Echocardiography
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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.
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The right ventricle (RV) can be described in terms of 3 components: the inlet, the apex, and the infundibulum. In the normal adult, the RV shows an arrangement suited for pumping blood against low resistance, with a mass about one sixth that of left ventricle (LV) mass, and a larger volume than the LV. The RV is able to manage a progressive increase in the afterload by increasing contractility and remodeling. ⋯ In case of increased afterload, the RA is enlarged, denoting high RA pressure, as a consequence of elevated RV diastolic pressure. RA area is a strong predictor of adverse clinical outcome in pulmonary arterial hypertension. In patients with severe pulmonary hypertension, in several congenital heart diseases, and in Eisenmenger syndrome, symptoms and prognosis are greatly dependent on RV function and its ability to adapt to a chronic increase in afterload.
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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.