–2014). During this period, the percentage of patients who had heart failure with CS requiring HT increased 5-fold from 0.1% (1994–1997) to 0.5% (2010–2014), P<0.001. Similarly, the contribution of CS as a cause of restrictive cardiomyopathy increased from 5.0% (1994–1997) to 15.22% (2010–2014), P<0.001.
Among patients in the United States with CS requiring HT, the median age at listing is 54 years, and 92 (61.3%) were male, and 90 (60.0%) were whites, 56 (37.3%) were blacks, and 4 (2.8%) were others. The use of an implantable cardioverter defibrillator is currently at 92.6% and waitlist mortality has decreased by an average of 11% per year (hazard ratio, 0.890; 95% confidence interval, 0.810–0.977; P=0.014) after adjusting for listing status, with 1-year and 3-year waitlist mortality at 89% and 77%, respectively. Correspondingly, posttransplant survival of CS patients is 86% at 1 year, 84% at 5 years, and 58% at 10 years, comparable with other HT recipients (P=0.296).
Our findings suggest that the trends described in Finland by Kandolin et al1 are also occurring in the United States and that CS patients are being increasingly considered for HT with good outcomes. Whether the higher prevalence of CS is a reflection of improved diagnostic modalities or increased incidence of the disease itself needs further investigation.
Sadeer G. Al-Kindi, MD
Guilherme H. Oliveira, MD
Advanced Heart Failure and Transplantation Center
Harrington Heart & Vascular Institute
University Hospitals Case Medical Center
Case Western Reserve University School of Medicine
- © 2015 American Heart Association, Inc.
- Kandolin R,
- Lehtonen J,
- Airaksinen J,
- Vihinen T,
- Miettinen H,
- Ylitalo K,
- Kaikkonen K,
- Tuohinen S,
- Haataja P,
- Kerola T,
- Kokkonen J,
- Pelkonen M,
- Pietilä-Effati P,
- Utrianen S,
- Kupari M