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11/16. Noninvasive evaluation of intracardiac pressures using Doppler ultrasound: a case study of panvalvular regurgitation.

    Doppler velocity signals from regurgitant valve flow can be used to calculate pressure gradients across incompetent valves by a modification of the Bernoulli equation. Analysis of these gradients provides clinically useful, noninvasive information about cardiac chamber pressures. This study presents an unusual case of cardiomyopathy with panvalvular regurgitation which demonstrates the major methods of pressure analysis using Doppler signals from regurgitant valves.
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ranking = 1
keywords = cardiomyopathy
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12/16. Acquired tricuspid incompetence in children: pitfalls in the clinical diagnosis of its aetiology.

    Pitfalls in the clinical diagnosis of the aetiology of acquired tricuspid incompetence in children were exemplified in five patients. In three of them acute staphylococcal endocarditis, the cause of the valve defect, was obscured during life by co-existing sickle cell haemoglobinopathy, acute leukaemia and rheumatic mitral valvulitis, respectively. The fourth and fifth patients had clinical features suggestive of right ventricular endomyocardial fibrosis but necropsy revealed that the tricuspid incompetence was caused by dilated cardiomyopathy and constrictive pericarditis respectively. This series illustrates the need to suspect bacterial endocarditis if a child presents with signs of infection and valvular incompetence, and also dispels the widespread belief that pericardial constriction does not cause tricuspid incompetence.
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ranking = 1
keywords = cardiomyopathy
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13/16. Surgical treatment of tetralogy of fallot associated with congenital tricuspid insufficiency. A report of two cases.

    Among the congenital anomalies associated with tetralogy of fallot, tricuspid insufficiency is rare. In two children with tetralogy of fallot associated with congenital tricuspid insufficiency, one due to elongation of the anterior papillary muscle and resultant redundancy of the anterior cusp of the tricuspid valve and another due to dysplasia of the tricuspid valve, both tetralogy of fallot and tricuspid insufficiency were corrected simultaneously with success. The etiologic factors, clinical manifestations and surgical implications of this combination of anomalies are discussed. It is emphasized that tricuspid regurgitation must be repaired simultaneously in order to avoid low cardiac output syndrome in the case of tetralogy of fallot associated with tricuspid regurgitation, particularly in which pulmonary regurgitation might occur after relief of the outflow tract obstruction.
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ranking = 0.41381821201989
keywords = dysplasia
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14/16. Familial cardiomyopathy, hypogonadism, and collagenoma.

    A patient with tricuspid regurgitation by clinical evaluation, cardiac catheterization, and angiography was found at autopsy to have a cardiomyopathy involving both ventricles but with predominant involvement of the right ventricle. He also had primary testicular failure and a distinctive type of collagenoma. The patient's two brothers were examined and found to have similar collagenomas and testicular failure. Evidence for a mild to moderate degree of cardiomyopathy was also apparent by findings on physical examination, chest roentgenogram, electrocardiogram, and echocardiogram. The father was known to have had a similar skin lesion and congestive heart failure. The aspects of the clinical presentation of this patient and the findings in the two brothers are discussed. A common genetic link in this familial syndrome is not elucidated.
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ranking = 6
keywords = cardiomyopathy
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15/16. Cardiopathy and ocular abnormalities in noonan syndrome.

    We describe three children with noonan syndrome with cardiopathy. One female child had cardiopathy and ocular abnormalities. The other two male children had congenital heart disease of which one had uncommon association of tricuspid valve dysplasia with regurgitation associated with endocardial cushion defect. Karyotypes of the female and one of the male children were normal. The growth hormone and thyroid hormone studies in the first and second male children were normal. All the three children were managed conservatively and followed-up.
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ranking = 0.41381821201989
keywords = dysplasia
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16/16. Isolated congenital tricuspid valve dysplasia: a rare condition mimicking persistent pulmonary hypertension of the newborn.

    Isolated congenital tricuspid valve dysplasia is a rare and potentially lethal congenital heart disease that can be easily confused with persistent pulmonary hypertension of the newborn. We describe a neonate with isolated congenital tricuspid valve dysplasia who did not respond to mechanical ventilation but improved by tolazoline. Clinicians should be aware that the initial fulminant course of this condition may be reversed by reducing the pulmonary vascular resistance, thereby allowing time for spontaneous recovery.
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ranking = 2.4829092721193
keywords = dysplasia
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