1/33. dna polymorphism analysis in transfusion-associated graft-versus-host disease.During cardiac surgery for transposition of the great arteries at age 7 weeks, a female infant received blood, fresh frozen plasma and platelet transfusions. Eleven days postoperatively, she developed bloody diarrhoea, fever, an erythematous macular rash, hepatomegaly, seizures and pancytopaenia. A clinical diagnosis of transfusion related graft-versus-host disease (GVHD) was supported by skin histopathology. dna polymorphism studies confirmed that circulating lymphocytes in peripheral blood and infiltrating cells in the skin were foreign in origin and were derived from transfused blood cells. No underlying immunodeficiency was identified. Treatment with steroids cyclosporin and antithymocyte globulin was unsuccessful and death occurred 2 months after surgery. The features of fever, rash, diarrhoea, liver dysfunction and pancytopaenia which characterize GVHD may mimic drug reactions or viral infection. In addition to histological features on skin biopsy. dna polymorphism studies on skin and blood samples provide a unique and sensitive method to confirm GVHD. Irradiation of blood products should be considered for acutely compromised infants requiring urgent cardiac surgery.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/33. Arterial switch operation after left ventricular retraining in the adult.Retraining the morphological left ventricle in transposition of the great arteries has been successfully reported in infancy, while older age seems to be a contraindication. A 23-year-old woman with inverted question markS,D,D inverted question mark transposition of the great arteries and ventricular septal defect developed severe right systemic ventricular dysfunction 22 years after Mustard procedure and ventricular septal defect closure. Hemodynamic investigation revealed moderate pulmonary hypertension and preserved left ventricular function. A pulmonary artery band was applied to obtain a left-right ventricular pressure ratio of 0.91. Her postoperative course was characterized by biventricular failure, treated effectively with inotropic support. Six months later, she underwent a Mustard baffle takedown and arterial switch procedure. Her postoperative course was uneventful. She was discharged home on postoperative day 15. At 24-months follow-up, she is in excellent clinical condition; echocardiographic evaluation shows good left ventricular function (ejection fraction: 0.69) with left ventricular volume within normal limits (70 ml/m2). Our experience demonstrates that, despite adult age, a staged arterial switch operation can be performed successfully in selected patients when left ventricular function is preserved.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
3/33. Combined atrial and arterial switch operations for congenitally corrected transposition.Conventional repair of congenitally corrected transposition of the great arteries (CCTGA) is directed at eliminating the associated defects and leaves the right ventricle in a systemic position. The long-term outcome of this procedure may involve deterioration of right ventricular function with tricuspid regurgitation and failure of the conduction system. We describe two consecutive patients with CCTGA, one of whom had apicocaval juxtaposition. The patients were aged 19 and 16 months, respectively, and both underwent a combination of atrial and arterial switch. These are the first two reported cases of successful completion of this type of operation in taiwan. Our review of previously reported cases suggested that no significant difference exists in the outcome of patients with this condition who undergo either arterial switch or Rastelli-type repair plus atrial redirection. However, reported patients who underwent anatomic repair had lower early mortality, late mortality, and incidence of complete heart block than those who underwent conventional repair. The present two cases and our review of the literature suggest that, among patients with apicocaval juxtaposition, 1) Mustard operation is optimal for patients with small atrial volume; 2) one-and-one-half ventricular repair may be helpful to the outcome, especially when treatment is combined with Rastelli-type repair; and 3) excellent access to the ventricular septal defect through the tricuspid valve is afforded via a left atriotomy. From the present two cases and our review of the literature, we conclude that anatomic repair is superior to conventional repair of CCTGA in terms of protection against dysfunction and failure of the anatomic right ventricle, tricuspid valve, and conduction system. Long-term follow-up is mandatory.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
4/33. Corrected transposition of the great arteries diagnosed in an 84-year-old woman.Corrected transposition of the great arteries without associated cardiac anomalies is a rare cardiac malformation. Few patients with this anomaly survive beyond 50 years of age because of systemic ventricular dysfunction or development of AV valvular regurgitation or conduction disturbance. We describe an autopsied, uncomplicated corrected transposition of the great arteries case in which the patient died at 84 years of age. We believe this patient to be the longest surviving corrected transposition of the great arteries associated person in the world.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
5/33. Systemic right ventricular failure after atrial switch operation: midterm results of conversion into an arterial switch.BACKGROUND: Failure of the systemic right ventricle after atrial switch operation can be treated by conversion into an arterial switch operation. methods: Four patients, age 38 to 59 months, presented with right ventricular failure after Senning operation and ventricular septal defect closure. One patient had elevated left ventricular pressure; in the other three patients the left ventricle was retrained to a left ventricular/right ventricular pressure ratio of 0.8 or greater by pulmonary artery banding in 12 to 24 months. RESULTS: Postoperative course after arterial switch operation was prolonged, but clinical condition was good at discharge. Fractional shortening ranged from 20% to 28%. Trace-to-moderate aortic regurgitation was present; only 1 patient had preserved sinus rhythm. After a mean follow-up of 43.5 months 1 patient had died due to left ventricular dysfunction. The survivors are in new york Heart association functional class I to II. Fractional shortening has improved (29% to 37%); aortic regurgitation has not increased. No patient has undisturbed sinus rhythm. CONCLUSIONS: Conversion of an atrial into an arterial switch is an alternative to cardiac transplantation in childhood. However, the procedure is demanding. Long-term morbidity is caused by rhythm disturbances. aortic valve performance and left ventricular function require close observation.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
6/33. Corrected transposition of the great arteries with several associated anomalies in a 68-year-old patient.Few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. We report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. The patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. Despite all these associated anomalies, the patient developed class III cardiac decompensation only at the age of 68 years, which makes this case a rarity. The patient was clinically treated, and was discharged from the hospital in good condition.- - - - - - - - - - ranking = 615.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
7/33. Congenitally corrected transposition of the great arteries in a 65-year-old woman.A 65-year-old Japanese woman was admitted to hospital because of exertional dyspnea. Transthoracic echocardiography showed diffuse hypokinesis of the left-sided ventricular wall, but was not clear enough to provide useful information because of the rotation of the cardiac apex and the presence of lung tissue. Systemic ventriculography showed that the left-sided ventricle with heavy trabeculations was morphologically similar to a normal right ventricle. Magnetic resonance imaging (MRI) clearly revealed corrected transposition of the great arteries. Because this patient had no severe associated cardiac anomalies, systemic ventricular dysfunction is thought to be the major cause of exertional dyspnea. MRI is a useful non-invasive method for the rapid evaluation of cardiac morphology.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
8/33. Acute myocardial infarction late after Mustard procedure for dextrotransposition of the great arteries.Systemic right ventricular dysfunction has been closely linked to late mortality and sudden cardiac death in patients with Mustard procedure for dextrotransposition of the great arteries. Two young patients with dextrotransposition of the great arteries late after Mustard procedure who presented with acute transmural myocardial infarction and sudden cardiac death (one patient) without prior exertional angina or causative coronary abnormalities are reported. It is surmised that acute coronary emboli originating from a severely dilated, hypocontractile systemic ventricle were the cause of transmural myocardial infarction. This phenomenon may be an important and as yet unrecognized factor in late morbidity and mortality in such patients.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
9/33. Left ventricular assist device for right side assistance in patients with transposition.Right (systemic) ventricular dysfunction is well described after Senning operations for transposition of the great arteries, and patients with congenitally corrected transposition of the great arteries. transplantation remains the only definitive therapy for refractory heart failure, however patients may deteriorate clinically prior to the availability of a donor heart. This report details the implantation of a TCI Heartmate (Thoratec Corp., Pleaston, CA) as a morphologic right ventricular assist device to bridge these patients to transplantation.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
10/33. Arterial switch operation after multiple-stage left ventricular retraining.Right ventricular dysfunction and tricuspid valve incompetence after atrial repair for transposition of the great arteries is an increasingly frequent problem. Left ventricular retraining to convert an atrial switch to an arterial switch is a well-known surgical option but can require a multiple-stage surgical approach. We report our successful multiple-stage experience with a 5-year-old girl.- - - - - - - - - - ranking = 614.68720468128keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
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