pulmonary hypertension newborn

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No abstract text is available yet for this article.

Pivotal trials investigating the use of inhaled nitric oxide (iNO) in the 1990s led to approval by the Food and Drug Administration in 1999. Inhaled nitric oxide is the only approved pulmonary vasodilator for persistent pulmonary hypertension of the newborn (PPHN). Selective pulmonary vasodilation with iNO in near-term and term neonates with PPHN is safe, and targeted use of iNO in less mature neonates with pulmonary hypertension (PH) can be beneficial. This review addresses a brief history of iNO, clinical features of neonatal PH, and the clinical application of iNO...

Oxygen is a specific pulmonary vasodilator. Hypoxemia causes pulmonary vasoconstriction, and normoxia leads to pulmonary vasodilation. However, hyperoxia does not enhance pulmonary vasodilation but causes oxidative stress. There are no clinical trials evaluating optimal oxygen saturation or Pao2 in pulmonary hypertension. Data from translational studies and case series suggest that oxygen saturation of 90% to 97% or Pao2 between 50 and 80 mm Hg is associated with the lowest pulmonary vascular resistance...

Pulmonary hypertension (PH) in neonates, originating from a range of disease states with heterogeneous underlying pathophysiology, is associated with significant morbidity and mortality. Although the final common pathway is a state of high right ventricular afterload leading to compromised cardiac output, multiple hemodynamic phenotypes exist in acute and chronic PH, for which cardiorespiratory treatment strategies differ. Comprehensive appraisal of pulmonary pressure, pulmonary vascular resistance, cardiac function, pulmonary and systemic blood flow, and extrapulmonary shunts facilitates delivery of individualized cardiovascular therapies in affected newborns...

Long-term outcomes of persistent pulmonary hypertension of newborn (PPHN) depend on disease severity, duration of ventilation, and associated anomalies. Congenital diaphragmatic hernia survivors may have respiratory morbidities and developmental delay. The presence of PPHN is associated with increased mortality in hypoxic-ischemic encephalopathy, though the effects on neurodevelopment are less clear. Preterm infants can develop pulmonary hypertension (PH) early in the postnatal course or later in the setting of bronchopulmonary dysplasia (BPD)...

Inhaled nitric oxide (iNO) is a pulmonary vasodilator considered standard of care to treat persistent pulmonary hypertension of the newborn. However, not all infants respond to iNO. The authors performed a systematic review to examine methodology, outcomes, and challenges of randomized controlled trials testing pulmonary vasodilator medications adjunctive to iNO. The 5 trials identified showed heterogeneity in eligibility criteria and outcomes assessed. No trial achieved recruitment goals, limiting conclusions regarding efficacy, safety, and pharmacology...

Persistent Pulmonary Hypertension of the Newborn (PPHN) is more common in Low and middle income countries (LMICs) due to high incidence of sepsis, perinatal asphyxia and meconium aspiration syndrome. Presence of hypoxic respiratory faillure and greater than 5% difference in preductal and post ductal saturation increases clinical sucipision for PPHN. The availability of Inhaled nitric oxide and extracorporaeal membrane oxygenation is limited but pulmonary vasodilators such as sildenafil are readily available in most LMICs...

Diverse genetic developmental lung diseases can present in the neonatal period with hypoxemic respiratory failure, often associated with with pulmonary hypertension. Intractable hypoxemia and lack of sustained response to medical management should increase the suspicion of a developmental lung disorder. Genetic diagnosis and lung biopsy are helpful in establishing the diagnosis. Early diagnosis can result in optimizing management and redirecting care if needed. This article reviews normal lung development, various developmental lung disorders that can result from genetic abnormalities at each stage of lung development, their clinical presentation, management, prognosis, and differential diagnoses...

Neonatal pulmonary hypertension (PH) is a devastating disorder of the pulmonary vasculature characterized by elevated pulmonary vascular resistance and mean pulmonary arterial pressure. Occurring predominantly because of maldevelopment or maladaptation of the pulmonary vasculature, PH in neonates is associated with suboptimal short-term and long-term outcomes because its pathobiology is unclear in most circumstances, and it responds poorly to conventional pulmonary vasodilators. Understanding the pathogenesis and pathophysiology of neonatal PH can lead to novel strategies and precise therapies...

Preterm infants with bronchopulmonary dysplasia (BPD) are prone to develop pulmonary hypertension (PH). Strong laboratory and clinical data suggest that antenatal factors, such as preeclampsia, chorioamnionitis, oligohydramnios, and placental dysfunction leading to fetal growth restriction, increase susceptibility for BPD-PH after premature birth. Echocardiogram metrics and serial assessments of NT-proBNP provide useful tools to diagnose and monitor clinical course during the management of BPD-PH, as well as monitoring for such complicating conditions as left ventricular diastolic dysfunction, shunt lesions, and pulmonary vein stenosis...

Pulmonary hypertension (PH) in preterm neonates has multifactorial pathogenesis with unique characteristics. Premature surfactant-deficient lungs are injured following exposure to positive pressure ventilation and high oxygen concentrations resulting in variable phenotypes of PH. The prevalence of early PH is variable and reported to be between 8% and 55% of extremely preterm infants. Disruption of the lung development and vascular signaling pathway could lead to abnormal pulmonary vascular transition. The management of early PH and the off-label use of selective pulmonary vasodilators continue to be controversial...

This review provides a comprehensive summary of the current understanding of pulmonary hypertension (PH) in congenital diaphragmatic hernia, outlining the underlying pathophysiologic mechanisms, methods for assessing PH severity, optimal management strategies, and prognostic implications.

Neonates with a perinatal hypoxic insult and subsequent neonatal encephalopathy are at risk of acute pulmonary hypertension (aPH) in the transitional period. The phenotypic contributors to aPH following perinatal asphyxia include a combination of hypoxic vasoconstriction of the pulmonary vascular bed, right heart dysfunction, and left heart dysfunction. Therapeutic hypothermia is the standard of care for neonates with moderate-to-severe hypoxic ischemic encephalopathy. This review summarizes the underlying risk factors, causes of aPH in neonates with perinatal asphyxia, discusses the unique phenotypical contributors to disease, and explores the impact of the initial insult and subsequent therapeutic hypothermia on aPH...

Pulmonary hypertension in the neonatal population can be acute or chronic and carries significant risk for morbidity and mortality. It can be idiopathic but more often is associated with comorbid pulmonary and heart disease. There are several pharmacotherapeutics aimed at pulmonary vasodilation. This review highlights the most common agents as well as those on the horizon for the treatment of pulmonary hypertension in the neonate.

Fetal lungs have fewer and smaller arteries with higher pulmonary vascular resistance (PVR) than a newborn. As gestation advances, the pulmonary circulation becomes more sensitive to changes in pulmonary arterial oxygen tension, which prepares them for the dramatic drop in PVR and increase in pulmonary blood flow (PBF) that occur when the baby takes its first few breaths of air, thus driving the transition from fetal to postnatal circulation. Dynamic and intricate regulatory mechanisms control PBF throughout development and are essential in supporting gas exchange after birth...

INTRODUCTION: Antenatal antibiotic exposure has been suggested as a risk factor for bronchopulmonary dysplasia (BPD). We aimed to summarize the evidence from randomized controlled trials (RCTs) and observational studies on this potential association. METHODS: PubMed/Medline and Embase databases were searched. BPD was classified as BPD28 (supplemental oxygen during 28 days or at postnatal day 28), BPD36 (supplemental oxygen at 36 weeks postmenstrual age), BPD36 or death, and BPD-associated pulmonary hypertension (BPD-PH)...

BACKGROUND: Right ventricular dysfunction, typically qualitatively diagnosed (Q-RVd) in preterm infants, requires echocardiography which is not always acutely available. We aimed to identify clinical indices of Q-RVd in very preterm infants (gestational age, GA <32 weeks) with persistent pulmonary hypertension of newborn (PPHN) and examine the reliability and validity of Q-RVd. METHODS: Forty-seven infants with mean ± SD GA of 26.8 ± 2...

Newborns with intrauterine growth restriction (IUGR) have a higher likelihood of developing pulmonary arterial hypertension (PAH) in adulthood. While there is increasing evidence suggesting that pericytes play a role in regulating myofibroblast transdifferentiation and angiogenesis in malignant and cardiovascular diseases, their involvement in the pathogenesis of IUGR-related PH and the underlying mechanisms remain incompletely understood. To address this issue, a study was conducted utilizing a Sprague-Dawley (SD) rat model of IUGR-related PH...

Heterozygous activin receptor-like kinase 1 (ALK1) mutations are associated with two vascular diseases: hereditary hemorrhagic telangiectasia (HHT) and more rarely pulmonary arterial hypertension (PAH). Here, we aimed to understand the impact of ALK1 mutations on BMP9 and BMP10 transcriptomic responses in endothelial cells. Endothelial colony-forming cells (ECFCs) and microvascular endothelial cells (HMVECs) carrying loss of function ALK1 mutations were isolated from newborn HHT and adult PAH donors, respectively...