Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark.

BACKGROUND: The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. METHODS AND RESULTS: All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients' unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05-3.46, P=0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed. CONCLUSIONS: The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality.

Cause-Specific Mortality in Patients During Long-Term Follow-Up After Atrial Switch for Transposition of the Great Arteries.

Background Little is known about the cause of death (CoD) in patients with transposition of the great arteries palliated with a Mustard or Senning procedure. The aim was to describe the CoD for patients with the Mustard and Senning procedure during short- (<10 years), mid- (10-20 years), and long-term (>20 years) follow-up after the operation. Methods and Results This is a retrospective, descriptive multicenter cohort study including all Nordic patients (Denmark, Finland, Norway, and Sweden) who underwent a Mustard or Senning procedure between 1967 and 2003. Patients who died within 30 days after the index operation were excluded. Among 968 patients with Mustard/Senning palliated transposition of the great arteries, 814 patients were eligible for the study, with a mean follow-up of 33.6 years. The estimated risk of all-cause mortality reached 36.0% after 43 years of follow-up, and the risk of death was highest among male patients as compared with female patients (P=0.004). The most common CoD was sudden cardiac death (SCD), followed by heart failure/heart transplantation accounting for 29% and 27%, respectively. During short-, mid-, and long-term follow-up, there was a change in CoD with SCD accounting for 23.7%, 46.6%, and 19.0% (P=0.002) and heart failure/heart transplantation 18.6%, 22.4%, and 46.6% (P=0.0005), respectively. Conclusions Among patients corrected with Mustard or Senning transposition of the great arteries, the most common CoD is SCD followed by heart failure/heart transplantation. The CoD changes as the patients age, with SCD as the most common cause in adolescence and heart failure as the dominant cause in adulthood. Furthermore, the risk of all-cause mortality, SCD, and death attributable to heart failure or heart transplantation was increased in men >10 years after the Mustard/Senning operation.

Improved early results after heart valve surgery over the last decade.

OBJECTIVES: This study was undertaken to investigate time trends in early mortality, morbidity and clinical characteristics of patients undergoing heart valve surgery over the last decade. METHODS: A regional, prospectively collected, study comprising all patients (2,327) undergoing valve surgery in a defined geographical area from January 1990 to December 1999 was conducted. Data were collected from 1,746 patients submitted to aortic valve replacement (AVR), 432 to mitral valve replacement (MVR), 78 to double valve replacement (DVR) and 71 to mitral valve repairs. Logistic regression was used to identify risk factors for early mortality. Time trends of early mortality, morbidity and clinical characteristics were analysed. RESULTS: The total early mortality rate was 5.9%; for AVR it was 4.8%, MVR 9%, DVR 14% and mitral valve repair 1.4%. The risk factor profiles for early mortality were similar in all groups of valve interventions, with shock, age over 70 years and advanced NYHA class as the strongest risk factors. There was a decrease in early mortality over the period which remained after correction for risk factors. The proportion of patients over 70 years of age and of patients with diabetes increased, whereas other risk factors were not altered during the study period. CONCLUSION: It is confirmed that early risks for death after heart valve surgery have decreased. This improvement was consistent after adjustment for risk factors.

Severe mitral regurgitation-relations between magnetic resonance imaging, echocardiography and natriuretic peptides.

BACKGROUND: Assessment of the severity of mitral regurgitation by echocardiography can be technically demanding in certain patients and supplementary methods are therefore desirable. This study addressed the agreement between magnetic resonance imaging (MRI) and echocardiography, and their relations to natriuretic peptides (NT-proANP and NT-proBNP), in quantifying severe mitral regurgitation. METHODS: Eighteen patients with severe mitral regurgitation scheduled for surgery underwent MRI, echocardiography and assay of natriuretic peptides preoperatively for clinical assessment. RESULTS: MRI and echocardiography were comparable in measuring severity of regurgitation qualitatively but not quantitatively, mitral regurgitant fraction (mean difference 27.5 (11) ml). There was a correlation between increasing regurgitant fraction on MRI and increased levels of plasma NT-proANP and NT-proBNP. In echocardiography, increasing vena contracta width and increasing PISA correlated to increased levels of plasma NT-proANP and NT-proBNP. No other correlation was found between measures on MRI and echocardiography and natriuretic peptides. CONCLUSIONS: MRI and echocardiography were comparable grading the severity of mitral regurgitation with qualitative measures but not with quantitative measures. MRI might be a complement to echocardiography when a more distinct measure of the regurgitant volume is needed, as in paravalvular leakage.

Survival after mitral valve replacement: rationale for surgery before occurrence of severe symptoms.

BACKGROUND: This study was undertaken to evaluate survival after mitral valve replacement, with a focus on the impact of age and preoperative symptoms. METHODS: Survival was analyzed in 784 patients undergoing mitral valve replacement from 1980 through 2000. Relative survival was estimated by relating the observed survival to the expected survival in a cohort, comparable regarding sex, age group, and calendar period, in the general Swedish population. RESULTS: Early mortality was 9.7%. Relative survival rates after 5, 10, and 15 years were 83%, 70%, and 54%, respectively. The corresponding rates for observed survival were 75%, 56%, and 36%. Advanced New York Heart Association class, but not high age, was a risk factor for early mortality, whereas both factors decreased survival. An association between age and New York Heart Association class was found, the majority of old patients also being severely symptomatic. Patients in New York Heart Association classes I and II showed excellent relative survival. CONCLUSIONS: Survival is reduced after mitral valve replacement in patients with severe preoperative symptoms, whereas patients with less-severe symptoms show excellent survival. Older patients were more often severely symptomatic at the time of surgery. Irrespective of age, surgery before the occurrence of advanced symptoms should improve the long-term outcome.