Major adverse events and atrial tachycardia in Ebstein's anomaly predicted by cardiovascular magnetic resonance.

OBJECTIVES: Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. METHODS: Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT). RESULTS: CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007). CONCLUSION: CMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Dyssynchrony and electromechanical delay are associated with focal fibrosis in the systemic right ventricle - Insights from echocardiography.

BACKGROUND: Systemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity. METHODS AND RESULTS: Twenty-two consecutive patients, mean age 28±8years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P=0.002 &P=0.049), prolonged systemic RV isovolumic contraction time (P=0.024) and reduced systemic RV free wall and septal excursion (P=0.027 &P=0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r=0.73 &P=0.004 and r=0.62 &P=0.041, respectively, and QRS duration r=0.68, P<0.01) and was inversely related to systolic RV free wall shortening velocity (r=-0.59 &P=0.042). The presence of LGE was also related to lower exercise capacity, ≥mild tricuspid regurgitation and more arrhythmia (P=0.008, P=0.014 and P=0.040). RV free wall excursion and systolic tissue Doppler velocity were related to CMR derived RV ejection fraction (r=0.51, P=0.015, and r=0.77, P=<0.001, respectively). CONCLUSION: Post Mustard repair, myocardial fibrosis is related to dyssynchrony, RV long axis dysfunction and tricuspid regurgitation. Echocardiographic measurements of systemic RV function can be confidently used in serial follow-up following Mustard operation.

Impaired Right, Left, or Biventricular Function and Resting Oxygen Saturation Are Associated With Mortality in Eisenmenger Syndrome: A Clinical and Cardiovascular Magnetic Resonance Study.

BACKGROUND: Patients with Eisenmenger syndrome (ES) have better survival, despite similar pulmonary vascular pathology, compared with other patients with pulmonary arterial hypertension. Cardiovascular magnetic resonance (CMR) is useful for risk stratification in idiopathic pulmonary arterial hypertension, whereas it has not been evaluated in ES. We studied CMR together with other noninvasive measurements in ES to evaluate its potential role as a noninvasive risk stratification test. METHODS AND RESULTS: Between 2003 and 2005, 48 patients with ES, all with a post-tricuspid shunt, were enrolled in a prospective, longitudinal, single-center study. All patients underwent a standardized baseline assessment with CMR, blood test, echocardiography, and 6-minute walk test and were followed up for mortality until the end of December 2013. Twelve patients (25%) died during follow-up, mostly from heart failure (50%). Impaired ventricular function (right or left ventricular ejection fraction) was associated with increased risk of mortality (lowest quartile: right ventricular ejection fraction, <40%; hazard ratio, 4.4 [95% confidence interval, 1.4-13.5]; P=0.01 and left ventricular ejection fraction, <50%; hazard ratio, 6.6 [95% confidence interval, 2.1-20.8]; P=0.001). Biventricular impairment (lowest quartile left ventricular ejection fraction, <50% and right ventricular ejection fraction, <40%) conveyed an even higher risk of mortality (hazard ratio, 8.0 [95% confidence interval, 2.5-25.1]; P=0.0004). No other CMR or noninvasive measurement besides resting oxygen saturation (hazard ratio, 0.90 [0.83-0.97]/%; P=0.007) was associated with mortality. CONCLUSIONS: Impaired right, left, or biventricular systolic function derived from baseline CMR and resting oxygen saturation are associated with mortality in adult patients with ES. CMR is a useful noninvasive tool, which may be incorporated in the risk stratification assessment of ES during lifelong follow-up.

Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries.

BACKGROUND: We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery. These patients have a systemic right ventricle (RV) and are at risk of arrhythmia, premature RV failure, and sudden death. METHODS AND RESULTS: Fifty-five patients (aged 27±7 years) underwent LGE cardiovascular magnetic resonance and were followed for a median 7.8 (interquartile range, 3.8-9.6) years in a prospective single-center cohort study. RV LGE was present in 31 (56%) patients. The prespecified composite clinical end point comprised new-onset sustained tachyarrhythmia (atrial/ventricular) or decompensated heart failure admission/transplantation/death. Univariate predictors of the composite end point (n=22 patients; 19 atrial/2 ventricular tachyarrhythmia, 1 death) included RV LGE presence and extent, RV volumes/mass/ejection fraction, right atrial area, peak Vo(2), and age at repair. In bivariate analysis, RV LGE presence was independently associated with the composite end point (hazard ratio, 4.95 [95% confidence interval, 1.60-15.28]; P=0.005), and only percent predicted peak Vo(2) remained significantly associated with cardiac events after controlling for RV LGE (hazard ratio, 0.80 [95% confidence interval, 0.68-0.95]; P=0.009/5%). In 8 of 9 patients with >1 event, atrial tachyarrhythmia, itself a known risk factor for mortality, occurred first. There was agreement between location and extent of RV LGE at in vivo cardiovascular magnetic resonance and histologically documented focal RV fibrosis in an explanted heart. There was RV LGE progression in a different case restudied for clinical indications. CONCLUSIONS: Systemic RV LGE is strongly associated with adverse clinical outcome especially arrhythmia in transposition of the great arteries, thus LGE cardiovascular magnetic resonance should be incorporated in risk stratification of these patients.

Utility of noninvasive arrhythmia mapping in patients with adult congenital heart disease.

Arrhythmia management in patients with adult congenital heart disease (ACHD) is a challenge on many levels, as tachycardic episodes may lead to hemodynamic impairment in otherwise compensated patients even if episodes are only transient. Recently several technical advances, including 3-dimensional (3D) image integration, 3D mapping, and remote magnetic navigation, have been introduced to facilitate curatively intended ablation procedures in patients with ACHD. This review attempts to outline the role of a novel technology of simultaneous, noninvasive mapping in this patient cohort, and gives details of the authors' single-center experience.

d-Dimer and simplified pulmonary embolism severity index in relation to right ventricular function.

BACKGROUND: Right ventricular (RV) involvement in pulmonary embolism (PE) is an ominous sign. The aim of this study was to investigate the extent to which the d-dimer level or simplified PE severity index (sPESI) indicates RV dysfunction in patients with preserved systemic arterial pressure. METHODS: Right ventricular function was studied in 34 consecutive patients with acute nonmassive PE by echocardiography including Doppler tissue imaging within 24 hours after arrival to the hospital. d-Dimer and sPESI were assessed upon arrival. RESULTS: d-Dimer correlated with RV pressure (Rs, 0.60; P < .001) and pulmonary vascular resistance (PVR; Rs, 0.68; P < .0001) and tended to be related to myocardial performance index (MPI; Rs, 0.31; P = .067). Compared to a level less than 3.0 mg/L, patients with d-dimer 3.0 mg/L or higher had lower systolic tricuspid annular velocity (11.3 ± 2.7 vs 13.5 ± 2.7 cm/s; P < .05), a prolonged MPI (0.8 ± 0.3 vs 0.5 ± 0.2; P < .01), increased RV pressure (58 ± 13 vs 37 ± 12 mm Hg; P < .001), and increased PVR (3.3 ± 1.1 vs 1.8 ± 0.4 Woods units; P < .001). Patients in the high-risk sPESI group had higher filling pressure than those in the low risk sPESI group. CONCLUSIONS: In the acute stage of PE, a d-dimer level 3 mg/L or higher may identify nonmassive PE patients with RV dysfunction and thereby help to determine their risk profile. We found no additional value for sPESI in this context.

Right ventricular function in patients with pulmonary embolism: early and late findings using Doppler tissue imaging.

BACKGROUND: Assessments of right ventricular (RV) function using myocardial velocities in patients with pulmonary embolism (PE) may add vital information. METHODS: Thirty-four patients with PE were studied in the acute stage and 3 months afterward. Tricuspid annular velocity was recorded using pulsed-wave Doppler tissue imaging. RESULTS: At the time of diagnosis, tricuspid annular velocities were significantly decreased in patients compared with controls in systole (12.9 vs 14.8 cm/s, P < .05) and early diastole (11.9 vs 15.3 cm/s, P < .01) and normalized during follow-up. Decreases in tricuspid annular velocity were most pronounced in patients with increased RV pressure. The myocardial performance index was prolonged and pulmonary vascular resistance was higher in patients with increased RV pressure. The ratio of tricuspid flow to myocardial velocity (E/Em) was also increased compared with controls (4.5 vs 3.5, P < .05). CONCLUSION: RV dysfunction in patients with PE was common in the acute phase but normalized within 3 months. Patients presenting with normal RV pressure had normal systolic but disturbed diastolic function.

Echocardiographic evaluation of right ventricular function in patients with acute pulmonary embolism: a study using tricuspid annular motion.

Assessment of right ventricular (RV) function is a challenge due to complex anatomy. We studied systolic and diastolic tricuspid annular excursion and longitudinal RV fractional shortening as geometry-independent measures in patients with acute pulmonary embolism (PE). Forty patients with PE were studied within 24 hours after admission and after 3 months, and compared to 23 healthy subjects used as controls. We recorded tricuspid annular plane systolic (TAPSE) and diastolic (TAPDE) excursion from the four-chamber view and calculated RV fractional shortening as TAPSE/RV diastolic length. The diastolic RV function was defined as the ratio of the amplitude of tricuspid annular plane excursion during atrial systole to total tricuspid annular plane diastolic excursion (atrial/total TAPDE). In the acute stage, the TAPSE was decreased in PE compared to healthy subjects (19 +/- 5 vs. 26 +/- 4 mm, P < 0.001), with greater reduction in patients with increased, compared to normal, RV pressure (16.6 +/- 5 vs. 20.5 +/- 5 mm, P < 0.05). The atrial/total TAPDE was increased in patients compared to healthy subjects (47 +/- 13% vs. 38 +/- 7%, P < 0.001) and normalized during the follow-up. Although the patients were asymptomatic after 3 months, the TAPSE recovered incompletely as compared to healthy subjects (21.4 +/- 4 vs. 26 +/- 4 mm, P < 0.001). Both systolic and diastolic RV function are impaired in acute PE. Diastolic function recovers faster than systolic; therefore, the atrial contribution to RV filling may be a useful measure to follow changes in diastolic function in PE.