1/9. Congestive heart failure in unoperated tetralogy of fallot: can hypoxia be a cause?A 10-year-old boy with tetralogy of fallot and congestive heart failure underwent a right-sided modified Blalock-Taussig anastomosis because of severe biventricular dysfunction and repeated hypercyanotic spells. Postoperatively, there was improvement in systemic oxygen saturation and myocardial function. We postulate that congestive heart failure occurred because of severe myocardial hypoxia and its elimination resulted in markedly improved cardiac performance.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
2/9. Aortopulmonary window coexisting with tetralogy of fallot: echocardiographic diagnosis.Aortopulmonary window coexisting with tetralogy of fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
3/9. Postoperative cardiopulmonary failure in tetralogy of fallot with absent pulmonary valve syndrome treated by extracorporeal membrane oxygenation.When tetralogy of fallot with absent pulmonary valve syndrome leads to respiratory failure in early infancy, total repair has been advocated. respiratory insufficiency may persist despite a surgically adequate repair. An infant with this syndrome underwent total surgical correction. extracorporeal membrane oxygenation was employed in the early postoperative period for intractable pulmonary insufficiency and myocardial dysfunction.- - - - - - - - - - ranking = 0.0035624699997817keywords = dysfunction (Clic here for more details about this article) |
4/9. Second operations for pulmonary stenosis or insufficiency after repair of tetralogy of fallot.Twenty-four patients with previous surgery for repair of tetralogy of fallot were in need of subsequent operative procedures for either residual pulmonary stenosis or pulmonary valve incompetence. The second operation was performed 1 to 21 years after the initial repair. The patients with pulmonary valve incompetence had evidence of marked right ventricular dysfunction and required pulmonary valve replacement. The patients with residual pulmonary stenosis had modifications of their pulmonary outflow tract to reduce the gradient. Five of the 15 patients with stenosis required pulmonary valve replacement. Clinical improvement has been good, but objective reversal of right ventricular dysfunction has been difficult to document.- - - - - - - - - - ranking = 2keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
5/9. Successful heart transplantation with acquired pulmonary artery atresia.Orthotopic cardiac transplantation was performed successfully in a patient with acquired atresia of the left pulmonary artery 19 years after repair of tetralogy of Fallot. Only the right lung could be incorporated into the cardiopulmonary circulation at transplantation, resulting in transient right ventricular dysfunction, which resolved with vasodilator therapy. perfusion of a single pulmonary vascular bed does not preclude successful heart transplantation, provided there is a low pulmonary vascular resistance and pulmonary artery architecture free of stenoses.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
6/9. The use of the right atrial free wall for reconstruction of the outflow tract in a patient with Fallot's tetralogy: a case report.We report the use of the lateral wall of the right atrium, as a free graft, for the reconstruction of the narrow outflow tract in a patient with Fallot's tetralogy. pericardium was used for closure of the ventricular septal defect, widening of the narrow pulmonary trunk and reconstruction of the right atrium. Postoperative echocardiographic studies showed cyclic shortening of the transplanted atrial tissue, a 40% increase in right ventricular ejection fraction and preserved (e) and (a) waves denoting normal blood flow pattern across the tricuspid value. Electrophysiological study carried out 6 months postoperatively recorded ventricular potential at the site of patch. The application of this technique may be considered, ona limited series of patients, for possible amelioration of the postoperative right ventricular dysfunction, whenever widening of its infundibulum is indicated.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
7/9. Severe hypoxic biventricular dysfunction in tetralogy of fallot: is Blalock shunt the answer?In a 4-year-old boy with tetralogy of fallot, a right modified Blalock-Taussig anastomosis was created because of severe myocardial dysfunction and repeated hypercyanotic spells. Post-operatively, systemic oxygen saturation improved and also myocardial function. It is postulated that hypoxia was responsible for myocardial dysfunction previously, and its elimination has resulted in markedly improved cardiac performance.- - - - - - - - - - ranking = 4.0071249399996keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
8/9. Phenotypic discordance in monozygotic twins with 22q11.2 deletion.We report on male monozygotic twins with 22q11.2 deletion and discordant phenotypes. The twins had twin-to-twin transfusion syndrome. Twin 1, the smaller of the pair, had tetralogy of fallot, a characteristic facial appearance, swallowing dysfunction, anal atresia, short stature, and mental retardation, whereas twin 2 had a characteristic facial appearance but no other signs of the 22q11 deletion syndrome. fluorescence in situ hybridization analysis showed a microdeletion on chromosome 22q11.2 in both twins. Zygosity analysis gave a probability of monozygosity greater than 99.999%. These observations indicate that environmental factors or postzygotic events play a role in the phenotypic variability in the twins.- - - - - - - - - - ranking = 0.0035624699997817keywords = dysfunction (Clic here for more details about this article) |
9/9. Right ventricular dysfunction after tetralogy repair in a pediatric patient with successful ECMO support--a case report.Right ventricular dysfunction after surgical repair of tetralogy is a serious life threatening complication. Although pulmonary vasodilators and inotropes can be used for cardiac support in tetralogy repair, these drugs may be ineffective in some patients. To date extracorporeal membrane oxygenation (ECMO) support may be used effectively in these patients when medical therapy fails. We report a case with successful ECMO support in right ventricular dysfunction and pulmonary hypertension after total surgical correction of Fallot's tetralogy. ECMO circuit was instituted in the operating room due to difficulty in weaning from cardiopulmonary bypass in spite of aggressive medical therapy. We reviewed and discussed the indications, contraindications of application of ECMO and patient survival with treatment of ECMO. At the same time we wish to share our limited experience we gained in our first attempt of ECMO support.- - - - - - - - - - ranking = 6keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |