30-Year Trends in the Incidence, Characteristics, and Outcome of Cardiac Sarcoidosis in a Nationwide Cohort

Pauli Pöyhönen, MD, MSc; Jukka Lehtonen, MD; Diana Velikanova, MD; Piia Simonen, MD; Valtteri Uusitalo, MD; Henriikka Mälkönen, MD; Hanna-Kaisa Nordenswan, MD; Tapani Vihinen, MD; Kari Kaikkonen, MD; Petri Haataja, MD; Tuomas Kerola, MD; Tuomas T. Rissanen, MD; Ville Vepsäläinen, MD; Aleksi Alatalo, MD; Päivi Pietilä-Effati, MD; Markku Kupari, MD

JACC: Advances (Aug 2024)

30-Year Trends in the Incidence, Characteristics, and Outcome of Cardiac Sarcoidosis in a Nationwide Cohort

Pauli Pöyhönen, MD, MSc,
Jukka Lehtonen, MD,
Diana Velikanova, MD,
Piia Simonen, MD,
Valtteri Uusitalo, MD,
Henriikka Mälkönen, MD,
Hanna-Kaisa Nordenswan, MD,
Tapani Vihinen, MD,
Kari Kaikkonen, MD,
Petri Haataja, MD,
Tuomas Kerola, MD,
Tuomas T. Rissanen, MD,
Ville Vepsäläinen, MD,
Aleksi Alatalo, MD,
Päivi Pietilä-Effati, MD,
Markku Kupari, MD

Affiliations

Pauli Pöyhönen, MD, MSc: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland; Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland; Address for correspondence: Dr Pauli Pöyhönen, Heart and Lung Center, Helsinki University Hospital, 00029 HUS, Helsinki, Finland.
Jukka Lehtonen, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Diana Velikanova, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Piia Simonen, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Valtteri Uusitalo, MD: Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland; Clinical Physiology and Nuclear Medicine, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Henriikka Mälkönen, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Hanna-Kaisa Nordenswan, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
Tapani Vihinen, MD: Heart Center, Turku University Hospital, Turku, Finland
Kari Kaikkonen, MD: Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland
Petri Haataja, MD: Heart Hospital, Tampere University Hospital, Tampere, Finland
Tuomas Kerola, MD: Department of Internal Medicine, Päijät-Häme Central Hospital, Lahti, Finland
Tuomas T. Rissanen, MD: Heart Center, North Karelia Central Hospital, Joensuu, Finland
Ville Vepsäläinen, MD: Heart Centre, Kuopio University Hospital, Kuopio, Finland
Aleksi Alatalo, MD: South Ostrobothnia Central Hospital, Seinäjoki, Finland
Päivi Pietilä-Effati, MD: Vaasa Central Hospital, Vaasa, Finland
Markku Kupari, MD: Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland

Journal volume & issue: Vol. 3, no. 8
p. 101102

Abstract

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Background: Cardiac sarcoidosis (CS) is a rare but potentially fatal inflammatory cardiomyopathy. Objectives: The authors studied temporal changes in the incidence, characteristics, and outcome of CS. Methods: A retrospective analysis was made of a 30-year nationwide cohort of CS. Results: The cohort comprised 511 patients with a median age of 52 years and female preponderance (69%). Altogether 77, 166, and 268 cases of CS were diagnosed in years 1988 to 2009, 2010 to 2014, and 2015 to 2019, respectively; the 5-year count of 2015 to 2019 was 134-fold the count of 1990 to 1994 (268/2) and 18-fold the count of 2000 to 2004 (268/15). Prior to 2010, compared with the later periods, CS presented more often with ventricular tachycardia/fibrillation (prevalence 36% vs 19% in 2010-2014 and 11% in 2015-2019, P < 0.001), left ventricular ejection fraction <50% (49%, 35%, and 31%; P = 0.010), and elevation of natriuretic peptides (87%, 57%, and 49%; P < 0.001). On magnetic resonance imaging, late gadolinium enhancement involved a median of 15% (IQR: 11%-22%) of left ventricular mass in studies of 1988 to 2009 (n = 16), 15% (IQR: 9%-22%) in studies of 2010 to 2014 (n = 87), and 11% (IQR: 5%-19%) in studies of 2015 to 2019 (n = 150) (P = 0.031). The respective 5-year incidences of the composite of death, heart transplantation, left ventricular–assisted device implantation, or ventricular tachyarrhythmia were 40% (95% CI: 29%-51%), 32% (95% CI: 25%-39%), and 23% (95% CI: 16%-30%) (P = 0.002). The prognostic trend disappeared after adjustment for differences in the presenting phenotype. Conclusions: Diagnoses of incident CS have increased exponentially in Finland. Concurrently, the phenotype has turned milder and prognosis better, suggesting detection of CS at an earlier stage of its course.

Published in JACC: Advances

ISSN: 2772-963X (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the circulatory (Cardiovascular) system; Medicine: Internal medicine: Medical emergencies. Critical care. Intensive care. First aid
Website: https://www.sciencedirect.com/journal/jacc-advances

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