Diminished right ventricular function at diagnosis of pulmonary hypertension is associated with mortality in bronchopulmonary dysplasia

Abstract

Pulmonary vascular disease and resultant pulmonary hypertension (PH) have been increasingly recognized in the preterm population, particularly among patients with bronchopulmonary dysplasia (BPD). Limited data exist on the impact of PH severity and right ventricular (RV) dysfunction at PH diagnosis on outcome. The purpose of this study was to evaluate if echocardiography measures of cardiac dysfunction and PH severity in BPD-PH were associated with mortality. The study is a retrospective analysis of the echocardiography at three months or less from time of PH diagnosis. Survival analysis using a univariate Cox proportional hazard model is presented and expressed using hazard ratios (HR). We included 52 patients with BPD and PH of which 16 (31%) died at follow-up. Average gestational age at birth was 26.3 ± 2.3 weeks. Echocardiography was performed at a median of 43.3 weeks (IQR: 39.0–54.7). The median time between PH diagnosis and death was 117 days (range: 49–262 days). Multiple measures of PH severity and RV performance were associated with mortality (sPAP/sBP: HR 1.02, eccentricity index: HR 2.02, tricuspid annular plane systolic excursion Z-score: HR 0.65, fractional area change: HR 0.88, peak longitudinal strain: HR 1.22). Hence, PH severity and underlying RV dysfunction at PH diagnosis were associated with mortality in BPD-PH patients. While absolute estimation of pulmonary pressures is not feasible in every screening echocardiography, thorough evaluation of RV function and other markers of PH may allow to discriminate the most at-risk population and should be considered as standard add-ons to the current screening at 36 weeks.

Pulmonary hypertension in bronchopulmonary dysplasia

“…About 25% of infants with moderate to severe BPD develop PH 3,4 that affects heart and lungs, greatly increasing mortality (47% of BPD infants die 2 years after diagnosis of PH). [5][6][7] Compared to BPD without PH, BPD-PH is associated with suboptimal somatic growth and neurodevelopmental outcome, 8,9 and higher rates of tracheostomy, increased use of supplemental oxygen, feeding problems, and frequent hospital admissions. 10 Young adults born preterm (very low birth weight ≤1500 g; average gestational age 28 weeks) are at increased risk for PVD, PH, and right ventricular (RV) dysfunction.…”

Cardiopulmonary Function Abnormalities in Cohort of Adults following Bronchopulmonary Dysplasia as Preterm Infants

“…Premature newborns, and particularly those with BPD, are at-risk for PH and RV dysfunction. 12,13,17,[43][44][45] Specifically, degree of RV dysfunction at diagnosis of PH seems to be associated with poorer outcomes in this vulnerable population. 17 The RV primarily contracts in a longitudinal fashion, with the tricuspid valve plunging toward the RV outflow tract; measure of RV function, therefore, includes the TAPSE which measures the longitudinal distance of movement of the tricuspid valve during systole.…”

“…12,13,17,[43][44][45] Specifically, degree of RV dysfunction at diagnosis of PH seems to be associated with poorer outcomes in this vulnerable population. 17 The RV primarily contracts in a longitudinal fashion, with the tricuspid valve plunging toward the RV outflow tract; measure of RV function, therefore, includes the TAPSE which measures the longitudinal distance of movement of the tricuspid valve during systole. [46][47][48][49] STE uses the pixels composing the ECHO image to track cardiac movement during the cardiac cycle.…”

“…Adult colleagues should pay specific attention to those patients with described risk factors in pediatric cohorts such as pulmonary vein stenosis, intrauterine growth restriction, maternal hypertensive disorders, duration of respiratory support, severe necrotizing enterocolitis, postnatal steroid exposure, severe airway anomalies, tracheostomy use, history of home oxygen therapy, cigarette or vape use, asthma, as well as known cardiovascular comorbidities (diabetes, hypertension, obesity, and concerning family history for early cardiovascular events). [11][12][13]17,[43][44][45]53 Factors beyond postnatal care alone are likely important contributors to respiratory and cardiac morbidities in adulthood; strategies that address all stages of neonatal development are thus needed to identify those at highest risk of developing BPD and chronic morbidity. With the improved survival of extremely preterm infants, physicians are likely to encounter adult survivors of BPD.…”

Role of functional echocardiographic parameters in the diagnosis of bronchopulmonary dysplasia-associated pulmonary hypertension

“…In a small pediatric PH study, strain was found to be an earlier predictor of RV dysfunction [ 50 ]. A recent study in neonates with BPD-PH has suggested that an RV peak longitudinal strain greater than −14 is a discriminative marker of BPD-PH [ 51 ]. The limitations of strain and strain rate measurements are the relatively low temporal solution of 2D speckle tracking in the presence of high heart rates and the long post-processing times [ 25 ].…”