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Contains fulltext : 89513.pdf (Publisher’s version ) (Closed access) BACKGROUND: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established. METHODS: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries. The primary end point is the change in right ventricular ejection fraction as measured by cardiovascular magnetic resonance or multidetector row cardiac computed tomography in case of pacemaker patients. CONCLUSION: This large prospective, double-blind, randomized, placebo-controlled trial will establish the role of angiotensin II receptor blockers (valsartan) in the treatment of patients with a systemic right ventricle. 01 november 2010

Seven patients with corrected transposition were studied by qualitative radionuclide angiocardiography. In four patients with situs solitus with apex on the left, the pulmonary artery was in a medial and posterior position, to the right of the aorta, with its root attached to the morphological left ventricle positioned on the right. The aorta arose from the ventricle placed on the left and had an anterior position. The ventricle located on the right has a triangular shape. In the case of situs solitus with dextroversion, the pulmonary artery was to the right of the aorta and had a right-to-left direction. The ventricle placed on the right was ovoid. In one single case of situs inversus with levoversion, the pulmonary artery was on the left, emerging from the morphological left ventricle placed on the left, and was ovoid in shape, while the aorta was on the right arising from the morphological right ventricle. In one case of situs inversus with dextrocardia, atrioventricular relationships were the same as in levoversion but the apex was on the right and the morphological left ventricle was triangular. Qualitative radionuclide angiocardiography is a simple and non-invasive method for obtaining morphological data for the precise diagnosis of corrected transposition.

Two cases of corrected transposition of the great vessels have been reported: one with an associated “mitral incompetence” and a complete atrioventricular block, and one with mild pulmonary stenosis and Grade I atrioventricular block. In both cases, proper evaluation of the physical findings, ECG, and roentgenologic appearance enabled us to reach the diagnosis which was confirmed by heart catheterization.

The angiocardiograms of 29 patients with positional anomalies of the heart are presented. Dextroversion of the ventricles was present in 21, mesocardia with solitus atria in 5, and levoversion of the ventricles in 3. A normal relationship of the great vessels was observed in only 10 patients, whereas transposition was observed in 19. When the proximal connections of the great arteries could be established, corrected transposition was more common than complete transposition. Associated defects were present in 27 cases. These defects tended to be milder when the great arteries were normally related than when they were transposed. In all patients the aortic arch was situated on the same side as the morphologic left atrium. The morphology of the ventricular chambers was difficult to recognize, since both tended to be smooth walled in outline. In cases in which two ventricles were present, these chambers lay side by side. The significance of assessing the atrial situs as the basis for the recognition of positional anomalies of the heart has been stressed.

Six autopsied cases of corrected transposition of the great arteries with VSD were examined from the surgical exposure. Two anatomic types concerning the relative height of the two semilunar valves and the position of the VSD were recognized. In four cases (Type I), the two semilunar valves are at the same height viewed from the anteroposterior direction, the parietal band is hypoplastic, and the VSD is located immediately below the aortic and pulmonary valves. In the other two cases (Type II), the aortic valvular level is higher than the pulmonary valvular level and the parietal band in these cases is well-developed. The VSD located beneath the pulmonary valve is clearly separated from the aortic valve by a well-developed parietal band. The first type has an excellent exposure viewed from the left-sided ventricle, while the second has an extremely poor exposure. Therefore, right-sided ventriculotomy is recommended in the latter type. Furthermore, these two anatomic types can be recognized and differentiated in angiocardiographic findings by the relative height of the two semilunar valves and the presence of a filling defect formed by the crista supraventricularis.

1. 1. A case of situs inversus with levocardia associated with duodenal atresia is presented. 2. 2. A review of the literature indicates that seventeen similar cases have been reported. 3. 3. All such cases have had bizarre anomalies of the heart and great vessels, usually either a transposition of the great vessels or a corrected transposition with reversal of the atria.

Autopsy specimens are studied of two cases in which there was a variety of corrected transposition of the great vessels associated with dextrocardia and auricular and ventricular septal defects. The embryologic basis of this entity is analyzed and the possibility of diagnosing the altered reciprocal relationship of the heart chambers is discussed on the basis of the electrocardiographic and radiologic findings. Comments on the surgical treatment of one of the cases are made.

days after an acute myocardial infarction, the LC and R arose from the RM and the RM was severely narrowed by an atherosclerotic plaque (Fig. 1). In the second patient, a 60-year-old man who died shortly after aortocoronary bypass grafting for severe angina pectoris, the LAD and R arose from the RM which also was severely narrowed by atherosclerotic plaque (Fig. 2), as were all major coronary arteries. Origin of the LC from the R coronary artery is the most frequent major congenital anomaly of the coronary arteries. Origin of the LAD from the RM, however, is extremely rare. Reports of five necropsy patients with this type anomaly appear only in a thesis’ and, to our knowledge, in no published reports. Severe narrowing by atherosclerotic plaque of the RM coronary artery-the true equivalent of severe narrowing of the LM coronary artery-has not been reported previously, and indeed the concept of a RM coronary artery, except possibly in the congenital condition, corrected transposition of the great arteries (ltransposition), has not been presented previously.

1. 1. The authors have reviewed the normal development of the bulboventricular loop, the ventricles, and the great vessels in the human embryo. 2. 2. Corrected transposition of the great vessels in any of its varieties is a complex malformation in which two embryonic elements are involved: the bulboventricular loop and the truncoconal septum. 3. 3. An embryologic explanation for the different varieties of corrected transposition of the great vessels is proposed. The anatomic features of all these varieties (in normally situated heart, in mirror-image dextrocardia, in one type of levocardia, and in the case of dextrorotation) are also given. 4. 4. A new theory based on recent embryologic findings is offered to explain the development of corrected transpositions of the great vessels.

The various clinical, electrocardiographic, radiologic, angiocardiographic, and cardiac catheterization features in a case of ◀functionally totally corrected transposition” without any associated defects are presented. To our knowledge, this is the first case in which detailed studies are available and in which a diagnosis has been feasible ante mortem.

This is a brief review of the clinical and pathologic features of corrected transposition of the great vessels of the heart. In this condition, the aorta and pulmonary artery are transposed, with the aorta lying anterior to the pulmonary artery; however, the aorta arises from the left-sided ventricle and carries oxygenated blood, while the pulmonary artery arises from the right-sided ventricle and carries venous blood. There is usually, also, localized inversion of the ventricles and the coronary arteries. Clinical manifestations result from the frequently associated cardiac anomalies, especially interventricular septal defect. Three case reports of corrected transposition are presented. These were studied both clinically and at autopsy.

This report describes a patient with corrected transposition of the great vessels and with an aneurysm of the membranous portion of the ventricular septum, which was surgically repaired. The aneurysm caused obstruction of the outflow tract of the venous ventricle, a hemodynamic complication that is essentially inevitable when an aneurysm of significant size develops in the presence of ventricular inversion. Subvalvular pulmonic stenosis was also present.