M. A. Slager

AIMS: To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair. METHODS AND RESULTS: This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status was obtained of 86 patients. Median follow-up was 35 (IQR 34-38) years. Cumulative survival was 84% after 10 years, 80% after 20 years, 77% after 30 years, and 68% after 39 years. Cumulative survival free of events (i.e. heart transplantation, arrhythmias, reintervention, and heart failure) was 19% after 39 years. Reinterventions were mainly required for baffle-related problems. Supraventricular and ventricular arrhythmias occurred in 28 and 6% of the patients, respectively. Pacemaker and/or ICD implantation was performed in 39%. Fifty survivors participated in the current in-hospital investigation including electrocardiography, 2D-echocardiography, cardiopulmonary-exercise testing, NT-proBNP measurement, Holter monitoring, and cardiac magnetic resonance. Right ventricular systolic function was impaired in all but one patient at last follow-up, and 14% developed heart failure in the last decade. NT-proBNP levels [median 31.6 (IQR 22.3-53.2) pmol/L] were elevated in 92% of the patients. Early postoperative arrhythmias were a predictor for late arrhythmias [HR 3.8 (95% CI 1.5-9.5)], and development of heart failure [HR 8.1 (95% CI 2.2-30.7)]. Also older age at operation was a predictor for heart failure [HR 1.26 (95% CI 1.0-1.6)]. CONCLUSION: Long-term survival after Mustard repair is clearly diminished and morbidity is substantial. Early postoperative arrhythmias are a predictor for heart failure and late arrhythmias.

CONTEXT: Corticosteroid-binding globulin (CBG) and albumin transport circulating cortisol. Cleavage of high-affinity CBG (haCBG) by neutrophil elastase at inflammatory sites causes cortisol release into tissues, facilitating immunomodulatory effects. OBJECTIVE: To determine whether depletion of haCBG is related to mortality in septic shock. DESIGN: A single-center prospective observational cohort study of patients recruited with critical illness or septic shock, using serum samples collected at 0, 8, 24, 48 and 72 hours. Serum total and haCBG, and total and free cortisol were assayed directly. Glucocorticoid treatment was an exclusion criterion. Mortality was assessed at 28 days from Intensive Care Unit admission. RESULTS: Thirty septic shock (SS) and 42 nonseptic critical illness (CI) patients provided 195 serum samples. SS/CI patients had lower total CBG, haCBG and low-affinity CBG (laCBG) than controls. Total CBG and haCBG were significantly lower in septic shock patients who died than in those that survived (P < 0.009, P = 0.021, respectively). Total and free cortisol were higher in septic than nonseptic individuals. Free/total cortisol fractions were higher in those with low haCBG as observed in septic shock. However, cortisol levels were not associated with mortality. Albumin levels fell in sepsis but were not related to mortality. CONCLUSIONS: Low circulating haCBG concentrations are associated with mortality in septic shock. These results are consistent with an important physiological role for haCBG in cortisol tissue delivery in septic shock.

Purpose: To describe mortality and morbidity in the total cohort of patients who underwent a Mustard operation for transposition of the great arteries (TGA) between 1971-1980 in our center and were followed prospectively every 10 years. Methods: Survival status was obtained from the Dutch National Population Registry. Patients who participated in one or both of the previous studies were invited for thorough in-hospital clinical investigation. Patients who could or would not participate were asked to fulfill a written questionnaire. Events were defined as death or heart transplantation, re-intervention, arrhythmia, heart failure, pacemaker or ICD implantation and endocarditis. Results: Information on survival status was obtained in 86 patients (95%). Mean follow-up was 35±2 years, with a range of 32-39 years. Cumulative survival was 80% after 20 years, 77% after 30 years and 68% after 37 years. Event free survival was 36%, 25% and 19% after 20, 30 and 37 years respectively. In the last 10 years 2 patients died due to ventricular fibrillation and 1 patient underwent heart transplantation, three patients (6%) underwent re-interventions for baffle stenosis, 5 (10%) had their first episode of supraventricular tachycardia, three (6%) needed a pacemaker (1 for sick sinus syndrome and 2 for AV-blok) and 3 (6%) received an ICD (1 for secondary and 2 for primary prevention), 5 patients (10%) were treated for de novo heart failure. Survival after Mustard operation for TGA Conclusion: Long-term outcome of Mustard patients is clearly hampered, with 68% survival and only 19% event-free survival after 37 years.