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Relationship Between Pulmonary Arterial Resistance and Compliance in Patients with Down Syndrome
Yuka Iwaya1, Jun Muneuchi2, Yuka Inoue1
1Division of Pediatric Cardiology, Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishiku, Kitakyushu, Fukuoka, 806-8501, Japan.
Insights
Individuals with Down syndrome (DS) exhibit unique pulmonary vasculature with lower pulmonary compliance (Cp). This low Cp is linked to the need for postoperative home oxygen therapy (HOT) in DS infants after corrective surgery for congenital heart disease.
Area of Science:
- Cardiology
- Pediatrics
- Pulmonology
Background:
- Down syndrome (DS) is associated with an increased risk of pulmonary arterial hypertension (PAH).
- Pulmonary arterial resistance (Rp)-compliance (Cp) coupling is crucial for cardiovascular health.
- Understanding pulmonary vascular characteristics in DS is vital for managing PAH risk.
Purpose of the Study:
- To investigate the pulmonary arterial resistance (Rp)-compliance (Cp) coupling in infants with Down syndrome (DS).
- To compare hemodynamic parameters before and after corrective surgery in DS infants and controls with congenital heart disease and PAH.
- To identify factors associated with postoperative outcomes in DS patients.
Main Methods:
- Retrospective analysis of cardiac catheterization data from 85 DS infants and 85 controls with congenital heart disease and PAH.
- Comparison of pulmonary arterial resistance (Rp) and compliance (Cp) before and after corrective surgery.
- Multivariate logistic regression analysis to identify predictors of postoperative home oxygen therapy (HOT).
Main Results:
- Preoperative pulmonary compliance (Cp) was significantly lower in DS patients compared to controls.
- No significant differences in mean pulmonary arterial pressure or pulmonary arterial resistance (Rp) were observed preoperatively.
- Postoperative regression analysis showed a downward shift in the Rp-Cp relationship in DS patients, and low preoperative Cp and DS were significant predictors of postoperative HOT.
Conclusions:
- Individuals with Down syndrome possess distinct pulmonary vasculature characterized by low pulmonary compliance (Cp).
- Low preoperative pulmonary compliance (Cp) is a significant predictor of the need for postoperative home oxygen therapy (HOT) in DS patients.
- These findings highlight unique pulmonary vascular characteristics in DS that influence postoperative management.
Abstract:
This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)-compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6-4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62-3.0) vs. 2.50 (1.86-3.31) mL/mmHg/m2, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.
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