Pulmonary Hypertension and Vascular Abnormalities in Bronchopulmonary Dysplasia

Abstract

Advances in the care of preterm infants have improved survival of infants born at earlier gestational ages. Yet, these infants remain at risk for the chronic lung disease of infancy, bronchopulmonary dysplasia (BPD), which results in prolonged need for supplemental oxygen, recurrent respiratory exacerbations, and exercise intolerance. Recent investigations have highlighted the important contribution of the developing pulmonary circulation to lung development, demonstrating that these infants are also at risk for pulmonary vascular disease (PVD), including pulmonary hypertension (PH) and pulmonary vascular abnormalities, which contributes significantly to morbidity and mortality. In the past few years, several epidemiological studies have delineated the incidence of PH in preterm infants and the impact on outcomes. However, these studies have also highlighted gaps in our understanding of PVD in BPD, including universally accepted definitions, approaches to diagnosis and treatment, and patient outcomes. Associated pulmonary vascular and cardiac abnormalities are increasingly recognized complications contributing to PH in these infants, but incidence of these lesions and degree of contribution to disease remains unknown. Therapeutic strategies for PVD in BPD are largely untested, but recent evidence presents the rationale for the approach to diagnosis and treatment of BPD infants with PH that can be evaluated in future studies.

Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome

“…40 BPD is a neonatal inflammatory condition of immature pulmonary capillary beds 41 that is also associated with abnormal pulmonary vasculature. 42 ROP is caused by altered retinal vasculogenesis that can incited by the inflammatory state. 43 Alterations in the mechanism of cell signaling and inflammation regulation due to the low cholesterol and elevated 7-DHC levels in SLOS may play a role in the predisposition of children with SLOS for the development of the vascular intimal proliferation seen in PVS.…”

El rol de la hipertensión pulmonar en la displasia broncopulmonar

“…Por lo tanto, no sólo nos encontramos frente a una alteración de daño alveolar con simplificación de su septación, sino que también dentro de la fisiopatología de la DBP existe una enfermedad vascular pulmonar, donde el desarrollo anormal de la microvasculatura junto al remodelamiento de la vasculatura pulmonar resultan en una disminución del área de sección transversal y con ello un aumento de la resistencia vascular pulmonar. Estas anomalías persistentes del crecimiento vascular pulmonar contribuyen al desarrollo de enfermedad vascular pulmonar, la cual se vuelve cada vez más sintomática, pudiendo llegar a su expresión más severa en la HP 7 .…”

“…Por otro lado, la cateterización cardíaca permite también la detección de lesiones como estenosis de vena pulmonar y colaterales que pueden presentarse en DBP, pero que no son fáciles de diagnosticar mediante ecografía. Es por esto que las guías de Hipertensión Pulmonar publicadas por la American Heart Association recomienda considerar el cateterismo cardiaco en algunos casos específicos como: 1) signos persistentes de enfermedad cardiorrespiratoria severa o deterioro clínico no explicado directamente por otra evaluación diagnóstica; 2) sospecha de tener HP significativa a pesar de manejo óptimo; 3) consideración de terapia vasodilatadora crónica; 4) presentación de edema pulmonar recurrente inexplicado 7,12 .…”

Pulmonary hypertension in the premature infant

“…In addition, recent data suggest that detection of early PH is a predictor for greater severity of BPD (7)*. A correlation between the diagnosis of BPD and PH is not surprising, given that alveolarization during lung growth requires pulmonary vascular development (8). Consequently, understanding the pathophysiology of PH in the BPD population involves an understanding of the interactions of these two processes as well as how other factors such as inflammation can influence these interactions (9).…”

“…However, there is still much to be learned in terms of the detection and management of infants with BPD-associated PH (BPD-PH). Current practice focuses on minimizing secondary insults to the developing lungs as well as maximizing nutrition to support optimal growth of the infant (8). PH-specific medications are used in this infant population, but there is little evidence to guide best practice in medication selection and dosing (11).…”