2026 Volume 13 Pages 175-180
Cardiac sarcoidosis elicits cardiac disorders due to granuloma formation in cardiac tissue; it can lead to cerebral infarcts. We encountered a 72-year-old Japanese male with isolated cardiac sarcoidosis diagnosed after a cardiogenic cerebral infarction. He suffered a sudden-onset disturbance of consciousness, aphasia, and right hemiparesis. His National Institutes of Health Stroke Scale score was 17. The diagnosis was left middle cerebral artery occlusion due to cardiogenic embolism. Percutaneous thrombectomy achieved thrombolysis and recanalization of the cerebral infarction; his National Institutes of Health Stroke Scale score improved to 0. Cardiac ultrasonography revealed an enlarged left atrial diameter, a thinned inferior ventricular wall, a ventricular aneurysm, and left ventricular systolic dysfunction. During the clinical course, ventricular tachycardia was noted; sarcoidosis was suspected as the underlying disease. As no definitive diagnosis could be made, he was discharged on the 54th day of hospitalization. 18F-fluorodeoxyglucose positron emission tomography in the outpatient department showed localized abnormal accumulation in the left ventricle, and he was diagnosed with isolated cardiac sarcoidosis. Steroid treatment was added to anticoagulation therapy. There have been no recurrent cerebral infarctions or lesions in other organs. Cardiac sarcoidosis can be 1 cause of cardiogenic cerebral infarction.
Cardiac sarcoidosis elicits cardiac disorders such as atrioventricular block, fatal arrhythmias, and heart failure due to conduction system disorders attributable to granuloma formation in cardiac tissue. Cerebral infarction in patients with cardiac sarcoidosis has been reported; however, its detection can be difficult.1-7) When cardiac sarcoidosis is accompanied by cardiogenic cerebral infarction, both conditions must be addressed. We report a patient with isolated cardiac sarcoidosis diagnosed after cerebral infarction.
The patient was a 72-year-old male with a history of smoking, first-degree atrioventricular block, hypertension, and membranous nephropathy. His mother had died of heart failure; no details were available. He was admitted to our hospital due to a sudden-onset disturbance of consciousness and right hemiparesis. His level of consciousness was II-10 on the Japanese Coma Scale. He presented with aphasia and right hemiparesis; his National Institutes of Health Stroke Scale (NIHSS) score was 17. Blood tests revealed no coagulation or fibrinolytic abnormalities; his pro-brain natriuretic peptide level was 480.3 pg/mL. An electrocardiogram showed atrial fibrillation (AF). Computed tomography (CT) cerebral angiography revealed occlusion of the left middle cerebral artery. CT perfusion using Vitrea software® (Canon Medical Systems, Japan) depicted the penumbra and the cerebral infarction area; the necrotic core was 0.8 cc, and the penumbra was 28.7 cc (mismatch ratio, 36.42) (Figure 1).
Computed tomography perfusion images.
a. Summary map showing the penumbra area in yellow (28.7 cc) and the necrotic core area in red (0.8 cc) on automatically processed images.
(b-f: Vitrea software®; Canon Medical Systems, Japan)
b. Cerebral blood volume.
c. Time to peak.
d. Cerebral blood flow.
e. Mean transit time.
f. Time to maximum.
g. CT angiogram.
CT: computed tomography
The diagnosis was left middle cerebral artery occlusion due to cardiogenic cerebral embolism. We performed percutaneous thrombectomy and administered tissue plasminogen activator. Recanalization was achieved with thrombolysis, resulting in grade 2B reperfusion (Figure 2). The next day, his NIHSS score was 0; brain magnetic resonance imaging (MRI) revealed cerebral infarction in the insular cortex (Figure 2). Administration of edaravone (60 mg/day) and heparin (10,000 U/day) was started. A transthoracic echocardiogram obtained on the day of admission showed no thrombus; the left atrial diameter was dilated to 4.2 cm. The ratio of diastolic transmitral flow velocity to early diastolic mitral annular velocity was 16.9, and the left atrial volume index was 62 mL/m2, indicating atrial diastolic dysfunction. In addition, there was reduced wall motion, thinning of the inferior ventricular wall, a ventricular aneurysm, and left ventricular (LV) systolic dysfunction. To prevent intraventricular and intra-atrial thrombus formation, warfarin (1.5 mg) was started on day X+1; the prothrombin time-international normalized ratio was maintained at 2.0-3.0, and the warfarin dose was adjusted accordingly.8)
Cerebral angiograms of the left internal carotid artery.
a. Before treatment.
b. Post-thrombectomy.
Brain MRI on the day after admission.
c. Diffusion-weighted image.
d. Fluid-attenuated inversion recovery image.
e. Magnetic resonance angiogram.
MRI: magnetic resonance imaging
On day X+7, we noted ventricular tachycardia (VT). A screening blood test revealed an elevation of high-sensitivity troponin T (0.7 ng/mL); coronary angiography showed no coronary artery stenosis. Despite the administration of mexiletine (300 mg/day), VT recurred multiple times. He underwent catheter ablation on day X+19, and an implantable cardioverter defibrillator was placed on day X+47. Thereafter, he suffered recurrent sustained VT. A second catheter ablation eliminated the arrhythmia. Based on his clinical course, we suspected cardiac sarcoidosis, myocarditis, or cardiac amyloidosis as the underlying disease. Due to the lack of recovery of his cardiac function, no remarkable elevation of troponin T, and no echocardiographic findings suggestive of cardiac edema, myocarditis was ruled out, as was cardiac amyloidosis because typical findings such as LV hypertrophy and low-voltage electrocardiogram were absent. The Kumamoto diagnostic score,9) used to diagnose cardiac amyloidosis, was 0 or 1; nuclear imaging was not performed. On day X+28, cardiac contrast MRI showed reduced motion of the LV inferior wall; there were no areas of delayed enhancement. Methoxyisobutylisonitrile scintigraphy identified only reduced blood flow in the inferior-posterior wall, with no reduced accumulation or defects in the ventricular septum or anterior wall base, which are common sites of cardiac sarcoidosis. Given that gallium scintigraphy failed to reveal any accumulation in the heart, the diagnosis of cardiac sarcoidosis was not confirmed. Blood tests showed that angiotensin-converting enzyme and soluble interleukin-2 receptor levels were within the normal range. Bilateral hilar lymphadenopathy, skin symptoms, and uveitis were absent. Chest CT showed no abnormalities. We did not perform bronchoalveolar lavage. For treatment stabilization, we changed anticoagulant therapy to edoxaban. The patient was discharged on day X+53 without any symptoms.
With his consent, we performed 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) 9 months later. It revealed localized abnormal accumulation in the LV (Figure 3). Based on clinical guidelines (Supplement)10) a diagnosis of isolated cardiac sarcoidosis was made, and he was started on oral prednisolone (30 mg/day for 35 days; 25 mg/day for 28 days; 20 mg/day for 28 days); 12.5 mg/day for 31 days, 10 mg/day for 47 days; the maintenance dose was 5 mg/day. After starting steroid treatment, he initially experienced depression, which subsequently improved, and he continued to receive the maintenance dose. At the last follow-up, 23 months post-onset, there was no recurrence of cerebral infarction, no deterioration of cardiac function, and no evidence of cardiac sarcoidosis.
An 18F-fluorodeoxyglucose positron emission tomography (axial image).
Abnormal localized accumulation in the left ventricle. The diagnosis was cardiac sarcoidosis.
We report a patient with isolated cardiac sarcoidosis diagnosed after a cardiogenic cerebral infarct. The administration of steroids and anticoagulants avoided worsening of his heart disease, and he suffered no recurrent cerebral infarction.
The clinical characteristics of sarcoidosis, such as affected organs and its incidence and severity, vary by geography and ethnicity. Its incidence is low in Japan and tends to be less severe than in Western countries. However, ocular and cardiac involvement is higher than in the West.11-14) Among North American patients with sarcoidosis, 20 - 27% present with cardiac involvement; the reported rate is as high as 58% among Japanese patients.14,15)
Cardiac sarcoidosis is elicited by the infiltration of inflammatory cells into cardiac tissue; this results in the formation of granulomas that damage the conduction system and myocardium. Impairment of the conduction system can lead to atrioventricular block and myocardial damage, fatal arrhythmias, dyspnea, and other symptoms of heart failure.
Patients with cardiac sarcoidosis who only have cardiac involvement and no systemic symptoms are considered to have isolated cardiac sarcoidosis; its definitive diagnosis is difficult because there are no systemic symptoms.5) The rate of patients with only cardiac involvement ranges from 3% to 65% and depends on the diagnostic criteria.16-18) In a 2024 study of 475 Japanese patients with sarcoidosis, the incidence was 25.1%.5) Ventricular aneurysms were detected in 8%-10% of autopsies, and concurrent ventricular arrhythmias have been reported.19,20) Given that our patient was free of coronary artery disease, we hypothesized that inflammatory myocardial damage due to cardiac sarcoidosis led to a decrease in localized ventricular wall motion and that remodeling of the inflammatory area resulted in LV systolic dysfunction and aneurysm formation that elicited VT.
Patients with progressive cardiac sarcoidosis may develop AF. Mechanisms by which cardiac sarcoidosis may lead to AF include inflammation and atrial scarring due to granulomatous infiltration. The incidence of AF was higher in patients with cardiac sarcoidosis whose FDG-PET uptake extended to the atrium.21) Others21,22) suggested that AF is associated with atrial remodeling due to an increase in left atrial diastolic pressure elicited by sarcoid infiltration into the lungs and ventricles. In our patient, PET showed no accumulation of infiltrates in the atria, but accumulation was present in the left ventricle. Therefore, rather than an incidental onset of AF in our case, we speculate that it developed due to increased end-diastolic pressure caused by sarcoid infiltration into the ventricle and the progression of atrial remodeling.
Cardiac sarcoidosis can elicit cardiac cerebral infarction. There are several previous reports of such cases;6,7,23) some authors4-7,23-25) suggested that the infarcts are attributable to thrombus formation after the development of an aneurysm in the ventricular wall and/or to wall motion abnormalities. To our best knowledge, there have been 3 reported cases of cerebral infarction attributable to cardiac sarcoidosis in Japan (Table 1).3,4,24) The 4 cases (including ours) can be summarized as follows: 3 males with varying ages at onset were identified. Three presented with stroke, and 1 was diagnosed with an asymptomatic cerebral infarction associated with heart failure. Echocardiography revealed LV systolic dysfunction (n = 3), LV thrombus (n = 2) or LV aneurysm (n = 2), and wall dyskinesis (n = 2). The diagnoses were based on cardiac MRI (n = 2), FDG-PET (n = 3), and pathological findings (n = 1). In 2 of the 4 patients, the disease was systemic, and in the other 2, it was isolated cardiac sarcoidosis. Treatment included thrombectomy in 3 patients; all 4 underwent steroid therapy, and none suffered recurrence. Only our patient manifested AF and VT; additional cardiac evaluation revealed a LV aneurysm and inferior ventricular wall thinning, yielding a definitive diagnosis of isolated cardiac sarcoidosis.
Summary of Reports of Cerebral Infarction Associated With Cardiac Sarcoidosis in Japan
| Cases | Age/ sex | Symptom | AF/ VT | Echocardiography | Brain MRI | Systemic lesions | Diagnosis of sarcoidosis | Treatment | Follow up (months) | Recurrence or worsening |
|---|---|---|---|---|---|---|---|---|---|---|
| AF: atrial fibrillation; DOC: disturbance of consciousness; FDG-PET: fluorodeoxyglucose positron emission tomography; Lt: left; lv: left ventricle; MCA: middle cerebral artery; MRI: magnetic resonance imaging; VT: ventricular tachycardia | ||||||||||
| Kanemitsu et al. (3) | 31/ male | congestive heart failure | -/- | LV systolic dysfunction, lt ventricle apex thrombus, mild mitral insufficiency | asymptomatic small occipital infarction | + | pathological findings(LV free wall and mediastinal lymph node) | Surgical thrombectomy, warfarin, predonisolone | not described | not described |
| Kuwabara et al. (4) | 42/ male | temporary dysarthria, hemiparalysis | -/- | lt ventricular thrombus, apical partial dyskinesis | multiple cerebral infarcts | - | cardiac MRI, FDG-PET | prednisolone | 6 | - |
| Minomo et al. (24) | 70/ female | aphasia, hemiparesis | -/- | LV systolic dysfunction, focal wall dyskinesia, ventricular aneurysm | lt. MCA occlusion | + | cardiac MRI, FDG-PET | thrombectomy, warfarin, glucocorticoid | 3 | - |
| Our case | 72/ male | aphasia, hemiparesis, DOC | +/+ | LV systolic dysfunction, ventricular aneurysm, atrial diastolic dysfunction, inferior ventricular wall thinning | lt. MCA occlusion | - | FDG-PET | thrombectomy, warfarin, prednisolone | 23 | - |
A registry study of 512 patients with cardiac sarcoidosis performed by Nabeta et al.26) revealed that the estimated 10-year mortality rate was 18.0% and that patients with a low LV ejection fraction, high B-type natriuretic peptide levels, VT, and a history of AF requiring ablation to treat VT were at high risk. Patients with cardiac sarcoidosis and cerebral infarction require steroid and anticoagulant therapy to avoid cardiogenic cerebral embolism. In our patient, transthoracic echocardiography revealed no obvious thrombus, and it is unclear whether an intra-atrial thrombus due to AF or an intraventricular thrombus due to cardiac sarcoidosis was implicated. Our treatment for cardiac sarcoidosis and the administration of anticoagulants successfully addressed his disease.
ConclusionWe report a patient with isolated cardiac sarcoidosis diagnosed after the development of a cardiogenic cerebral infarct. Steroid and anticoagulant therapy prevented the recurrence of cerebral infarction and the worsening of cardiac disease. It should be noted that cardiac sarcoidosis can result in cardiogenic cerebral infarction.
All authors have no conflict of interest.
