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Multicenter Study
, 145 (4), 1018-1027.e3

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

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Multicenter Study

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 et al. J Thorac Cardiovasc Surg.

Abstract

Background: A bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC).

Methods: Between 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study. Late EC and FHS were assessed following repair (mean 14 years) using standardized exercise testing and 3 validated FHS instruments. Relationships between FHS, EC, morphology, and 3 end states (ie, BV, univentricular [UV], or 1.5-ventricle repair [1.5V]) were evaluated.

Results: One hundred two of 271 end state survivors participated (63 BV, 25 UV, and 14 1.5V). Participants had lower FHS scores in domains of physical functioning (P < .001) compared with age- and sex-matched normal controls, but scored significantly higher in nearly all psychosocial domains. EC was higher in 1.5V-repair patients (P = .02), whereas discrete FHS measures were higher in BV-repair patients. Peak oxygen consumption was low across all groups, and was positively correlated with larger initial tricuspid valve z-score (P < .001), with an enhanced effect within the BV-repair group.

Conclusions: Late patient-perceived physical FHS and measured EC are reduced, regardless of PAIVS repair pathway, with an important dichotomy whereby patients with PAIVS believe they are doing well despite important physical impediments. For those with smaller initial tricuspid valve z-score, achievement of survival with BV repair may be at a cost of late deficits in exercise capacity, emphasizing that better outcomes may be achieved for borderline patients with a 1.5V- or UV-repair strategy.

Figures

FIGURE 1
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.
FIGURE 2
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).
FIGURE 3
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.

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