Pulmonary hypertension is an important co‐morbidity in developmental lung diseases of infancy: Bronchopulmonary dysplasia and congenital diaphragmatic hernia

Abstract

Bronchopulmonary dysplasia (BPD) following preterm birth and congenital diaphragmatic hernia (CDH) are both forms of developmental lung disease that may result in persistent pulmonary and pulmonary vascular morbidity in childhood. The pulmonary vascular disease (PVD) which accompanies BPD and CDH is due to developmental abnormalities and ongoing perinatal insults. This may be accompanied by evidence of elevated right heart pressures and pulmonary vascular resistance, leading to diagnosis of pulmonary hypertension (PH). The development of PH in these conditions is associated with increased morbidity and mortality in the vulnerable BPD and CDH populations. We present a review of PVD pathogenesis and evaluation in BPD and CDH and discuss management of related sequelae of PH co‐morbidity for affected infants.

Full Issue PDF

“…The P(a-et)co 2 gradient, which is the difference between the partial pressure of carbon dioxide on an arterial blood gas (Paco 2 ) minus the end tidal co 2 on capnography, fails to decline or rather, increases in patients with PAH that are exercising. 34 In patients with severe ILD with severe PH (mPAP ≥ 40 mm Hg), there is a lower Vo 2 peak, higher Ve/Vco 2 slope, and lower peak exercise Petco 2 compared to ILD patients without PH. 35…”

“…33 Findings similar to those in IPF have been reproduced in more general ILD population with reduced 6MWD and survival noted in patients with PH-ILD. 24,34 The severity of PH in the context of ILD is believed to generally be biased toward mild to moderate elevations in pulmonary arterial pressures. 3 In addition to fundamental pathophysiologic differences, other explanations for this include classification bias (patients with severe PH are classified as group 1) and survivorship bias (patients with advanced PH and ILD do not survive or are transplanted).…”

“…38,39 As mentioned earlier, patients with PH-ILD have decreased exercise tolerance as measured by 6MWD. 34 Though 6MWD is not routinely part of the ILD follow-up protocols, when performed, a decrease in exercise capacity, particularly if not associated with progression of the underlying ILD, can be a signal of progression of pulmonary vascular disease. Other measurements obtained during 6MWT may also be telling: abnormal heart rate recovery at 1 minute has also been found to be predictive of both the presence of PH and survival in patients with IPF.…”

“…Pulsed inhaled nitric oxide was associated with improved moderate-vigorous physical activity and oxygenation in a phase 2b trial in fibrotic PH-ILD, and a phase 3 trial is currently enrolling (Clinical Trials.gov identifier NCT03267108). 34 Furthermore, as efforts to better phenotype and understand PH-ILD continue through programs like PVODMICs, this will hopefully provide a future basis for the clearer delineation of study populations and endpoints reflective of individual disease burden.…”

PH Professional Network: 360-degree Care for the Bronchopulmonary Dysplasia Infant with Pulmonary Hypertension: A Comprehensive Review

“…These irregularities are reflected in the reported prevalence of PH-BPD. 34 PH guidelines released by the American Heart Association in conjunction with the American Thoracic Society recommend echocardiography screening of all PH-BPD infants with moderate to severe BPD at 36 weeks gestational age, and the Pediatric Pulmonary Hypertension Network expanded on this recommendation with clinical specifics. 33,35,36 Despite these consensus statements, implementation remains variable.…”