Augmentation index and the evolution of aortic disease in marfan-like syndromes.

BACKGROUND: The augmentation index at a heart rate of 75 beats/min (AIx@HR75) and central pulse pressure (CPP) can be measured noninvasively with applanation tonometry (APT). In this observational study, we investigated the relationship between AIx@HR75, CPP and aortic disease in patients with Marfan-like syndromes. METHODS: We performed APT in 78 consecutive patients in whom classic Marfan syndrome (MFS) had been excluded (46 men and 32 women aged 34 +/- 13 years). These patients comprised 9 persons with MFS-like habitus, 6 with a bicuspid aortic valve (BAV), 5 with MASS phenotype, 3 with vascular type of Ehlers-Danlos syndrome (EDS), 3 with familial thoracic aortic aneurysm, 2 with Loeys-Dietz syndrome (LDS), 1 with mitral valve prolapse syndrome, 1 with familial ectopia lentis, and 48 persons with Marfan-like features but no defined syndrome. During 20 +/- 18 months after APT, we observed progression of aortic diameters in 15 patients, and aortic surgery or aortic dissection in 3 individuals. RESULTS: All 11 patients with Marfan-like syndromes and progression of aortic disease exhibited AIx@HR75 > or =11%, including 8 individuals with aortic diameters < or =95th percentile of normal at baseline. Similarly, all 7 individuals without any defined syndrome but progression of aortic diameters exhibited AIx@HR75 >11%, including 6 individuals with aortic diameters < or =95th percentile at the time of APT. Aortic disease did not evolve at AIx@HR75 <11%. CPP is also related to aortic disease progression. CONCLUSIONS: Aortic disease evolution relates to AIx@HR75 and CPP in Marfan like syndromes. Larger studies with comprehensive clinical and echocardiographic follow-up over long time intervals will be required to establish APT for prediction of aortic disease evolution in Marfan-like syndromes.

Comparison of antero-lateral versus antero-posterior electrode position for biphasic external cardioversion of atrial flutter.

External cardioversion is an established and very important tool to terminate symptomatic atrial flutter. The superiority of the biphasic waveform has been demonstrated for atrial flutter, but whether electrode position affects the efficacy of cardioversion in this population is not known. The aim of this trial was to evaluate whether anterior-lateral (A-L) compared with anterior-posterior (A-P) electrode position improves cardioversion results. Of 130 screened patients, 96 (72 men, mean age 62 +/- 12 years) were included and randomly assigned to a cardioversion protocol with either A-L or A-P electrode position. In each group, 48 patients received sequential biphasic waveform shocks using a step-up protocol consisting of 50, 75, 100, 150, or 200 J. The mean energy (65 +/- 13 J for A-L vs 77 +/- 13 J for A-P, p = 0.001) and mean number of shocks (1.48 +/- 1.01 for A-L vs 1.96 +/- 1.00 for A-P, p = 0.001) required for successful cardioversion were significantly lower in the A-L group. The efficacy of the first shock with 50 J in the A-L electrode position (35 of 48 patients [73%]) was also highly significantly greater than the first shock with 50 J in the A-P electrode position (18 of 48 patients [36%]) (p = 0.001). In conclusion, the A-L electrode position increases efficacy and requires fewer energy and shocks in external electrical cardioversion of common atrial flutter. Therefore, A-L electrode positioning should be recommended for the external cardioversion of common atrial flutter.

Augmentation index relates to progression of aortic disease in adults with Marfan syndrome.

BACKGROUND: Noninvasive applanation tonometry (APT) is useful to assess aortic stiffness and pulse wave reflection. Moreover, APT can predict outcome in many conditions such as arterial hypertension. In this study, we test whether APT measurements relate to progression of aortic disease in Marfan syndrome (MFS). METHODS: We performed APT in 50 consecutive, medically treated adults with MFS (19 men and 31 women aged 32 +/- 13 years), who had not undergone previous cardiovascular surgery. During 22 +/- 16 months of follow-up, 26 of these patients developed progression of aortic disease, which we defined as progression of aortic root diameters >or=5 mm/annum (18 individuals), aortic surgery >or=3 months after APT (seven individuals), or onset of acute aortic dissection any time after APT (one individual). RESULTS: Univariate Cox regression analysis suggested an association of aortic disease progression with age (P = 0.001), total cholesterol levels (P = 0.04), aortic root diameter (P = 0.007), descending aorta diameter (P = 0.01), aortic root ratio (P = 0.02), and augmentation index (AIx@HR75; P < 0.006). Multivariate Cox regression analysis confirmed an independent impact on aortic disease progression exclusively for baseline aortic root diameters (hazard ratio = 1.347; 95% confidence interval (CI) 1.104-1.643; P = 0.003) and AIx@HR75 (hazard ratio = 1.246; 95% CI 1.029-1.508; P = 0.02). In addition, Kaplan-Meier survival curve analysis illustrated significantly lower rates of aortic root disease progression both with lower AIx@HR75 (P = 0.025) and with lower pulse wave velocity (PWV) values (P = 0.027). CONCLUSIONS: We provide evidence that APT parameters relate to aortic disease progression in medically treated patients with MFS. We believe that APT has a potential to improve risk stratification in the clinical management of MFS patients.

Predicting recurrence of vasovagal syncope: a simple risk score for the clinical routine.

BACKGROUND: Predictors for recurrence of syncope are lacking in patients with vasovagal syncope. The aim of this study was to identify risk factors for recurrence of syncope and develop a simple prognostic risk score of clinical value. METHODS: Two hundred seventy-six patients with a history of vasovagal syncope were prospectively followed for 2 years. Diagnosis of vasovagal syncope was based on clinical history and negative standard work-up. Inclusion in the study was independent from the result of the head-up tilt test, which was performed in all cases. Risk factors for syncope recurrence were evaluated by the Cox proportional hazards regression model and implemented in a risk score, which was validated with the log-rank test and an internal cross-validation. RESULTS: The Cox-regression analysis identified the number of previous syncopal events, history of bronchial asthma, and female gender as predictors for syncope recurrence (all P < 0.05). In contrast, head-up tilt test response had no predictive value (P = 0.881). Developing a risk score, study patients were identified as having high (recurrence rate during 2 years of follow-up: 37.2%), intermediate (24.8%), and low (6.5%) risk for syncope recurrence (receiver operating characteristic [ROC] of score 0.83, P < 0.01; Log-rank test for event-free survival, P < 0.005). CONCLUSIONS: In patients with vasovagal syncope, risk of recurrence can be stratified and is predictable based on a simple risk score.

Tissue Doppler imaging identifies myocardial dysfunction in adults with Marfan syndrome.

BACKGROUND: Successful prevention of aortic complications has lead to improved survival of Marfan syndrome (MFS). With increasing age, however, ventricular arrhythmia and heart failure are emerging as life-threatening manifestations of myocardial dysfunction. HYPOTHESIS: We sought to investigate whether echocardiography with tissue Doppler imaging (TDI) identifies myocardial dysfunction in adults with MFS. METHODS: We performed two-dimensional (2-D) and Doppler echocardiography with TDI in 141 individuals with suspected MFS and competent heart valves, including 28 persons with MFS who had not undergone surgery and 86 healthy controls without inherited connective tissue disorders. RESULTS: Demographic profile, 2-D, mitral and pulmonary venous flow indices, and left ventricular ejection fractions were similar in both groups. Conversely, isovolumic relaxation time (p < 0.001) and deceleration time of E velocity (p = 0.005) were longer, and atrial reversal velocities (p = 0.02), and systolic and early diastolic TD velocities were slower in MFS than in controls (p = 0.01). Multiple linear regression analysis excluded association of reduced systolic and early diastolic TD velocities with mitral valve prolapse or other clinical or echocardiographic features of MFS. CONCLUSIONS: Our study identifies reduced systolic and early diastolic TD velocities in adults with MFS. Further studies are mandatory to elucidate whether TD velocities predict arrhythmia and heart failure in MFS.

Predictors of aneurysmal formation after surgical correction of aortic coarctation.

OBJECTIVES: We sought to identify the predictors of aneurysmal formation after surgical correction of aortic coarctation. BACKGROUND: In 9% of patients, aneurysms develop late after corrective surgery of coarctation of the aorta, with a 36% mortality rate if left untreated. However, the predictors of postsurgical aneurysmal formation are unknown. METHODS: Of 25 aortic aneurysms requiring corrective surgery 152 +/- 78 months after the initial coarctation repair, 8 were located in the ascending aorta (type A) and 17 at the site of previous repair (local type). Seventy-four patients without progression of the aortic diameter within 189 +/- 71 months after coarctation repair were used for categorical data analysis in an attempt to identify the predictors of postsurgical aneurysmal formation. RESULTS: Advanced age at coarctation repair (p = 0.004) and patch graft technique (p < 0.0005) independently predicted local aneurysmal formation. Type A aneurysm was univariately associated with the presence of a bicuspid aortic valve (p = 0.02), advanced age at coarctation repair (p = 0.044) and a high preoperative peak systolic pressure gradient of 74 +/- 21 mm Hg (p = 0.041). Conversely, multivariate analysis confirmed only the presence of a bicuspid aortic valve (p = 0.015) as an independent predictor of type A aneurysm. Receiver operating characteristic curve analysis revealed that 72% of patients with a postsurgical aneurysm had an operation at age 13.5 years or more, whereas 69% with no postsurgical aneurysm had an operation at a younger age. CONCLUSIONS: Use of the patch graft technique and late correction of coarctation can predict aneurysmal formation at the site of coarctation repair, although patients with a bicuspid aortic valve may be at risk for an aneurysm developing in the ascending aorta, particularly after late repair of aortic coarctation with high preoperative pressure gradients.