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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.

Adolescent↗

Neonatal mechanical bridging to total orthotopic heart transplantation.

BACKGROUND: Until recently, newborns with medically intractable cardiac failure caused by congenital malformations were mostly doomed to death because of the severity of the disease, which precludes a palliative operation, or because of fatal deterioration before availability of a suitable donor heart. METHODS: The recently developed paracorporeal pneumatically driven Medos HIA ventricular assist device offers a therapeutic option for these small infants because it is manufactured in various sizes and is even suitable for cardiac assistance in neonates with a body surface area less than 0.3 m2. RESULTS: We report our initial experience with this device, which we used for univentricular bridging to total orthotopic cardiac transplantation in 3 infants. The device was inserted to support the left ventricle in two instances and to support the right heart in one. Successful bridging to transplantation was achieved in 2 infants for periods of 2 and 7 weeks. CONCLUSIONS: Our experience demonstrates the feasibility of univentricular mechanical support followed by successful cardiac transplantation in infants and newborns.

Aortic Valve Stenosis↗

Chronic afterload reduction in infants and children with primary myocardial disease.

The purpose of this study was to determine the long-term effects of chronic afterload reduction with oral hydralazine therapy in patients with primary myocardial disease (PMD). Twenty-six children aged 3 to 48 months with the diagnosis confirmed by M-mode and two-dimensional echocardiograms and angiograms were given digitalis and diuretics. Fourteen of these patients also received hydralazine orally in doses up to 4.0 mg/kg/day in four divided doses. Echocardiograms were initially repeated at 1- to 3-month intervals and subsequently at 6-month intervals. Long-term follow-up data were available in 10 patients given hydralazine and eight control patients; the follow-up interval ranged from 3 to 48 months. In the hydralazine group the shortening fraction rose from 14.5 +/- 4.9 to 23.2 +/- 7.5 (P less than 0.01), and the ratio of pre-ejection period to ejection time (0.52 +/- 0.05 to 0.35 +/- 0.06, P less than 0.001) and left ventricular size, normalized to body surface area (116 +/- 7 to 87 +/- 21, P less than 0.01), decreased. Significant improvement was demonstrated by echocardiography after 12 months of hydralazine therapy. There was no significant change in any of these values in the control group. We conclude that hydralazine therapy is a useful adjunct in the management of primary myocardial disease in infancy and childhood.

Administration, Oral↗

[Papillary fibroelastoma: a rare but treatable cause of cerebral infarction].

INTRODUCTION: Papillary fibroelastoma is a benign cardiac tumor which can be associated with serious embolic complications. EXEGESIS: We report on a 42-year-old man admitted for an ischemic stroke in the left middle cerebral artery region. Transesophageal echocardiography revealed a mitral valve tumor. Surgical excision and histological examination showed a papillary fibroelastoma. Clinical course was uneventful. CONCLUSION: We consider the high embolic potential of this tumor, which represents a surgically treatable cause of ischemic stroke.

Adult↗

The histopathology of endocardial sclerosis.

This histological study of endocardial thickening in human hearts revealed that as in adult hearts, the proliferation in fetal, neonatal, and infant hearts consisted of collagen, elastin, and smooth muscle cells. Variation in severity from chamber to chamber and site to site indicated that severity is not an aging phenomenon and that predominantly local blood flow conditions determine localization and progression of proliferation. The similarity to endocardial thickening of cardiac valves and to intimal proliferation in blood vessels was remarkable. In old age and in chronic rheumatic heart disease the proliferation exhibited hyalinization, cell depletion, loss and fragmentation of elastin, lipid accumulation, and thrombosis, indicative of a similar pathogenesis to atherosclerotic changes in valvular endocardium and blood vessels. It was concluded that these chronic hemodynamically induced degenerative changes in the endocardium, including cardiac valves, should be classified as endocardial atherosclerosis analogous to that in arteries and veins and that severity is aggravated by high blood pressure, cardiac malformations, and dysfunction or damage caused by other disease processes.

Adolescent↗

[Endocardial fibrosis in neuroendocrine tumors of the gastroenteropancreatic system].

Clinical characteristics, echocardiographic and Doppler echocardiographic findings, as well as serum levels of chromogranin A were recorded on 62 patients (27 women, 35 men; mean age 55 [11-83] years) with histologically confirmed tumours of the gastroenteropancreatic (GEP) system. Changes in the right heart were found in 14 patients (22%), club-like thickening of tricuspid leaflets in 13, tricuspid regurgitation in 14, stenosis in 2 and right atrial or right ventricular dilatation in 11 and 5, respectively. There was no difference between the patients with or without right-heart changes in regard to age, presence of carcinoid syndrome, duration of symptoms, primary tumour site, pattern of metastases, treatment or chromogranin A level. Two patients with nonfunctioning tumours had right-heart changes. While clinical and biochemical parameters did not identify patients with right-heart changes, echocardiography demonstrated all haemodynamically significant endocardial changes. Even patients with nonfunctioning GEP tumours should be regularly monitored by echocardiography.

Adolescent↗