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1/26. Evolution of left ventricular diseasein the fetus. Case report.

    A fetal case is described that showed a rapid progression from the features of initial left ventricular fibroelastosis at 20 weeks of gestation to a more marked dilation at 22 weeks and finally to a hypoplastic left ventricle with aortic stenosis at 24 weeks of gestation. This case confirms the evolutive character of left ventricular disease during fetal life.
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2/26. Late-diastolic forward flow in the aorta induced by left atrial contraction.

    Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography.
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3/26. Secondary endocardial fibroelastosis associated with Pompe disease and multicystic dysplastic kidney.

    An infant with secondary endocardial fibroelastosis (EFE) associated with glycogen storage disease II (Pompe disease) and multicystic dysplastic kidney (MCDK) is described. She had had refractory heart and renal failure from the early neonatal period. In spite of administration of cathecholamines and diuretics, ventilator support, and peritoneal dialysis, her heart failure due to reduction of left ventricular contractility progressively worsened. She died on the 40th day after admission. Histological examination of a left ventricular autopsy specimen showed prominent thickening of the endocardium due to fibroelastosis, and a lacework-like structure due to accumulation of glycogen in the cardiomyocytes. The EFE was derived from degeneration of the smooth muscle in the endocardium and cardiomyocytes due to glycogen storage. In addition, we supposed that the renal failure due to MCDK made the preload for the ventricles increase and accelerated her heart failure.
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4/26. Electron beam computed tomography appearance of endocardial fibroelastosis EBCT appearance of endocardial fibroelastosis.

    Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.
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5/26. Familial nonobstructive cardiomyopathy with endocardial fibroelastosis beyond infancy.

    A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle. The sister of this patient had followed a similar course at 13 years of age with death within six months of the onset of congestive failure. Her postmortem examination also showed endocardial fibroelastosis. The clinical presentation of familial endocardial fibroelastosis in the preteen and teenage years is a rare event. Probably the endocardial fibroelastosis was secondary to a familial nonobstructive cardiomyopathy.
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6/26. heart transplantation in a patient with isolated noncompaction of the left ventricular myocardium.

    We describe a patient with isolated noncompaction of the left ventricle who presented with worsening congestive heart failure and was successfully treated with heart transplantation. The prognosis for these patients is poor because of accelerated event rates of fatal arrhythmias, thromboemboli, and profound left ventricular decompensation. Only 7 patients with isolated noncompaction of the left ventricle have been reported to have undergone heart transplantation. Herein we describe a patient with isolated noncompaction of the left ventricle who underwent successful heart transplantation.
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7/26. Double primary left ventricular and aortic valve papillary fibroelastoma.

    Papillary fibroelastomas of the heart are relatively rare benign tumors and although they can be symptom free, symptoms such as cerebral ischemia and cardiac infarction can be lethal. It is important to diagnose this tumor using echocardiography, because lethal embolisms can be prevented by surgical resection. recurrence of this tumor has not been reported and multiple tumors are rare. The present report is a case of double primary aortic valve fibroelastoma with aortic regurgitation diagnosed 4 years after surgery for a left ventricular fibroelastoma with mitral valvular disease.
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8/26. A rare case of a cardiac papillary fibroelastoma of the pulmonary valve diagnosed by echocardiography.

    Primary cardiac tumors are rare. The majority of primary tumors of the heart are benign with papillary fibroelastomas being the third most common after myxomas and lipomas respectively. Papillary fibroelastomas represent the most common valvular tumor of the heart. Although benign, papillary fibroelastomas have the potential to cause turbulent flow, thrombus formation, and severe cerebral or coronary embolic disease. The majority of fibroelastomas have been described on the left side of the heart involving the aortic and mitral valves. We present a rare case of a papillary fibroelastoma occurring on the pulmonic valve.
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9/26. endocardial fibroelastosis with coronary artery thromboembolus and myocardial infarction.

    We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy and myocarditis. After gradual improvement in heart size and function with pharmacologic therapy, he developed a terminal episode of respiratory distress and cardiogenic shock, with ECG findings of an anterolateral infarct. At autopsy it was found that endocardial fibroelastosis with mural thrombi in the left ventricle had been complicated by thromboembolism to the left anterior descending coronary artery, resulting in transmural infarction of the anteroseptal region of the left ventricle. myocardial infarction is a potential but unusual thromboembolic complication of endocardial fibroelastosis. A high index of suspicion for coronary artery thromboemboli should be maintained in pediatric patients with cardiomyopathy and suspected myocardial infarction.
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10/26. Balloon dilatation of the aortic valve in the fetus: a report of two cases.

    Because they had irreversible damage to the left ventricular myocardium none of 12 patients with critical aortic stenosis diagnosed prenatally survived after postnatal treatment. This experience prompted three attempts at intrauterine balloon dilatation of the aortic valve in two fetuses with this condition. On each attempt the balloon catheter was successfully delivered to the left ventricle. In the first fetus the aortic valve was not crossed and the fetus died the next day. In the second fetus the balloon was correctly positioned across the aortic valve and inflated in the valve ring. After delivery, a further balloon angioplasty was performed; this relieved the stenosis but the patient died five weeks later from persisting left ventricular dysfunction related to endocardial fibroelastosis. Balloon angioplasty is feasible in fetal life but the prognosis depends on the ability of the relief of stenosis to limit, prevent, or allow regression of left ventricular damage before delivery.
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