1/108. Clinical manifestation and survival of patients with idiopathic bilateral atrial dilatation.We studied the histories of eight patients who lacked clear evidence of cardiac abnormalities other than marked bilateral atrial dilatation and atrial fibrillation, which have rarely been discussed in the literature. From the time of their first visit to our hospital, the patients' chest radiographs and electrocardiograms showed markedly enlarged cardiac silhouettes and atrial fibrillation, respectively. Each patient's echocardiogram showed a marked bilateral atrial dilatation with almost normal wall motion of both ventricles. In one patient, inflammatory change was demonstrated by cardiac catheterization and endomyocardial biopsy from the right ventricle. Seven of our eight cases were elderly women. Over a long period after the diagnosis of cardiomegaly or arrhythmia, diuretics or digitalis offered good results in the treatment of edema and congestion in these patients. In view of the clinical courses included in the present study, we conclude that this disorder has a good prognosis.- - - - - - - - - - ranking = 1keywords = ventricle (Clic here for more details about this article) |
2/108. Neonatal marfan syndrome: a case report.A case of neonatal marfan syndrome is presented. The patient was noted to have cardiomegaly and tricuspid regurgitation on antenatal ultrasound scan. She was born with long, slender fingers and toes, an aged appearance and non-paralytic hypotonia. Echocardiogram revealed a dilated right atrium, right ventricle, dysplastic tricuspid valve and severe tricuspid regurgitation. She subsequently died of severe heart failure. Post-mortem examination showed the pathological features of lobar emphysema and cystic medial necrosis of the aorta. These features supported the diagnosis of neonatal marfan syndrome. Nucleotide sequencing showed substitution of G by A at codon 1032 in exon 25 located in the long arm of chromosome 15. This resulted in the substitution of a cysteine by a tyrosine. A de novo mutation is suggested by the absence of affected family members.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
3/108. pulmonary atresia with intact ventricular septum--a report of two cases with review of literature.Two perinatal autopsy cases are reported where a rare congenital anomaly, namely pulmonary atresia with intact ventricular septum in association with tricuspid stenosis and a hypoplastic right ventricle was encountered.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
4/108. Pathological and neuropathological findings in two males with fragile-X syndrome.The present paper addresses post mortem pathological and neuropathological findings in two males with fragile-X syndrome, aged 67 and 87 years. Both subjects died from sudden, unexpected cardiovascular causes, and both showed abnormalities of the mitral valve, ventricular hypertrophy and cardiomegaly. Both cases demonstrated macrocephaly characteristic of the classical Martin-Bell phenotype in FRAXA. There was increased brain weight in both cases: macroscopically, both cerebral and cerebellar hemispheres appeared normal, but dilated lateral ventricles were seen; and microscopic examination of the brain in case 2 showed normal hexalaminar architecture and no gross neuronal dropout. The hippocampus showed mild CA4 pyramidal cell loss and associated gliosis. The cerebellum showed focal Purkinje cell loss and corresponding Bergmann gliosis. Whilst there is a need to delineate the microscopic features of fragile-X syndrome from those of the ageing process, there is an urgent need for more systematic neuropathological studies of fragile-X syndrome; the increased brain weight and Purkinje cell loss in autism and fragile-X syndrome reopens the debate on these two conditions. The case for further research into the cardiac anomalies in fragile-X syndrome is also strengthened by the findings. Finally, the present report confirms the role of interstitial cell hyperplasia as the major cause of megalo-testes in this condition.- - - - - - - - - - ranking = 0.51227810451473keywords = ventricle, cerebral, brain (Clic here for more details about this article) |
5/108. Successful treatment of a neonate with vein of Galen aneurysmal malformation.We present a follow-up study on a 5-year-old boy who was born with vein of Galen aneurysmal malformation (VGAM) accompanied by extreme cardiovascular distress. He was successfully treated by the staged endovascular technique during the neonatal stage. Postoperative angiographic study confirmed complete occlusion of the VGAM and the establishment of normal brain perfusion. At 5 years of age, the child is growing normally without neurological deficits or developmental retardation. We suggest that the restoration of normal brain perfusion in neonates with VGAM can cause normal brain development.- - - - - - - - - - ranking = 0.0046007446075632keywords = brain (Clic here for more details about this article) |
6/108. Fatal arrhythmia in a juvenile athlete due to myocardial hypertrophy and infarction.This report is a case history of a 16-year-old highly trained athlete who suffered from ventricular fibrillation during exhaustive physical activity. After resuscitation and admission into hospital ECG revealed posterior wall infarction. thrombolytic therapy was advised and ST-segment elevation reversed. Within 48 h cerebral edema evolved due to hypoxic brain damage and the subject deceased after 16 days despite prolonged maximum antiedematous therapy. autopsy confirmed the diagnosis of concentric myocardial hypertrophy (total heart weight 568 g) without signs of coronary artery disease. Systemic inflammatory diseases and drug abuse were ruled out by lab studies, evidence for viral infection was not found. Thus, relative coronary insufficiency in regard to myocardial hypertrophy during excessive athletic activity must be viewed as cause for the fatal arrhythmia.- - - - - - - - - - ranking = 0.0092109414430179keywords = cerebral, brain (Clic here for more details about this article) |
7/108. Further case of Cantu syndrome: exclusion of cryptic subtelomeric chromosome aberrations.Cantu syndrome consists of hypertrichosis, osteochondrodysplasia, and cardiomegaly, and has been reported in 18 patients to date. We report an infant with Cantu syndrome. In addition to typical findings, he had relatively mild radiological and cardiological manifestations. Previously undescribed findings included pyloric stenosis and elevated alkaline phosphatase levels. brain scans showed bilateral calcification of the Arteriae thalamostriatae and widening of the outer liquor spaces and lateral ventricles. Because the propositus is the youngest patient reported to date, our findings refine the clinical spectrum of Cantu syndrome in neonates and young infants. The etiology and mode of inheritance of Cantu syndrome are unknown. Most cases are sporadic. Microdeletions have been discussed as a possible cause of Cantu syndrome. Recently, several syndromes with multiple congenital anomalies and mental retardation have been shown to be caused by subtelomeric chromosome aberrations. We excluded the presence of a cryptic subtelomeric chromosome anomaly in our patient by fluorescence in situ hybridization (FISH) screening with locus-specific probes.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
8/108. Severe heart failure from light chain cardiomyopathy (cardiac amyloidosis).Primary (AL)amyloidosis is characterized by clonal production of immunoglobulin with subsequent deposition in several organs. We describe the clinical features of a 66-year old female who was referred to our department for congestive heart failure. One year before, she was examined and found to have diastolic dysfunction of the left ventricle. We could evaluate the diagnosis of primary amyloid cardiomyopathy by echocardiography, doppler echocardiography and laboratory findings and confirmed the diagnosis by biopsy of the rectum mucosa. Clinical outcome was poor, because therapy is poor.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
9/108. Hypertrophic non-obstructive cardiomyopathy caused by disorder of the myofiber texture.A case of hypertrophic non-obstructive cardiomyopathy caused by a disorder of the myofiber texture was observed in a 10 year old boy. The heart weighed 390 g and showed concentric hypertrophy of the left and right ventricles as well as of the ventricular septum. Additional findings consisted in an abnormal septum membranaceum and a accessory tricuspid valve leaflet. Severe cardiac hypertrophy was associated with prominent interstitial and subendocardial fibrosis, and pronounced intimal fibrosis of the intramural arteries. Electron microscopy revealed various degrees and stages of hypertrophy of the myocardial cells combined with severe degenerative changes. Additional changes of the sinus node and conduction system were responsible for a tachycardia-bradycardia syndrome, complete left bundle branch block and final total AV-block.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
10/108. Rapidly progressive obstructive cardiomyopathy in infants with Noonan's syndrome. Report of two cases.Two patients with hypertrophic obstructive cardiomyopathy and Noonan's syndrome are presented. Both patients were found at postmortem examination to have gross malformation of the mitral valve and obliteration of the left ventricle due to muscle hypertrophy. Each case demonstrated similar clinical, echocardiographic, and angiographic findings. The poor response to medical and surgical therapy are noted.- - - - - - - - - - ranking = 0.5keywords = ventricle (Clic here for more details about this article) |
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