Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: A Congenital Heart.

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Long-term Functional Health Status and Exercise Test Variables for Patients with Pulmonary Atresia with Intact Ventricular Septum: A Congenital Heart Surgeons.

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Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: A Congenital Heart Surgeons Society study Tara Karamlou, MD, MSc, Jeffrey A. Poynter, MD, Henry L. Walters, MD, Jonathan Rhodes, MD, Igor Bondarenko, MD, Sara K. Pasquali, MD, Stephanie M. Fuller, MD, Linda M. Lambert, RN, Eugene H. Blackstone, MD, Marshall L. Jacobs, MD, Kim Duncan, MD, Christopher A. Caldarone, MD, William G. Williams, MD, Brian W. McCrindle, MD, MPH The Journal of Thoracic and Cardiovascular Surgery Volume 145, Issue 4, Pages 1018-1027.e3 (April 2013) DOI: 10.1016/j.jtcvs.2012.11.092 Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 Flowchart depicting cross-sectional study participants segregated by current end state. BV, Biventricular repair; UV, univentricular repair; 1.5V, 1.5-ventricle repair. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 A, Influence of end state on selected functional health status (FHS) scores. This histogram demonstrates age- and sex-adjusted values for the 3 end state groups in selected domains. In general, age- and sex-adjusted FHS measures were similar in patients undergoing univentricular (UV) repair compared with patients undergoing biventricular (BV) repair. B, Histogram depicting influence of repair pathway on exercise test results. Age- and sex-adjusted means for percent predicted peak oxygen consumption (VO2) and maximum heart rate (Max heart rate). The BV group had lower peak VO2 than the 1.5-ventricle repair (1.5V) group. The 1.5V group also achieved a higher max heart rate than the UV repair group. *Overall P value not significant; †Overall P < .05; α = P < .01 compared with the reference group (ie, 1.5V-repair group); ‡P < .05 compared with reference group (ie, 1.5V-repair group). The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 3 A, Percent predicted late peak oxygen consumption (VO2) across the spectrum of initial tricuspid valve z-scores for all participants. Peak VO2 increased linearly with increasing initial tricuspid valve z-score for all participants (P < .001). Solid line is a best-fit regression lines enclosed by 95% confidence limits. Individual dots represent individual data points. B, Percent predicted late peak VO2 among the 3 end state groups over the range of initial tricuspid valve z-scores with risk-adjusted best-fit regression lines for each group. Peak VO2 was higher for those with larger initial tricuspid valve z-score for all end state groups (P < .001). It appears that biventricular (BV) repair in patients with the highest values of initial tricuspid valve z-score (> 0) was associated with higher peak VO2 than was observed in other treatment groups. This is based on a very small number of patients in the BV group with initial tricuspid valve z-score values higher than all patients in the other treatment groups. Importantly, peak VO2 in the BV group with low initial tricuspid valve z-scores (eg, < −2) tended to be lower than peak VO2 in the 1.5–ventricle-repair group with comparable initial tricuspid valve z-scores. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure E1 Flowchart depicting the final end states and survival among the entire cohort of patients with pulmonary atresia with intact ventricular septum (PAIVS). BV, Biventricular repair; UV, univentricular repair; 1.5V, 1.5-ventricle repair. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure E2 A, Histograms demonstrating functional health status results obtained from the Child Health Questionnaire (CHQ) for all participants with pulmonary atresia with intact ventricular septum (PAIVS) compared with age- and sex-matched normal controls. Participants scored lower than age- and sex-matched normal controls in physical functioning, but they performed better in psychosocial and relationship domains. Such evidence is important because it demonstrates that patients with PAIVS believe they are doing well despite their important physical impediments. B, Histograms demonstrating functional health status results obtained from the Pediatric Quality of Life Inventory 4.0 (PedsQL) for patients with PAIVS compared with age- and sex-matched normal controls. Concordant with the CHQ, participants with PAIVS scored lower in domains of physical functioning compared with normal control subjects, but were equivalent in psychosocial domains. PF, Physical functioning; RS-E, role social-emotional; RS-B, role social behavioral; BP, bodily pain; BE, behavioral; SE, self-esteem; GH, general health; FC, family cohesion. *P ≤ .05. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure E3 Percent predicted late maximum heart rate among the 3 end state groups over the range of initial tricuspid valve z-scores with risk-adjusted best-fit regression lines for each group. Although the 1.5-ventricle (1.5V) repair group achieved higher maximum heart rate than the biventricular (BV) repair group, there was no influence of initial tricuspid valve z-score. UV, Univentricular. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure E4 Risk-adjusted nomogram of peak oxygen consumption (VO2) across the range of participant ages demonstrates 3 solutions for the multivariable peak VO2 equation for a hypothetical male patient with an initial tricuspid valve z-score of −2. Although peak VO2 increases with increasing age in all end state groups, the 1.5-ventricle repair group had improved peak VO2 compared with both the biventricular-repair and univentricular-repair groups, although this difference was significant only between the 1.5–ventricle-repair group and biventricular-repair group. The Journal of Thoracic and Cardiovascular Surgery 2013 145, 1018-1027.e3DOI: (10.1016/j.jtcvs.2012.11.092) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions