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OBJECTIVE:

To examine the risk of thoracic aortic disease (TAD) when one or more first-degree relatives are affected, and to relate the risk of family history to the risk of other cardiopulmonary comorbidity.

DESIGN:

Population-based, matched, case-control study.

SETTING:

Registry-based investigation. PATIENTS: All cases, nationwide, of TAD diagnosed 2001-2005 in individuals born 1932 or later (n=2436) were identified, and a random control-group (n=12 152) matched for age, sex and geography was generated. First-degree relatives were identified in the Multigeneration Registry. Family history of TAD was assessed by cross-linking nationwide health registries.

INTERVENTIONS:

None.

RESULTS:

Family history was present in 108 cases (4.4%), compared with 93 (0.77%) controls (p<0.0001). The risk of TAD increased with number of affected relatives: OR 5.8 (95% CI 4.3 to 7.7) vs OR 20 (2.2 to 179) with one versus two or more affected relatives. The relative risk of TAD was highest in the youngest (≤49 years) age group and slightly more pronounced in women than in men (OR 7.2 (4.2 to 12) vs OR 5.5 (3.9 to 7.7)). Among cardiopulmonary comorbidities, heart failure conferred the highest relative risk, OR 6.3 (4.1 to 9.8).

CONCLUSIONS:

Family history confers a significantly increased (sixfold to 20-fold) relative risk of TAD. The effect is more pronounced in women and in younger subjects, and is not conveyed by cardiopulmonary comorbidity. Knowledge of family history is important to counselling, treatment indications, surveillance and screening protocols.

Objective: To assess surgical and long-term mortality in a large, contemporary, unselected cohort of patients undergoing operations on the proximal thoracic aorta. Methods: Patients in the Swedish Heart Surgery register operated 1992-2004 were identified and data cross-linked with the in-hospital and cause-of-death registers. Factors associated with surgical, intermediate, and long-term mortality were studied with separate Cox analyses. Long-term survival was estimated by Kaplan-Meier analysis. Results: 2634 patients (68% men, mean age 60 years) were operated for aortic aneurysm (n = 1821, 69%) or aortic dissection (n = 813, 31%). Overall, increased age, aortic dissection, emergency operation, coronary artery bypass grafting, postoperative stroke, and postoperative renal failure were independently associated with surgical mortality. Only age was independently associated with long-term mortality. Later era of treatment (1998-2004 vs 1992-1997) was associated with lower risk only for aneurysm patients, despite similar changes in surgical approach. Long-term survival for all patients was 83% at 1 year, 77% at 5 years, and 73% at 10 years and identical for aneurysm and dissection when adjusted for surgical mortality. Conclusions: Increased age was associated with increased mortality across follow-up, implicating early surgery when possible. Results improved over time for aneurysms but not dissections; however, long-term survival was equal.

BACKGROUND: Selective antegrade cerebral perfusion has been introduced as a strategy of cerebral protection in operations on the aortic arch with hypothermic circulatory arrest. Several techniques of unilateral and bilateral cerebral perfusion have been described with varying results.

METHODS: Patients underwent either unilateral cerebral perfusion with a cannula in the right subclavian artery or bilateral cerebral perfusion, with an additional cannula in the left carotid artery. A simplified Seldinger-type technique for subclavian artery cannulation was employed. Results were analyzed with multivariable logistic regression analysis and propensity score analysis to adjust for nonrandomized treatment assignment.

RESULTS: Of 65 patients, 17 (26%) had unilateral cerebral perfusion. Mortality was 11% (n = 7); 14% (n = 9) had a stroke. In multivariable analysis, unilateral cerebral perfusion was significantly associated with stroke (odds ratio 6.6 [1.2 to 36]). Age more than 70 years was associated with in-hospital death (odds ratio 12 [1.3 to 113]), and concomitant coronary artery bypass graft surgery was associated with adverse outcome (odds ratio 23 [1.8 to 299]). Balancing variables in a propensity score analysis, stroke remained significantly more common with unilateral brain perfusion (29% versus 0%, p = 0.045). Complications associated with subclavian artery cannulation were encountered in 1 patient (1.5%).

CONCLUSIONS: The described cannulation technique is safe and effective. Bilateral cerebral perfusion is easily achieved and is associated with decreased stroke risk, and should be the preferred brain protection strategy.

Thirty-eight patients (10 cases and 28 controls) were included in a case-control study of Staphylococcus aureus bloodstream infection after cardiac surgery in 833 patients. All bacterial strains were found to be unique by pulsed-field gel electrophoresis. In multivariable risk-factor analysis, only valve prosthesis implantation was associated with bloodstream infection. The early and late case mortality rate was 0%.

Objectives. To review the data quality and validity in the nationwide Swedish Heart Surgery register for patients operated on the proximal thoracic aorta. Design. Medical records from a random sample of 300 patients in The Swedish Heart Surgery register were reviewed with register data items systematically re-reported. Variable reporting frequency, proportion of adequately reported data, and number and correctness of diagnostic and procedural codes were analysed. Results. After exclusions, 251 patients (84%) remained in the analysis. Reporting frequency for individual items varied from 12% to 100% (median 61%). For core variables, reporting frequency was 96%-100%. In 40 of 43 (93%) reviewed variables, registry data were at least 85% correct. A total of 485 diagnoses and 673 procedures were reported, compared to 617 diagnoses and 758 procedures identified in the review process. Conclusions. The register data quality and validity for patients operated on the proximal thoracic aorta was satisfactory overall, but need further improvement for complications. The register coverage and completeness was very high. Register-based reports should be accompanied by review of data quality.

BACKGROUND: Current knowledge of prevalence, incidence, and survival in thoracic aortic diseases (aneurysm and dissection) is based on small studies from a dated era of treatment and diagnostic procedures. The objective of the present study was to reappraise epidemiology and long-term outcomes in subjects with thoracic aortic disease in a large contemporary population. METHODS AND RESULTS: All subjects with thoracic aortic aneurysm or dissection identified in Swedish national healthcare registers from 1987 to 2002 were included in the present study. Of 14,229 individuals with thoracic aortic disease, 11,039 (78%) were diagnosed before death. Incidence of thoracic aortic disease rose by 52% in men and by 28% in women to reach 16.3 per 100,000 per year and 9.1 per 100,000 per year, respectively. Operations increased 7-fold in men and 15-fold in women over time. Of the 2455 patients who underwent operation, 389 (16%) died within 30 days, with older age and thoracic aortic rupture as risk factors. In Cox analysis, increasing age was the only variable associated with long-term mortality. Both short- and long-term mortality improved over time. In patients who underwent operation, actuarial survival (95% CI) at 1, 5, and 10 years was 92% (91% to 93%), 77% (75% to 80%), and 57% (53% to 61%), respectively. The cumulative incidence of thoracic aortic reoperations was 7.8% at 10 years. CONCLUSIONS: The prevalence and incidence of thoracic aortic disease was higher than previously reported and increasing. The annual number of operations increased substantially. Surgical (30-day) and long-term survival improved significantly over time to form a growing cohort of patients needing counseling, management decisions, operations, and extended postoperative surveillance.