Although inflammatory myofibroblastic tumors (IMTs) can be found in virtually every major organ, cardiac origin is rare. After recently providing care to a child who presented with a significant myocardial infarction, interest in this rare tumor was piqued. We describe a comprehensive review of cardiac IMT, including information on nomenclature, epidemiology, clinical features, pathogenesis, gross/histological features, immunohistochemical profile, diagnosis, treatment, and prognosis. Fifty-seven cases were identified in the literature. Interestingly, our case represents the seventh case of coronary artery involvement reported. Moreover, it was found that an initial presentation of sudden death most commonly involves the coronary arteries.

Cardiac inflammatory myofibroblastic tumor: a comprehensive review of the literature.

Behcet's disease is an inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Many abnormal echocardiographic findings have been previously reported in patients with Behcet's disease. Some of these; namely, aortic root dilation, aortic valve regurgitation, intracardiac thrombi, and pulmonary artery aneurysm may precede other manifestations of the disease and occasionally require surgical intervention for proper management. Compared to patients without Behcet's disease, management of these complications in patients with the disease is challenging and requires different treatment strategies. If diagnosis of Behcet's disease is missed prior to surgical intervention, higher incidence of surgical failure, recurrence, and even higher postoperative mortality may occur. In this review, the author discusses these major cardiac complications of Behcet's disease.

Behçet's Disease, Echocardiographers, and Cardiac Surgeons: Together is Better

Purpose

To describe the echocardiographic findings detected as first manifestations of Behcet's disease (BD) and compare these findings with those detected in established cases of BD receiving regular medical treatment.



Methods

Two groups of patients were studied. Group 1 comprised 41 patients with BD on regular medical treatment. Group 2 comprised 5 previously healthy patients who presented to the cardiology department because of cardiac symptoms and were diagnosed in retrospect during hospitalization as BD. Thirty-two age- and sex-matched individuals served as control subjects (control group). All patients and controls underwent complete M-mode, two-dimensional, and Doppler transthoracic echocardiographic examinations.



Results

Of the 41 treated patients with BD (mean age: 32 ± 8 years, 90% males, mean duration since diagnosis: 14.5 years), only 1 patient was found to have severe aortic regurgitation secondary to aortic root dilation. On the other hand, the 5 previously healthy patients who presented with cardiac symptoms (mean age: 24 ± 6 years, all males) had significant cardiac involvement and evident echocardiographic findings (P < 0.001). Four cases had intracardiac masses: 3 in the right atrium (RA), 1 in the right ventricle (RV), while the last patient had pericardial effusion (PE). All these patients were diagnosed in retrospect as BD. The RA masses disappeared on medical therapy, while the RV mass was surgically excised and proved to be multiple thrombi histopathologically. The patient with PE had recurrent attacks of massive effusion so a pericardial window was performed surgically.



Conclusion

Diagnosis of BD might be initially suspected by the cardiologists based on certain echocardiographic findings, namely the presence of right-sided masses. Diagnosis of BD in such patients has important therapeutic implications and accordingly prognostic value.

Value of certain echocardiographic findings in the initial suspicion of Behçet's disease.

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and immunohistologic features, and outcome is presented. Recognition of this rare abnormality is important in order to initiate prompt surgical intervention.

Inflammatory myofibroblastic tumor of the aortic valves causing sudden cardiac death: a case report and review of the literature.

OBJECTIVES Cardiothoracic interventions for cardiovascular complications of Behcet's disease have not been sufficiently elucidated. METHODS A comprehensive literature search of cardiovascular complications of Behcet's disease was made for year range 2000-2013. The articles on the cardiothoracic procedures for cardiovascular complications of Behcet's disease were screened and analysed. RESULTS The 221 major cardiothoracic procedures performed in this patient setting included 176 (79.6%) cardiac, 9 (4.1%) thoracic, 31 (14.0%) interventional and 5 (2.3%) hybrid procedures (χ2=478.03, p<0.0001). Of the major cardiac operations, there were 74 (42%) valvular, 58 (33%) aneurysmal, 23 (13.1%) thrombotic, 10 (5.7%) coronary and 11 (6.3%) miscellaneous procedures. The postoperative morbidity, recurrence and mortality rates were 21.4%, 11.7% and 15.0%, and the reintervention rates were 15.4% for recurrence, and 43.2% for morbidity patients. Dehiscence of the prosthetic valve was the major morbidity (52.3%) and the major cause of death (63.6%). The cardiac surgical patients carried the highest mortality rate comparing with thoracic, interventional and hybrid treatment patients. CONCLUSIONS Cardiovascular operations prevailed thoracic and interventional procedures for the cardiovascular complications of Behcet's disease. Postoperative complications and recurrence rates were high. Aortic valve regurgitation, pulmonary artery aneurysm, and intracardiac and great vessel thrombosis were the most common indications for a cardiothoracic intervention. Dehiscence of the prosthetic valve was the main cause of death of the cardiothoracic interventions. Intense immunosuppressive treatment may reduce the postoperative complications and the need for reinterventions.

Cardiothoracic interventions in Behçet's disease

Inflammatory pseudotumor is a rare benign neoplasm. It is common in children and has been reported in various locations throughout the body but rarely in the heart. Behcet's disease is a multisystemic, recurrent, inflammatory disorder. We report a 35-year-old Behcet's disease patient with pseudotumor of the right ventricle and multiple pulmonary emboli. Transthoracic echocardiography showed dilation of right atrium, right ventricle, and a mass in the right ventricle. Multislice computed tomography revealed a large, poorly defined mass infiltrating the groove and multiple pulmonary emboli. Surgery was performed with diagnostic and therapeutic intent. Following sternotomy, we identified a mass in the anterior wall of right ventricle, the outflow tract, and the inflow tract of right ventricle. The histopathologic analysis identified an inflammatory infiltrate composed of lymphocytes, plasma cells, and other inflammatory cells, without mitosis. And there were also some thrombus on top of the mass.

Shengli Yin M.D.

Papers

Inflammatory Pseudotumor of the Right Ventricle in a 35‐Year‐Old Woman with Behçet's Disease: A Case Report