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Exercise Capacity in Patients with Pulmonary Atresia with Intact Ventricular Septum: Does the Type of Surgical Repair Matter?

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Abstract

Two standard surgical palliative options for neonates born with pulmonary atresia and intact ventricular septum (PA/IVS) include uni-or biventricular repair. Whenever feasible, the biventricular repair is considered to have better exercise capacity (XC) and outcomes. However, there is a paucity of data comparing objective XC between these two surgical techniques. Our aim was to compare XC, including longitudinal changes in patients with PA/IVS following uni-biventricular repair. We performed a single-center retrospective study of survivors with repaired PA/IVS who underwent comprehensive treadmill cardiopulmonary exercise testing. Initial and latest exercise parameters were compared for longitudinal analysis. Demographic and exercise parameters were collated. Peak oxygen uptake (VO2 in ml/kg/min), an indicator of maximal aerobic capacity, peak heart rate, and other measures of spirometry performed at the same time were collected. Recorded parameters included, (a) Percentage of predicted VO2 (% VO2) normalized for age, weight, height, and gender, (b) % oxygen (O2) pulse, (c) anaerobic threshold (AT), (d) Chronotropic index (CI), (e) % Breathing reserve, (f) Forced vital capacity (FVC), (g) % Forced Expiratory volume in 1 s (FEV1), (h) Maximum voluntary ventilation (MVV), and (i) VE/VCO2. Appropriate statistical tests were performed, and a p value < 0.05 was considered significant. A total of 35 patients (43% male, 57% univentricular repair) were included, with a mean (SD) age of 20.1(7.5) years. Patients with univentricular palliation demonstrated significantly impaired peak heart rate, chronotropic index (0.50 ± 0.2 vs. 0.90 ± 0.1, p = 0.02), VE/VCO2 (35.4 ± 5.0 vs. 30.2 ± 2.8, p = 0.001), and %FVC (78.3 ± 8.3 vs. 88.6 ± 15.1, p = 0.02). There was a trend towards reduction in % VO2 in the Fontan patients though it was statistically similar between the groups (68.4 ± 21.4 vs. 81.2 ± 18.9, p = 0.07). Longitudinal data were available for 11 patients in each group, and there was no longitudinal decline in their exercise parameters over similar intermediate follow-up duration [6.8 (UV) vs. 5.3 (BV) years]. We conclude that young survivors with PA/IVS with prior univentricular palliation demonstrated an objective impairment in their chronotropic parameters compared with the biventricular repair. However, this did not translate into a significant difference in their exercise capacity. There was no longitudinal decline in exercise capacity or other parameters over intermediate follow-up.

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Abbreviations

ACE:

Angiotensin-converting enzyme

AV:

Atrioventricular

BMI:

Body mass index

BP:

Blood pressure

BV:

Biventricular

CHD:

Congenital heart disease

CI:

Chronotropic index

CO2 :

Carbon dioxide

EKG:

Electrocardiogram

FEV1:

Forced expiratory volume in 1 s

FVC:

Forced vital capacity

HR:

Heart rate

IRB:

Institutional review board

LV:

Left ventricle

METS:

Metabolic equivalents

MV:

Minute ventilation

MVV:

Maximum voluntary ventilation

O2 :

Oxygen

PA:

Pulmonary artery

PA/IVS:

Pulmonary atresia/intact ventricular septum

PFT:

Pulmonary function test

RV:

Right ventricle

RVOT:

Right ventricular outflow tract

SD:

Standard deviation

SpO2 :

Oxygen saturation

TOF:

Tetralogy of fallot

UV:

Univentricular

VAT:

Ventilatory anaerobic threshold

VCO2 :

Carbon dioxide elimination

VE:

Pulmonary ventilation

VO2 :

Peak oxygen uptake

XC:

Exercise capacity

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI, 48201, USA

    Anusha Konduri, Chenni Sriram, Deemah Mahadin & Sanjeev Aggarwal

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  1. Anusha Konduri
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Contributions

Anusha Konduri: Acquisition of data, analysis and interpretation of data, drafting the manuscript Sanjeev Aggarwal: Conception and design of study, analysis and interpretation of data, drafting the manuscript Chenni Sriram: Analysis and interpretation of data, drafting the manuscript Deemah Mahadin: Analysis and interpretation of data, drafting the manuscript All authors reviewed the manuscript.

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Correspondence to Anusha Konduri.

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The authors do not have any conflict of interest, financial or otherwise, to disclose. No Grants were utilized.

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Konduri, A., Sriram, C., Mahadin, D. et al. Exercise Capacity in Patients with Pulmonary Atresia with Intact Ventricular Septum: Does the Type of Surgical Repair Matter?. Pediatr Cardiol 44, 556–563 (2023). https://doi.org/10.1007/s00246-022-02943-8

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