Congenital heart disease
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Congenital heart disease · Jan 2012
ReviewCan "inoperable" congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality?
The decision whether to repair congenital heart defects in patients with raised pulmonary vascular resistance to alleviate pulmonary hypertension is a complex one. The degree of pulmonary vascular disease is of paramount importance. Operating on patients with pulmonary vascular resistance above a certain threshold runs the risk of postoperative persistent pulmonary hypertension and a worse long-term prognosis. This review focuses on patients deemed "borderline inoperable" or "inoperable" due to pulmonary vascular disease and asks whether they can be "converted to an operable status" with pulmonary arterial hypertension-specific drugs that potentially modify the pulmonary vascular lesions and resistance.
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Congenital heart disease · Jan 2012
Comparative StudyComparison of longitudinal myocardial deformation and dyssynchrony in children with left and right ventricular morphology after the Fontan operation using two-dimensional speckle tracking.
Two-dimensional speckle tracking can assess ventricular deformation independent of ventricular geometry. Using this technique, the aim of our study was therefore to compare global and regional longitudinal deformation and intraventricular dyssynchrony between children with left and right ventricular morphology after Fontan operation. ⋯ Despite minor regional differences in longitudinal deformation and dyssynchrony, overall ventricular longitudinal deformation was not different between morphologic right and left ventricles. These findings may reflect similar adaptation of longitudinal function of both ventricular morphologies to the single-ventricle circulation in our cohort, albeit relatively early after Fontan surgery.
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Congenital heart disease · Jan 2012
Case ReportsScimitar syndrome with right hemianomalous pulmonary venous drainage into superior vena cava/right atrium junction.
Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.