Neonatal expression of Ebstein's anomaly

Freedom, Robert M.; Benson, Leland N.

doi:10.1016/1058-9813(93)90044-z

Cited by 7 publications

(3 citation statements)

References 28 publications

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“…Some patients without significant RV outflow obstruction spontaneously improve as the pulmonary vascular resistance (PVR) drops in the early neonatal period. 33 The use of pulmonary vasodilators such as oxygen and inhaled nitric oxide can help the drop in PVR and can also assist in determining if there is patency of the pulmonary valve. 34,35 Sildenafil has been used in a similar manner to decrease PVR and promote antegrade pulmonary blood flow.…”

Section: Managementmentioning

confidence: 99%

Preoperative Physiology, Imaging, and Management of Ebstein’s Anomaly of the Tricuspid Valve

Morray

2015

Semin Cardiothorac Vasc Anesth

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Ebstein's anomaly of the tricuspid valve (TV) refers to an embryological derangement of TV formation causing tethering of the septal and posterior leaflets of the valve to the underlying myocardium and apical displacement of the effective valve annulus, resulting in significant TV insufficiency and dilation of the right heart structures. The pathological abnormalities of the valve can vary significantly, resulting in a wide range of clinical presentations. Fetal diagnosis and neonatal presentations of the disease are typically the most severe and are associated with the highest mortality rates. Patients with less-severe disease will present later in life with symptoms of right heart failure and tachyarrhythmias. Medical and surgical management strategies are driven by the age at presentation, severity of disease, and any associated cardiac abnormalities. There are an increasing number of surgical options focused on valve repair.

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Section: Managementmentioning

confidence: 99%

Preoperative Physiology, Imaging, and Management of Ebstein’s Anomaly of the Tricuspid Valve

Morray

2015

Semin Cardiothorac Vasc Anesth

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“…Its leading edge, nonetheless, may be inserted in linear fashion in such a way as to obstruct the normal outflow beneath its leading edge to the right ventricular outflow tract. 32,37 Thus, while the most recognized functional correlate of the tricuspid valve deformed by Ebstein's malformation is that of regurgitation, the abnormal valves can also exhibit some degree of functional stenosis. Indeed, in rare circumstances, the valve deformed by Ebstein's malformation can be imperforate, producing a variant of tricuspid atresia.…”

Section: Segmental and Sequential Analysismentioning

confidence: 99%

“…Indeed, in rare circumstances, the valve deformed by Ebstein's malformation can be imperforate, producing a variant of tricuspid atresia. 32,[37][38][39][40] The hearts seen with tricuspid stenosis or atresia, however, do not typically show exorbitant cardiac enlargement, and hence do not fall within the category of "wall-to-wall" heart.…”

Section: Segmental and Sequential Analysismentioning

confidence: 99%

The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum

et al. 2006

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D ISCUSSION AND DEBATE CONTINUES AS TO THE congenital cardiac malformation currently warranting the dubious distinction as representing the worst disease. At one time, Roberts et al. 1 considered hearts with aortic atresia as constituting the worst disease, at least in terms of survival. The emergence of strategies for functionally univentricular palliation, along with transplantation, for these patients, with ever improving outcomes, has markedly altered this perception. 2 In a recent review, 3 we discussed the continuing disappointing outcomes for patients with isomerism of the right atrial appendages, certainly justifying the nomination of this lesion as one of the current worst forms of disease. Few would deny, however, that the subset of patients with pulmonary atresia and intact ventricular septum with florid tricuspid regurgitation, the complication producing the so-called "wall-to-wall" heart (Figs 1 and 2), also have a very grim prognosis. 4-6 The outcomes for the patients making up this subset, however, have received scant attention in comparison to that devoted to the more commonly encountered patients with hypoplastic ventricular cavities, severe right ventricular hypertension, and ventriculo-coronary arterial connections, another fascinating combination that received our recent attention. 7 It is our intention to correct the neglect for those with dilated right ventricles in the present review. Historical Comment As indicated above, we recently reviewed the findings in those patients with pulmonary atresia and intact ventricular septum complicated by ventriculocoronary arterial connections, emphasizing their Continuing Medical Education The "wall-to-wall" heart in the patient with pulmonary atresia and intact ventricular septum

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References

2003

The Natural and Modifed History of Congenital Heart Disease

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Neonatal expression of Ebstein's anomaly

Cited by 7 publications

References 28 publications

Preoperative Physiology, Imaging, and Management of Ebstein’s Anomaly of the Tricuspid Valve

Preoperative Physiology, Imaging, and Management of Ebstein’s Anomaly of the Tricuspid Valve

The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum

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