Multidisciplinary approach for primary cardiac lymphoma associated with hemodynamic failure caused by tricuspid valve obstruction.

Primary cardiac lymphoma (PCL) comprises rare cardiac tumors and exhibits rapid growth and poor prognosis. We report the case of a 65-year-old man with PCL associated with unstable hemodynamics caused by tricuspid valve obstruction. Generally, chemotherapy is the first choice of treatment for patients with PCL. This patient required emergency tumor reduction as he was at risk of having acute hemodynamic failure caused by tricuspid valve obstruction. Therefore, he underwent a 2-staged treatment: urgent surgery to avoid sudden death by tricuspid valve obstruction as well as pulmonary embolism during chemotherapy, followed by early chemotherapy. Pathological findings showed diffuse large B-cell lymphoma, and rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, prednisolone (R-CHOP) therapy was initiated to treat any residual tumor infiltrating the myocardial wall. The patient showed a marked clinical improvement. We conclude that surgical tumor reduction and early chemotherapy might be an effective treatment for PCL patients with hemodynamic compromise. .

A case of cardiac calcified amorphous tumor (cardiac CAT) causing acute embolism in right common iliac artery.

A 68-year-old man was admitted to our hospital for the further examination of intermittent claudication. He had been on continuous ambulatory peritoneal dialysis for 2 years. Screening transthoracic echocardiography (TTE) revealed a club-shaped tumor and a round-shaped tumor attached to mitral annulus calcification (MAC). The club-shaped tumor was swinging and plunged into the left ventricle at diastolic phase. Because of the risk of fatal embolism, we planned early surgical resection of the tumors. However, 13 days after admission, his intermittent claudication was getting worse and some part of the club-shaped tumor had vanished by TTE. Urgent iliac angiography showed that the tumor had embolized the right common iliac artery. Although we tried embolectomy using a Fogarty catheter, it was unsuccessful. We therefore treated the iliac artery stenosis by endovascular therapy and the procedure was successful. Three months later, he suffered from unstable angina and was treated by percutaneous coronary intervention. However, subacute stent thrombosis occurred after one month. After urgent treatment, we decided to treat him by coronary artery bypass graft and surgical resection of the residual tumor on MAC. The operation was performed successfully. Finally, the tumor was diagnosed as cardiac calcified amorphous tumor by its histologic features. .

Gender differences in the clinical features of hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations.

BACKGROUND: Although gender may be one of the important factors modifying phenotypic expression in hypertrophic cardiomyopathy (HCM), there has been little information on it. METHODS AND RESULTS: We investigated gender differences in the clinical features of HCM caused by cardiac myosin-binding protein C gene (MYBPC3) mutations. Sixty-one subjects (28 families) carrying MYBPC3 mutations were studied. Of the 61 subjects with MYBPC3 mutations, 50 patients including 23 female patients were phenotype-positive by echocardiography. Disease penetrance in subjects aged ≤40 years old was 92% in males and 67% in females. Females showed delayed onset of left ventricular hypertrophy compared with males in subjects who were genotype-positive. Female patients were more symptomatic at diagnosis than were males (mean New York Heart Association class: 1.7±0.8 versus 1.2±0.4, p=0.012). From a longitudinal point of view by age, no significant gender difference in cardiovascular deaths or cardiovascular events was found. During the follow-up period after diagnosis of HCM (13±8 years), female patients who were phenotype-positive had significantly more frequent heart failure events than did phenotypically affected male patients (p=0.028). CONCLUSIONS: Although females with MYBPC3 mutations showed later onset of the disease, female patients were more symptomatic at diagnosis and had more frequent heart failure events once they had developed hypertrophy.

A case of percutaneous transluminal renal angioplasty for partial coverage of a renal artery by a stent graft after endovascular aneurysm repair.

An elderly woman was admitted to our hospital for evaluation of an abdominal aortic aneurysm (AAA) and we decided to treat her AAA with endovascular aneurysm repair. Her renal function became worse after the operation and a renogram suggested flow disturbance of her right renal artery. We performed angiography and checked her right renal artery using intravascular ultrasound. The ultrasound revealed thrombus formation and severe stenosis caused by the stent graft. We performed percutaneous transluminal renal angioplasty at the ostium of her right renal artery. After this procedure, her renal function rapidly improved.

Inferior acute myocardial infarction due to acute cardiac herniation after right pneumonectomy.

A 69-year-old man underwent right intrapericardial pneumonectomy for lung cancer. After 24 h, he went into shock with inferior acute myocardial infarction. We performed urgent coronary angiography, which revealed total occlusion of the mid-right coronary artery. Intravascular ultrasound showed that the artery seemed to be compressed from the pericardial side. We implanted a coronary stent because the lesion was refractory to balloon dilatation. After the procedure, we performed computed tomography and cardiac herniation was diagnosed. Emergency thoracotomy was performed to return the herniated heart to its normal position. This patient was discharged 38 days after initial surgery.

Complex venous malformation in an elderly female: A case report.

A 76-year-old female taking oral medications for chronic atrial fibrillation and hypertension was admitted to our hospital for examination of bilateral leg edema. Transthoracic echocardiography showed an enlarged coronary sinus of about 40 mm. Contrast-enhanced computed tomography revealed absence of the left innominate vein, a persistent left superior vena cava, and absence of the hepatic segment of the inferior vena cava. The interrupted inferior vena cava connected with the hemiazygos vein. The left superior vena cava also continued to the hemiazygos vein with reflux to the enlarged coronary sinus. A diagnosis of heterotaxy syndrome associated with polysplenia was made. No other congenital cardiac abnormalities were found. The patient was also diagnosed as having right cardiac failure due to atrioventricular valve regurgitation associated with chronic atrial fibrillation. The bilateral leg edema improved rapidly with oral medications. Although complex venous malformations such as those described above are rarely observed in the elderly, absence of the hepatic segment of the inferior vena cava, as in this case, is a particularly pathognomonic malformation in left isomerism in heterotaxy syndrome.

Hemolytic anemia in a patient with hypertrophic obstructive cardiomyopathy.

A 66-year-old woman was referred for further evaluation and treatment of normocytic and normochromic anemia with hemoglobin level of 8.6 g/dL. A peripheral blood smear showed fragmented erythrocytes. The patient was then referred to the department of cardiology because of systolic murmur, ECG abnormality, and red cell fragmentation. Transthoracic echocardiography revealed hypertrophic cardiomyopathy with particularly increased interventricular septal thickness of 24 mm and a hyperkinetic wall motion, resulting in marked obstruction to left ventricular outflow tract (pressure gradient of 200 mmHg). Mitral regurgitation due to systolic anterior motion of the mitral valve leaflets was also seen. The cause of anemia was thought to be mechanical intravascular hemolysis due to left ventricular outflow tract obstruction and mitral regurgitation. She was treated with atenolol and the class Ia antiarrhythmic drug cibenzoline to relieve the outflow tract obstruction, and the pressure gradient was reduced to 70 mmHg. After 3 months of treatment, her hemoglobin level had increased to 11.4 g/dL without additional treatment for anemia.