Neuroblastoma in an XYY male.
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Biomedical subjects
Publications and source records attributed to Takashi Shima.
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A 76-year-old man developed congestive heart failure due to severe mitral regurgitation after episodes of vasospastic angina. Echocardiography demonstrated left ventricular apical akinesis with ballooning and deformity of the anterior mitral leaflet becoming concave toward the left atrium. The acetylcholine provocation test induced diffuse coronary vasospasm in the distal segments of both right and left coronary arteries and reproduced severe mitral regurgitation. Follow-up echocardiography demonstrated decreased mitral regurgitation with ameliorated apical wall motion. Coronary vasospasm remained refractory to antivasospastic medications and severe mitral regurgitation relapsed 1 month after discharge. Mitral valve annuloplasty with a Carpentier-Edwards physio ring was performed, and no recurrence of mitral regurgitation was observed despite some episodes of vasospastic angina. We speculate that vasospastic angina and the resultant apical wall motion abnormality caused tethering of the mitral subvalvular apparatus, leading to inappropriate mitral coaptation and severe regurgitation.
A 62-year-old male with emphysema developed recurrent episodes of transient left ventricular ballooning occurring in different regions. Left ventriculography revealed symmetric mid-ventricular ballooning when he was 60 years old, and he also developed mid-ventricular ballooning of larger extent at the age of 62 years. Furthermore, as he was treated for severe asthma attack 3 months later, left ventricular apical ballooning occurred. Echocardiography also demonstrated akinetic wall motion in the right ventricular apex. These episodes showed myocardial infarction-like onset, ST elevations on electrocardiography, no significant increases in cardiac enzymes, wall motion abnormalities incompatible with coronary artery disease, and complete recovery within a few weeks. From these findings, we speculate that the recurrent left ventricular wall motion abnormalities including the mid-ventricular ballooning were so-called takotsubo-like left ventricular dysfunction.
In rare cases, patients with heterozygous hemophilia show bleeding tendency due to a low factor VIII activity of <20%. Here we describe a case of heterozygous hemophilia that developed during pregnancy. The patient was a 33-year-old Japanese woman whose father and brother both showed elevated nasal bleeding tendencies. However, they had received no medication for this condition. At 26 weeks' gestation she experienced heavy nasal bleeding. The prothrombin time and platelet count were normal, but the activated partial thromboplastin time was prolonged. Premature rupture of the membrane occurred, and an emergency cesarean section was carried out at 28 weeks' gestation. A diagnosis of heterozygous hemophilia was made after the cesarean section. Hemophilia crisis and heterozygous hemophilia require special obstetric care, with close liaison with a hematologist. Guidelines for the management of such cases should be available and observed.
We encountered complications of septic shock and retroperitoneal hemorrhage which occurred after implantation of a retrievable Günther Tulip filter. A 79-year-old woman was diagnosed as right femoral vein thrombosis, and a retrievable Günther Tulip filter was deployed prior to a total knee replacement surgery. However, the patient developed septicemia due to Citrobacter freundii, followed by thrombocytopenia and retroperitoneal hemorrhage. A large hematoma was formed along the lateral side of the filter struts. We considered that the filter should be removed to control retroperitoneal hemorrhage and prevent secondary infection of the hematoma. Removal of the retrievable filter was effective in managing these complications.
A 76-year-old man with previous antero-septal myocardial infarction and aorto-coronary bypass surgery developed exertional dyspnea. Echocardiography revealed diffuse left ventricular hypokinesis and aortic stenosis with a mean pressure gradient of 29 mmHg. Coronary angiography showed no significant lesions in the bypass grafts and total occlusions of the proximal left anterior descending artery and mid circumflex artery. Dobutamine stress echocardiography was performed to evaluate the severity of aortic stenosis and left ventricular functional reversibility. Administration of dobutamine increased the mean pressure gradients to 48 mmHg and increased the stroke volume by 28% without change in aortic valve area of about 0.5 cm2. We considered that our patient had severe aortic stenosis with contractile reserve. After aortic valve replacement, he improved with better left ventricular function.
A 83-year-old man with an implanted pacemaker was admitted to our hospital for dyspnea. Echocardiography revealed left ventricular dilation and diffuse hypokinesis mimicking dilated cardiomyopathy. Multiple trabeculations and deep intertrabecular recesses were prominent in the apical and posterior walls. Computed tomography with contrast medium also showed prominent trabeculations. Thallium-201 and iodine-123-beta-methyl-p-iodophenyl-pentadecanoic acid scintigraphy demonstrated fixed defects in the inferior wall. These findings were consistent with isolated noncompaction of the ventricular myocardium. The patient's symptoms of heart failure improved in response to treatment, including carvedilol. Isolated noncompaction of the ventricular myocardium is recognized as a rare congenital disorder of infancy with fatal outcomes, with some reports of cases in adults. The present patient is the most elderly described to date.
A 60-year-old man underwent emergent cardiac catheterization because of anterior chest oppression. He had been treated with calcium antagonist and nitrate. Coronary angiography showed no organic stenosis. However, left ventriculography revealed that the basal and the apical areas were hyperkinetic and the mid portion was akinetic. Echocardiography showed that the bizarre-shaped left ventricular dysfunction had completely recovered after a week. Serial electrocardiography demonstrated initial ST-segment elevation in the precordial leads followed by T-wave inversion in II, III, a VF and V4-V6. Coronary artery spasm provocation tests were negative. Endomyocardial biopsy revealed focal myocardial depletion and mild fibrosis. The present case may be an atypical form of tako-tsubo-like left ventricular dysfunction in which the left ventricular contraction is symmetric with both hyperkinetic and akinetic areas.