Effect of bosentan therapy on ventricular and atrial function in adults with Eisenmenger syndrome. A prospective, multicenter study using conventional and Speckle tracking echocardiography.

BACKGROUND: The effect of bosentan on the ventricular and atrial performance in patients with Eisenmenger syndrome is unclear. In adult patients with Eisenmenger syndrome, we aimed to evaluate the midterm effect of bosentan on physical exercise, ventricular and atrial function, and pulmonary hemodynamics. METHODS: Forty adult patients before and after 24 weeks bosentan therapy underwent 6 min walk test, two-dimensional speckle tracking echocardiography, plasma NT-proBNP measurement and cardiac catheterization. RESULTS: After 24 weeks, bosentan therapy an improvement was observed regarding the 6 min walk distance from a median (quartile 1-quartile 3) of 382.5 (312-430) to 450 (390-510) m (p = 0.0001), NT-proBNP from 527.5 (201-1,691.25) to 369 (179-1,246) pg/ml (p = 0.021), right ventricular mean longitudinal systolic strain from 18 (13-22) to 19 (14.5-25) % (p = 0.004), left ventricular mean longitudinal systolic strain from 16 (12-21) to 17 (16-22) % (p = 0.001), right atrial mean peak longitudinal strain from 26 (18-34) to 28 (22-34) % (p = 0.01) and right atrial mean peak contraction strain from 11 (8-16) to 13 (11-16) % (p = 0.005). The invasively obtained Qp:Qs and Rp:Rs did not significantly change under bosentan therapy. CONCLUSIONS: In adult patients with Eisenmenger syndrome, bosentan therapy improves ventricular and atrial functions resulting in enhancement of physical exercise and reduction in the NT-proBNP level, while the pulmonary vascular resistance does not change substantially.

Altered right ventricular function in the long-term follow-up evaluation of patients after delayed aortic reimplantation of the anomalous left coronary artery from the pulmonary artery.

This study aimed to evaluate regional and global ventricular functions in the long term after aortic reimplantation of the anomalous left coronary artery from the pulmonary artery (ALCAPA) and to assess whether the time of surgical repair influences ventricular performance.The study examined 20 patients with a median age of 15 years (range 3-37 years) who had a corrected ALCAPA and 20 age-matched control subjects using echocardiography and tissue Doppler imaging (TDI). The median follow-up period after corrective surgery was 6 years (range 2.6-15 years). Seven patients underwent surgery before the age of 3 years (early-surgery group), whereas 13 patients had surgery after that age (late-surgery group). The TDI-derived myocardial strain of the interventricular septum (IVS), lateral wall of the left ventricle (LV), and lateral wall of the right ventricle (RV) in the basal and mid regions were examined, and a mean was calculated. The pulsed Doppler-derived Tei index was used to assess global left ventricular function. No significant differences were found between the early-surgery group and the control group regarding the regional myocardial strain or the Tei index. Compared with the early-surgery group, the late-surgery group had a significantly higher Tei index (mean 0.37; range 0.31-0.42 vs. mean 0.52; range 0.39-0.69; p < 0.005), a lower strain percentage of the lateral wall of the LV (mean 29; range 17-30 vs. mean 9; range 7-23), IVS (mean 23; range 21-31 vs. mean 19; range 13-25), and lateral wall of the RV (mean 23; range 21-31 vs. mean 19; range 13-25). The age at operation correlated significantly with the Tei index (r = 0.84, p < 0.001) and inversely with the mean strain of the lateral wall of the LV (r = -0.53, p = 0.028), IVS (r = -0.68, p = 0.003), and lateral wall of the RV (r = -0.68, p = 0.003). At the midterm follow-up evaluation after corrective surgery of ALCAPA, not only the left but also the right ventricular function seemed to be affected in patients with delayed diagnosis and late surgical repair but preserved among the younger patients with early diagnosis and corrective surgery.

Regional analysis of longitudinal systolic function of the right ventricle after corrective surgery of tetralogy of Fallot using myocardial isovolumetric acceleration index.

To assess regional longitudinal systolic function of the right ventricle in patients with repaired tetralogy of Fallot (TOF) by tissue Doppler imaging-derived isovolumetric acceleration (IVA) index and determine the effect of right-ventricular (RV) enlargement on regional systolic function. In 30 consecutive TOF patients and 30 age-matched controls, myocardial velocity of the RV ventricular free wall in the basal and middle regions were examined in the apical four-chamber view. Peak myocardial velocity during IVA was recorded on the free RV wall. IVA index was calculated as the difference between baseline and peak velocity divided by their time interval. In 23 of the studied TOF patients, magnetic resonance imaging was performed on the same day to determine global RV volume and ejection fraction. IVA index of the RV lateral free wall was significantly lower in the basal (8.31 ± 6.00 vs. 19.00 ± 10.85 m/s(2), p = 0.0001) and middle segments (6.56 ± 5.22 vs. 16.17 ± 7.44 m/s(2), p = 0.0001) in patients than in controls. Among TOF patients, a negative correlation was found between IVA index in the middle segment and RV end-diastolic volume/body surface area (r = -0.549, p < 0.01). Similar to other longitudinal RV wall parameters, the IVA index showed a decreased value in the RV free wall, which is related to the impaired regional and global longitudinal RV systolic dysfunction. RV enlargement adversely affects regional longitudinal systolic function.

Diastolic asynchrony and myocardial dysfunction in patients with univentricular heart after Fontan operation.

BACKGROUND: We aimed to assess the existence of myocardial dysfunction and intra-univentricular diastolic asynchrony in patients after Fontan operation. METHODS: Twenty patients after Fontan procedure and 30 age-matched controls were included in the study. The global function of the univentricular heart was analyzed by the Tei index. Regional myocardial velocities and strain of the univentricular heart including the rudimentary right ventricle (RV) were quantified by tissue Doppler imaging. Intra-univentricular or intra left ventricular (LV) diastolic delay was measured from the difference of diastolic intervals (time to peak early diastolic velocity), measured at LV lateral wall and the rudimental RV wall in patients, or LV lateral wall and the ventricular septum in controls. RESULTS: Compared to the control group, patients after Fontan operation had significantly elevated Tei index (0.24 ± 0.02 vs. 0.41 ± 0.1, p < 0.001). On the other hand, the regional myocardial velocities and strains of the univentricular heart including the rudimentary RV were significantly reduced (p < 0.001). Among patients, there was a significant correlation between the Tei index of the univentricular ventricle and rudimentary RV strain (r = -0.66, p = 0.01). The heart rate-corrected intra-univentricular diastolic delay was significantly prolonged among patients when compared to the intra-LV diastolic delay in controls (0.01 ± 0.9 vs. 1 ± 1.1, p = 0.005). CONCLUSIONS: Myocardial dysfunctions and intra-univentricular diastolic asynchrony of the univentricular heart in patients after Fontan procedure are evident. The rudimentary RV in patients after Fontan procedure plays an important role in the determination of the global function of the univentricular heart.

Preserved regional atrial contractile function following extra-atrial rather than intra-atrial type Fontan operation: a tissue Doppler imaging study.

OBJECTIVE: To determine the univentricular and atrial function in patients following Fontan operation using tissue Doppler echocardiography (TDE). SETTING: Hospital-based outpatient clinic and inpatient unit. PATIENTS: Thirty-six patients (mean age 13 ys [2-34 ys]) after Fontan procedure and 30 healthy individuals matched for age and heart rate. MAIN OUTCOME MEASURES: Pulsed wave Doppler derived Tei index was determined for global univentricular function. Tissue-Doppler derived strain rate and strain were measured for regional ventricular function. Different planimetric approaches were used to evaluate the global atrial function. Strain rate was measured for regional atrial contractile function. RESULTS: The Tei index, reflecting global univentricular function, was significantly higher in Fontan patients than in the control group (p<0.001). The regional ventricular contractile function, which was determined by TDE derived strain and strain rate, was significantly lower in Fontan patients than in healthy controls (p<0.001). The global atrial function evaluated planimetrically, and the regional atrial function assessed using the TDE strain rate, were significantly lower in Fontan patients than in normal controls (p<0.001, p<0.001, respectively). A comparison of different types of Fontan operations with extra-atrial or intra-atrial baffle showed a better regional left atrial wall strain rate in patients with extra-atrial baffle than with intra-atrial baffle. CONCLUSION: Patients who have undergone the Fontan procedure may present with altered ventricular and atrial performance independent of whether the extra-atrial or intraatrial type Fontan operation was performed. However, the regional atrial contractile function seems to be better preserved following the extra-atrial type Fontan operation.

Detection of left ventricular asynchrony in patients with right bundle branch block after repair of tetralogy of Fallot using tissue-Doppler imaging-derived strain.

OBJECTIVES: We aimed to investigate whether patients after tetralogy of Fallot (TOF) repair with right bundle branch block have left ventricular (LV) asynchrony and to assess the influence of ventricular asynchrony on regional and global LV function. BACKGROUND: Patients after TOF repair usually have right bundle branch block. However, no data regarding LV asynchrony in this group are available. METHODS: Twenty-five patients after TOF repair and 25 age-matched healthy control subjects were studied. The regional myocardial deformation of the interventricular septum (IVS) and the LV lateral wall were examined using tissue-Doppler-derived strain. The time interval between the onset of QRS complex and the peak strain was measured for each wall. According to the difference between LV and septum time intervals among the normal subjects, a normal range (mean +/- 2 SD) was plotted, and TOF patients in whom the difference was beyond the normal range were considered to have LV asynchrony. The Tei index was used to assess global LV function. RESULTS: Thirteen (52%) of the examined patients after TOF repair had LV asynchrony. Patients after TOF repair with LV asynchrony had a significantly reduced regional septal systolic strain (p < 0.001) and significantly elevated Tei index (p < 0.001) compared with those without. CONCLUSIONS: Left ventricular asynchrony may exist in patients after TOF repair with right bundle branch block. This LV asynchrony is associated with a reduction of both regional and global LV function.

Quantitative analysis of right atrial performance after surgical repair of tetralogy of Fallot.

We aimed to assess the right atrial performance in patients after surgical correction of tetralogy of Fallot, and to clarify the relationship between the pump function of the right atrium and right ventricular systolic function. We included in the study 50 asymptomatic patients following corrective surgery of tetralogy of Fallot, comparing them to 30 normal subjects. Right atrial areas were measured by echocardiography, and the active fractional area of emptying was expressed, in percentages, as the area measured at the onset of atrial contraction, minus the minimal area, divided by the area at the onset of atrial contraction. We used this value to assess the atrial pump function. Right atrial peak strain rates were measured by tissue Doppler imaging. Compared to controls, patients with tetralogy of Fallot had a significantly reduced right atrial active fractional area of emptying (p = 0.005), along with a reduced peak late diastolic strain rate (p = 0.002). Among 20 patients who underwent magnetic resonance tomographic examination, a relatively higher right atrial peak late diastolic strain rate was shown in patients with a right ventricular ejection fraction of less than 50% (p = 0.021). Right atrial performance is reduced in patients after surgical correction of tetralogy of Fallot. When facing right ventricular systolic dysfunction, nonetheless, the right atrial pump function may be relatively enhanced. Tissue Doppler derived strain rate can provide quantitative analysis of regional right atrial performance.