Isolated cardiac sarcoidosis associated with coronary vasomotion abnormalities: a case report
Shigeo Godo1, Yuhi Hasebe1, Jun Takahashi1
1Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Japan.
Insights
Cardiac sarcoidosis can cause coronary artery spasm, affecting heart function. Treatment with corticosteroids and calcium-channel blockers may reduce spasm and inflammation.
Area of Science:
- Cardiology
- Inflammatory Diseases
- Medical Imaging
Background:
- Cardiac sarcoidosis is a serious inflammatory heart condition leading to heart failure and arrhythmias.
- Diagnosis of isolated cardiac sarcoidosis can be achieved using imaging techniques, avoiding invasive biopsy.
- Coronary vasomotion abnormalities are common in cardiovascular and inflammatory conditions, but their link to cardiac sarcoidosis was unclear.
Observation:
- A patient with cardiac sarcoidosis experienced cardiac arrest due to ventricular fibrillation.
- Coronary angiography revealed normal arteries, but acetylcholine testing showed epicardial and microvascular spasm.
- Diagnosis of isolated cardiac sarcoidosis was confirmed through established criteria, including imaging findings.
Findings:
- Active myocardial inflammation in cardiac sarcoidosis is associated with both epicardial and coronary microvascular spasm.
- Treatment combining corticosteroids for sarcoidosis and calcium-channel blockers for spasm led to reduced coronary artery spasm.
- Therapy resulted in regression of myocardial inflammation alongside improved coronary vasomotion.
Implications:
- Coronary vasomotion abnormalities should be considered in patients with cardiac sarcoidosis.
- Combined treatment strategies can effectively manage coronary spasm and inflammation in cardiac sarcoidosis.
- This highlights a potential therapeutic target for improving outcomes in cardiac sarcoidosis patients.
Background:
Cardiac sarcoidosis is a chronic, inflammatory disease that can affect the heart and often results in heart failure and lethal arrhythmias. A multimodality imaging approach without endomyocardial biopsy allows for the diagnosis of isolated cardiac sarcoidosis. Coronary vasomotion abnormalities are highly prevalent in various cardiovascular and inflammatory diseases. It remains unknown whether active myocardial inflammation due to cardiac sarcoidosis is associated with coronary vasomotion abnormalities.
Case Summary:
A 68-year-old man without a past medical history experienced an out-of-hospital cardiac arrest due to ventricular fibrillation and was successfully resuscitated without neurologic sequelae. Coronary angiography showed normal coronary arteries; however, intracoronary acetylcholine provocation testing demonstrated both epicardial coronary and coronary microvascular spasm. He was diagnosed with isolated cardiac sarcoidosis by fulfilling the diagnostic criteria proposed by the Japanese Circulation Society 2016 diagnostic guidelines, including fatal ventricular arrhythmia, focal left ventricular wall asynergy, increased myocardial fluorodeoxyglucose uptake by positron emission tomography, and late gadolinium enhancement by cardiac magnetic resonance in the heart. He was treated with calcium-channel blocker for coronary artery spasm and prednisolone for cardiac sarcoidosis and underwent implantation of an implantable cardioverter-defibrillator for secondary prevention. Following the treatment, the severity of coronary artery spasm was reduced along with regression of the myocardial inflammation.
Discussion:
Epicardial coronary artery and coronary microvascular spasm can be accompanied by active myocardial inflammation of isolated cardiac sarcoidosis, and the treatment with corticosteroid and calcium-channel blocker may be effective for relieving the severity of coronary artery spasm in association with regression of myocardial inflammation of the disease.
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