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Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report.

ABSTRACT: Background:Sarcoidosis is a multi-organ granulomatous disease of unknown aetiology. Adverse outcome related with cardiac involvement, makes early diagnosis of cardiac sarcoidosis crucial. Case summary:In a 55-year-old man presenting with recurrent pulmonary infections, computed tomography (CT) showed several enlarged mediastinal lymph nodes and no lung pathology. Subsequent mediastinoscopy revealed the diagnosis of sarcoidosis. Further screening for organ involvement showed multifocal cardiac involvement both on cardiac magnetic resonance (CMR) and 18-F-fluorodeoxyglucose-positron emission tomography-computed tomography (18F-FDG PET-CT). Because of the lack of functional deterioration and clinical symptoms, no steroid treatment was initiated and regular follow-up of cardiac abnormalities was performed by CMR. Unremarkable progression of cardiac involvement during the first 2?years of follow-up turned into a dramatic involvement after 4?years, with the increase in the number and size of lesions at late gadolinium enhancement (LGE) CMR. Late gadolinium enhancement areas matched the regions of strongly increased 18F-FDG uptake. For the first time, the patient started complaining on shortness of breath, electrocardiography showed an atrioventricular block Grade 1. Cardiac biomarkers and cardiac function were still preserved. Steroid treatment was started. Although an electrophysiology study was negative, Holter monitoring showed ventricular arrhythmia. Cardioverter-defibrillator was implanted. Discussion:This case shows the progression of cardiac sarcoidosis on CMR in an asymptomatic untreated patient over a 4-year period, and rises the awareness of possible severe cardiac damage even in the absence of clinical signs of cardiac involvement. Combination of PET and CMR is appealing to better understand the evolution of cardiac sarcoidosis and may help in the management of such patients.

SUBMITTER: Degtiarova G

PROVIDER: S-EPMC6764548 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report.

Degtiarova Ganna G Gheysens Olivier O Van Cleemput Johan J Wuyts Wim W Bogaert Jan J

European heart journal. Case reports 20190705 3

<h4>Background</h4>Sarcoidosis is a multi-organ granulomatous disease of unknown aetiology. Adverse outcome related with cardiac involvement, makes early diagnosis of cardiac sarcoidosis crucial.<h4>Case summary</h4>In a 55-year-old man presenting with recurrent pulmonary infections, computed tomography (CT) showed several enlarged mediastinal lymph nodes and no lung pathology. Subsequent mediastinoscopy revealed the diagnosis of sarcoidosis. Further screening for organ involvement showed multif ...[more]

PMID: 31660477

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Project description: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.

Project description:BackgroundSarcoidosis is a rare multiorgan inflammatory disorder of unknown aetiology, characterized by the formation of non-caseating granulomas in the affected organs. Cardiac involvement is underrecognized and observed in up to 25% of cases in autopsy studies, and is associated with a high mortality rate, especially due to sudden cardiac death due to ventricular arrhythmias.Case summaryA 41-year-old man well known to our hospital because of his father's diagnosis of cardiac amyloidosis, and carrier of transthyretin (TTR) gene mutation, was hospitalized following a resuscitated cardiac arrest. The patient was hospitalized a month before for a syncopal episode with demonstration of preserved left ventricular ejection fraction (LVEF) with akinetic basal septum at heart ultrasound and normal coronary. Chest computed tomography, performed in the emergency department, was significant for hilar lymphadenopathies and pulmonary nodules highly suggestive of sarcoidosis. A subsequent 18-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed multiorgan phlogistic involvement, including the myocardium. After the diagnosis of cardiac sarcoidosis, the patient was started on steroids therapy and underwent ICD implantation. A follow-up 18-FDG-PET showed a reduction of organs glucose uptake and a follow-up echo an improvement in LVEF. Despite that, he occurs occasional recording of repetitive ventricular arrhythmias and one appropriate ICD shock during the next 12 months.DiscussionCardiac sarcoidosis is an insidious disease. Its diagnosis can be challenging, with no specific finding in echocardiography. The best strategy would be multi-modality imaging involving both magnetic resonance imaging with late gadolinium enhancement and 18-FDG-PET, followed by biopsy to confirm the diagnosis. Multi-modality imaging should be further used to evaluate the response to treatment and assess prognosis. Since the patient was a known carrier of the TTR gene mutation, many efforts were made in order to come up with the correct diagnosis considering that both cardiac amyloidosis and cardiac sarcoidosis are non-ischaemic cardiomyopathy with systemic involvement.

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Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report.

Publications

Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report.

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