Echocardiography
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Ebstein's anomaly is a complex congenital lesion which primarily involves the tricuspid valve. The tricuspid leaflets are tethered to varying degrees to the right ventricular free wall and the ventricular septum often resulting in significant tricuspid regurgitation and a small functioning right ventricular chamber. ⋯ Although two-dimensional echocardiography represents the primary modality for the diagnosis of this anomaly, three-dimensional echocardiography provides incremental value in characterizing the extent and severity of tethering of individual tricuspid valve leaflets. This information is useful in surgical decision making whether to repair or replace the tricuspid valve.
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The overall risk of cancer is higher in people exposed to computed tomography (CT) scans in childhood or adolescence compared to adults. Transthoracic contrast echocardiography (TTCE) has recently been used to screen for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT), but the value of TTCE to rule out PAVMs and avoid chest CT radiation has yet to be discussed. ⋯ A low-grade classification (Barzilai 0 or 1) could presumably exclude the presence of PAVMs and allow CT irradiation to be avoided in children and adolescents. The screening algorithm using TTCE first would allow more than 40% of the pediatric patients screened for PAVMs to be spared the radiation dose of CT.
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In addition to infundibular muscular hypertrophy and anomalous muscle bundles in the right ventricular body, large membranous septal aneurysms (MSA), sinus of valsalva aneurysms, and aneurysms derived from the embryonic venous valve protruding through the tricuspid valve can cause right ventricular outflow obstruction in a patient with ventricular septal defect. Here we describe an adult with a small perimembranous ventricular septal defect complicated by a large MSA causing severe right ventricular outflow obstruction evaluated with real time three dimensional transesophageal echocardiography and cardiac catheterisation. The patient underwent surgical correction of the abnormality and is asymptomatic on follow up.