Cases reported "Heart Diseases"

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11/66. QT prolongation and syncopal attacks. A case of the romano-ward syndrome.

    The romano-ward syndrome is very rare as hereditary disease. Only 5 families have been reported in japan before the present report. The patient, a 15-year-old female, had been treated for epilepsy due to syncopal attacks. electrocardiography revealed occurrence of an augmented and labile U wave and prolongation of the Q-T interval, with recordings of frequent multifocal extrasystoles and transient ventricular fibrillation. Also noticed was sinus bradycardia. Clinical examinations presented no results coinciding with those specific ECG findings. It was inferred through surveys of her family tree that the present case had an autosomal dominant trait, heterozygously affected through the male line. Severe arrhythmias detected on her ECGs were suppressed by oral administration of 30 mg/day propranolol. Judging from the fact, propranolol may be the first to be administered among a variety of anti-arrhythmic agents in the romano-ward syndrome.
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12/66. T-wave alternans associated with heart failure and hypomagnesemia in alcoholic cardiomyopathy.

    Marked T wave abnormality developed in a patient with alcoholic cardiomyopathy. The T negativity was of giant size and occurred in an alternating sequence in the presence of sinus rhythm. This change was rapidly transient, disappearing in 3 days. The complete electrocardiographic recovery was temporally related to successful treatment of severe heart failure, normalization of initially low serum magnesium level, and abolition of recurrent ventricular fibrillation.
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13/66. Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990 to 2000).

    Cardiac papillary fibroelastoma (PFE) is a rare, benign, slow-growing tumor of the endocardium. Whether it represents a reactive tumoral lesion or a true neoplasm remains a matter of debate. However, an anecdotal association of PFEs with previous cardiac surgery has been reported. The current study was undertaken to determine the frequency and nature of iatrogenic events associated with PFEs and to provide a comprehensive review of the topic. The study group comprised 12 cases seen between 1990 and 2000, with specimens from 7 women and 5 men. Six developed postoperatively and 6 developed after thoracic irradiation. The 9 Mayo cases represented 18% of all surgically excised PFEs during the study period. The mean age at operation was 54 years (range, 29 to 79 years). The mean interval between the iatrogenic event and excision of the tumor was 18 years (range, 9 to 31 years). In 58% of cases, the presence of multiple tumors was either confirmed pathologically (41.7%) or strongly suggested by echocardiography (16.6%). Among patients who had undergone previous cardiac surgery, PFEs were found in the chamber closest to the procedure. Similarly, in patients who had received radiation therapy, tumors developed in the left atrium, in the right ventricle and atrium, and on the tricuspid valve within the radiation field. In conclusion, iatrogenic PFEs may be relatively common among all such tumors, are frequently multiple, and often involve nonvalvular endocardial surfaces.
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14/66. The hemodynamic simulation of mitral regurgitation in ventricular septal defect after myocardial infarction.

    The development of a ventricular septal defect (VSD) following myocardial infarction is an uncommon complication which clinically can be confused with mitral insufficiency due to infarction of a papillary muscle. The clinical and hemodynamic records of six patients with documented acute VSD secondary to myocardial infarction were analyzed to determine which descriptors would be of value in clinically separating these two entities. All six of our patients had a right heart catheterization showing an oxygen step-up consistent with a VSD, and five had a large pulmonary wedge V wave suggesting concomitant mitral insufficiency. The echocardiogram showed only nonspecific chamber enlargement. Since these patients were being considered for open heart surgery to close the VSD, left and right cardiac catheterization including selective coronary arteriography was done. Despite large V waves being present in the pulmonary wedge and/or left atrial pressure tracing in five of the six patients, no mitral insufficiency was present on the left ventricular cineangiograms. It is concluded that a large pulmonary wedge and/or left atrial V wave does not necessarily indicate mitral insufficiency. Since both a VSD and mitral insufficiency are surgically correctable, patients who develop new holosystolic murmurs following myocardial infarction should have complete right and left heart catheterizations with LV angiography for accurate diagnosis if surgical correction of the lesion is contemplated.
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15/66. Left ventricular apical thrombus formation in a patient with suspected tako-tsubo-like left ventricular dysfunction.

    A 74-year-old woman with hypertension and bronchial asthma had chest discomfort at rest and 4 days later was admitted to her nearby hospital because of the sudden onset of right hemiparesis. The hemiparesis had almost disappeared within 24 h of onset, but because an electrocardiogram showed sinus tachycardia and diffuse symmetrical T-wave inversion, she was referred for cardiac examination. coronary angiography did not reveal any significant coronary artery stenosis, but left ventriculography revealed severe hypokinesis of the left ventricular apical region, which contained a 4 x 4-mm solid thrombus moving freely with a wavy motion. Moreover, the activity of both protein c and protein S had decreased. The thrombus disappeared after 2 weeks of anticoagulant treatment with warfarin. Her clinical course suggested that the transient cerebral ischemic attack was caused by embolism of the left ventricular thrombus associated with 'tako-tsubo-like left ventricular dysfunction'.
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16/66. Right atrial thrombus migration during echocardiography: a case for urgent intervention.

    A 65-year-old woman presented with increasing shortness of breath, chest pain and ST-T wave abnormalities on the electrocardiogram suggestive of unstable angina. Cross-sectional echocardiography performed to assess wall-motion abnormality and left ventricular function revealed a pedunculated right atrial thrombus prolapsing into the right ventricle which suddenly dislodged and migrated into the pulmonary circulation during the examination. A diagnosis of recurrent pulmonary thromboembolism was made, necessitating urgent pulmonary angiography with infusion of streptokinase. The patient made an uneventful recovery.
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17/66. Cardiac involvement in the Kugelbert-Welander syndrome.

    Two cases of the Kugelberg-Welander syndrome (juvenile form of progressive spinal muscular atrophy) associated with cardiomyopathy and cardiomegaly are presented. The first patient, a 24 year old man, had atrial flutter with complete atrioventricular (A-V) block due to A-H block. echocardiography revealed an increase in the left atrial and right ventricular dimensions. The second patient was a 26 year old man whose electrocardiogram revealed an A-V junctional rhythm, deep Q wave in leads I, aVL and V5 to V6 and an RS pattern in lead V1. Histologic examination of the myocardium in Case 2 showed slight interstitial fibrosis. review of previously reported cases shows that (1) the atrium, the ventricular myocardium and A-V conducting tissue may be involved, and (2) atrial arrhythmias, A-V conduction disturbances and congestive heart failure may occur in the Kugelberg-Welander syndrome.
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18/66. Intra-cardial thrombosis with systemic and pulmonary embolism as main symptoms in a patient with protein s deficiency.

    This report describes the unusual occurrence of both left and right atrial thrombosis with peripheral arterial and pulmonary embolism, respectively, as presenting symptoms of congenital protein s deficiency in a 31-year-old man. The coagulation study performed in the coumarin-treated propositus indicated a heterozygous protein S state. The finding of reduced free protein S antigen and protein S activity levels with normal total protein S and C4B-bp levels in five other family members (father, sister, and three relatives on the paternal side) confirmed the inherited nature of the defect. Since there is an increased frequency of arterial thrombosis in patients suffering from protein s deficiency, any case of idiopathic intra-cardial thrombosis requires careful haemostatic screening. In addition, the possibility of intra-cardial thrombosis should be considered in any thromboembolic event seen in inherited protein s deficiency.
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19/66. Embolus in transit.

    Since the advent of echocardiography, embolus in transit, historically found during surgical exploration or on postmortem examination, has been found with increasing frequency on antemortem examination. There is an inherent high mortality rate with this condition and awareness of the association between deep venous thrombosis and embolus in transit is paramount. On echocardiography the embolus is typically seen as a pleomorphic mass moving in a tumbling fashion. The most frequent symptoms are dyspnea and near syncopal episodes. The most common signs are diastolic "tumor plop" and a systolic ejection murmur heard on auscultation. Despite the success of some medical interventions, surgery should be strongly considered in patients with embolus in transit.
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20/66. Cardiac disease in children with primary glomerular disorders-role of focal segmental glomerulosclerosis.

    Anecdotal reports suggest a higher frequency of serious cardiac complications, particularly cardiomyopathy and congestive heart failure (CHF), in children with focal segmental glomerulosclerosis (FSGS). We report the occurrence of cardiac disease in children with FSGS compared with other glomerular causes of primary nephrotic syndrome (NS). A chart review was performed on all patients evaluated at the Schneider Children's Hospital between 1985 and 2003 with a diagnosis of membranoproliferative glomerulonephritis (MPGN), membranous nephropathy (MN), focal global glomerulosclerosis (FGGS), and FSGS. Clinical and demographic data were compiled, specifically whether or not the patient had clinically evident cardiac disease. The blood pressure (BP) and hematocrit in patients with FSGS and chronic renal failure (CRF) (glomerular filtration rate <30 ml/min per 1.73 m(2)) in the 3 months prior to the development of cardiac complications were compared with the values in FSGS patients with CRF but no cardiac complications, and in patients with the other causes of primary NS in whom CRF developed. There were 48 patients with FSGS, 22 with MPGN, 19 with MN, and 4 with FGGS. Cardiac disease occurred in 6 children (mean age 11 years), all with FSGS. Four of these patients were black and 5 were female. CHF occurred in all patients, cardiomyopathy in 4, and left ventricular hypertrophy in 5 patients. There was no significant difference in the BP and the hematocrit levels between the 6 patients with both FSGS and cardiac disease, 3 patients with FSGS and CRF but no cardiac disease, and the 5 patients with the other glomerulopathies in whom CRF occurred ( P>0.1). Our findings suggest that there is a clinical association between FSGS and cardiac disease in pediatric patients. We speculate that the immune mechanism responsible for the development of FSGS may also affect the heart.
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