Cardiac Sarcoidosis: A Clinical Overview

Alba, Ana Carolina; Gupta, Shubham; Kugathasan, Lakshmi; Ha, Andrew C.T.; Ochoa, Alejandro; Balter, Meyer; Liprandi, Álvaro Sosa; Liprandi, María Inés Sosa

doi:10.1016/j.cpcardiol.2021.100936

Cited by 5 publications

(12 citation statements)

References 85 publications

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“…In our case, neurohormonal treatment has been maintained with the four current pharmacological pillars that include the use of sacubitril/valsartan, beta-blocker, sodium-glucose cotransporter 2 (SGLT2) inhibitors, and mineralocorticoid receptor antagonist. Although treatment for ICS has not been evaluated in randomized studies, it is assumed that early immunosuppressive treatment and treatment of derived cardiac complications (left ventricular systolic dysfunction and ventricular arrhythmias) could clearly reduce symptoms and improve short- and long-term prognosis [ 12 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

Ventricular Tachycardia as a Presentation of Isolated Cardiac Sarcoidosis: How to Manage It When We Do Not See Granulomas

Puglla Sánchez,

De Escalante Yangüela,

Escota-Villanueva

et al. 2023

Cureus

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A 47-year-old male was referred for rapid palpitations and an electrocardiogram compatible with sustained monomorphic ventricular tachycardia (VT) that required synchronized electrical cardioversion due to hemodynamic instability. After the initial clinical certainty, an etiological search is carried out. The transthoracic echocardiogram (TTE) revealed moderate dilatation and left ventricular systolic dysfunction due to global hypokinesia. Coronary angiography did not show significant coronary stenosis. Cardiac magnetic resonance (CMR) guarantees a nonischemic dilated cardiomyopathy with moderate systolic dysfunction and a pattern of subepicardial and intramyocardial late gadolinium enhancement (LGE) in medial-lateral and median inferolateral segments. Lastly, a positron emission tomography-computed tomography (PET-CT) scan showed diffuse fixation of the radiotracer in the left ventricular (LV) walls, with greater uptake on the lateral and inferolateral surfaces of inflammatory origin. After ruling out other alternative pathologies and according to current diagnostic criteria, the clinical judgment of probable isolated cardiac sarcoidosis (ICS) is established. An implantable cardioverter-defibrillator was implanted as secondary prevention of the acute arrhythmic event. Specific treatment for systolic dysfunction was prescribed, as well as immunosuppressive therapy with corticosteroids and methotrexate, after which the patient remained in clinical remission, with disappearance of active inflammation on cardiac imaging tests and progressive ventricular systolic function. The initial diagnosis of isolated cardiac sarcoidosis can be complex and challenging, especially in those patients in whom the diagnosis of extracardiac sarcoidosis has not been previously established. The limitations of endomyocardial biopsy in this entity make it necessary to have a high index of clinical suspicion with the early use of new cardiac imaging techniques and to include this picture in the differential diagnosis of patients with sustained ventricular arrhythmias or left ventricular systolic dysfunction of nonspecific etiology clarified. Early initiation of aggressive immunosuppressive therapy has been shown to prevent disease progression and limit its potential cardiac complications.

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Section: Discussionmentioning

confidence: 99%

Ventricular Tachycardia as a Presentation of Isolated Cardiac Sarcoidosis: How to Manage It When We Do Not See Granulomas

Puglla Sánchez,

De Escalante Yangüela,

Escota-Villanueva

et al. 2023

Cureus

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“…Sarcoidosis can affect multiple organs, including the lungs, liver, kidneys, brain, heart, eyes, skin, and sinuses, and can cause a variety of complications. Among them, cardiovascular complications, such as conduction abnormalities, arrhythmias, heart failure, and PH, are associated with higher mortality ( 14 , 44 , 45 ). SAPH is closely associated with poor prognosis, conferring up to a 10-fold increase in mortality in patients with sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis

Zhang

Xiang

Zhang

et al. 2022

Front. Cardiovasc. Med.

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BackgroundSarcoidosis-associated pulmonary hypertension (SAPH) is associated with poor prognosis, conferring up to a 10-fold increase in mortality in patients with sarcoidosis, but the actual prevalence of SAPH is unknown.MethodsThe PubMed, Embase, and Cochrane Library databases were systematically searched for epidemiological studies reporting the prevalence of SAPH up to July 2021. Two reviewers independently performed the study selection, data extraction, and quality assessment. Studies were pooled using random-effects meta-analysis.ResultsThis meta-analysis included 25 high-quality studies from 12 countries, with a pooled sample of 632,368 patients with sarcoidosis. The prevalence of SAPH by transthoracic echocardiography in Europe, the United States and Asia was 18.8% [95% confidence interval (CI): 11.1–26.5%], 13.9% (95% CI: 5.4–22.4%) and 16.2% (95% CI: 7.1–25.4%) separately, and the overall pooled prevalence was 16.4% (95%CI: 12.2–20.5%). By right heart catheterization (RHC), the pooled prevalence of SAPH was 6.4% (95% CI: 3.6–9.1%) in general sarcoidosis population, and subgroup analyses showed that the prevalence of SAPH was 6.7% (95% CI: 2.4–11.0%) in Europe and 8.6% (95% CI: −4.1 to 21.3%) in the United States. Further, the prevalence of pre-capillary PH was 6.5% (95% CI: 2.9–10.2%). For the population with advanced sarcoidosis, the pooled prevalence of SAPH and pre-capillary PH by RHC was as high as 62.3% (95% CI: 46.9–77.6%) and 55.9% (95% CI: 20.1–91.7%), respectively. Finally, the pooled prevalence of SAPH in large databases with documented diagnoses (6.1%, 95% CI: 2.6–9.5%) was similar to that of RHC. Substantial heterogeneity across studies was observed for all analyses (I2 > 80%, P < 0.001).ConclusionsThe sarcoidosis population has a relatively low burden of PH, mainly pre-capillary PH. However, as the disease progresses to advanced sarcoidosis, the prevalence of SAPH increases significantly.

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“…Clinically, about 3%–39% of patients with systemic sarcoidosis have cardiac involvement, while the incidence of isolated CS in patients with CS is about 27%–54% ( 69 ). About 5%–8% of patients with CS have clinical manifestations, including conduction abnormalities, ventricular arrhythmias, and heart failure (HF), while 20%–25% of patients have asymptomatic cardiac involvement ( 63 , 70 ). The CS diagnosis is mainly based on the combination of serological markers, such as serum ACE, imaging examinations, including cardiac magnetic resonance imaging and positron emission tomography, and endomyocardial biopsy (EMB) following the Japanese Circulation Society (JCS) protocols ( 71 , 72 ).…”

Section: Cardiac Sarcoidosismentioning

confidence: 99%

Re-evaluating serum angiotensin-converting enzyme in sarcoidosis

Zheng,

Du,

Dong

2023

Front. Immunol.

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Sarcoidosis is a systemic inflammatory disease of unknown etiology, which mainly affects the lungs and lymph nodes, as well as extrapulmonary organs. Its incidence, and prevalence rate, and disease course largely vary with regions and populations globally. The clinical manifestations of sarcoidosis depend on the affected organs and the degree of severity, and the diagnosis is mainly based on serum biomarkers, radiographic, magnetic resonance, or positron emission tomography imaging, and pathological biopsy. Noncaseating granulomas composing T cells, macrophages, epithelioid cells, and giant cells, were observed in a pathological biopsy, which was the characteristic pathological manifestation of sarcoidosis. Angiotensin-converting enzyme (ACE) was first found in the renin–angiotensin–aldosterone system. Its main function is to convert angiotensin I (Ang I) into Ang II, which plays an important role in regulating blood pressure. Also, an ACE insertion/deletion polymorphism exists in the human genome, which is involved in the occurrence and development of many diseases, including hypertension, heart failure, and sarcoidosis. The serum ACE level, most commonly used as a biomarker in diagnosing sarcoidosis, in patients with sarcoidosis increases. because of epithelioid cells and giant cells of sarcoid granuloma expressing ACE. Thus, it serves as the most commonly used biomarker in the diagnosis of sarcoidosis and also aids in analyzing its therapeutic effect and prognosis in patients with sarcoidosis.

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Cardiac Sarcoidosis: A Clinical Overview

Cited by 5 publications

References 85 publications

Ventricular Tachycardia as a Presentation of Isolated Cardiac Sarcoidosis: How to Manage It When We Do Not See Granulomas

Ventricular Tachycardia as a Presentation of Isolated Cardiac Sarcoidosis: How to Manage It When We Do Not See Granulomas

Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis

Re-evaluating serum angiotensin-converting enzyme in sarcoidosis

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