We report a successfully treated case of double valve replacement for mucopolysaccharosis in a 27-year-old woman. Mucopolysaccharosis had been suspected since she was aged 11. Symptoms of heart failure and chest pain suggested valvular disease and she was referred to us. Echocardiography, aortography and cardioangiography showed aortic regurgitation (grade IV/IV) and mitral regurgitation (grade III/IV). She received double valve replacement and was discharged on the 38th postoperative day with symptom improvement. Although urinalysis was positive for heparan-sulfate, this case could not be diagnosed definitively as mucopolysaccharosis due to normal lymphocytic enzyme-activity. However, large amounts of mucopolysaccharoid deposits were present in her removed aortic and mitral valve leaflets, and her clinical picture corresponded with mucopolysaccharosis. Thus, it was considered that her ultimate diagnosis was combined cardiac valvular disease due to mucopolysaccharosis.

Bacterial endopthalmitis is associated with risk for poor visual prognosis, however, it is rarely combined with infective endocarditis. A 66-year-old man underwent pacemaker implantation and received antibiotic therapy due to persistent fever. A month after the pacemaker implantation, he was admitted to our hospital because of disturbance of vision and consciousness. Disseminated intravascular coagulation (DIC) with decrease of platelet count was also present. His eyes were reddish and swelled, and the conjunctiva were turbid and edematous in both sides. Transesophageal echocardiography demonstrated 18×13mm pendulous verruca originating from the tricuspid annulus. The patient underwent concomitant resection and repair of the tricuspid valve and removal of both infected eyes after DIC treatment. The postoperative course was uneventful and he was discharged from the hospital 43 days after the operation. We conclude that careful observation of the eyes may be needed for patients with infective endocarditis when they have some visual symptoms.

Septic pulmonary embolization (SPE) is an uncommon pulmonary disorder. The diagnosis of SPE is frequently delayed because of its nonspecific chest roentgenological features. A 76-year-old woman who underwent pacemaker implantation one year previously received antibiotic therapy under a diagnosis of infectious colitis. She suffered septic shock and disseminated intravascular coagulation (DIC) and was admitted to our hospital. Methicillin-resistant Staphylococcus aureus (MRSA) was cultured from her blood and echocardiography demonstrated 13×16 mm vegetation originating from the tricuspid valve. Multiple peripheral nodules with cavitation were found on chest computed tomography and she was given a diagnosis of SPE. She rapidly presented acute respiratory failure and mechanical ventilation was inevitable for 23 days before surgery. She underwent removal of the entire pacing system, resection and repair of the tricuspid valve and epicardial pacemaker lead implantation. Tracheostomy and long-term mechanical ventilation (16 days) was required after surgery, however, she was discharged from our hospital without any complication.

We report a case of a caseous calcification lesion originating from a calcified anterior mitral annulus. A 59-year-old woman on chronic hemodialysis was referred to our hospital due to an elevated brain natriuretic peptide value. Transthoracic echocardiography demonstrated moderate aortic valve stenosis with regurgitation and a pendulous mass in the left ventricular outflow tract, and therefore we perfomed. The patient underwent resection of the mass with aortic valve replacement. Pathological examination of the mass revealed interstitial calcium deposits but without tumors or inflammatory cells. We speculated that the cardiac mass was caseous calcification which originated from a severely calcified mitral annulus based on its echocardiographic and pathological features.

Primary malignant tumors of the heart are rare and are associated with very poor survival. We retrospectively analyzed early and late results for five primary malignant tumors of the heart. There were two operative deaths and two late deaths, and the mean survival of patients who survived operation was 18.3 months. No operative survivors had symptoms of congestive heart failure during follow up period. One patient who underwent histologic biopsy received postoperative chemotherapy and is alive without recurrence 36 months after operation. The operative mortality of primary malignant tumors of the heart was high and unsatisfactory, however, surgical treatment prevented congestive heart failure during follow up and contributed to the selection of postopeative therapeutic options, with or without complete resection of the tumors.

A 68-year-old man was taken to our hospital by ambulance due to syncope. He was in shock with cardiac tamponade. Pericardial drainage was performed. Aortic valve regurgitation gradually increased and surgery was performed at 25 days after onset. Surgical finding showed that there was a detachment of the commissure between the right and non coronary cusps of the aortic valve. An intimal tear was detected in the same place and aortic root replacement was required. The patient had a good recovery and he was discharged 14 days after surgery.

A 78-year-old woman was referred to our hospital because of progressive exertional dyspnea due to nonrheumatic severe aortic valve stenosis and moderate mitral valve stenosis with mitral annular calcification. We subsequently performed aortic valve replacement and mitral anterior leaflet decalcification. During surgery, we found that the cause of mitral valve stenosis was calcification of A2 aortic curtain-medial trigon through aortic valve annulus and resected calcification with SONOPET. The postoperative echocardiography revealed good mitral valve motion with mild mitral valve stenosis.