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Undiagnosed cardiac sarcoidosis presenting as complete heart block and ventricular arrhythmia.

ABSTRACT: Cardiac involvement in sarcoidosis has been reported to be as high as 50% of cases and it is well documented that it is associated with a poorer prognosis. Early recognition and treatment is key to reducing the risk of fatal arrhythmias and heart failure. We report a case of undiagnosed systemic sarcoidosis in a young man who initially presented to the emergency department with complete heart block in the context of preserved biventricular systolic function, and then again with ventricular tachycardia and moderately impaired left ventricular systolic function. We discuss how 18F-fluorodeoxyglucose positron emission tomography with CT was used for accurate diagnosis and how its role in the management of sarcoidosis has expanded with recent evidence suggesting it to be a mainstay of management and disease monitoring in cardiac sarcoidosis.

SUBMITTER: Mcbeath K

PROVIDER: S-EPMC4550861 | biostudies-other | 2015 Aug

REPOSITORIES: biostudies-other

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Project description:BackgroundComplete heart block (CHB) as a first presentation of acute viral myocarditis is a rare occurrence associated with increased morbidity and mortality. In such cases, an endomyocardial biopsy is recommended to make a clear histological diagnosis aiding to differentiate from other possible conditions such as sarcoiditic myocarditis, giant cell myocarditis, and eosinophilic myocarditis. Insertion of a permanent pacemaker may be considered on a case-to-case basis.Case presentationA previously healthy 21-year-old female presented to the emergency department after having suffered two episodes of syncope on a background of a few days' history of myalgias, chills, and rigors. Electrocardiogram showed high-grade Mobitz II block with intermittent periods of CHB. A bedside echocardiogram upon admission demonstrated normal biventricular systolic function. Given the patient's unstable haemodynamic status and lack of obvious reversible causes for the CHB, a permanent dual-chamber pacemaker was inserted urgently. Initial blood investigations indicated an ongoing inflammatory process highlighting the possibility of myocarditis as a cause of the CHB. Therefore, a troponin level was taken and was noted to be elevated confirming the suspicion of myocarditis. The left ventricular ejection fraction (LVEF) decreased over the following days to approximately 20%, clinically resulting in pulmonary oedema and acute shortness of breath. The patient required aggressive intravenous diuresis and anti-heart failure medication. An endomyocardial biopsy (EMB) confirmed the diagnosis of lymphocytic myocarditis. The patient's condition improved secondary to an improvement in LVEF and resolution of the heart block. A cardiac magnetic resonance (CMR) imaging performed 6 weeks from admission reported an improved LVEF of 51% with no late gadolinium enhancement (LGE). Based on the reassuring CMR findings and the resolution of CHB on follow-up pacemaker checks, it was deemed safe to explant the pacemaker.ConclusionsAcute myocarditis may be complicated with high-degree AV block and cardiogenic shock necessitating close observation in a critical care unit. A permanent pacemaker may provide atrio-ventricular synchrony which helps stabilise the patient's condition and protect from a prolonged period of heart block. Early myocardial fibrosis on EMB and degree of LGE on CMR are indicators of persistent atrioventricular block. Guideline-directed treatment of heart failure is essential.

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Undiagnosed cardiac sarcoidosis presenting as complete heart block and ventricular arrhythmia.

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