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Background: Pulmonary hypertension (PH), right ventricular (RV) dysfunction, and tricuspid regurgitation (TR) are commonly present in patients with mitral regurgitation (MR) and known to impair prognosis. This systematic review and meta-analysis aimed to determine the prognostic value of PH, RV function, and TR on mortality after transcatheter mitral valve repair (TMVR)., Methods: A systematic search was performed to identify studies investigating PH, RV function, or TR in patients who underwent TMVR. Studies were included for pooled analysis if hazard ratios (HR) for all-cause mortality were given., Results: A total of 8,672 patients from 21 selected studies were included (PH, 11 studies; RV function, nine studies; TR, 10 studies). Mean follow-up was 2.7±1.6 years. The HRs and 95% CIs for all-cause mortality of PH (dichotomised: HR 1.70, 95% CI 1.00-2.87; per 10 mmHg increase in systolic PAP: HR 1.17, 95% CI 1.07-1.29), RV function (dichotomised: HR 1.86, 95% CI 1.45-2.38; per 5 mm decrease in TAPSE: HR 1.18, 95% CI 0.97-1.43) and TR (HR 1.51, 95% CI 1.28-1.79) indicated a significant association., Conclusion: Prognosis after TMVR is worse in patients with significant MR when concomitant PH, RV dysfunction, or TR are present. Careful assessment of these parameters should therefore precede clinical decision-making for TMVR. The current results encourage investigation into whether (1) intervention at an earlier stage of MR reduces incidence of PH, RV dysfunction, and TR; and (2) transcatheter treatment of concomitant TR can improve clinical outcome and prognosis for these patients., (Copyright © 2021 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Maternal right ventricular (RV) dysfunction (measured by echocardiography) is associated with impaired uteroplacental circulation, however echocardiography has important limitations in the assessment of RV function. We therefore aimed to investigate the association of pre-pregnancy RV and left ventricular (LV) function measured by cardiovascular magnetic resonance with uteroplacental Doppler flow parameters in pregnant women with repaired Tetralogy of Fallot (ToF)., Methods: Women with repaired ToF were examined, who had been enrolled in a prospective multicenter study of pregnant women with congenital heart disease. Clinical data and CMR evaluation before pregnancy were compared with uteroplacental Doppler parameters at 20 and 32 weeks gestation. In particular, pulsatility index (PI) of uterine and umbilical artery were studied., Results: We studied 31 women; mean age 30 years, operated at early age. Univariable analyses showed that reduced RV ejection fraction (RVEF; P = 0.037 and P = 0.001), higher RV end-systolic volume (P = 0.004) and higher LV end-diastolic and end-systolic volume (P = 0.001 and P = 0.003, respectively) were associated with higher uterine or umbilical artery PI. With multivariable analyses (corrected for maternal age and body mass index), reduced RVEF before pregnancy remained associated with higher umbilical artery PI at 32 weeks (P = 0.002). RVEF was lower in women with high PI compared to women with normal PI during pregnancy (44% vs. 53%, p = 0.022). LV ejection fraction was not associated with uterine or umbilical artery PI., Conclusions: Reduced RV function before pregnancy is associated with abnormal uteroplacental Doppler flow parameters. It could be postulated that reduced RV function on pre-pregnancy CMR (≤2 years) is a predisposing factor for impaired placental function in women with repaired ToF.

Objectives: In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or with an atrial switch corrected transposition of the great arteries. The aim of the current study was to investigate the longer-term clinical outcomes after valsartan treatment., Methods: From 2006 to 2009, 88 adults were randomly allocated 1:1 to either valsartan or placebo for three consecutive years. Endpoints were defined as overall survival and freedom from clinical events (arrhythmia, heart failure, tricuspid valve surgery, death)., Results: Cardiac drug use and median follow-up after trial close-out (8.3 years) was similar between the randomization groups. Six patients (valsartan n = 3, placebo n = 3) died in 364 and 365 person-years (P = 0.999). No difference in the composite or separate clinical endpoints was found between the randomization groups, with corresponding long-term event-free survival rates of 50% and 34%. Nevertheless, in symptomatic patients valsartan significantly reduced the risk for events compared to placebo (HR 0.37, 95% CI 0.17-0.92). Analysis for repeated events and on-treatment analysis with any renin-angiotensin-aldosterone-system-inhibitor did not alter these results., Conclusions: Valsartan treatment in systemic RV patients did not result in improved survival at longer-term follow-up, but was associated with decreased risk of events in symptomatic patients., (Copyright © 2018. Published by Elsevier B.V.)

Background: The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot., Methods: The REDEFINE trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) is an investigator-initiated, multicenter, prospective, 1:1 randomized, double-blind, placebo-controlled study. Adults with repaired tetralogy of Fallot and RV dysfunction (RV ejection fraction [EF] <50%) but without severe valvular dysfunction were eligible. Patients were randomly assigned between losartan (150 mg daily) and placebo with target treatment duration between 18 and 24 months. The primary outcome was RV EF change, determined by cardiovascular MRI in intention-to-treat analysis., Results: Of 95 included patients, 47 patients received 150 mg losartan daily (age, 38.0±12.4 years; 74% male), and 48 patients received placebo (age, 40.6±11.4 years; 63% male). Overall, RV EF did not change in patients allocated to losartan (n=42) (44.4±5.1% to 45.2±5.0%) and placebo (n=46) (43.2±6.3% to 43.6±6.9%). Losartan did not significantly improve RV EF in comparison with placebo (+0.51%; 95% confidence interval, -1.0 to +2.0; P =0.50). No significant treatment effects were found on secondary outcomes: left ventricular EF, peak aerobic exercise capacity, and N-terminal pro-brain natriuretic peptide ( P >0.30 for all). In predefined subgroup analyses, losartan did not have a statistically significant impact on RV EF in subgroups with symptoms, restrictive RV, RV EF<40%, pulmonary valve replacement, or QRS fragmentation. However, in a post hoc analysis, losartan was associated with improved RV EF in a subgroup (n=30) with nonrestrictive RV and incomplete remodeling (QRS fragmentation and previous pulmonary valve replacement) (+2.7%; 95% confidence interval, +0.1 to +5.4; P =0.045)., Conclusions: Losartan had no significant effect on RV dysfunction or secondary outcome parameters in repaired tetralogy of Fallot. Future larger studies may determine whether there might be a role for losartan in specific vulnerable subgroups., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02010905., (© 2017 American Heart Association, Inc.)

Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Importance: Adults late after total correction of tetralogy of Fallot (TOF) are at risk for major complications. Cardiovascular magnetic resonance (CMR) imaging is recommended to quantify right ventricular (RV) and left ventricular (LV) function. However, a commonly used risk model by Khairy et al requires invasive investigations and lacks CMR imaging to identify high-risk patients., Objective: To implement CMR imaging in noninvasive risk stratification to predict major adverse clinical outcomes., Design, Setting, and Participants: This multicenter study included 575 adult patients with TOF (4.083 patient-years at risk) from a prospective nationwide registry in whom CMR was performed. This study involved 5 tertiary referral centers with a specialized adult congenital heart disease unit. Multivariable Cox hazards regression analysis was performed to determine factors associated with the primary end point. The CMR variables were combined with the noninvasive components of the Khairy et al risk model, and the C statistic of the final noninvasive risk model was determined using bootstrap sampling. The data analysis was conducted from January to December 2016., Main Outcomes and Measures: The composite primary outcome was defined as all-cause mortality or ventricular arrhythmia, defined as aborted cardiac arrest or documented ventricular fibrillation and ventricular tachycardia (lasting ≥30 seconds or recurrent symptomatic)., Results: Of the 575 patients with TOF, 57% were male, and the mean (SD) age was 31 (11) years. During a mean (SD) follow-up of 7.1 (3.5) years, the primary composite end point occurred in 35 patients, including all-cause mortality in 13 patients. Mean (SD) RV ejection fraction (EF) was 44% (10%), and mean (SD) LV EF was 53% (8%). There was a correlation between RV EF and LV EF (R, 0.36; 95% CI, 0.29-0.44; P < .001). Optimal thresholds for ventricular function (RV EF <30%: hazard ratio, 3.90; 95% CI, 1.84-8.26; P < .001 and LV EF <45%: hazard ratio, 3.23; 95% CI, 1.57-6.65; P = .001) were independently predictive in multivariable analysis. Both thresholds were included in a point-based noninvasive risk model (C statistic, 0.75; 95% CI, 0.63-0.85) and combined with the noninvasive components of the Khairy et al risk model., Conclusions and Relevance: In patients with repaired TOF, biventricular dysfunction on CMR imaging was associated with major adverse clinical outcomes. The quantified thresholds (RV EF <30% and LV EF <45%) may be implemented in noninvasive risk stratification.

Renin-angiotensin-aldosterone system (RAAS) inhibition with angiotensin II receptor blockers or angiotensin-converting enzyme inhibitors is beneficial in patients with acquired left ventricular dysfunction. Adult patients with tetralogy of Fallot (TOF) with right ventricular (RV) dysfunction are at high risk for heart failure, arrhythmias, and sudden cardiac death. However, the efficacy of RAAS inhibition has not been established in these patients., Methods: The REDEFINE is an investigator-initiated, multicenter, prospective, randomized, double-blind, placebo-controlled trial to study the effects of the angiotensin II receptor blocker losartan (target dosage of 150 mg once daily) in adult patients with TOF. Patients with RV dysfunction in the absence of severe valvular dysfunction are eligible for inclusion. The primary end point is the change in RV ejection fraction after 18 to 24 months, as measured by cardiovascular magnetic resonance imaging. In addition, laboratory measurements, echocardiography, and cardiopulmonary exercise testing are performed., Conclusion: The REDEFINE trial will study the effects of RAAS inhibition with losartan in TOF patients with RV dysfunction., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: In patients with congenitally corrected transposition of the great arteries (ccTGA) or after atrial (Mustard or Senning) correction for transposition of the great arteries (acTGA), the right ventricle (RV) supports the systemic circulation. The tricuspid valve (TV) (systemic atrioventricular valve) is prone to regurgitation in these patients and this is associated with impending RV failure and decreased survival. This study evaluates mid-term functional improvements, echocardiographic findings and survival after TV surgery in this patient group., Methods: From July 1999 to November 2014, 26 patients (mean age 37.1 ± 12.3 years, 14 females) with ccTGA (n = 15) or acTGA (n = 11) had TV surgery. All patients had RV dysfunction and more-than-moderate TV regurgitation (TR); 14 underwent TV replacement (TVR) and 12 had valvuloplasty (TVP). Main outcomes were New York Heart Association (NYHA) functional class, TR and RV dysfunction at 1 year postoperatively and at latest follow-up. Complications and freedom from the composite end-point of death or recurrent TR were analysed., Results: The median follow-up time was 5.9 years (range, 0-16.1 years). Mean NYHA functional class significantly improved to 1.7 [95% confidence interval (CI): 1.3-2.1] at 1 year (P= 0.004) and was 2.1 (95% CI: 1.7-2.6) at latest follow-up (P= 0.14). TV competence significantly improved to a mean TR grade of 1.1 (95% CI: 0.5-1.7) at latest follow-up (P< 0.001). The mean grade for RV function at latest follow-up was 2.7 (95% CI: 2.3-3.0). Most encountered postoperative complications were arrhythmias and temporary haemodynamic instability due to low cardiac output. Early mortality was 11.5% (n = 3); late mortality was 15.4% (n = 4). Estimated freedom from the composite end-point of death or recurrent TR was 76.9% (95% CI: 55.7-88.9%) at 1 year and 64.8% (95% CI: 43.2-79.9%) at 5 years. In TVP patients, TV function at 1 year and at latest follow-up was significantly worse than in TVR patients (P< 0.001 and P= 0.003, respectively). Also, TVP patients had a significantly lower composite end-point survival curve compared with TVR patients (P= 0.018)., Conclusions: In this patient group, TV surgery showed stabilization of RV function and improvement of NYHA functional class for at least several years. In this series, TVR appears superior to TVP with respect to occurrence of recurrent TR. Early and late mortality after TV surgery is substantial, and we believe that patients with significant TR should be referred earlier for surgery for better outcome., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability., Methods: This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR., Results: A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively)., Conclusions: Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objectives: The aim of the present study is to determine the long-term effects of a ten-week exercise training program in adult patients with a systemic right ventricle., Methods: All patients who participated in a 2009 randomized controlled trial were approached. At approximately three years of follow-up from initial baseline, patients underwent cardiopulmonary exercise testing, filled out two quality of life questionnaires, and NT proBNP levels were measured. All examinations were performed according to the protocols of the 2009 trial. In addition, patients were asked about their current sports habits., Results: Of the 54 patients who were randomized in the 2009-trial 40 participated in the current re-evaluation (male 50%, ccTGA 35%, age 36 ± 10 years, intervention group n=22, control group n=18). After three years, no persistent effect of exercise training on V'O₂peak training remained (-2% of predicted, 95% CI -3% to 5%; p=.56). However, patients who already participated in regular sports or exercise at baseline (n=23/40 (58%)) showed higher V'O₂peak of 13% of predicted (95% CI 4% to 23%; p>.01) and a decrease of 62% in plasma NT-proBNP (95% CI -115% to -10%; p>.03) during follow-up, when compared to patients who did not. Moreover, sports were associated with a lower incidence of clinical events (p=.032)., Conclusion: Short-term beneficial effects of exercise training did not persist over a three-year follow-up period. However, sports participation at baseline was associated with better exercise capacity, lower neurohormone levels, and increased event-free survival., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: Chronic mitral regurgitation (MR) often leads to diminished right ventricular (RV) function due to long-standing pressure and volume overload. Surgical intervention often adds to the preexisting RV dysfunction. Percutaneous mitral valve (MV) repair can reduce MR, but to what extent this affects the right ventricle is unknown., Methods: Consecutive patients scheduled for percutaneous MV repair using the MitraClip system underwent transthoracic echocardiography at baseline and at 1- and 6-month follow-up. RV systolic function was evaluated using five echocardiographic parameters. RV afterload was evaluated using systolic pulmonary arterial pressure and the mean MV pressure gradient. Residual MR was defined as grade ≥ 3 and mitral stenosis (MS) as a mean MV pressure gradient ≥ 5 mm Hg., Results: Sixty-eight patients (52% men; mean age, 75 ± 10 years) were included. Six months after MitraClip implantation, there were no significant changes in any of the RV parameters. MR decreased (P < .01) and the mean MV pressure gradient increased during follow-up (2.3 ± 1.4 mm Hg at baseline vs 4.5 ± 2.7 mm Hg at 6 months, P < .01). Patients with both residual MR and MS 6 months after MitraClip implantation showed significantly higher systolic pulmonary arterial pressure values (P < .01) and lower New York Heart Association functional classes (P < .01) compared with patients without residual MR or MS., Conclusions: Percutaneous MV repair, in contrast to surgical repair or replacement, does not negatively affect RV function. After repair, RV afterload and New York Heart Association functional class are improved in the case of successful repair but adversely affected in the presence of both residual MR and MS., (Copyright © 2014 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events., Background: In patients with congenitally or atrially corrected transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia., Methods: At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events., Results: A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m(2) and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors., Conclusions: Patients with a right ventricular end-diastolic volume index above 150 ml/m(2) and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Right ventricular (RV) failure after cardiac surgery is a clinical entity with high morbidity and mortality. Patients with congenital heart disease (CHD) often undergo right-sided cardiac surgery. The authors aimed to identify determinants of RV failure after cardiac surgery to differentiate patients with increased risk., Design: A retrospective chart review., Setting: University hospital., Participants: Adults with CHD operated on between January 2001 and January 2011., Interventions: Clinical characteristics, laboratory tests, surgical data, and intensive care unit outcome were obtained from medical records., Measurements and Main Results: The diagnosis of clinical RV failure was made by careful review of the medical records by 2 independent physicians. Patients only were identified as having RV failure if (1) they had elevated jugular venous pressure, (2) they had impaired postoperative RV function on transthoracic echocardiography, and (3) a diagnosis of RV failure was documented clearly in the medical charts by the treating physician. Data of 412 consecutive patients (median age 36 [range 18-74] years, 56% male) were studied. Eighteen patients had clinical RV failure (4.4%) postoperatively, of whom 6 patients died. Patients undergoing left- and both-sided surgery had an equal risk of developing clinical RV failure as compared with patients undergoing right-sided surgery. In multivariate logistic regression analysis, preoperative impaired RV function, supraventricular tachycardia, and cardiopulmonary bypass time >150 minutes were the strongest determinants of clinical RV failure (p<0.05, for all)., Conclusions: RV failure after cardiac surgery is a serious complication, and occurs regardless of the side of surgery. A tailored approach in patients with CHD at highest risk of RV failure should be considered., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: To assess whether exercise training in adult patients with a systemic right ventricle (RV) improves exercise capacity and quality of life and lowers serum N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels., Design: Multi-centre parallel randomized controlled trial., Participants: Patients with a systemic RV due to congenitally or surgically corrected transposition of the great arteries., Methods: Fifty-four adult patients with a systemic RV, were randomized using unmarked opaque envelopes to an intervention group (n = 28) with three training sessions per week for 10 consecutive weeks, and a control group (n = 26). Randomization was stratified by participating centre. At baseline, and follow-up, we determined maximal exercise capacity (V'O(2peak)), serum NT-proBNP levels, and quality of life by means of the SF-36, and the TAAQOL Congenital Heart Disease questionnaires. The final analysis was performed by linear regression, taking into account the stratified randomization., Results: Forty-six patients were analysed (male 50%, age 32 ± 11 years, intervention group n = 24, control group n = 22). Analysis at 10 weeks showed a significant difference in V'O(2peak) (3.4 mL/kg/min, 95% CI: 0.2 to 6.7; P = 0.04) and resting systolic blood pressure (-7.6 mmHg, 95% CI: -14.0 to -1.3; P = 0.03) in favour of the exercise group. No significant changes were found in serum NT-proBNP levels or quality of life in the intervention group or in the control group nor between groups. None of the patients in the intervention group had to discontinue the training programme due to adverse events., Conclusion: In adult patients with a systemic RV exercise training improve exercise capacity. We recommend to revise restrictive guidelines, and to encourage patients to become physically active. (, Trial Registration: The study was registered at http://trialregister.nl. Identifier: NTR1909.).

Right ventricular function (RVF) is often selectively declined after coronary artery bypass graft surgery. In adult patients with congenital heart disease (CHD) the incidence and persistence of declined RVF after cardiac surgery is unknown. The current study aimed to describe RVF after cardiac surgery in these patients. Adult CHD patients operated between January 2008 and December 2009 in the Academic Medical Centre in Amsterdam were studied. Clinical characteristics, laboratory tests, surgical data and intensive care unit outcome were obtained from medical records. RVF was measured by trans-thoracic echocardiography (TTE) and expressed by tricuspid annular plane systolic excursion (TAPSE), tissue Doppler imaging (RV S') and myocardial performance index (MPI) pre-operatively and direct, at intermediate and late follow up. Of a total of 185 operated, 86 patients (mean age 39 ± 13 years, 54% male) had echo data available. There was a significant fall in RVF after cardiac surgery. TAPSE and RV S' were significantly higher and MPI was significantly lower pre-operatively compared to direct post-operative values (TAPSE 22 ± 5 versus 13 ± 3 mm (P < 0.01), RV S' 11 ± 4 versus 8 ± 2 cm/s (P < 0.01) and MPI 0.36 ± 0.14 vs 0.62 ± 0.25; P < 0.01). There were no significant differences in left ventricular function pre-operatively compared to post-operative values. Right-sided surgery was performed in 33, left-sided surgery in 37 and both sided surgery in 16 patients. Decline in RVF was equal for those groups. Patients with severe decline in RVF, were patients who underwent tricuspid valve surgery. Decline in RVF was associated with post-operative myocardial creatine kinase level and maximal troponin T level. There was no association between decline in RVF and clinical outcome on the intensive care unit. 18 months post-operatively, most RVF parameters had recovered to pre-operative values, but TAPSE which remained still lower (P < 0.01). CHD patients have a decline in RVF directly after cardiac surgery, regardless the side of surgery. Although a gradual improvement was observed, complete recovery was not seen 18 months post-operatively.

Aims: Right ventricular (RV) failure in patients with chronic thromboembolic pulmonary hypertension (CTEPH), and other types of pulmonary arterial hypertension is associated with right-to-left ventricle (LV) delay in peak myocardial shortening and, consequently, the onset of diastolic relaxation. We aimed to establish whether RV pacing may resynchronize the onsets of RV and LV diastolic relaxation, and improve haemodynamics., Methods and Results: Fourteen CTEPH patients (mean age 63.7 ± 12.0 years, 10 women) with large (≥60 ms) RV-to-LV delay in the onset of diastolic relaxation (DIVD, diastolic interventricular delay) were studied. Temporary RV pacing was performed by atrioventricular (A-V) sequential pacing with incremental shortening of A-V delay to advance RV activation. Effects were assessed using tissue Doppler echocardiography and LV pressure-conductance catheter measurements in a subset of patients. Compared with right atrial pacing, RV pacing at optimal A-V delay (average 140 ± 22 ms, range 120-180 ms) resulted in significant DIVD reduction (59 ± 19 to 3 ± 22 ms, P < 0.001), and increase in LV stroke volume as measured by LV outflow tract velocity-time integral (14.9 ± 2.8 to 16.9 ± 3.0 cm, P < 0.001), along with enhanced global RV contractility and LV diastolic filling., Conclusion: Right-to-left ventricle resynchronization of the onset of diastolic relaxation results in stroke volume increase in CTEPH patients. Whether RV pacing may be a novel therapeutic target in RV failure following chronic pressure overload remains to be investigated.

Objectives: We sought to study whether patients with right ventricular failure (RVF) secondary to chronic thromboembolic pulmonary hypertension (CTEPH) have reduced left ventricular (LV) mass, and whether LV mass reduction is caused by atrophy., Background: The LV in patients with CTEPH is underfilled (unloaded). LV unloading may cause atrophic remodeling that is associated with diastolic and systolic dysfunction., Methods: We studied LV mass using cardiac magnetic resonance imaging (MRI) in 36 consecutive CTEPH patients (before/after pulmonary endarterectomy [PEA]) and 11 healthy volunteers selected to match age and sex of patients. We studied whether LV atrophy is present in monocrotaline (MCT)-injected rats with RVF or controls by measuring myocyte dimensions and performing in situ hybridization., Results: At baseline, CTEPH patients with RVF had significantly lower LV free wall mass indexes than patients without RVF (35 ± 6 g/m(2) vs. 44 ± 7 g/m(2), p = 0.007) or volunteers (42 ± 6 g/m(2), p = 0.006). After PEA, LV free wall mass index increased (from 38 ± 6 g/m(2) to 44 ± 9 g/m(2), p = 0.001), as right ventricular (RV) ejection fraction improved (from 31 ± 8% to 56 ± 12%, p < 0.001). Compared with controls, rats with RVF had reduced LV free wall mass and smaller LV free wall myocytes. Expression of atrial natriuretic peptide was higher, whereas that of α-myosin heavy chain and sarcoplasmic reticulum calcium ATPase-2 were lower in RVF than in controls, both in RV and LV., Conclusions: RVF in patients with CTEPH is associated with reversible reduction in LV free wall mass. In a rat model of RVF, myocyte shrinkage due to atrophic remodeling contributed to reduction in LV free wall mass., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Ventricular systolic and diastolic function, as measured by echocardiography, are diminished in patients with a systemic right ventricle (RV). As the clinical implications of these finding remained unknown, we aimed to identify echocardiographic parameters of systolic and diastolic ventricular function that are independent determinants of the clinical condition in these patients., Methods: Forty-six adult patients (61% male; mean age 33 [range 18-69] years) with a systemic RV underwent echocardiography to assess qualitative and quantitative systolic and diastolic function of the systemic RV and the subpulmonary left ventricle (LV). Uni- and multivariate linear regression analyses were performed to identify independent echocardiographic determinants for NYHA class, maximal exercise capacity (V'O(2peak)) and NT-proBNP levels., Results: We found qualitative assessment of RV and LV function to be significantly associated with NYHA class (RV: β= 0.26; P = 0.05 and LV: β= 0.82; P < 0.01), V'O(2peak) (RV: β=-10.4; P < 0.05 and LV: β=-18.4; P < 0.05) and NT-proBNP levels (RV: β= 0.58; P < 0.01 and LV: β= 1.40; P < 0.001). Tricuspid annulus plane systolic excursion (TAPSE) was significantly associated with NYHA class (β=-0.92; P = 0.001), V'O(2peak) (β= 18.5; P = 0.05), and serum NT-proBNP levels (β=-1.00; P < 0.05). Associations between quantitative parameters of systolic subpulmonary LV function and clinical parameters were less distinct. We found no associations between RV and LV diastolic function and clinical parameters., Conclusions: Qualitative function of the systemic RV and the subpulmonary LV, and TAPSE, are determinants of clinical condition in patients with a systemic RV. These patients' clinical condition could not be determined by echocardiographically measured diastolic RV function, and systolic and diastolic LV function., (© 2010, Wiley Periodicals, Inc.)

Background: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established., Methods: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries. The primary end point is the change in right ventricular ejection fraction as measured by cardiovascular magnetic resonance or multidetector row cardiac computed tomography in case of pacemaker patients., Conclusion: This large prospective, double-blind, randomized, placebo-controlled trial will establish the role of angiotensin II receptor blockers (valsartan) in the treatment of patients with a systemic right ventricle., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Background: Previous studies demonstrated that ventricular response to stress cardiovascular magnetic resonance (CMR) is frequently abnormal in patients with a systemic right ventricle (RV). However, the clinical implications of these findings remained unknown. We sought to evaluate whether abnormal response to stress CMR predicts adverse outcome in patients with a systemic RV., Methods: Thirty-nine adult patients (54% male; mean age 26, range 18-65 years) with a systemic RV underwent stress CMR to determine the response of RV volumes and ejection fraction (EF). During follow-up, cardiac events, defined as hospitalization for heart failure, cardiac surgery, aborted cardiac arrest, or death, were recorded. The prognostic value of an abnormal response to stress, defined as lack of a decrease in RV end-systolic volume (ESV) or lack of an increase in RV EF, was assessed., Results: We frequently observed an abnormal response to stress, as RV ESV did not decrease in 17 patients (44%), and RV EF did not increase in 15 patients (38%). After a mean follow-up period of 8.1 years, 8 (21%) patients had reached the composite end point. The inability to decrease RV ESV during stress was predictive for cardiac events with a hazard ratio of 2.3 (95% CI 1.19-88.72, P = .034), as was the inability to increase RV EF with a hazard ratio of 2.3 (95% CI 1.31-81.59, P = .027)., Conclusions: Stress CMR potentially has important prognostic value in patients with a systemic RV. Patients with a systemic RV who show abnormal cardiac response to stress have a substantially higher risk of adverse outcome., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Background: Conflicting data have been published on the differences in cardiac response to exercise between patients with an atrially switched transposition of the great arteries (TGA), and patients with a congenitally corrected TGA (ccTGA). The purpose of our study was to evaluate the differences in cardiac response to exercise in these 2 patient groups with a systemic right ventricle (RV)., Methods: Thirty four patients (62% male; mean 35, range 21-69 years) with a systemic RV (20 with an atrially switched TGA and 14 with a ccTGA) were included. Exercise tests with Portapres measurements were performed to assess maximal exercise capacity (V'O(2peak)), cardiac index, stroke volume index, and heart rate augmentation. Cardiac function was assessed by Cardiovascular Magnetic Resonance or Computed Tomography, and serum NT-proBNP levels., Results: We found that both groups were able to significantly augment cardiac index during exercise. Cardiac index augmentation during exercise was positively related to V'O(2peak) (r=0.37, p<0.05), and independent of resting cardiac function. Although the increase in cardiac index during exercise was similar in both patients with TGA and with ccTGA, mechanisms to achieve this increase differed between groups. ccTGA patients increased both stroke volume and heart rate during exercise. Atrially switched TGA patients augmented heart rate during exercise, but failed to increase stroke volume., Conclusion: Mechanisms to achieve cardiac output augmentation differ between ccTGA patients and TGA patients. We suggest that therapeutic approaches should be tailored to the specific patient group to avoid counterproductive effects., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

Aims: Despite improvement in prognosis for ST-elevation myocardial infarction (STEMI) patients, mortality remains high in STEMI patients presenting with cardiogenic shock (CS). Right ventricular (RV) dysfunction is an established independent predictor for adverse prognosis in STEMI patients without CS. The purpose of our study was to determine the prognostic value of RV dysfunction on admission in STEMI patients presenting in CS., Methods and Results: Two hundred and ninety-two consecutive STEMI patients with CS on admission were treated by primary percutaneous coronary intervention (PCI) from January 1997 through March 2005. RV function was assessed by measurement of tricuspid annular plane systolic excursion (TAPSE) on early echocardiography in 184 of 292 patients. Right ventricular dysfunction was defined as a TAPSE of

Background: Delayed left ventricle (LV)-to-right ventricle (RV) peak shortening results in cardiac output reduction in patients with chronic thromboembolic hypertension (CTEPH) and other types of pulmonary arterial hypertension. Why the synchrony between LV and RV is lost is unknown. We hypothesized that RV electrophysiological remodeling, notably, conduction slowing and action potential prolongation, contribute to this loss in synchrony., Methods and Results: We conducted epicardial mapping during pulmonary endarterectomy in 26 patients with CTEPH and compared these findings with clinical, hemodynamic, and echocardiographic variables. We consecutively placed a multielectrode grid on the epicardium of the RV free wall and LV lateral wall. These regions corresponded to RV and LV areas where echocardiographic Doppler sample volumes were placed to measure RV-to-LV diastolic interventricular delay. RV and LV epicardial action potential duration was assessed by measuring activation-recovery interval. Onset of diastolic relaxation of RV free wall with respect to LV lateral wall (diastolic interventricular delay) was delayed by 38+/-31 ms in patients with CTEPH versus -12+/-13 ms in control subjects (P<0.001), because, in patients with CTEPH, RV completed electric activation later than LV (65+/-20 versus 44+/-7 ms, P<0.001) and epicardial action potential duration, as assessed by activation-recovery interval measurement, was longer in RV free wall than in LV lateral wall (253+/-29 versus 240+/-22 ms, P<0.001)., Conclusions: Additive effects of electrophysiological changes in RV, notably, conduction slowing and action potential prolongation, assessed by epicardial activation-recovery interval, contribute to diastolic interventricular delay in patients with CTEPH.

Background: The method used to delineate the boundary of the right ventricle (RV), relative to the trabeculations and papillary muscles in cardiovascular magnetic resonance (CMR) ventricular volume analysis, may matter more when these structures are hypertrophied than in individuals with normal cardiovascular anatomy. This study aimed to compare two methods of cavity delineation in patients with systemic RV., Methods: Twenty-nine patients (mean age 34.7 +/- 12.4 years) with a systemic RV (12 with congenitally corrected transposition of the great arteries (ccTGA) and 17 with atrially switched (TGA) underwent CMR. We compared measurements of systemic RV volumes and function using two analysis protocols. The RV trabeculations and papillary muscles were either included in the calculated blood volume, the boundary drawn immediately within the apparently compacted myocardial layer, or they were manually outlined and excluded. RV stroke volume (SV) calculated using each method was compared with corresponding left ventricular (LV) SV. Additionally, we compared the differences in analysis time, and in intra- and inter-observer variability between the two methods. Paired samples t-test was used to test for differences in volumes, function and analysis time between the two methods. Differences in intra- and inter-observer reproducibility were tested using an extension of the Bland-Altman method., Results: The inclusion of trabeculations and papillary muscles in the ventricular volume resulted in higher values for systemic RV end diastolic volume (mean difference 28.7 +/- 10.6 ml, p < 0.001) and for end systolic volume (mean difference 31.0 +/- 11.5 ml, p < 0.001). Values for ejection fraction were significantly lower (mean difference -7.4 +/- 3.9%, p < 0.001) if structures were included. LV SV did not differ significantly from RV SV for both analysis methods (p = NS). Including structures resulted in shorter analysis time (p < 0.001), and showed better inter-observer reproducibility for ejection fraction (p < 0.01)., Conclusion: The choice of method for systemic RV cavity delineation significantly affected volume measurements, given the CMR acquisition and analysis systems used. We recommend delineation outside the trabeculations for routine clinical measurements of systemic RV volumes as this approach took less time and gave more reproducible measurements.