Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-systemic vasculitis, with cardiac involvement being one of its most serious manifestations. We aimed to systematically review and analyze the limited case reports of EGPA with cardiac involvement. f EGPA with cardiac involvement. Methods: Based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we performed a systematic literature search for the case reports of EGPA with cardiac involvement in the MEDLINE database from 2011 until 2018. For each case, clinical data including sex, age, clinical presentation, electrocardiographic and cardiac imaging findings, the type of cardiac involvement, the available laboratory data (cardiac biomarkers, white blood cell count, eosinophilic count, erythrocyte sedimentation rate, C-reactive protein, and antineutrophil cytoplasmic antibody positivity), therapeutic regimen, and the outcome of the patients were collected and analyzed. Results: A total number of 62 cases were included. The mean age was 48.29±15.60 years, and 51.6% were male. All the cases were in the active disease state. Cardiac symptoms, electrocardiographic abnormalities, abnormal biomarkers, and abnormal echocardiography were detected in 82.3%, 68.5%, 77.4%, and 96.8%, respectively. Cardiac magnetic resonance was done in 46.8% of the patients, and it was abnormal in all. The most common abnormal findings in echocardiography were systolic left ventricular dysfunction (83.9%) and pericardial effusion (37.1%). The most common type of clinical presentation was clinical heart failure (51.6%). Only 6.5% of the patients presented with tamponade. The overall prognosis was good. Conclusion: Any part of the heart could be involved by EGPA. The results emphasize the necessity of in-depth cardiac evaluation in these patients.
The IgG4‐related disease is a distinct, steroid‐responsive fibro‐inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4‐positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri‐aortitis, coronary arteritis, and pericarditis. Intra‐cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4‐related disease with a long‐time lag between initiation of symptoms to proper diagnosis with biopsy‐proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.
Intramyocardial dissection (IMD) with ventricular septal rupture (VSR) following myocardial infarction (MI) is a rare subacute form of cardiac rupture. The evidence available in this regard is scarce. We aimed to share our experience and conduct a systematic review of previous cases. We searched the literature and performed a systematic review of previous cases. A total of 37 cases of IMD with VSR were included (1 our original and 36 literature cases). Mean age was 68 ± 8 years and 20 (54.1%) patients were male. Anterior and inferior MI were observed in 14 (37.8%) and 23 (62.2%) cases, respectively. The dissected area was the septum, RV, both septum and RV, or LV apex in 21 (56.8%), 9 (24.3%), 5 (13.5%), and 2 (5.4%), respectively. Apicoseptal and inferoseptal VSR were observed in 15 (40.5%) and 22 (59.5%) cases, respectively. At least one occluded artery was observed in 29 (90.6%) of cases. Reperfusion therapy was done for 15 (40.5%) cases before the VSR occurred.Surgery, percutaneous, and medical therapy were done for 26 (70.3%), 3 (8.1%), and 7 (18.9%) cases, respectively. The mortality rate was significantly higher in the medical versus surgical-treated group (85.7% versus 42.3%, P = .027). There was a trend to higher mortality in the group with dissection of both septum and RV (P = .15). We concluded that echocardiography has a critical role in diagnosing this complication.Surgery is mandatory in IMD with VSR.
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