Transposition of the great arteries
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček in Congenital Heart Disease in Adults, 2008
Transposition of the great arteries refers to a condition where the position of the aorta and the pulmonary artery is reversed. Transposition of the great arteries after Mustard correction; transesophageal echo, transversal view. Long-term follow-up after intra-atrial repair for complete transposition shows that postoperative morphological and hemodynamic abnormalities exist in many patients. The risk of infectious endocarditis is high, especially in patients with valvular dysfunction and if foreign material has been used during the operation. Transposition of the great arteries after Rastelli correction; transthoracic echo, pulsed Doppler flow in the left ventricular outflow tract shows laminar flow without obstruction from the left ventricle through original ventricular septal defeat redirected to aorta. The operation consists of dividing the aorta and pulmonary artery above the valvular level, connecting the aorta to the left ventricle, and the pulmonary artery to the right ventricle.
Ebstein’s anomaly of the tricuspid valve
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček in Congenital Heart Disease in Adults, 2008
Ebstein’s anomaly is a rare congenital heart anomaly of the tricuspid valve and right ventricle. It is characterized by abnormal adherence of the tricuspid valve leaflets to the underlying myocardium, and displacement of the functional tricuspid valve orifice more apically and anteriorly into the right ventricle and towards the right ventricular outflow trace. In adults with Ebstein’s anomaly, echocardiography is difficult to perform because of the heart dislocation and dilatation. Adequate depth of the examination has to be selected. The critical feature for establishing a diagnosis of Ebstein’s anomaly is the apical shift of the tricuspid leaflet coaptation site, which is ideally verified using color Doppler mapping in the presence of tricuspid regurgitation. One-and-half ventricular repair is usually performed in high-risk patients with a severely reduced size of the right ventricle, or a severely dysfunctional and enlarged right ventricle, extended atrialized right ventricle and long-standing atrial fibrillation.
Functionally single ventricle, Fontan procedure – univentricular heart/circulation
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček in Congenital Heart Disease in Adults, 2008
Some patients with a functionally single ventricle may survive without surgery until adulthood, in the presence of favorable hemodynamics and balanced pulmonary artery flow associated with significant pulmonary stenosis. Under certain circumstances, Fontan procedure or some of its modifications can be performed, even in adulthood. This chapter describes the most common complex congenital heart diseases (CHD) with univentricular circulation, which may be surgically managed by Fontan circulation, usually total cavo-pulmonary connection (TCPC). Tricuspid atresia (TA) accounts for some 0.7% of all CHD. In TA, the tricuspid valve is absent, and there is no anatomic connection and direct communication between the right atrium and right ventricle; a fibrotic septum is usually present at the site of the tricuspid valve. Double-outlet right ventricle encompasses virtually the entire spectrum of congenital cardiac anatomy and physiology. Both great arteries arise predominantly (>50%) from the morphologically right ventricle.
Detection of Early Cardiac Dysfunction in Patients with β-Thalassemia Major and Thalassemia Trait by Tissue Doppler Echocardiography
Published in Pediatric Hematology and Oncology, 2011
Yasemin Isik Balci, Dolunay Gurses
Cardiac complications are the leading cause of death in β-thalassemia major (TM) patients. The aim of this study was to investigate the impact of iron overload on ventricular functions using conventional and tissue Doppler imaging (TDI) in patients with TM and compare them with children with thalassemia trait (TT) and healthy controls. This prospective study includes 3 groups: group 1: 29 patients with β-TM; group 2: 28 patients with TT; group 3: 29 healthy controls. Peak late relaxation velocity determined by conventional echocardiography for the right ventricle was significantly higher and the E/A ratio for the right ventricle and left ventricle were significantly lower in TM patients than the other groups (P < .05). Peak late relaxation velocity determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly higher in TM patients than the TT subjects and controls (P < .001). The E/A ratio determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly lower in group 1 than the other 2 groups (P < .001). There was a negative correlation between the ferritin level and E/A ratio for the left ventricle, interventricular septum, and right ventricle using TDI (P < .05). Conventional echocardiographic techniques have failed to distinguish ventricular functions of asymptomatic patients with TM from the subjects with TT and from normal controls when global functions were examined. The present study indicates that TDI should be used for screening of TM and TT subjects’ cardiac functions.
The Interpretation of Intracardiac Phonocardiography of a Continuous Murmur Developed after Radical Operation for Tetralogy of Fallot
Published in Scandinavian Journal of Thoracic and Cardiovascular Surgery, 1968
A patient with tetralogy of Fallot developed a continuous flow-like murmur after radical operation. At right-heart catheterization with intracardiac phonocardio-graphy, the continuous murmur was recorded in the entire inflow tract of the right ventricle and was erroneously interpreted as being due to an arteriovenous fistula to the right ventricle. Thoracic aortography demonstrated aortic insufficiency and a residual ventricular septal defect with left-to-right shunt as the cause of the continuous murmur. The diagnostic value of intracardiac phonocardiography in such cases is based on the assumption that the diastolic component of aortic insufficiency is not recordable in the right ventricle; the reported case indicates that intracardiac phonocardiography may be of limited diagnostic value after radical operation for tetralogy of Fallot.
Double-chamber right ventricle in a 63-year-old woman
Published in Acta Cardiologica, 2000
Eugenio Simarro, Carlos Simarro, Cesar Moris
Double-chamber right ventricle (DCRV) is a rare congenital heart disease consisting in right ventricular obstruction due to one or several anomalous muscle bundles that divide the right ventricle into two chambers. Because of the rarity of this anomaly in adults, we present the case of a 63-year-old woman suffering from this heart disease, being on the other hand, one of the few cases described in the literature in such an old patient.