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Improved exercise performance and quality of life after percutaneous pulmonary valve implantation
International Journal of Cardiology 173 (2014) 388–392
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International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Improved exercise performance and quality of life after percutaneous pulmonary valve implantation Jan Müller ⁎, Andrea Engelhardt, Sohrab Fratz, Andreas Eicken, Peter Ewert, Alfred Hager Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Germany
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Article history: Received 20 June 2013 Received in revised form 20 January 2014 Accepted 1 March 2014 Available online 5 March 2014 Keywords: Congenital heart disease PPVI Melody® Sapien® Cardiopulmonary exercise test Quality of life Intervention Percutaneous pulmonary valve implantation
a b s t r a c t Objective: Percutaneous pulmonary valve implantation (PPVI) has emerged as a new approach to treat patients with dysfunctional pulmonary valve conduits. Short- and midterm results have outlined hemodynamic improvements and increase in exercise performance. However, there is a lack of knowledge about quality of life at short term follow-up. Patients and methods: From July 2007 to March 2013, we investigated 59 patients (17 female; median age 22.8 years) undergoing PPVI in our institution. 46 had predominant pulmonary stenosis (PS) and 13 had predominant pulmonary regurgitation (PR). They answered the quality of life questionnaire (SF-36) and underwent a cardiopulmonary exercise test and Cardiovascular Magnetic Resonance before and 6 months after PPVI. Results: Peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p b .0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Improvements were seen in both, the PS (71.9 to 78.3 %predicted; p b .0001) and PR (62.7 to 73.0 %predicted; p b .0001) group. Self-estimated quality of life was good already before PPVI but increased in almost all domains 6 months after PPVI in PS and PR group. Significant improvements developed in “physical function”, “general health perception” and “health transition” in both groups, and “physical role functioning”, “vitality” and “mental health” only in the PS group. Conclusions: In patients with dysfunctional pulmonary valve conduits exercise performance and quality of life improve substantially 6 months after successful percutaneous pulmonary valve implantation. © 2014 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Patients and methods 2.1. Study subjects
Percutaneous pulmonary valve implantation (PPVI) is a new treatment option for selected patients with dysfunctional right ventricular to pulmonary artery conduits [1]. During PPVI, the degenerated pulmonary valve is overstented using either a, in a stent mounted, bovine jugular vein (Melody®) or a bovine pericardial valve (Sapien®). Short and midterm follow-ups of patients after PPVI have shown improved and sustained hemodynamics and an increase in exercise capacity, especially in those patients with predominant pulmonary stenosis before intervention [2–5]. However, there is a lack of knowledge about quality of life changes with this procedure and how the patients themselves report the results of the procedure.
⁎ Corresponding author at: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr, 36, D-80636 München, Germany. Tel.: +49 89 1218 3009; fax: +49 89 1218 3003. E-mail address: [email protected] (J. Müller).
http://dx.doi.org/10.1016/j.ijcard.2014.03.002 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
rFrom July 2007 to March 2013 we prospectively investigated all 128 consecutive patients undergoing PPVI at our institution (Fig. 1). In 20 patients the 6 month follow-up is still pending. 22 patients were lost for the initial or follow-up investigation (11 patients from abroad). Two patients died. In those 84 patients eligible at follow-up 18 patients were younger than 14 years and not included into the study because the quality of life instrument is not validated for children. Furthermore, 7 patients with syndromes (Down syndrome, 22q11) or psychomotor disability had to be excluded. Our inclusion criteria for PPVI were described previously [2]. In total 59 patients (17 female, median: 22.8 (1st quartile: 17.0; 3rd quartile: 29.7) years) received a quality of life assessment and a cardiopulmonary exercise test (CPET) 1 (1; 20) day before PPVI and 6.2 (5.8; 6.7) months after PPVI (Tables 1a and 1b). The indication for PPVI was isolated stenosis in 39 patients (peak instantaneous pressure gradient N50 mm Hg at echocardiography) and isolated pulmonary regurgitation (N25% at cardiovascular magnetic resonance) in 9 patients. A mixed conduit dysfunction was present in 11 patients. From those 11 patients seven were assigned to the stenosis group and four to the regurgitation group according to their predominant indication derived from Cardiovascular Magnetic Resonance or echocardiography (Tables 1a and 1b). Of the total 59 patients, 30 patients had repaired tetralogy of Fallot followed by a conduit replacement, 10 had a common arterial trunk with a conduit repair, 2 pulmonary stenosis with a preceding conduit repair, 7 aortic stenosis with preceding Ross procedure, 3 patients with congenitally corrected transposition of the great arteries, and 7 patients with transposition of the great arteries after Rastelli procedure.
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2.4. Cardiopulmonary exercise test (CPET) All patients underwent a symptom limited cardiopulmonary exercise test on a bicycle ergometer in an upright position as previously described [8]. In short, after a 3 min rest to define baseline, patients had a 3 min warm up without load, followed by a ramp wise increase of load with 10, 15, 20, or 30 W/min depending on the expected individual physical capacity estimated by the investigator. With this protocol a cycling duration of about 8 to 12 min after warm up should be reached. The end of the CPET was marked by symptom limitation and was followed by a 5-min recovery period, with the first 2–3 min cycling with minimal load and 2 min rest. The exercise test featured a breath-by-breath gas exchange analysis using a metabolic chart (Vmax 229, SensorMedics, Viasys Healthcare, Yorba Linda, California). Peak oxygen uptake (V˙ O2 ) was defined as the highest mean uptake of any 30-s time interval during exercise. Reference values for age, body mass, body height, and gender, expressed in “% predicted” were calculated like previously described [9]. Ventilatory threshold was estimated manually with the V-slope method according to Beaver et al. [10] and corrected by the V˙ E = V˙ CO2 curve. Ventilatory efficiency was displayed as V˙ E =V˙ CO2 slope confined to the linear part of the curve, excluding values beyond the respiratory compensation point. Peak O2 pulse was defined as peak V˙ O2 divided by peak heart rate from the same time interval. The reference value was calculated from the peak V˙ O2 reference value divided by the expected peak heart rate estimated by (220 − age) × 0.925 [11]. 2.5. Quality of life CPET: cardiopulmonary exercise test
Fig. 1. Patient inclusion. CPET: cardiopulmonary exercise test.
The study was in accordance with the Declaration of Helsinki (revision 2008). Patients agreed to the anonymous publication of their data. Some of the patients were also included in the previous report on the feasibility and early hemodynamic results of PPVI [2]. 2.2. Echocardiography Echocardiography was performed on a VingMed Vivid 5® (GE Healthcare). As part of a standardized investigation the right ventricular outflow tract was visualized in a 2-D Bmode enhanced by color encoded Doppler signal in the parasternal short axis view, in an apical long axis view, and from the sternal notch. The maximal and the mean flow was measured by cw Doppler signal and converted to a peak and mean instantaneous gradient by the Bernoulli equation, respectively. 2.3. Cardiovascular magnetic resonance Flow through the pulmonary artery was measured as previously described [6,7]. In brief, a conventional phase-sensitive gradient echo sequence was used in a double-oblique plane perpendicular to the dominant flow direction in the main pulmonary artery to measure antegrade and retrograde flow volumes. The following acquisition parameters were used for phase velocity magnetic resonance: 25/6 ms; slice thickness, 5 mm; flip angle, 30°; receiver bandwidth, 31.25 kHz; rectangular field of view, 260 to 400 mm; matrix, 256 × 256; and number of excitations, 3. At the beginning of each study, velocity encoding, according to the clinical expectation, was chosen. Immediately after acquiring phase velocity magnetic resonance flow maps, they were checked for aliasing. If aliasing was detected, the scan was repeated using a higher velocity encoding. This approach resulted in final velocity encodings between 2.0 and 4.5 m/s. Data were reconstructed to provide 30 magnitude (anatomic) and phase (velocity-mapped) images per cardiac cycle. Data analysis was performed offline using commercially available software (Argus®, Siemens Healthcare, Erlangen, Germany). In three patients CMR could not be conducted because of implanted pacemaker or ICD.
The medical outcome study 36 item short form (SF-36) was used. It has an acceptable internal consistency and has proven useful in various specialties of medicine without any bias for symptoms of a specific disease [12,13]. The SF-36 measures eight health constructs with scores ranging from 0 (worst) to 100 (best) using subscales with two to 10 items per subscale and one single item about health transition. The dimensions are physical functioning (the extent to which health limits daily physical activities), role functioning physical (the extent of which physical health interferes with work or other daily activities), bodily pain (the extent of bodily pain and its effect on daily life), general health perception, vitality, social functioning (the extent to which health interferes with social activities), role functioning emotional (the extent to which emotional problems interfere with daily activities), and mental health (a rough score for depression, affect, anxiety). A single item component assesses health transition (health changes in the past year). In this study the German version of the self-report form with a window of 4 weeks was used [12,13]. 2.6. Data analyses Since data were skewed, all descriptive data were expressed in median values and interquartile ranges (Q1; Q3). Wilcoxon's signed rank tests and chi-square tests were calculated to find differences between patient variables before and 6 months after PPVI. Non-parametric Spearman rank correlations were calculated to find relations of the quality of life variables and the hemodynamic variables. Mann–Whitney-U tests were calculated to find differences between patients with initially predominant pulmonary stenosis and pulmonary regurgitation. All analyses were performed using SPSS 20.0 software (IBM Inc, Armonk, New York, USA). P-values b0.05 in a two-sided analysis were considered significant.
3. Results 3.1. Hemodynamics Hemodynamic parameters according to the PS and PR group are displayed in Tables 1a and 2a). Cardiovascular magnetic resonance revealed no substantial pulmonary regurgitation after PPVI (b15% in all patients) and
Table 1a Study subjects and hemodynamic parameters of the 46 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary stenosis group.
Sex Age Body mass index Peak instantaneous pressure gradient Mean instantaneous pressure gradient Pulmonary regurgitation Right ventricular EF Right ventricular EDVI Right ventricular EDV Left ventricular EDVI RVEDV: LVEDV
♂/♀ years kg/m2 mm Hg mm Hg % % ml/m2 ml ml –
n
Before PPVI
Six months after PPVI
p-value*
46 46 46 46 37 43 43 43 43 43 43
11/35 21.2 (16.8; 28.6) 22.6 (20.1; 27.5) 70 (53; 84) 39 (28; 48) 7 (1; 12) 52 (42; 61) 90 (72; 113) 162 (111; 215) 135 (102; 159) 1.21 (0.96; 1.37)
11/35 21.8 (17.5; 29.1) 23.0 (20.4; 27.6) 28 (23; 37) 15 (11; 20) 1 (0; 2) 56 (47; 61) 82 (67; 100) 152 (116; 200) 144 (120; 165) 1.01 (0.90; 1.33)
– – .042 b.0001 b.0001 b.0001 .075 .049 .046 .006 b.0001
p-values <0.05 were displayed in bold. EF: ejection fraction, EDVI: end-diastolic volume index; EDV: end-diastolic volume.
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Table 1b Exercise performance of the 46 patients before and 6 months after percutaneous pulmonary valve implantation in the pulmonary stenosis group. p-values <0.05 were displayed in bold.
PeakV˙ O2 Ventilatory threshold Peak O2 pulse Peak work load V˙ E =V˙ CO2 slope Peak heart rate Systolic blood pressure at peak exercise SpO2 at peak exercise Respiratory exchange ratio at peak exercise
ml/min/kg % predicted ml/min/kg % predicted W W/kg – bpm mm Hg % –
n
Before PPVI
Six months after PPVI
p-value*
46 46 44 46 46 46 46 46 45 41 46
28.2 (20.8; 34.4) 71.9 (56.7; 86.0) 16.2 (12.4; 18.6) 81.1 (70.5; 93.2) 148.5 (109.5; 192.0) 2.2 (1.9; 2.7) 28.4 (24.6; 32.8) 170 (150; 184) 164 (144; 180) 97.0 (94.0; 98.5) 1.10 (1.06; 1.15)
30.1 (22.7; 35.3) 78.3 (69.3; 93.3) 16.6 (13.1;23.0) 97.1 (80.0; 116.3) 159.0 (118.0; 214.7) 2.3 (1.9; 3.0) 25.7 (22.9; 28.9) 172 (140; 186) 167 (145; 184) 96.0 (93.0; 98.0) 1.09 (1.04; 1.13)
.001 b.0001 .017 b.0001 b.0001 .023 b.0001 .689 .648 .312 .575
echocardiographic pressure gradients improved significantly 6 months after PPVI (Tables 1a and 1b). 3.2. Exercise capacity The results of the CPET are displayed in detail in Tables 1b and 2b. Respiratory exchange ratio showed adequate exhaustion at the end of exercise without a significant difference between the two examinations 1.09 (1.05; 1.13) before vs. 1.10 (1.06; 1.15) after PPV (p = .993). For the total group, peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p b .0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Primarily, the increase in peakV˙O2 (%predicted) was present in all subgroups without any difference between those with leading pulmonary stenosis (n = 46, p b .0001) or those with leading pulmonary regurgitation (n = 13, p b .0001; comparison between groups p = .228). In addition, there was no correlation of improvements in peak V˙O2 and the reduction of pulmonary stenosis (r = −.054, p = .690) or regurgitation (r = −.096, p = .518). 3.3. Quality of life Six patients were excluded due to language barrier (n = 6). Self-estimated quality of life improved in almost all domains in both groups 6 months after intervention for PPVI (Table 3). Six months after PPVI 16 patients (30%) rated their self-reported health condition in the question on health transition similar to that before PPVI, 27 (51%) better, and 10 (19%) much better. Most improvements were seen in the more physical domains of quality of life, where the patients report on improvements regarding physical concerns in their daily life. Significant improvements were
found in “physical function”, “general health perception” and “health transition” in both groups, and “physical role functioning”, “vitality” and “mental health” only in the PS group (Table 3). Improvements in quality of life were not associated with any exercise or hemodynamic variable.
4. Discussion This study showed that patients profit from percutaneous pulmonary valve implantation not only with regard to hemodynamics and exercise performance, but also with regard to quality of life. In concordance to recent reports [2–5] pulmonary regurgitation was reduced to almost zero after PPVI and pressure gradients significantly improved. This proves the concept that percutaneous pulmonary valve implantation is feasible and restores pulmonary conduit function successfully. However, several studies [3–5] have suggested that these hemodynamic improvements cannot be automatically translated in an improvement of exercise capacity. They report on no improvement in standard measures of exercise performance in the whole study group of patient with PPVI. In the subgroups, however, where patients with predominant stenosis were compared with those with predominant regurgitation [3,4], significant improvement in peak oxygen uptake was confined to patients that were scheduled for PPVI with predominant pulmonary stenosis. Patients admitted to PPVI because of pulmonary regurgitation did not profit from intervention with regard to their exercise capacity [3,4]. Moreover, the reduction in right ventricular outflow tract gradient was the only predictor of improved peak oxygen uptake [3]. Our study could not confirm these results. We saw improvements in all indication groups for PPVI, even in the small subgroup of patients with predominantly pulmonary regurgitation. As a further result of the relief of the stenosis, O2 pulse, as a surrogate of stroke volume, improved due to the reduction in afterload after PPVI
Table 2a Study subjects and hemodynamic parameters of the 13 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary regurgitation group.
Sex Age Body mass index Peak instantaneous pressure gradient Mean instantaneous pressure gradient Pulmonary regurgitation Right ventricular EF Right vventricular EDVI Right ventricular EDV Left ventricular EDV RVEDV:LVEDV
♂/♀ years kg/m2 mm Hg mm Hg % % ml/m2 ml ml –
n
Before PPVI
Six months after PPVI
p-value*
13 13 13 11 11 11 11 11 11 11 11
6/7 25.9 (19.7; 29.9) 21.9 (17.9; 24.5) 44 (26; 57) 31 (18; 36) 35 (24; 41) 46 (37; 52) 112 (103; 164) 206 (187; 298) 136 (108; 157) 1.61 (1.33; 2.01)
6/7 26.4 (20.2; 30.7) 21.9 (19.7; 24.4) 26 (20; 30) 14 (11. 17) 2 (1; 5) 50 (36; 52) 92 (81; 113) 169 (144; 213) 142 (118; 169) 1.21 (0.96; 1,37)
– b.0001 .859 b.0001 b.0001 b.0001 .553 .005 .005 .192 .005
p-values <0.05 were displayed in bold. EF: ejection fraction, EDVI: end-diastolic volume index; EDV: end-diastolic volume.
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Table 2b Exercise performance of the 13 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary regurgitation group. p-values <0.05 were displayed in bold.
PeakV˙ O2 Ventilatory threshold Peak O2 pulse Peak work load V˙ E =V˙CO2 slope Peak heart rate Systolic blood pressure at peak exercise SpO2 at peak exercise Respiratory exchange ratio at peak exercise
ml/min/kg % predicted ml/min/kg % predicted W W/kg – bpm mm Hg % –
n
Before PPVI
Six months after PPVI
p-value*
13 13 13 13 13 13 13 13 13 12 13
22.1 (16.5; 32.5) 62.7 (54.4; 70.1) 14.7 (10.0; 20.5) 72.4 (55.6; 78.9) 125.0 (97.5; 187.0) 2.1 (1.5; 2.9) 27.4 (24.9; 31.4) 169 (154; 178) 144 (130; 162) 97.0 (92.3; 98.8) 1.07 (1.05; 1.15)
24.8 (22.1; 33.8) 73.0 (64.6; 91.6) 16.5 (11.7; 20.2) 92.7 (80.4; 100.6) 145.0 (119.0; 191.0) 2.3 (1.8; 2.7) 26.1 (22.4; 29.2) 171 (156; 183) 160 (148; 180) 96.5 (94.3; 98.0) 1.10 (1.07; 1.16)
.039 b.0001 .367 .005 .016 .152 .069 .115 .039 .765 .209
[3]. In addition, the merit of PPVI is mirrored more in the improvement of ventilatory efficiency, which is a relevant prognostic risk factor in various cardiac conditions [14–17]. This improvement in ventilatory efficiency is probably due to better pulmonary perfusion which is a result of an increase in right ventricular output [5]. In agreement to Lurz et al. [3,4] we found these improvements in V˙ E =V˙ CO2 slope in our cohort, dominated by patients with pulmonary stenosis. However, improvements in V˙ E =V˙ CO2 slope, albeit not significant, were also seen in those patients with pulmonary regurgitation [3–5].
4.1. Quality of life Many studies have focused on quality of life in patients with CHD [9,18–24], but there are only few reports [25,26] with a longitudinal design. For PPVI, the outcome in quality of life is not known at all. In our study, self-reported quality of life was good already before PPVI (Table 3). The patients in our cohort seemed to be only very little compromised by the substantial pulmonary stenosis or pulmonary regurgitation. Although showing good quality of life, 6 months after PPVI, patients rated their quality of life in almost all quality of life domains better than before the intervention with the most significant improvements in the physical domains. Those improvements hold true for patients with pulmonary stenosis as well as pulmonary regurgitation. However, improvements in the latter group were limited by the small sample size and the general good quality of life before PPVI that permits less room for improvement. Albeit improvements in hemodynamics, exercise capacity, and quality of life happened in parallel with PPVI, the improvements in quality of life, surprisingly, did not correlate with any of the investigated hemodynamic or exercise variables. This lack of correlation has already previously been observed by us in a mixed population of patients with different forms of
congenital heart disease [25]. In this data set patients after interventions had an increase in quality of life that is inadequate for their changes in exercise capacity (unpublished data). Now, this is confirmed by the current study. It seems that the subjective rating of the patient whether or not the treatment was a success does not primarily depend on hemodynamic or exercise variables. Maybe the patients simply like the concept that a cardiac problem that could be only solved by surgery a few years before, can now be treated in the catheter laboratory, needing only 2–3 days of hospitalization, and showing an improved general condition already a few days after the procedure. However, this speculation does not diminish the primary result of the study that, in addition to an improved hemodynamic situation and exercise performance 6 months after PPVI, patients benefit from PPVI also with regard to their quality of life.
5. Limitation The whole study group was biased by the composition of our study group, which was driven by the clinical decision which patient was regarded eligible for PPVI. This resulted in a highly selected group of patients with most of the patients suffering from pulmonary stenosis. This reduces the power of the subgroup analyses for patients with pulmonary regurgitation substantially.
Conflict of interests P. Ewert is proctor for the Melody Valve® (Medtronic) and the Edwards Sapien Pulmonic Valve® (Edwards Lifescience). A. Eicken is proctor for the Melody Valve® (Medtronic). A. Hager has received speaker's honoraries from Medtronic.
Table 3 Quality of life of the 53 patients before and 6 months after percutaneous pulmonary valve implantation according to diagnostic subgroups. Pulmonary stenosis (n = 40)
Physical function Physical role functioning Bodily pain General health perceptions Vitality Social role functioning Emotional role functioning Mental health Health transition
Pulmonary regurgitation (n = 13)
Before PPVI
Six months after PPVI
p-values*
Before PPVI
Six months after PPVI
p-values*
90 (70; 95) 100 (75;100) 100 (92; 100) 72 (56; 82) 65 (55; 75) 100 (75;100) 100 (100; 100) 84 (70; 88) 50 (25; 50)
95 (90; 100) 100 (100; 100) 100 (100; 100) 77 (66; 88) 70 (64; 80) 100 (100;100) 100 (100; 100) 86 (75; 92) 75 (50; 75)
b.0001 .003 .091 .035 .039 .079 .979 .039 b.0001
85 (57; 93) 100 (75;100) 100 (100; 100) 77 (51; 87) 60 (36; 84) 100 (91;100) 100 (100; 100) 82 (73; 91) 25 (25; 50)
95 (88; 100) 100 (100; 100) 100 (100; 100) 92 (62; 97) 75 (48; 88) 100 (81;100) 100 (100; 100) 88 (62; 94) 75 (63; 100)
.005 .705 .180 .059 .301 .453 .317 .421 .003
p-values <0.05 were displayed in bold. *comparing the data by a paired Wilcoxon test. PPVI: percutaneous pulmonary valve implantation.
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International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Improved exercise performance and quality of life after percutaneous pulmonary valve implantation Jan Müller ⁎, Andrea Engelhardt, Sohrab Fratz, Andreas Eicken, Peter Ewert, Alfred Hager Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Germany
a r t i c l e
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Article history: Received 20 June 2013 Received in revised form 20 January 2014 Accepted 1 March 2014 Available online 5 March 2014 Keywords: Congenital heart disease PPVI Melody® Sapien® Cardiopulmonary exercise test Quality of life Intervention Percutaneous pulmonary valve implantation
a b s t r a c t Objective: Percutaneous pulmonary valve implantation (PPVI) has emerged as a new approach to treat patients with dysfunctional pulmonary valve conduits. Short- and midterm results have outlined hemodynamic improvements and increase in exercise performance. However, there is a lack of knowledge about quality of life at short term follow-up. Patients and methods: From July 2007 to March 2013, we investigated 59 patients (17 female; median age 22.8 years) undergoing PPVI in our institution. 46 had predominant pulmonary stenosis (PS) and 13 had predominant pulmonary regurgitation (PR). They answered the quality of life questionnaire (SF-36) and underwent a cardiopulmonary exercise test and Cardiovascular Magnetic Resonance before and 6 months after PPVI. Results: Peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p b .0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Improvements were seen in both, the PS (71.9 to 78.3 %predicted; p b .0001) and PR (62.7 to 73.0 %predicted; p b .0001) group. Self-estimated quality of life was good already before PPVI but increased in almost all domains 6 months after PPVI in PS and PR group. Significant improvements developed in “physical function”, “general health perception” and “health transition” in both groups, and “physical role functioning”, “vitality” and “mental health” only in the PS group. Conclusions: In patients with dysfunctional pulmonary valve conduits exercise performance and quality of life improve substantially 6 months after successful percutaneous pulmonary valve implantation. © 2014 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Patients and methods 2.1. Study subjects
Percutaneous pulmonary valve implantation (PPVI) is a new treatment option for selected patients with dysfunctional right ventricular to pulmonary artery conduits [1]. During PPVI, the degenerated pulmonary valve is overstented using either a, in a stent mounted, bovine jugular vein (Melody®) or a bovine pericardial valve (Sapien®). Short and midterm follow-ups of patients after PPVI have shown improved and sustained hemodynamics and an increase in exercise capacity, especially in those patients with predominant pulmonary stenosis before intervention [2–5]. However, there is a lack of knowledge about quality of life changes with this procedure and how the patients themselves report the results of the procedure.
⁎ Corresponding author at: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr, 36, D-80636 München, Germany. Tel.: +49 89 1218 3009; fax: +49 89 1218 3003. E-mail address: [email protected] (J. Müller).
http://dx.doi.org/10.1016/j.ijcard.2014.03.002 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
rFrom July 2007 to March 2013 we prospectively investigated all 128 consecutive patients undergoing PPVI at our institution (Fig. 1). In 20 patients the 6 month follow-up is still pending. 22 patients were lost for the initial or follow-up investigation (11 patients from abroad). Two patients died. In those 84 patients eligible at follow-up 18 patients were younger than 14 years and not included into the study because the quality of life instrument is not validated for children. Furthermore, 7 patients with syndromes (Down syndrome, 22q11) or psychomotor disability had to be excluded. Our inclusion criteria for PPVI were described previously [2]. In total 59 patients (17 female, median: 22.8 (1st quartile: 17.0; 3rd quartile: 29.7) years) received a quality of life assessment and a cardiopulmonary exercise test (CPET) 1 (1; 20) day before PPVI and 6.2 (5.8; 6.7) months after PPVI (Tables 1a and 1b). The indication for PPVI was isolated stenosis in 39 patients (peak instantaneous pressure gradient N50 mm Hg at echocardiography) and isolated pulmonary regurgitation (N25% at cardiovascular magnetic resonance) in 9 patients. A mixed conduit dysfunction was present in 11 patients. From those 11 patients seven were assigned to the stenosis group and four to the regurgitation group according to their predominant indication derived from Cardiovascular Magnetic Resonance or echocardiography (Tables 1a and 1b). Of the total 59 patients, 30 patients had repaired tetralogy of Fallot followed by a conduit replacement, 10 had a common arterial trunk with a conduit repair, 2 pulmonary stenosis with a preceding conduit repair, 7 aortic stenosis with preceding Ross procedure, 3 patients with congenitally corrected transposition of the great arteries, and 7 patients with transposition of the great arteries after Rastelli procedure.
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2.4. Cardiopulmonary exercise test (CPET) All patients underwent a symptom limited cardiopulmonary exercise test on a bicycle ergometer in an upright position as previously described [8]. In short, after a 3 min rest to define baseline, patients had a 3 min warm up without load, followed by a ramp wise increase of load with 10, 15, 20, or 30 W/min depending on the expected individual physical capacity estimated by the investigator. With this protocol a cycling duration of about 8 to 12 min after warm up should be reached. The end of the CPET was marked by symptom limitation and was followed by a 5-min recovery period, with the first 2–3 min cycling with minimal load and 2 min rest. The exercise test featured a breath-by-breath gas exchange analysis using a metabolic chart (Vmax 229, SensorMedics, Viasys Healthcare, Yorba Linda, California). Peak oxygen uptake (V˙ O2 ) was defined as the highest mean uptake of any 30-s time interval during exercise. Reference values for age, body mass, body height, and gender, expressed in “% predicted” were calculated like previously described [9]. Ventilatory threshold was estimated manually with the V-slope method according to Beaver et al. [10] and corrected by the V˙ E = V˙ CO2 curve. Ventilatory efficiency was displayed as V˙ E =V˙ CO2 slope confined to the linear part of the curve, excluding values beyond the respiratory compensation point. Peak O2 pulse was defined as peak V˙ O2 divided by peak heart rate from the same time interval. The reference value was calculated from the peak V˙ O2 reference value divided by the expected peak heart rate estimated by (220 − age) × 0.925 [11]. 2.5. Quality of life CPET: cardiopulmonary exercise test
Fig. 1. Patient inclusion. CPET: cardiopulmonary exercise test.
The study was in accordance with the Declaration of Helsinki (revision 2008). Patients agreed to the anonymous publication of their data. Some of the patients were also included in the previous report on the feasibility and early hemodynamic results of PPVI [2]. 2.2. Echocardiography Echocardiography was performed on a VingMed Vivid 5® (GE Healthcare). As part of a standardized investigation the right ventricular outflow tract was visualized in a 2-D Bmode enhanced by color encoded Doppler signal in the parasternal short axis view, in an apical long axis view, and from the sternal notch. The maximal and the mean flow was measured by cw Doppler signal and converted to a peak and mean instantaneous gradient by the Bernoulli equation, respectively. 2.3. Cardiovascular magnetic resonance Flow through the pulmonary artery was measured as previously described [6,7]. In brief, a conventional phase-sensitive gradient echo sequence was used in a double-oblique plane perpendicular to the dominant flow direction in the main pulmonary artery to measure antegrade and retrograde flow volumes. The following acquisition parameters were used for phase velocity magnetic resonance: 25/6 ms; slice thickness, 5 mm; flip angle, 30°; receiver bandwidth, 31.25 kHz; rectangular field of view, 260 to 400 mm; matrix, 256 × 256; and number of excitations, 3. At the beginning of each study, velocity encoding, according to the clinical expectation, was chosen. Immediately after acquiring phase velocity magnetic resonance flow maps, they were checked for aliasing. If aliasing was detected, the scan was repeated using a higher velocity encoding. This approach resulted in final velocity encodings between 2.0 and 4.5 m/s. Data were reconstructed to provide 30 magnitude (anatomic) and phase (velocity-mapped) images per cardiac cycle. Data analysis was performed offline using commercially available software (Argus®, Siemens Healthcare, Erlangen, Germany). In three patients CMR could not be conducted because of implanted pacemaker or ICD.
The medical outcome study 36 item short form (SF-36) was used. It has an acceptable internal consistency and has proven useful in various specialties of medicine without any bias for symptoms of a specific disease [12,13]. The SF-36 measures eight health constructs with scores ranging from 0 (worst) to 100 (best) using subscales with two to 10 items per subscale and one single item about health transition. The dimensions are physical functioning (the extent to which health limits daily physical activities), role functioning physical (the extent of which physical health interferes with work or other daily activities), bodily pain (the extent of bodily pain and its effect on daily life), general health perception, vitality, social functioning (the extent to which health interferes with social activities), role functioning emotional (the extent to which emotional problems interfere with daily activities), and mental health (a rough score for depression, affect, anxiety). A single item component assesses health transition (health changes in the past year). In this study the German version of the self-report form with a window of 4 weeks was used [12,13]. 2.6. Data analyses Since data were skewed, all descriptive data were expressed in median values and interquartile ranges (Q1; Q3). Wilcoxon's signed rank tests and chi-square tests were calculated to find differences between patient variables before and 6 months after PPVI. Non-parametric Spearman rank correlations were calculated to find relations of the quality of life variables and the hemodynamic variables. Mann–Whitney-U tests were calculated to find differences between patients with initially predominant pulmonary stenosis and pulmonary regurgitation. All analyses were performed using SPSS 20.0 software (IBM Inc, Armonk, New York, USA). P-values b0.05 in a two-sided analysis were considered significant.
3. Results 3.1. Hemodynamics Hemodynamic parameters according to the PS and PR group are displayed in Tables 1a and 2a). Cardiovascular magnetic resonance revealed no substantial pulmonary regurgitation after PPVI (b15% in all patients) and
Table 1a Study subjects and hemodynamic parameters of the 46 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary stenosis group.
Sex Age Body mass index Peak instantaneous pressure gradient Mean instantaneous pressure gradient Pulmonary regurgitation Right ventricular EF Right ventricular EDVI Right ventricular EDV Left ventricular EDVI RVEDV: LVEDV
♂/♀ years kg/m2 mm Hg mm Hg % % ml/m2 ml ml –
n
Before PPVI
Six months after PPVI
p-value*
46 46 46 46 37 43 43 43 43 43 43
11/35 21.2 (16.8; 28.6) 22.6 (20.1; 27.5) 70 (53; 84) 39 (28; 48) 7 (1; 12) 52 (42; 61) 90 (72; 113) 162 (111; 215) 135 (102; 159) 1.21 (0.96; 1.37)
11/35 21.8 (17.5; 29.1) 23.0 (20.4; 27.6) 28 (23; 37) 15 (11; 20) 1 (0; 2) 56 (47; 61) 82 (67; 100) 152 (116; 200) 144 (120; 165) 1.01 (0.90; 1.33)
– – .042 b.0001 b.0001 b.0001 .075 .049 .046 .006 b.0001
p-values <0.05 were displayed in bold. EF: ejection fraction, EDVI: end-diastolic volume index; EDV: end-diastolic volume.
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Table 1b Exercise performance of the 46 patients before and 6 months after percutaneous pulmonary valve implantation in the pulmonary stenosis group. p-values <0.05 were displayed in bold.
PeakV˙ O2 Ventilatory threshold Peak O2 pulse Peak work load V˙ E =V˙ CO2 slope Peak heart rate Systolic blood pressure at peak exercise SpO2 at peak exercise Respiratory exchange ratio at peak exercise
ml/min/kg % predicted ml/min/kg % predicted W W/kg – bpm mm Hg % –
n
Before PPVI
Six months after PPVI
p-value*
46 46 44 46 46 46 46 46 45 41 46
28.2 (20.8; 34.4) 71.9 (56.7; 86.0) 16.2 (12.4; 18.6) 81.1 (70.5; 93.2) 148.5 (109.5; 192.0) 2.2 (1.9; 2.7) 28.4 (24.6; 32.8) 170 (150; 184) 164 (144; 180) 97.0 (94.0; 98.5) 1.10 (1.06; 1.15)
30.1 (22.7; 35.3) 78.3 (69.3; 93.3) 16.6 (13.1;23.0) 97.1 (80.0; 116.3) 159.0 (118.0; 214.7) 2.3 (1.9; 3.0) 25.7 (22.9; 28.9) 172 (140; 186) 167 (145; 184) 96.0 (93.0; 98.0) 1.09 (1.04; 1.13)
.001 b.0001 .017 b.0001 b.0001 .023 b.0001 .689 .648 .312 .575
echocardiographic pressure gradients improved significantly 6 months after PPVI (Tables 1a and 1b). 3.2. Exercise capacity The results of the CPET are displayed in detail in Tables 1b and 2b. Respiratory exchange ratio showed adequate exhaustion at the end of exercise without a significant difference between the two examinations 1.09 (1.05; 1.13) before vs. 1.10 (1.06; 1.15) after PPV (p = .993). For the total group, peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p b .0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Primarily, the increase in peakV˙O2 (%predicted) was present in all subgroups without any difference between those with leading pulmonary stenosis (n = 46, p b .0001) or those with leading pulmonary regurgitation (n = 13, p b .0001; comparison between groups p = .228). In addition, there was no correlation of improvements in peak V˙O2 and the reduction of pulmonary stenosis (r = −.054, p = .690) or regurgitation (r = −.096, p = .518). 3.3. Quality of life Six patients were excluded due to language barrier (n = 6). Self-estimated quality of life improved in almost all domains in both groups 6 months after intervention for PPVI (Table 3). Six months after PPVI 16 patients (30%) rated their self-reported health condition in the question on health transition similar to that before PPVI, 27 (51%) better, and 10 (19%) much better. Most improvements were seen in the more physical domains of quality of life, where the patients report on improvements regarding physical concerns in their daily life. Significant improvements were
found in “physical function”, “general health perception” and “health transition” in both groups, and “physical role functioning”, “vitality” and “mental health” only in the PS group (Table 3). Improvements in quality of life were not associated with any exercise or hemodynamic variable.
4. Discussion This study showed that patients profit from percutaneous pulmonary valve implantation not only with regard to hemodynamics and exercise performance, but also with regard to quality of life. In concordance to recent reports [2–5] pulmonary regurgitation was reduced to almost zero after PPVI and pressure gradients significantly improved. This proves the concept that percutaneous pulmonary valve implantation is feasible and restores pulmonary conduit function successfully. However, several studies [3–5] have suggested that these hemodynamic improvements cannot be automatically translated in an improvement of exercise capacity. They report on no improvement in standard measures of exercise performance in the whole study group of patient with PPVI. In the subgroups, however, where patients with predominant stenosis were compared with those with predominant regurgitation [3,4], significant improvement in peak oxygen uptake was confined to patients that were scheduled for PPVI with predominant pulmonary stenosis. Patients admitted to PPVI because of pulmonary regurgitation did not profit from intervention with regard to their exercise capacity [3,4]. Moreover, the reduction in right ventricular outflow tract gradient was the only predictor of improved peak oxygen uptake [3]. Our study could not confirm these results. We saw improvements in all indication groups for PPVI, even in the small subgroup of patients with predominantly pulmonary regurgitation. As a further result of the relief of the stenosis, O2 pulse, as a surrogate of stroke volume, improved due to the reduction in afterload after PPVI
Table 2a Study subjects and hemodynamic parameters of the 13 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary regurgitation group.
Sex Age Body mass index Peak instantaneous pressure gradient Mean instantaneous pressure gradient Pulmonary regurgitation Right ventricular EF Right vventricular EDVI Right ventricular EDV Left ventricular EDV RVEDV:LVEDV
♂/♀ years kg/m2 mm Hg mm Hg % % ml/m2 ml ml –
n
Before PPVI
Six months after PPVI
p-value*
13 13 13 11 11 11 11 11 11 11 11
6/7 25.9 (19.7; 29.9) 21.9 (17.9; 24.5) 44 (26; 57) 31 (18; 36) 35 (24; 41) 46 (37; 52) 112 (103; 164) 206 (187; 298) 136 (108; 157) 1.61 (1.33; 2.01)
6/7 26.4 (20.2; 30.7) 21.9 (19.7; 24.4) 26 (20; 30) 14 (11. 17) 2 (1; 5) 50 (36; 52) 92 (81; 113) 169 (144; 213) 142 (118; 169) 1.21 (0.96; 1,37)
– b.0001 .859 b.0001 b.0001 b.0001 .553 .005 .005 .192 .005
p-values <0.05 were displayed in bold. EF: ejection fraction, EDVI: end-diastolic volume index; EDV: end-diastolic volume.
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Table 2b Exercise performance of the 13 patients before and six months after percutaneous pulmonary valve implantation in the pulmonary regurgitation group. p-values <0.05 were displayed in bold.
PeakV˙ O2 Ventilatory threshold Peak O2 pulse Peak work load V˙ E =V˙CO2 slope Peak heart rate Systolic blood pressure at peak exercise SpO2 at peak exercise Respiratory exchange ratio at peak exercise
ml/min/kg % predicted ml/min/kg % predicted W W/kg – bpm mm Hg % –
n
Before PPVI
Six months after PPVI
p-value*
13 13 13 13 13 13 13 13 13 12 13
22.1 (16.5; 32.5) 62.7 (54.4; 70.1) 14.7 (10.0; 20.5) 72.4 (55.6; 78.9) 125.0 (97.5; 187.0) 2.1 (1.5; 2.9) 27.4 (24.9; 31.4) 169 (154; 178) 144 (130; 162) 97.0 (92.3; 98.8) 1.07 (1.05; 1.15)
24.8 (22.1; 33.8) 73.0 (64.6; 91.6) 16.5 (11.7; 20.2) 92.7 (80.4; 100.6) 145.0 (119.0; 191.0) 2.3 (1.8; 2.7) 26.1 (22.4; 29.2) 171 (156; 183) 160 (148; 180) 96.5 (94.3; 98.0) 1.10 (1.07; 1.16)
.039 b.0001 .367 .005 .016 .152 .069 .115 .039 .765 .209
[3]. In addition, the merit of PPVI is mirrored more in the improvement of ventilatory efficiency, which is a relevant prognostic risk factor in various cardiac conditions [14–17]. This improvement in ventilatory efficiency is probably due to better pulmonary perfusion which is a result of an increase in right ventricular output [5]. In agreement to Lurz et al. [3,4] we found these improvements in V˙ E =V˙ CO2 slope in our cohort, dominated by patients with pulmonary stenosis. However, improvements in V˙ E =V˙ CO2 slope, albeit not significant, were also seen in those patients with pulmonary regurgitation [3–5].
4.1. Quality of life Many studies have focused on quality of life in patients with CHD [9,18–24], but there are only few reports [25,26] with a longitudinal design. For PPVI, the outcome in quality of life is not known at all. In our study, self-reported quality of life was good already before PPVI (Table 3). The patients in our cohort seemed to be only very little compromised by the substantial pulmonary stenosis or pulmonary regurgitation. Although showing good quality of life, 6 months after PPVI, patients rated their quality of life in almost all quality of life domains better than before the intervention with the most significant improvements in the physical domains. Those improvements hold true for patients with pulmonary stenosis as well as pulmonary regurgitation. However, improvements in the latter group were limited by the small sample size and the general good quality of life before PPVI that permits less room for improvement. Albeit improvements in hemodynamics, exercise capacity, and quality of life happened in parallel with PPVI, the improvements in quality of life, surprisingly, did not correlate with any of the investigated hemodynamic or exercise variables. This lack of correlation has already previously been observed by us in a mixed population of patients with different forms of
congenital heart disease [25]. In this data set patients after interventions had an increase in quality of life that is inadequate for their changes in exercise capacity (unpublished data). Now, this is confirmed by the current study. It seems that the subjective rating of the patient whether or not the treatment was a success does not primarily depend on hemodynamic or exercise variables. Maybe the patients simply like the concept that a cardiac problem that could be only solved by surgery a few years before, can now be treated in the catheter laboratory, needing only 2–3 days of hospitalization, and showing an improved general condition already a few days after the procedure. However, this speculation does not diminish the primary result of the study that, in addition to an improved hemodynamic situation and exercise performance 6 months after PPVI, patients benefit from PPVI also with regard to their quality of life.
5. Limitation The whole study group was biased by the composition of our study group, which was driven by the clinical decision which patient was regarded eligible for PPVI. This resulted in a highly selected group of patients with most of the patients suffering from pulmonary stenosis. This reduces the power of the subgroup analyses for patients with pulmonary regurgitation substantially.
Conflict of interests P. Ewert is proctor for the Melody Valve® (Medtronic) and the Edwards Sapien Pulmonic Valve® (Edwards Lifescience). A. Eicken is proctor for the Melody Valve® (Medtronic). A. Hager has received speaker's honoraries from Medtronic.
Table 3 Quality of life of the 53 patients before and 6 months after percutaneous pulmonary valve implantation according to diagnostic subgroups. Pulmonary stenosis (n = 40)
Physical function Physical role functioning Bodily pain General health perceptions Vitality Social role functioning Emotional role functioning Mental health Health transition
Pulmonary regurgitation (n = 13)
Before PPVI
Six months after PPVI
p-values*
Before PPVI
Six months after PPVI
p-values*
90 (70; 95) 100 (75;100) 100 (92; 100) 72 (56; 82) 65 (55; 75) 100 (75;100) 100 (100; 100) 84 (70; 88) 50 (25; 50)
95 (90; 100) 100 (100; 100) 100 (100; 100) 77 (66; 88) 70 (64; 80) 100 (100;100) 100 (100; 100) 86 (75; 92) 75 (50; 75)
b.0001 .003 .091 .035 .039 .079 .979 .039 b.0001
85 (57; 93) 100 (75;100) 100 (100; 100) 77 (51; 87) 60 (36; 84) 100 (91;100) 100 (100; 100) 82 (73; 91) 25 (25; 50)
95 (88; 100) 100 (100; 100) 100 (100; 100) 92 (62; 97) 75 (48; 88) 100 (81;100) 100 (100; 100) 88 (62; 94) 75 (63; 100)
.005 .705 .180 .059 .301 .453 .317 .421 .003
p-values <0.05 were displayed in bold. *comparing the data by a paired Wilcoxon test. PPVI: percutaneous pulmonary valve implantation.
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