Key result
Preoperative PVR/SVR ≥24% during NO inhalation predicts ~9% higher risk of abnormal postoperative hemodynamics.
Why the study?
Management of pediatric pulmonary hypertension associated with congenital heart disease is challenging and predicting persistently elevated pulmonary artery pressure after surgery is difficult.
Does oral sildenafil combined with surgical repair improve hemodynamics in pediatric patients with pulmonary hypertension associated with congenital heart disease?
Cohort (n=33)
Does oral sildenafil combined with surgical repair improve hemodynamics in pediatric patients with pulmonary hypertension associated with congenital heart disease?
Hazard Ratio: 1.09 (95% CI 1.01–1.18)
p-value: p=0.036
In pediatric patients with PHT-CHD, combined oral sildenafil and surgical repair significantly improves hemodynamics at 6 months, with outcomes predictable by preoperative NO response and histopathology.
Preoperative hemodynamics and vessel wall thickness may predict abnormal post-repair hemodynamics in PHT-CHD; leaves open whether these markers should guide sildenafil use or surgical timing.
Management of pediatric pulmonary hypertension associated with congenital heart disease (PHT‐CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT‐CHD received sildenafil orally pre‐ and postoperatively for six months and then were subjected to a catheter study. Thirty‐three patients were enrolled (age range = 4.6–37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9–7.2) Wood units × m 2 (median with IQR). Twenty‐two patients had a ≥ 20% decrease in PVR and pulmonary‐to‐systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles ( P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non‐responders had a ≥ 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0–4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower ( P < 0.001 vs. baseline), even in seven patients with Heath‐Edwards grade III/IV pulmonary vascular lesions ( P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U × m 2 , respectively). A preoperative PVR/SVR of ≥24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of ≥4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01–1.18], P = 0.036; and 0.69 [0.49–0.98], P = 0.040, respectively). Thus, in PHT‐CHD patients receiving oral sildenafil pre‐ and post‐surgical repair of cardiac lesions, mid‐term postoperative outcome is predictable to some extent.
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Thomaz et al. (2019) conducted a cohort in pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) (n=33). Preoperative PVR/SVR ≥24% during NO inhalation was evaluated on Abnormal postoperative hemodynamics (PAP >25 mmHg and PVR >3.0 U × m2) (HR 1.09, 95% CI 1.01-1.18, p=0.036). A preoperative PVR/SVR ≥24% during NO inhalation was identified as a risk factor for abnormal postoperative hemodynamics (HR 1.09; 95% CI 1.01-1.18; P=0.036) in pediatric patients with PHT-CHD.
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