Skip to main content
Key Specialties
Anesthesiology Cardiology Dermatology Diabetology Endocrinology Gastroenterology General Medicine Geriatrics Hematology Infectious Diseases Internal Medicine Nephrology Neurology Obstetrics and Gynecology Oncology Ophthalmology Pediatrics Psychiatry Radiology Respiratory Medicine Rheumatology Surgery Urology
Congress Coverage
ASH 2024 Annual Meeting 2024 ESMO Congress 2024 ERS Congress 2024 EASD Congress 2024 ESC Congress 2024 EAN Congress 2024 ASCO Annual Meeting 2024 ACC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on adolescent vaping

LIVE: Wednesday 29th January, 18:00-19:30 CET

Growing numbers of young people are using e-cigarettes, despite warnings of respiratory effects and addiction. How can doctors tackle the epidemic, and what health effects should you prepare to manage in your clinics?
ADVANCED SEARCH
Log in
Top
Netherlands Heart Journal

Open Access 09-01-2025 | Sarcoidosis | Original Article

Screening for cardiac sarcoidosis: diagnostic approach and long-term follow-up in a tertiary centre

Authors: Nikki van der Velde, Anne Poleij, Mattie J. Lenzen, Ricardo P. J. Budde, Tessa Brabander, Jelle R. Miedema, Arend F. L. Schinkel, Michelle Michels, Alexander Hirsch

Published in: Netherlands Heart Journal

Login to get access

Abstract

Background

Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. This study evaluated the results of a diagnostic approach in patients with known sarcoidosis and suspected cardiac involvement in a tertiary centre and their long-term outcomes.

Methods

We included 180 patients with sarcoidosis and a clinical suspicion of CS. In addition to an electrocardiogram (ECG)/transthoracic echocardiogram (TTE), cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) were performed in 66% and 37% of the patients, respectively. The diagnosis of CS was based on the Heart Rhythm Society criteria. Follow-up was performed, and a composite endpoint of sustained ventricular tachycardia, ventricular fibrillation, aborted sudden cardiac death, heart failure hospitalisation, heart transplantation or cardiac death was used for the survival analysis.

Results

Symptoms were present in 87% of the patients, and ECG/TTE abnormalities were found in 92/180 patients (51%). Using CMR and/or PET, 31/92 patients (34%) were diagnosed with CS. In 15 patients, an alternative diagnosis was found. CS was diagnosed in 11/88 patients (13%) without ECG/TTE abnormalities. During a median follow-up time of 4.4 years (interquartile range: 2.3–6.8), 11 composite endpoints occurred, more frequently in CS patients than in sarcoidosis patients without cardiac involvement (p < 0.001). Patients with ECG/TTE abnormalities at baseline had worse outcomes than those without abnormalities (p = 0.019).

Conclusion

CS was diagnosed in 23% of the referred sarcoidosis patients. ECG/TTE were of limited diagnostic value for screening for CS but seemed to have important prognostic value as patients with normal ECG/TTE results who did meet the diagnostic CS criteria had a very good prognosis. CMR/PET provided a good diagnostic yield and identified other cardiac diseases.
Appendix
Available only for authorised users
Literature
1.
Ungprasert P, Carmona EM, Utz JP, et al. Epidemiology of Sarcoidosis 1946–2013: A Population-Based Study. Mayo Clin Proc. 2016;91:183–8.CrossRefPubMed
2.
Rybicki BA, Major M, Popovich J Jr, et al. Racial Differences in Sarcoidosis Incidence: A 5-Year Study in a Health Maintenance Organization. Am J Epid. 1997;145:234–41.CrossRef
3.
Dubrey SW, Falk RH. Diagnosis and Management of Cardiac Sarcoidosis. Prog Cardiovasc Dis. 2010;52:336–46.CrossRefPubMed
4.
Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978;58:1204–11.CrossRefPubMed
5.
Iwai K, Tachibana T, Takemura T, et al. Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Pathol Int. 1993;43:372–6.
6.
Martusewicz-Boros MM, Boros PW, Wiatr E, et al. Prevalence of cardiac sarcoidosis in white population: a case-control study: Proposal for a novel risk index based on commonly available tests. Medicine. 2016;95:e4518.CrossRefPubMedPubMedCentral
7.
Baughman RP, Teirstein AS, Judson MA, et al. Clinical Characteristics of Patients in a Case Control Study of Sarcoidosis. Am J Resp Crit Care Med. 2001;164:1885–9.CrossRefPubMed
8.
Lynch JP, Hwang J, Bradfield J, et al. Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment. Semin Respir Crit Care Med. 2014;35:372–90.CrossRefPubMedPubMedCentral
9.
Slart RGA, Lancellotti P, Hyafil F, et al. A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and Inflammation &amp; Infection Committees of the European Association of Nuclear Medicine, the European Association of Cardiovascular Imaging, and the American Society of Nuclear Cardiology. Eur Heart J. 2017;18:1073–89.
10.
Birnie DH, Sauer WH, Bogun F, et al. HRS Expert Consensus Statement on the Diagnosis and Management of Arrhythmias Associated With Cardiac Sarcoidosis. Heart Rhythm. 2014;11:1304–23.CrossRef
11.
Błaut-Jurkowska J, Kaźnica-Wiatr M, Knap K, et al. Cardiac sarcoidosis—management and prognosis. 2017.
12.
Yazaki Y, Isobe M, Hiroe M, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Card. 2001;88:1006–10.CrossRefPubMed
13.
14.
Sekiguchi M, Hiroe M, Take M, Hirosawa K. Clinical and Histopathological Profile of Sarcoidosis of the Heart and Acute Idiopathic Myocarditis Concepts through a study employing endomyocardial biopsy II. Myocarditis: Symposium on Secondary Myocardial Disease. Jap Circ J. 1980;44:264–73.CrossRefPubMed
15.
Kim JS, Judson MA, Donnino R, et al. Cardiac sarcoidosis. Am Heart J. 2009;157:9–21.CrossRefPubMed
16.
Kopriva P, Griva M, Tüdös Z. Management of cardiac sarcoidosis—A practical guide. Cor Vasa. 2018;60:e155–e64.CrossRef
17.
Hulten E, Aslam S, Osborne M, et al. Cardiac sarcoidosis—state of the art review. Cardiovasc Diag Ther. 2016;6:50–63.
18.
Kouranos V, Tzelepis GE, Rapti A, et al. Complementary Role of CMR to Conventional Screening in the Diagnosis and Prognosis of Cardiac Sarcoidosis. Jacc: Cardiovasc Imaging. 2017;10:1437–47.PubMed
19.
Hiraga H, Iwai K. Guidelines for diagnosis of cardiac sarcoidosis: study report on diffuse pulmonary disease (in Japanese). Tokyo: The Japanese Ministry of Health and Welfare; 1993.
20.
Diagnostic standard and guidelines for sarcoidosis. Jpn J Sarcoidosis Granulomatous Disord. 2007;27:89–102.
21.
Darlington P, Gabrielsen A, Cederlund K, et al. Diagnostic approach for cardiac involvement in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2019;36:11–7.PubMedPubMedCentral
22.
Hunninghake GW, Costabel U, Ando M, et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16:149–73.PubMed
23.
Simpson R, Bromage D, Dancy L, et al. 6 Comparing echocardiography and cardiac magnetic resonance measures of ejection fraction: implications for HFMRF research. Heart. 2018;104(5):A3.
24.
Wagholikar KB, Fischer CM, Goodson A, et al. Extraction of Ejection Fraction from Echocardiography Notes for Constructing a Cohort of Patients having Heart Failure with reduced Ejection Fraction (HFrEF). J Med Syst. 2018;42:209.CrossRefPubMedPubMedCentral
25.
Petersen SE, Aung N, Sanghvi MM, et al. Reference ranges for cardiac structure and function using cardiovascular magnetic resonance (CMR) in Caucasians from the UK Biobank population cohort. J Cardiovasc Magn Res. 2017;19:18.CrossRef
26.
Vita T, Okada DR, Veillet-Chowdhury M, et al. Complementary Value of Cardiac Magnetic Resonance Imaging and Positron Emission Tomography/Computed Tomography in the Assessment of Cardiac Sarcoidosis. Circ Cardiovasc Imaging. 2018;11:e7030.CrossRefPubMedPubMedCentral
27.
Flamée L, Symons R, Degtiarova G, et al. Prognostic value of cardiovascular magnetic resonance in patients with biopsy-proven systemic sarcoidosis. Eur Radiol. 2020;30:3702–10.CrossRefPubMed
28.
Kiko T, Yoshihisa A, Kanno Y, et al. A Multiple Biomarker Approach in Patients with Cardiac Sarcoidosis. Int Heart J. 2018;59:996–1001.CrossRefPubMed
29.
Pour-Ghaz I, Kayali S, Abutineh I, et al. Cardiac Sarcoidosis: Pathophysiology, Diagnosis, and Management. Hearts. 2021;2:234–50.CrossRef
30.
Mathijssen H, Tjoeng TWH, Keijsers RGM, et al. The usefulness of repeated CMR and FDG PET/CT in the diagnosis of patients with initial possible cardiac sarcoidosis. Ejnmmi Res. 2021;11:129.CrossRefPubMedPubMedCentral
Metadata
Title
Screening for cardiac sarcoidosis: diagnostic approach and long-term follow-up in a tertiary centre
Authors
Nikki van der Velde
Anne Poleij
Mattie J. Lenzen
Ricardo P. J. Budde
Tessa Brabander
Jelle R. Miedema
Arend F. L. Schinkel
Michelle Michels
Alexander Hirsch
Publication date
09-01-2025
Publisher
BSL Media & Learning
Published in
Netherlands Heart Journal
Print ISSN: 1568-5888
Electronic ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-024-01925-0

Keynote webinar | Spotlight on menopause

Menopause can have a significant impact on the body, with effects ranging beyond the endocrine and reproductive systems. Learn about the broader systemic effects of menopause, so you can help patients in your clinics through the transition.   

Prof. Martha Hickey
Dr. Claudia Barth
Dr. Samar El Khoudary
Developed by: Springer Medicine
Watch now

A quick guide to ECGs

Improve your ECG interpretation skills with this comprehensive, rapid, interactive course. Expert advice provides detailed feedback as you work through 50 ECGs covering the most common cardiac presentations to ensure your practice stays up to date. 

PD Dr. Carsten W. Israel
Developed by: Springer Medizin
Start the cases

Keynote webinar | Spotlight on medication adherence

  • Webinar | 27-06-2024 | 18:00 (CEST)

Medication non-adherence is a major barrier to effective healthcare delivery; half of all patients do not follow their doctor’s recommendations or treatment plan.

Our experts explain the fundamentals with a practical discussion of how to manage non-adherence in two common scenarios: hypertension and asthma control.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Watch now
Image Credits