Diagnosis and treatment of pulmonary hypertension in infancy

Abstract

Normal pulmonary vascular development in infancy requires maintenance of low pulmonary vascular resistance after birth, and is necessary for normal lung function and growth. The developing lung is subject to multiple genetic, pathological and/or environmental influences that can adversely affect lung adaptation, development, and growth, leading to pulmonary hypertension. New classifications of pulmonary hypertension are beginning to account for these diverse phenotypes, and or pulmonary hypertension in infants due to PPHN, congenital diaphragmatic hernia, and bronchopulmonary dysplasia (BPD). The most effective pharmacotherapeutic strategies for infants with PPHN are directed at selective reduction of PVR, and take advantage of a rapidly advancing understanding of the altered signaling pathways in the remodeled vasculature.

The myocardial architecture changes in persistent pulmonary hypertension of the newborn in an ovine animal model

“…This leads to right-to-left extra-pulmonary shunting, resulting in hypoxemia. The histological changes in the lungs are well described (2). In contrast, its effects on the heart itself are relatively poorly understood, albeit that the notion of congestive heart failure due to elevated right ventricular afterload is relatively common (2,3).…”

“…The histological changes in the lungs are well described (2). In contrast, its effects on the heart itself are relatively poorly understood, albeit that the notion of congestive heart failure due to elevated right ventricular afterload is relatively common (2,3). Left ventricular dysfunction due to diastolic impairment is also described (4), while a spectrum of myocardial dysfunction was identified in a case-based study (5).…”

Accuracy and reliability of qualitative echocardiography assessment of right ventricular size and function in neonates

“…remains 8%-10% 3,4 and the rates of disability at 1 year of age remain high, regardless of inhaled nitric oxide (iNO) use. 5 Chronic PH (cPH) presents later secondary to pulmonary vascular remodeling, particularly in context of evolving chronic lung disease (CLD).…”

Multiple mitochondrial dysfunctions syndrome 1: An unusual cause of developmental pulmonary hypertension

“…There are no data in the literature on management of pHTN in MMDS1. In an attempt to treat her pHTN‐related cardiac dysfunction, Patient 2 was given a trial of sildenafil and later bosentan (Steinhorn, ). Sildenafil was well tolerated and may have stabilized her vascular disease, given improvement in echocardiogram studies, although initiation of other therapies, namely ketogenic diet, and proper nutrition may confound this assessment.…”