Importance of Early Diagnosis of Cardiac Sarcoidosis in Patients with Complete Atrioventricular Block

Kaida, Toyoji; Inomata, Takayuki; Minami, Yoshiyasu; Yazaki, Mayu; Fujita, Toshio; Iida, Yuichiro; Ikeda, Yuki; Nabeta, Takeru; Ishii, Shunsuke; Naruke, Tsuguo; Maekawa, Emi; Koitabashi, Toshimi; Ako, Junya

doi:10.1536/ihj.17-492

Cited by 13 publications

(15 citation statements)

References 22 publications

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“…Early recognition and treatment of underlying CS can prevent progressive LV dysfunction, reduce morbidity and mortality as well as prevent unnecessary device upgradation or cardiac transplantation. Some authors [5,11,12] have also reported similar cases, where underlying CS rather than PICM which was responsible for progressive LV dysfunction. It is indeed difficult to suspect CS among patients diagnosed of PICM as majority of do not have systemic involvement.…”

Section: Discussionmentioning

confidence: 91%

Severe left ventricular systolic dysfunction after permanent pacemaker implantation: Should we pause before upgrading to biventricular pacing?

Bera

Yalagudri

Devidutta

et al. 2019

Indian Pacing and Electrophysiology Journal

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Left ventricular (LV) systolic dysfunction leading to heart failure (HF) is known to occur after permanent pacemaker implantation (PPI) in a subset of patients. They are often treated by upgradation of the pacemaker to cardiac resynchronisation therapy (CRT). We report a case of progressive LV dysfunction and HF after PPI. Cardiac 18 FDG-PET-CT scan revealed abnormal myocardial FDG uptake suggestive of cardiac sarcoidosis (CS). Biopsy from FDG avid lymph node demonstrated non-caseating granuloma. Therapy with steroids resulted in resolution of HF symptoms accompanied by a significant improvement in LV function.

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Section: Discussionmentioning

confidence: 91%

Severe left ventricular systolic dysfunction after permanent pacemaker implantation: Should we pause before upgrading to biventricular pacing?

Bera

Yalagudri

Devidutta

et al. 2019

Indian Pacing and Electrophysiology Journal

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“…This is comparable with a previous report including CS patients presenting with a complete AV-block, where in none of the patients with delayed diagnosis 67 Ga Scintigraphy and/or 18 F-FDG-PET was performed. 19 …”

Section: Discussionmentioning

confidence: 99%

“…Last, the diagnosis might be confirmed by positive extracardiac histology with cardiac symptoms, but extracardiac symptoms are frequently absent. 3 , 19 However, despite absence of extracardiac complaints or involvement, it can be worthwhile to perform additional pulmonary diagnostic testing. A mediastinal lymphnode biopsy and/or bronchoalveolar lavage confirmed diagnosis in 84% of patients with suspected CS without extracardiac complaints, regardless of imaging findings.…”

Section: Discussionmentioning

confidence: 99%

“…Previously, in patients with CS presenting with complete AV-block, the same trend was observed with a benefit of early diagnosis. 19 Although patient populations are small, corticosteroid therapy seems to maintain cardiac function in patients with preserved function, improve function in patients with mild-moderate dysfunction and does not have effect in patients with severely decreased function. 21 These findings suggest that there may be a tipping point, after which cardiac function is beyond repair.…”

Section: Discussionmentioning

confidence: 99%

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The harm of delayed diagnosis of arrhythmogenic cardiac sarcoidosis: a case series

et al. 2020

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Abstract Aims Cardiac sarcoidosis (CS) is a known cause of ventricular tachycardia (VT). However, an arrhythmogenic presentation may not prompt immediate comprehensive evaluation. We aimed to assess the diagnostic and disease course of patients with arrhythmogenic cardiac sarcoidosis (ACS). Methods and results From the Leiden VT-ablation-registry, consecutive patients with CS as underlying aetiology were retrospectively included. Data on clinical presentation, time-to-diagnosis, cardiac function, and clinical outcomes were collected. Patients were divided in early (<6 months from first cardiac presentation) and late diagnosis. After exclusion of patients with known causes of non-ischaemic cardiomyopathy (NICM), 15 (12%) out of 129 patients with idiopathic NICM were ultimately diagnosed with CS and included. Five patients were diagnosed early; all had early presentation with VTs. Ten patients had a late diagnosis with a median delay of 24 (IQR 15–44) months, despite presentation with VT (n = 5) and atrioventricular block (n = 4). In 6 of 10 patients, reason for suspicion of ACS was the electroanatomical scar pattern. In patients with early diagnosis, immunosuppressive therapy was immediately initiated with stable cardiac function during follow-up. Adversely, in 7 of 10 patients with late diagnosis, cardiac function deteriorated before diagnosis, and in only one cardiac function recovered with immunosuppressive therapy. Six (40%) patients died (five of six with late diagnosis). Conclusion Arrhythmogenic cardiac sarcoidosis is an important differential diagnosis in NICM patients referred for VT ablation. Importantly, the diagnosis is frequently delayed, which leads to a severe disease course, including irreversible cardiac dysfunction and death. Early recognition, which can be facilitated by electroanatomical mapping, is crucial.

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“…Therefore, the primary role of immune‐suppression is secondary prevention of cardiac dysfunction and ventricular arrhythmias. Efficacy of corticosteroid therapy is enhanced in early stage CS with well‐preserved ventricular systolic function . However, the urgent initiation of immune‐suppression is often delayed due to the technical difficulty of obtaining histopathological evidence required in conventional guidelines.…”

Section: Discussionmentioning

confidence: 99%

Remaining challenges in the diagnosis of early stage cardiac sarcoidosis

Tsushima

Sasaki

Yuda

et al. 2019

Clinical Case Reports

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Key Clinical Message Despite the requirement for histopathological evidence to make a definite diagnosis of cardiac sarcoidosis, the sensitivity of endomyocardial biopsy is still low. Recently, Japanese Circulation Society suggests a new strategy that patients diagnosed clinically do not require the endomyocardial biopsy evidence. Physicians should familiarize themselves with such paradigm shifts.

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Importance of Early Diagnosis of Cardiac Sarcoidosis in Patients with Complete Atrioventricular Block

Cited by 13 publications

References 22 publications

Severe left ventricular systolic dysfunction after permanent pacemaker implantation: Should we pause before upgrading to biventricular pacing?

Severe left ventricular systolic dysfunction after permanent pacemaker implantation: Should we pause before upgrading to biventricular pacing?

The harm of delayed diagnosis of arrhythmogenic cardiac sarcoidosis: a case series

Remaining challenges in the diagnosis of early stage cardiac sarcoidosis

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