Search

Between 1 January 1975 and 31 December 1982, 111 patients with atrioventricular septal defect underwent surgical repair. Of these, 43 had the complete, 11 the intermediate, and 57 the partial form. The postoperative mortality rate was 37%, 9%, and 6% respectively. To determine which factors were independently responsible for the operative risk multivariate analysis of the surgical mortality was applied simultaneously to all three forms of the malformation. The form of defect, although strongly influencing the natural history and clinical presentation, was not by itself an operative risk factor. The risk was related primarily to failure to obtain a well functioning atrioventricular valve, to the presence of left ventricular dominance, to the degree of pulmonary vascular resistance, and, finally, to the technique of reconstructing a two leaflet left atrioventricular valve. The small size of the patient was also a significant incremental risk factor, but if the other factors were not unfavorable good results could be achieved in small infants with mortality rates less than 10%. Multivariate analysis showed that severe postoperative left atrioventricular valve malfunction was related to the technique used to reconstruct a "normal" two leaflet left atrioventricular valve. These findings support the policy of reconstructing the left atrioventricular valve as a three leaflet valve. Nevertheless, the implicit beneficial effect of this technique has not as yet proved to be statistically significant. [ABSTRACT FROM PUBLISHER]

AimsTo assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome.MethodsThe clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33·02±12·8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death.ResultsEighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18·6±11·3 vs 26·7±12·2) and shorter survival (25·8±7·9 vs 32·5±14·6 years). Thirty-eight (20·2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13·2%) had pulmonary thromboembolism at a mean age of 35·2±13·4 years. Fifteen (7·9%) had a stroke and 7 (3·7%) a cerebral abscess at a mean age of 31·4±15·7 and 24·1±4·9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2·04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23·5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35·8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29·5%), heart failure (22·9%) and from haemoptysis (11·4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis.Conclusionspatients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks [ABSTRACT FROM PUBLISHER]

Objectives: Surgical repair of complete A-V canal defects (CAVCD) is a well established procedure which is currently performed in infancy. The aim of this study is to evaluate surgical results of correction in early infancy in comparison to older age. Methods: From January 1985 to March 2001, 119 consecutive patients (age range 27 days to 83 months, mean 6.7 months) underwent repair of CAVCD in our Institution. Forms with unbalanced ventricles in association with Fallot''s tetralogy or heterotaxia were excluded from this series. Fifty-eight patients (49%) underwent correction before 3 months of age (Group A), and 61 patients (51%) after 3 months (Group B). Surgical repair was accomplished with a double patch technique in 100 patients (84%). Associated surgical lesions were treated simultaneously in 48 patients (40%). Results: There were 11 operative deaths (<30 days) (two in Group A (3.4%) and nine in Group B (15%)) (P=0.05). The remaining patients were discharged home in good haemodynamic condition. Reoperation for postoperative left A-V incompetence occurred in five patients in Group A and in eight patients in Group B. There were eight late deaths (three in Group A and five in Group B), of which four were non-cardiac related. At a mean follow-up time of 80 months (range 2–184 months) 100 patients are asymptomatic and well, and free from oral medication. Echocardiographic examination showed absent or mild residual left A-V valve incompetence in 91 patients (49 in Group A and 42 in Group B) and moderate left A-V valve incompetence in nine patients (four in Group A and five in Group B). Kaplan–Meier survival estimates at 10 years were 90% for Group A and 75% for Group B. Kaplan–Meier freedom from reoperation at 10 years was 89% for Group A and 84% for Group B. Conclusions: Our data demonstrate that repair of CAVCD under 3 months of age is the ideal approach to this malformation with a lower mortality rate at operation compared to older patients. Logistic analysis showed that an operative age >3 months is, compared to an age ≤3 months, an incremental risk factor for hospital mortality with an odds ratio of 4.8 (95% confidence limit 1–23.5) (P=0.05). In the long term, freedom from reoperation for left A-V valve incompetence is higher when compared to children repaired at an older age. [Copyright &y& Elsevier]

We studied 38 patients (mean age 32 +/- 14 years) with arrhythmogenic right ventricular cardiomyopathy (ARVC) to evaluate the clinical significance of histologic features on endomyocardial biopsy specimens as related to signal-averaged electrocardiography (SAECG), spontaneous ventricular arrhythmias, and hemodynamic features. Fifteen patients presented with ventricular tachycardia or fibrillation (sustained ventricular arrhythmias), 23 with other minor arrhythmias. SAECG variables and right ventricular ejection fraction (RVEF) were statistically correlated with the extent of myocardial fibrosis on biopsy in ARVC. An increased percentage of fibrous tissue (> or = 30%) was a significant univariate predictor of late potentials (p = 0.004) and reduced RVEF (p = 0.02). The 18 patients with late potentials had an increased percentage of fibrous tissue (p = 0.01), a reduced RVEF (p = 0.0004), and a higher risk for sustained ventricular arrhythmias (p = 0.05) than the 20 patients without late potentials. RVEF was the most powerful predictor of late potentials (p = 0.004) at multivariate analysis. Moreover, RVEF < or = 50% was associated with an increased risk for development of sustained ventricular arrhythmias (p = 0.02). A SAECG parameter, namely the root-mean-square voltage of the terminal 40 ms at 25 Hz, was an independent predictive factor for the occurrence of sustained ventricular arrhythmias (p = 0.02). Although fibrous tissue may contribute to delayed myocardial activation in ARVC, a reduced RVEF plays an essential role for spontaneous manifestation of sustained ventricular arrhythmias. [ABSTRACT FROM AUTHOR]