Quality of Life Among Patients With Congenital Heart Disease After Valve Replacement.

Most studies concerning valve replacement in congenital heart disease (CHD) focus on surgical morbidity and mortality. However, with the increased life expectancy of these patients, the focus shifts to quality of life (QOL). The aim of this study was to report and compare the QOL of CHD patients after valve replacement with the general population and to find factors associated with QOL. In a multicenter cross-sectional observational study of adults with CHD, QOL was measured with the RAND-36 questionnaire (a health-related QOL questionnaire, with 8 domains scoring from 0 to 100; higher scores indicate a better QOL). Functional status was measured with exercise capacity testing. Uni- and multivariable linear regression was used to find associations with QOL. In total, 324 patients with CHD and a prosthetic valve were included in this study. CHD patients with a valve replacement scored significantly lower than the general population on the general health, vitality, and social functioning domains (P < 0.05). On the bodily pain domain, they scored significantly higher (less pain) (P < 0.001). Higher NYHA class was associated with a lower QOL for all domains, reflecting the importance of functional capacity. Other variables related to aspects of QOL were age, gender, exercise capacity, and employment status. Adult patients with CHD and a prosthetic valve have lower scores on the QOL domains general health, vitality, and social functioning as compared to the general population. NYHA class was negatively associated with all QOL domains. Health care professionals should be aware of these patterns in counseling patients.

Aortic valve prosthesis-patient mismatch and exercise capacity in adult patients with congenital heart disease.

OBJECTIVES: To report the prevalence of aortic valve prosthesis-patient mismatch (PPM) in an adult population with congenital heart disease (CHD) and its impact on exercise capacity. Adults with congenital heart disease (ACHD) with a history of aortic valve replacement may outgrow their prosthesis later in life. However, the prevalence and clinical consequences of aortic PPM in ACHD are presently unknown. METHODS: From the national Dutch Congenital Corvitia (CONCOR) registry, we identified 207 ACHD with an aortic valve prosthesis for this cross-sectional cohort study. Severe PPM was defined as an indexed effective orifice area ≤0.65 cm2/m2 and moderate PPM as an indexed orifice area ≤0.85 cm2/m2 measured using echocardiography. Exercise capacity was reported as percentage of predicted exercise capacity (PPEC). RESULTS: Of the 207 patients, 68% was male, 71% had a mechanical prosthesis and mean age at inclusion was 43.9 years ±11.4. The prevalence of PPM was 42%, comprising 23% severe PPM and 19% moderate PPM. Prevalence of PPM was higher in patients with mechanical prostheses (p<0.001). PPM was associated with poorer exercise capacity (mean PPEC 84% vs. 92%; p=0.048, mean difference =-8.3%, p=0.047). Mean follow-up was 2.6±1.1 years during which New York Heart Association (NYHA) class remained stable in most patients. PPM showed no significant effect on death or hospitalisation during follow-up (p=0.218). CONCLUSIONS: In this study we report a high prevalence (42%) of PPM in ACHD with an aortic valve prosthesis and an independent association of PPM with diminished exercise capacity.

Pulmonary regurgitant volume is superior to fraction using background-corrected phase contrast MRI in determining the severity of regurgitation in repaired tetralogy of Fallot.

In the assessment of pulmonary regurgitation (PR) using phase contrast MRI, phase offset errors affect the accuracy of flow. This study evaluated the use of automated background correction for phase offset in the quantification of PR fraction and volume in patients with repaired tetralogy of Fallot (TOF), and to assess its clinical impact. We retrospectively analyzed 203 cardiac MRI studies, performed on 1.5-T scanner. Pulmonary flow (Q(P)) and systemic flow (Q(S)) was assessed both with and without background correction. Non-corrected and corrected Q(P) was correlated with Q(S). PR was correlated with (1) indexed right ventricular end-diastolic volume (RVEDVi) and (2) with differential right and left ventricular stroke volumes (PR(SV)). Both PR fraction and volume showed major change after correction (-43 to +36% and -13 to +13 ml/m(2)). Corrected Q(P) and Q(S) were stronger correlated with each other than non-corrected Q(P) and Q(S) [r = 0.78 vs. 0.73 (p < 0.001)]. Both PR fraction and volume were stronger correlated with RVEDVi, compared to their non-corrected counterparts (p < 0.001). PR volume was stronger correlated with RVEDVi, compared to PR fraction [r = 0.74 vs. 0.69 (p < 0.001)]. When patients were divided according to PR severity, 12% of patients reclassified after correction. Background correction for phase offset significantly changed the quantification of PR. Non-corrected assessment of PR may result in the misclassification of patients. Our data suggest that the use of PR volume is favourable in the follow-up of patients with repaired TOF.

Pulmonary valve replacement: twenty-six years of experience with mechanical valvar prostheses.

BACKGROUND: Although the thromboembolic risk after pulmonary valve replacement (PVR) with mechanical valves is presumed to be high, recent studies suggest promising short-term and mid-term results. However, large studies reporting long-term mortality and valve-related complications are missing. METHODS: We describe valve-related complications in 66 patients with a mechanical pulmonary valvar prosthesis implanted between 1987 and 2013. RESULTS: Mean follow-up duration was 5.9 ± 4.8 years (median 4.9). Mean age at time of implantation was 35 ± 13 years. The most frequent underlying cardiac diagnosis was tetralogy of Fallot (77%). Valvar thrombosis or pannus was reported in 7 patients (10%) of which 4 in the setting of inadequate anticoagulation or pregnancy. Redo PVR was performed in 6 patients. Freedom from redo PVR in survivors after 5 and 10 years was 96% and 89%, respectively. Survival after 5 and 10 years was 91% and 81%, respectively. Main cause of death was end-stage heart failure. CONCLUSIONS: Success of PVR using mechanical valvar prostheses over 26 years was limited because of valvar thrombosis (often in the setting of pregnancy or incompliance with anticoagulation therapy) or pannus. Performance of mechanical prostheses in the pulmonary position may improve when valvar thrombosis is prevented by patient selection, avoiding mechanical valves in patients at increased risk of valvar thrombosis, and by strict compliance to anticoagulation therapy.

Pressure overloaded right ventricles: a multicenter study on the importance of trabeculae in RV function measured by CMR.

Cardiac magnetic resonance (CMR) imaging is the preferred method to measure right ventricular (RV) volumes and ejection fraction (RVEF). This study aimed to determine the impact of excluding trabeculae and papillary muscles on RV volumes and function in patients with RV pressure and/or volume overload and healthy controls and its reproducibility using semi-automatic software. Eighty patients (pulmonary hypertension, transposition of the great arteries after arterial switch operation and after atrial switch procedure and repaired Tetralogy of Fallot) and 20 controls underwent short-axis multislice cine CMR. End diastolic volume (EDV), end systolic volume (ESV), RV mass and RVEF were measured using 2 methods. First, manual contour tracing of RV endo- and epi-cardial borders was performed. Thereafter, trabeculae were excluded from the RV blood volume using semi-automatic pixel-intensity based software. Both methods were compared using a Student T test and 25 datasets were re-analyzed for reproducibility. Exclusion of trabeculae resulted in significantly decreased EDV; ranging from -5.7 ± 1.7 ml/m(2) in controls to -29.2 ± 6.6 ml/m(2) in patients after atrial switch procedure. RVEF significantly increased in all groups, ranging from an absolute increase of 3.4 ± 0.8 % in healthy controls to 10.1 ± 2.3 % in patients after atrial switch procedure. Interobserver agreement of method 2 was equal to method 1 for RVEDV, RVESV and RVEF and superior for RV mass. In patients with overloaded RVs exclusion of trabeculae from the blood volume results in a significant change in RV volumes, RVEF and RV mass. Exclusion of trabeculae is highly reproducible when semi-automatic pixel-intensity based software is used.

Long-term tricuspid valve prosthesis-related complications in patients with congenital heart disease.

OBJECTIVES: In patients with acquired valvar disease, morbidity and mortality rates after tricuspid valve replacement (TVR) are high. However, in adult patients with congenital heart disease, though data concerning outcome after TVR are scarce, even poorer results are suggested in patients with Ebstein anomaly. To investigate the applicability of these results to a broader array of congenital heart disease patients, we report the long-term follow-up of prosthesis-related complications, including re-replacement of patients with a tricuspid valve prosthesis and congenital heart disease. METHODS: From the Dutch Congenital Corvitia (CONCOR) registry, we identified 20 patients with a biological or mechanical tricuspid valve prosthesis implanted between 1977 and 2012 (total of 31 prostheses). We analysed the tricuspid valve-related complications and mortality. RESULTS: Ten patients with a median age of 16.2 years at the time of surgery (interquartile range 13.2-28.2 years) received a bioprosthesis while 10 patients with a median age of 36.4 years (interquartile range 14.0-47.0) at the time of surgery received a mechanical prosthesis (P = 0.28). During a mean follow-up of 14 years, 50% needed a re-replacement because of valve-related complications (e.g. valve degeneration or valve thrombosis). The yearly percentage of patients with valve-related complications was 4.2% in patients with a bioprosthesis and 2.7% in those with a mechanical prosthesis. Within 20 years of implantation, the median duration of event-free survival was significantly shorter in 3 patients with a prosthesis-patient mismatch (PPM; 1.0 year; interquartile range 0.01-2.6), compared with 7 without mismatch (8.0 years; interquartile range 5.1-12.3; P = 0.02). CONCLUSIONS: Compared with previous literature on acquired valvar disease, we found a higher incidence of valve-related complications in patients with congenital heart disease that was unrelated to prosthesis material. Our data suggest that PPM may have a negative effect on the event-free interval.

Effect of right ventricular outflow tract obstruction on right ventricular volumes and exercise capacity in patients with repaired tetralogy of fallot.

Patients with tetralogy of Fallot and combined right ventricular outflow tract obstruction (RVOTO) and pulmonary regurgitation (PR) have a less dilated right ventricular (RV) and better RV function compared with patients without RVOTO. It is not known whether RVOTO is associated with improved exercise capacity. We compared cardiac magnetic resonance imaging, echocardiography, and exercise tests in 12 patients with RVOTO (Doppler peak RVOT gradient ≥30 mm Hg) and 30 patients without RVOTO. RV end-systolic and end-diastolic volumes were smaller in patients with RVOTO compared with patients without RVOTO (50 ± 16 vs 64 ± 18 ml/m(2) and 117 ± 24 vs 135 ± 28 ml/m(2), respectively) and patients with RVOTO had a higher RV mass (52 ± 14 vs 42 ± 11 ml/m(2)), p <0.05. RV ejection fraction was marginally significantly different between both groups (58 ± 8% vs 53 ± 7%), p = 0.051. Degree of PR, left ventricular volumes, and function did not differ significantly between both groups. Peak oxygen uptake in patients with RVOTO was significantly lower (25 ± 3 vs 32 ± 8 ml/kg/min) than in patients without RVOTO, as was the percentage of predicted peak oxygen uptake (63 ± 7% vs 79 ± 14%), p <0.001. Multivariate analysis showed that the peak RVOT gradient was the only independent predictor of exercise capacity. In conclusion, exercise capacity is lower in patients with RVOTO compared with those without RVOTO despite a less dilated RV and comparable degree of PR. Therefore, exercise capacity may be of importance and should additionally be taken in consideration to RV volumes and function in patients with tetralogy of Fallot and PR.

Improved cardiac MRI volume measurements in patients with tetralogy of Fallot by independent end-systolic and end-diastolic phase selection.

OBJECTIVES: To investigate to what extent cardiac MRI derived measurements of right ventricular (RV) volumes using the left ventricular (LV) end-systolic and end-diastolic frame misrepresent RV end-systolic and end-diastolic volumes in patients with tetralogy of Fallot (ToF) and a right bundle branch block. METHODS: Sixty-five cardiac MRI scans of patients with ToF and a right bundle branch block, and 50 cardiac MRI scans of control subjects were analyzed. RV volumes and function using the end-systolic and end-diastolic frame of the RV were compared to using the end-systolic and end-diastolic frame of the LV. RESULTS: Timing of the RV end-systolic frame was delayed compared to the LV end-systolic frame in 94% of patients with ToF and in 50% of control subjects. RV end-systolic volume using the RV end-systolic instead of LV end-systolic frame was smaller in ToF (median -3.3 ml/m(2), interquartile range -1.9 to -5.6 ml/m(2); p<0.001) and close to unchanged in control subjects. Using the RV instead of LV end-systolic and end-diastolic frame hardly affected RV end-diastolic volumes in both groups and ejection fraction in control subjects (54±4%, both methods), while increasing ejection fraction from 45±7% to 48±7% for patients with ToF (p<0.001). QRS duration correlated positively with the changes in the RV end-systolic volume (p<0.001) and RV ejection fraction obtained in ToF patients when using the RV instead of the LV end-systolic and end-diastolic frame (p = 0.004). CONCLUSION: For clinical decision making in ToF patients RV volumes derived from cardiac MRI should be measured in the end-systolic frame of the RV instead of the LV.

Impact of right ventricular endocardial trabeculae on volumes and function assessed by CMR in patients with tetralogy of Fallot.

The objective of this study was to assess the impact of right ventricular (RV) trabeculae and papillary muscles on measured volumes and function assessed by cardiovascular magnetic resonance imaging in patients with repaired tetralogy of Fallot. Sixty-five patients with repaired tetralogy of Fallot underwent routine cardiovascular magnetic resonance imaging. Endocardial and epicardial contours were drawn manually and included trabeculae and papillary muscles in the blood volume. Semi-automatic threshold-based segmentation software excluded these structures. Both methods were compared in terms of end-diastolic, end-systolic and stroke volume, ejection fraction and mass. Observer agreement was determined for all measures. Exclusion of trabeculae and papillary muscle in the RV blood volume decreased measured RV end-diastolic volume by 15 % (from 140 ± 35 to 120 ± 32 ml/m(2)) compared to inclusion, end-systolic volume by 21 % (from 74 ± 23 to 59 ± 20 ml/m(2)), stroke volume by 9 % (from 66 ± 16 to 60 ± 16 ml/m(2)) and relatively increased ejection fraction by 7 % (from 48 ± 7 to 51 ± 8 %) and end-diastolic mass by 79 % (from 28 ± 7 to 51 ± 10 g/m(2)), p < .01. Excluding trabeculae and papillary muscle resulted in an improved interobserver agreement of RV mass compared to including these structures (coefficient of agreement of 87 versus 78 %, p < .01). Trabeculae and papillary muscle significantly affect measured RV volumes, function and mass. Semi-automatic threshold-based segmentation software can reliably exclude trabeculae and papillary muscles from the RV blood volume.

Improving the reproducibility of MR-derived left ventricular volume and function measurements with a semi-automatic threshold-based segmentation algorithm.

To validate a novel semi-automatic segmentation algorithm for MR-derived volume and function measurements by comparing it with the standard method of manual contour tracing. The new algorithms excludes papillary muscles and trabeculae from the blood pool, while the manual approach includes these objects in the blood pool. An epicardial contour served as input for both methods. Multiphase 2D steady-state free precession short axis images were acquired in 12 subjects with normal heart function and in a dynamic anthropomorphic heart phantom on a 1.5 T MR system. In the heart phantom, manually and semi-automatically measured cardiac parameters were compared to the true end-diastolic volume (EDV), end-systolic volume (ESV) and ejection fraction (EF). In the subjects, the semi-automatic method was compared to manual contouring in terms of difference in measured EDV, ESV, EF and myocardial volume (MV). For all measures, intra- and inter-observer agreement was determined. In the heart phantom, EDV and ESV were underestimated for both the semi-automatic. As the papillary muscles were excluded from the blood pool with the semi-automatic method, EDV and ESV were approximately 20 ml lower in the patients, whereas EF was approximately 16 % higher. Intra- and inter-observer agreement was overall improved with the semi-automatic method compared to the manual method. Correlation between manual and semi-automatic measurements was high (EDV: R = 0.99, ESV: R = 0.96; EF: R = 0.80, MV: R = 0.99). The semi-automatic method could exclude endoluminal muscular structures from the blood volume with significantly improved intra- and inter-observer variabilities in cardiac function measurements compared to the conventional, manual method, which includes endoluminal structures in the blood volume.