Cases reported "Heart Diseases"

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1/69. Systemic lupus erythematosus with myocardial dysfunction due to microvasculopathy.

    A 25-year-old Japanese woman with systemic lupus erythematosus (SLE) had myocardial dysfunction. Heart catheterization showed normal coronary anatomy. Left ventricular cineangiography demonstrated hypokinesis in the anterior and posterior segments. Myocardial scintigraphy revealed patchy defects in the regions unrelated to coronary arteries. These data suggested that the myocardial dysfunction was due to microvasculopthy. In addition, it was speculated that the microvasculopathy was caused by vasculitis but not by thrombi, since she did not have antiphospholipid syndrome. In support of this speculation, corticosteroid therapy without any thrombolytic agents was effective. This report represents the first live patient with SLE in whom myocardial dysfunction due to microvasculopathy has been demonstrated.
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2/69. Aortic dissection complicated with aorto-right atrium fistula.

    Aorto-right atrium fistula associated with aortic dissection is a very rare complication. Here report a case of successful surgical repair of ascending aortic dissection complicated with aorto-right atrium fistula. A 65-year-old man was presented with sudden chest pain and dyspnea. Fifteen years ago, he had aortic valve replacement. An aortic dissection with fistula to the right atrium was diagnosed by echocardiography and cardiac catheterization. At operation, dense adhesion of the aortic root due to the previous cardiac operation was confirmed, and this was suggested as the cause for this rare complication.
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3/69. Iatrogenic left ventricular-right atrial fistula following mitral valve replacement and tricuspid annuloplasty: diagnosis by transthoracic and transesophageal echocardiography.

    Acquired left ventricle-to-right atrium communications are a known complication of valvular heart surgery. Previous reports have described the clinical features and diagnosis using cardiac catheterization. We report two cases of acquired left ventricle-to-right atrium fistula following mitral valve replacement. Particular emphasis is placed on the diagnosis using transthoracic and transesophageal echocardiography, obviating the need for cardiac catheterization before repair.
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4/69. Assessment of coronary morphology and flow in a patient with guillain-barre syndrome and ST-segment elevation.

    patients with guillain-barre syndrome often have cardiac disturbances as a manifestation of autonomic dysfunction. Such abnormalities consist of arrhythmias and disturbances of heart rate and blood pressure. We report a case of a patient with guillain-barre syndrome who developed ST-segment elevation in the inferolateral leads, suggestive of an acute coronary syndrome. cardiac catheterization revealed angiographically normal coronary arteries. Intracoronary ultrasound was also normal. Intracoronary Doppler flow measurements revealed an elevated baseline coronary flow velocity of up to 41 cm/s and decreased coronary flow reserve, particularly in the left circumflex artery. Myopericarditis as cause of the electrocardiographic changes could be ruled out by echocardiography and endomyocardial biopsy. We postulate that the intracoronary Doppler findings are caused by autonomic dysfunction with decrease of coronary resistance and redistribution of the transmural myocardial blood flow.
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5/69. Atrial thrombus and central venous dialysis catheters.

    A native arteriovenous fistula is the first choice for hemodialysis access. Despite improved catheter designs and the use of internal jugular veins, thrombotic complications still occur when tunneled central venous catheters are used as an alternative. Although right atrial thrombus (RAT) is a well-characterized complication of long-term central venous cannulation, particularly when used for parenteral nutrition and chemotherapy in pediatric practice, only 9 reported cases previously have been associated with the long-term use of central venous catheters for hemodialysis. We report five cases of RAT seen at our unit between 1994 and 1998 in patients who had been dialyzed using tunneled catheters. In four of five cases, the diagnosis was made during the investigation of hemoptysis or dyspnea. In the fifth case, a screening transthoracic echocardiogram revealed the thrombus. Three of five of the patients suffered pulmonary emboli, and a fourth patient had an unexplained electromechanical dissociation cardiac arrest without definite evidence of pulmonary embolus. Our experience suggests that anticoagulated patients with RAT remain at risk of pulmonary embolism. One of our patients successfully underwent atrial thrombectomy. In four of five of our cases and four of nine cases in the literature, the central venous catheter tip was within the right atrium. Positioning of the central venous catheter tip low down in the superior vena cava or in the right atrium has been advocated to improve dialysis adequacy and to reduce the incidence of catheter thrombosis. However, placement of the catheter tip within the right atrium may be associated with an increased risk of RAT.
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6/69. syncope with ST-segment abnormalities resembling brugada syndrome due to reversible myocardial ischemia.

    This report describes a case of syncope with an initial ECG that showed ST-segment elevation in the right precordial leads suggestive of brugada syndrome. procainamide infusion induced a significant increase in the ST-segment abnormalities, further increasing the suspicion for this syndrome. cardiac catheterization showed lesions in the proximal left anterior descending artery and distal right coronary artery. Following percutaneous coronary intervention at these sites, the ST-segment abnormalities resolved and a repeat procainamide challenge was negative. Electrophysiological study did not provoke any ventricular arrhythmias. Silent myocardial ischemia may result in ECG changes that resemble those seen in patients with brugada syndrome.
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7/69. Acute cardiomyopathy with recurrent pulmonary edema and hypotension following heroin overdosage.

    An 18-year-old man developed acute pulmonary edema following heroin overdose. Two days after initial improvement, there was recurrence of hypotension and pulmonary edema with severe hypoxemia refractory to mechanical ventilatory support utilizing positive and end-expiratory pressure. cardiac catheterization revealed elevated pulmonary capillary wedge pressure suggestive of left ventricular failure. The use of digitalis and diuretics resulted in prompt clinical improvement and ultimate recovery. Evidence is presented indicating that this patient represents an uncommon but important syndrome of acute cardiomyopathy with left ventricular failure which complicates the clinical course of certain cases of heroin overdose. Its physiologic diagnosis is of obvious importance in the choice of proper therapy, thereby increasing the patient's chances of recovery.
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8/69. 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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9/69. syncope--an unusual presentation of ventricle dysfunction in a patient with Fontan circulation.

    We report a case of recurrent syncope in association with moderate ventricular dysfunction and mild AV-valve regurgitation in an 18-year-old girl, 4 years after she underwent total cavopulmonary connection surgery. cardiac catheterization revealed a transpulmonary gradient of 1-2 mmHg. During exercise, a dramatic fall in blood pressure and blood oxygenation was observed, paralleled by an increase in heart rate and central venous pressure. Although a slight increase in pulmonary vascular resistance could not be excluded, the reaction was interpreted in terms of an extremely low transpulmonary gradient in association with ventricular dysfunction. Five months after heart transplantation, the patient has been completely free from syncope. Fontan circulation usually involves a delicate haemodynamic situation which may necessitate haemodynamic re-evaluations, including dynamic measurements of the central venous pressure during exercise. Also a moderate ventricular dysfunction may result in compromised pulmonary circulation which in turn may lead to syncope during exercise as a result of insufficient systemic circulation.
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10/69. Diffuse locally invasive lipomatosis of the pericardium.

    The chest radiographic, echocardiographic (transthoracic and transesophageal), MRI, CT and cardiac catheterization findings in a 72-year-old patient with extensive pericardial lipomatosis are presented. Diastolic pressures in the left heart were elevated. The massive lipomatous mass was partially resected surgically with good symptomatic relief. Histologically the neoplasm consisted of mature non-malignant adipose tissue. Cushing's Syndrome was absent.
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