Complete heart block in cardiac sarcoidosis reversed by corticosteroid therapy: time course of resolution

Tomdio, Anna; Syed, Huzaefah; Ellenbogen, Kenneth A.; Kron, Jordana

doi:10.1136/bcr-2020-240834

Cited by 6 publications

(4 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Interestingly, two cases were found in which AV block appeared to be reversed by corticosteroid therapy alone. In one case, a patient with complete heart block reversed to second degree AV block Mobitz type II and later to normal AV conduction after four weeks of corticosteroids treatment, and in the second case, a patient with complete heart block reversed to first degree AV block after two months of corticosteroid treatment [ 18 - 19 ]. While unable to be explored in our above case, it may be worth exploring the viability of medical monotherapy as a standalone treatment for CS, which offers the ultimate benefit of alleviating emotional, financial, and logistical burdens of cardiac device placement and its lifetime potential for further complications.…”

Section: Discussionmentioning

confidence: 99%

Unexpected Case of Cardiac Sarcoidosis in a Caucasian Male

Damito¹,

Liu-An²

2023

Cureus

View full text Add to dashboard Cite

Cardiac sarcoidosis (CS) is an underappreciated diagnosis in healthy patients presenting with recurrent syncope. This may be particularly limited in patients who do not meet common epidemiology and manifestations of sarcoidosis, which are typically African American women and pulmonary, respectively. In our case, we have a previously healthy middle-aged Caucasian American male who presented with recurrent syncope for one week. Initial electrocardiogram showed a right bundle branch block with a normal P-R interval. A few days into admission, the patient suffered another episode of syncope precipitated by micturition, and repeat electrocardiogram revealed evolution to complete atrioventricular block, necessitating emergent placement of a temporary permanent pacemaker. Transthoracic echocardiogram showed preserved left ventricular ejection fraction of 55%-60% with normal heart valves. Chest computerized tomography revealed few pulmonary nodules, prompting a weak concern for infiltrative disease, e.g. sarcoidosis. To evaluate for possible cardiac structural abnormalities, a cardiac magnetic resonance imaging (MRI) study was considered but precluded by the presence of the MRI-incompatible temporary pacemaker. Despite low suspicion, a fluorodeoxyglucose-positron emission tomography was obtained which unexpectedly revealed hypermetabolic lymph nodes in the perihilar, supraclavicular, and mediastinal regions as well as an area along the interventricular septum, consistent with atrioventricular (AV) conduction pathways. As the patient met major criteria for CS per Japanese Circulation Society guidelines, a tentative diagnosis was made, and a Biotronik single-chamber implantable cardiac defibrillator was ultimately placed. On outpatient follow-up, endobronchial ultrasound-guided fine needle biopsy of perihilar lymph nodes revealed only rare epithelioid histiocytes, rare alveolar macrophages, and benign bronchial cells, consistent with benign nodal tissue. Further attempts for histological confirmation were aborted due to profound calcification and location of affected lymph nodes. A decision was made to defer further biopsy, including the gold standard of diagnosis endomyocardial biopsy, due to the risks outweighing the benefits. He initiated medical therapy with prednisone and mycophenolate, as well as trimethoprimsulfamethoxazole for Pneumocystis prophylaxis. Unlike general sarcoidosis, which is often considered a benign systemic disease, CS has high potential for severe complications including arrhythmia, systolic heart failure, and sudden cardiac death. In general, males carry a higher risk of CS than females, especially those who are of African American descent as they carry a higher incidence of nonspecific sarcoidosis. Expectations related to our patient's demographic initially delayed diagnostic workup for infiltrative disease, primarily focusing on intracranial, orthostatic, and infectious causes. This case serves to inform clinicians on early manifestations of CS, raise awareness of its incidence in unexpected dem...

show abstract

Section: Discussionmentioning

confidence: 99%

Unexpected Case of Cardiac Sarcoidosis in a Caucasian Male

Damito¹,

Liu-An²

2023

Cureus

View full text Add to dashboard Cite

show abstract

“…Previous studies have reported that SN abnormalities were irreversible even in those who received immunosuppressants and that ICD implantation with significant atrial pacing was necessary for patients with probable late-stage CS. 3–5 Therefore, early diagnosis and prompt initiation of treatment are important to prevent the progression of early- to late-stage CS to aid with the recovery of SN and AV conduction 11 and to avoid pacemaker implantation, considering that CS may occasionally be rapidly progressive. 1 , 5 …”

Section: Discussionmentioning

confidence: 99%

A case report of recovery of sinus node abnormalities associated with right atrial involvement of ‘early-stage’ cardiac sarcoidosis

Nakahara

Takemoto

Fujishima

et al. 2022

European Heart Journal - Case Reports

View full text Add to dashboard Cite

Background Cardiac sarcoidosis (CS) is a chronic inflammatory disease characterised by impaired contractility of the myocardium secondary to cardiac conduction system abnormalities, which result in atrio-ventricular (AV) conduction block and ventricular tachyarrhythmias. Notably, sinus node (SN) abnormalities are rarely associated with CS. Case Summary We herein present a case of CS presenting with SN abnormalities associated with atrial involvement of the CS and describe the utility of cardiac magnetic resonance imaging (cMRI), fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18-FDG RET-CT) scans, and cardiac biopsy, in making an initial early diagnosis of early-stage CS. Fortunately, an initial appropriate immunosuppression therapy with methylprednisolone for the CS thus far can help the SN and AV conduction function recover and has provided a good clinical course without the implantation of a pacemaker or implantable cardio-defibrillator. Discussion Although the diagnosis of CS may be elusive, the initial clinical suspicion and use of advanced imaging may be important for an early diagnosis of CS. Further, because CS may sometimes rapidly progress, the early diagnosis and treatment of early-stage CS may also be important to help the SN and AV conduction function recover, and avoid an implantation of a pacemaker, as in this present case.

show abstract

“…Complete AV block has a prevalence of 23% to 30% in patients with cardiac sarcoid 51 . The administration of corticosteroids may reverse the AV block caused by sarcoid if active inflammation (as opposed to fibrosis) is present 65 …”

Section: Extrinsic Causesmentioning

confidence: 99%

“…51 The administration of corticosteroids may reverse the AV block caused by sarcoid if active inflammation (as opposed to fibrosis) is present. 65 Endocrine Diseases Among endocrine diseases, hyperthyroidism is rarely responsible for CHB. Focal myocarditis, interstitial inflammation, fibrosis, myocyte necrosis, in, and around the AV node have been reported.…”

Section: Infiltrative Diseasesmentioning

confidence: 99%

Lesions of the Cardiac Conduction System and Sudden Death

Cohle,

Wygant

2023

Am J Forensic Med Pathol

View full text Add to dashboard Cite

When a young previously healthy person dies suddenly, occasionally, the scene is noncontributory and the autopsy and drug screen are negative. In such cases, additional studies, including genetic assessment and cardiac conduction system examination, should be performed. We performed a literature search and reviewed our own material to identify possible or definite conduction system anomalies that may cause death. We identified intrinsic conduction system disease including cystic tumor of the atrioventricular node, atrioventricular node (cystic tumor of the AV node), and fibromuscular dysplasia of the atrioventricular node artery to be likely causes of death. Extrinsic causes, in which a generalized disease affects the conduction system, include tumors, autoimmune disease, infiltrative disorders, and others, are a second category of diseases that can affect the conduction system and cause atrioventricular block and sudden death.

show abstract

Complete heart block in cardiac sarcoidosis reversed by corticosteroid therapy: time course of resolution

Cited by 6 publications

References 7 publications

Unexpected Case of Cardiac Sarcoidosis in a Caucasian Male

Unexpected Case of Cardiac Sarcoidosis in a Caucasian Male

A case report of recovery of sinus node abnormalities associated with right atrial involvement of ‘early-stage’ cardiac sarcoidosis

Lesions of the Cardiac Conduction System and Sudden Death

Contact Info

Product

Resources

About