| A 47-year-old male Caucasian patient, with no previous relevant medical history, presented in September 1996 with persistent right lower quadrant abdominal pain. A tumor in the cecum was identified and the patient was submitted to ileocecal resection with ileocolic anastomosis. Histological examination showed a moderately differentiated adenocarcinoma. One year later he developed bloody diarrhea, urgency, and loss of weight. Based on clinical presentation and histology of large bowel biopsies, a diagnosis of ulcerative colitis (UC) was established. The previously resected surgical specimen was reevaluated, and lesions resembling UC were identified in the nonneoplastic mucosa. High levels of alkaline phosphatase and gamma-glutamyl transferase were detected. These alterations could be traced back to 1991. Endoscopic retrograde cholangiopancreatography was performed, showing diagnostic features of primary sclerosing cholangitis (PSC), and the patient was put on ursodeoxycholic acid therapy. In March 1999, he started to have progressive dyspnea and signs of cardiac failure. Endomyocardial biopsy was performed showing extensive lesions of endomyocardial fibrosis. This case illustrates a rather silent course of UC in the presence of PSC, and supports the postulated increased risk in the development of proximally located colorectal carcinoma in these patients. Additionally, the development of endomyocardial fibrosis constituted an unexpected finding, not previously reported in this setting. ( info) |
| The patient was a 29-year-old woman. When she consulted a local physician with chief complaints of fever and fatigue of the extremities, cerebral infarction was detected on MRI, in addition to abnormalities found on ECG. Ultrasonic cardiography revealed the presence of a tumor in the left ventricle. Therefore, tumorectomy and endocardectomy were performed under extracorporeal circulation based on a diagnosis of cardiac tumor. Inflammatory cell infiltration into the ventricular wall was pathologically confirmed, and eosinophilia was observed preoperatively. Therefore, the patient was diagnosed as having endomyocardial fibrosis, which is rarely observed in japan. The postoperative course of this patient was satisfactory, and the eosinophil count was normalized postoperatively. At present, this patient is being followed at the outpatient clinic. ( info) |
13/118. An unusual case of recurrent Loffler endomyocarditis of the aortic valve. Idiopathic hypereosinophilic syndrome is a rare systemic disease with an unexplained elevated eosinophil count. Loffler endomyocarditis is hypereosinophilic syndrome with endocardial fibrosis and restrictive cardiomyopathy. The atrioventricular valves are frequently involved, causing valvular regurgitation. Previously, there has been one case report of combined aortic and mitral valve involvement with Loffler endomyocarditis that was treated with bivalvular replacement. We describe a previously healthy 50-year-old man diagnosed with Loffler endomyocarditis complicated by peripheral thromboembolism and severe aortic regurgitation due to valve fibrosis and fibrotic vegetation on the aortic valve. He underwent embolectomy and aortic valve replacement in addition to treatment for his hypereosinophilia. He later presented with cardiomyopathy with severe aortic insufficiency due to the destruction of the aortic valve prosthesis by sterile fibrinous vegetation. To our knowledge, this is the second case in the literature in which Loffler endomyocarditis involves the aortic valve and the first patient in whom only the aortic valve is involved. ( info) |
| The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age. ( info) |
15/118. Fluorodeoxyglucose positron emission tomography excludes pericardial metastasis by recurrent lung cancer. Fluorodeoxyglucose (FDG) positron emission tomography (PET) has been shown to be useful in the diagnosis and staging of a variety of malignancies. Because of its high sensitivity, FDG PET frequently detects malignant lesions that are not demonstrated clearly by anatomic imaging modalities. FDG PET usually has high negative predictive value and, therefore, negative studies are highly suggestive of a benign process. The authors present a patient in whom transesophageal echocardiography and magnetic resonance imaging described pericardial metastasis from a recurrent lung cancer, which on FDG PET was shown correctly to suggest benign scar tissue. ( info) |
| The clinical and angiographic findings in 5 consecutive patients with congestive cardiac failure are presented to illustrate the pitfalls in the clinical diagnosis of endomyocardial fibrosis. In one patient the clinical diagnosis was confirmed at angiography while another patient who had angiographic evidence of early right ventricular endomyocardial fibrosis was diagnosed clinically as mitral stenosis. In 2 patients the clinical diagnosis was erroneous, there being no evidence of endomyocardial fibrosis on angiography. The fifth patient, who had angiographic evidence of idiopathic cardiomegaly, was diagnosed clinically as either idiopathic cardiomegaly or advanced left ventricular endomyocardial fibrosis. In tropical countries, where endomyocardial fibrosis, rheumatic heart disease, and idiopathic cardiomegaly are common, accurate clinical diagnosis of endomyocardial fibrosis is often difficult and angiographic studies are essential for confirmation. ( info) |
17/118. Functionally atrialized parchment-like right ventricle with extensive myocardial fibrosis of left ventricle. A 18-year-old female with unusual type of parchment-like right ventricle died of intractable congestive heart failure was reported. A catheter study revealed the absence of systolic contraction of the right ventricle. The pressure tracing curve in the right ventricle was virtually the same as in the right atrium. At autopsy, there was an extensive myocardial fibrosis of the left ventricle in addition to the almost total absence of the myocardium of the right ventricle. The case was considered to be a unique type of idiopathic cardiomyopathy. ( info) |
18/118. Right-sided endomyocardial fibrosis with recurrent pulmonary emboli leading to irreversible pulmonary hypertension. A 26 year old Saudi man with features of both Loeffer's endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli. Echocardiographic examination showed obliteration of the right ventricular apex by an attached mass. The results of haemodynamic studies were somewhat abnormal and medical treatment was started. Despite anticoagulation with warfarin the patient's condition deteriorated rapidly over a four month period after a further episode of pulmonary embolism and the development of pulmonary hypertension. Two haemodynamic studies performed four months apart were typical of pulmonary hypertension and later right ventricular failure; they showed none of the characteristics of restriction. Pulmonary embolectomy was attempted but there was no cleavage plane between the organised thrombi and the endothelium of the pulmonary artery. The patient died of severe pulmonary hypertension and right ventricular failure several days after operation. Surgical intervention in the early stages of right-sided endomyocardial fibrosis might have prevented the development of pulmonary embolism and pulmonary hypertension. ( info) |
19/118. Multichamber intracardiac thrombi in a patient without any predisposing cardiac or non-cardiac disease. A patient who developed multichamber (left ventricle, right ventricle, right atrium) intracardiac thrombi without having any predisposing cardiac or non-cardiac disease is discussed. The thrombi were diagnosed echocardiographically and confirmed histopathologically. The occurrence of multichamber mural thrombi without any predisposing disease is extremely rare. risk factors for their development have been reviewed. ( info) |
| We report two cases of multiple distal borderzone ischaemic strokes associated with hypereosinophilia due to idiopathic hypereosinophilic syndrome in one patient and to acute schistosoma mansoni infestation in the other. endomyocardial fibrosis (EMF) was documented pathologically, in one case at autopsy and in the other after cardiac surgery; and by cardiac CT, with initially negative echocardiography, in one patient. These observations suggest that so called borderzone infarcts may be due to microembolisms and that, in the context of hypereosinophilia, EMF diagnosis warrants complete cardiac investigation including cardiac CT and repeat echocardiography. ( info) |