Persistent Pulmonary hypertension of the newborn

INTRODUCTION: For patients with a congenital diaphragmatic hernia, conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation (HFOV) are used in initial ventilatory management. HFOV has recently been recommended as a rescue therapy; however, we use HFOV for initial ventilation management, with a preoperative challenge test for CMV conversion and respiratory function testing at the time of CMV conversion. We aimed to compare patient characteristics between CMV conversion- and HFOV-preferred treatment groups...

Persistent pulmonary hypertension of the newborn (PPHN) can be monitored theoretically by the difference of the partial pressure of arterial (PaCO2 ) to end-tidal CO2 (EtCO2 ). We aimed to test the hypothesis that the PaCO2 -EtCO2 gradient in infants with PPHN would be higher compared to infants without PPHN. Prospective, observational study of term-born ventilated infants with echocardiographically-confirmed PPHN with right-to-left shunting and term-born control infants without respiratory disease. The PaCO2 -EtCO2 gradient was calculated as the difference between the PaCO2 measured from indwelling arterial sample lines and EtCO2 measured by continuous Microstream sidestream capnography...

Pulmonary hypertension (PH) is the most severe complication in preterm infants with bronchopulmonary dysplasia (BPD) and associated with significant mortality. Diagnostic and treatment strategies, however, still lack standardization. By the use of a survey study (PH in BPD), we assessed clinical practice (diagnosis, treatment, follow-up) in preterm infants with early postnatal persistent pulmonary hypertension of the newborn (PPHN) as well as at risk for or with established BPD-associated PH between 06/2018 and 10/2020 in two-thirds of all German perinatal centers with >70 very low birthweight infants/year including their cardiology departments and outpatient units...

Background: Earlier studies on the use of inhaled nitric oxide (iNO) for premature infants born at <34 weeks of gestation requiring respiratory support did not provide conclusive evidence of benefit. National guidelines generally discouraged the use in this population. More recent national guidelines endorsed the use of iNO in premature infants with hypoxic respiratory failure (HRF) associated with persistent pulmonary hypertension of the newborn (PPHN). Recent Studies: Two recently published observational studies evaluated the effect of administering iNO on oxygenation in the first week of life...

BACKGROUND: This study aims to evaluate the effectiveness of treprostinil and oral sildenafil in managing persistent pulmonary hypertension of newborns (PPHN). METHODS: We conducted a retrospective cohort study of 42 neonates with PPHN treated with continuous intravenous treprostinil or oral sildenafil from January 2020 to October 2022 in China. Outcomes assessed included echocardiographic pulmonary artery systolic pressure (PASP), shunt direction, and arterial blood gas measures...

BACKGROUND: Neonates with persistent pulmonary hypertension of the newborn (PPHN) can present with hypoxia and right ventricular dysfunction with resultant inadequate oxygen delivery and end-organ damage. This study describes the use of prostaglandin-E1 (PGE) for ductal patency to preserve right ventricular systolic function and limit afterload in newborns with PPHN. METHODS: This is a retrospective cohort study that follows the hemodynamics, markers of end-organ perfusion, length of therapeutics, and echocardiographic variables of 57 newborns who used prostglandin-E1 in the setting of PPHN...

Despite improvements in the medical management of persistent pulmonary hypertension of the newborn (PPHN), a significant number of patients persist with inadequate gas exchange and are treated with extracorporeal membrane oxygenation (ECMO). Prolonged time to weaning ECMO can increase mortality risk. Therefore, multiple therapies are utilized for pulmonary hypertension treatment, including pharmacotherapy with pulmonary vasodilators, to improve the prognosis of these critical patients. We report a case of a 37 2/7-week neonate with severe PPHN refractory to triple pulmonary vasodilator therapy (inhaled nitric oxide (iNO), sildenafil, and milrinone) and required veno-venous (VV)-ECMO support to improve oxygenation...

INTRODUCTION: Pulmonary hypertension (PH) is a complex condition that encompasses an array of underlying disease processes and affects a diverse population of infants, including those with congenital heart disease, congenital diaphragmatic hernia, persistent PH of the newborn, and those with lung disease such as bronchopulmonary dysplasia. While there are treatments available to adults with PH, limited data exists for infants, especially for the newer medications. Therapies that target the three main pathophysiologic pathways of pulmonary hypertension appear to benefit infants, but which are best for each individual disease process is unclear...

Most extremely preterm infants (EPIs), who were born before 28 weeks of gestation, with pulmonary air leak syndrome (ALS) are symptomatic, often severe, and require drainage. EPIs with severe air leak syndrome (sALS) that require tube drainage or needle aspiration are at high risk of morbidities and mortality. This study aimed to investigate perinatal characteristics, morbidities, and mortality in EPIs with sALS, and to estimate the risk of mortality according to gestational age (GA). A prospective cohort study conducted from 2013 to 2020 compiled the Korean Neonatal Network database to evaluate the incidence, perinatal characteristics, and outcomes of sALS in EPIs born before 28 weeks of gestation...

OBJECTIVE: Pulmonary artery stiffness (PAS) is a strong and independent predictor of mortality in adult patients with pulmonary hypertension (PH). But the change in PAS during perinatal period remains unknown. Here, we aimed to explore the feasibility and performance of PAS on predicting persistent pulmonary hypertension of the newborn (PPHN). METHODS: 1325 fetuses underwent a dedicated echocardiography screening for fetal heart defects during second trimester, third-trimester and neonatal period with the measurement of acceleration time (PAAT) and maximal frequency shift (MFS) of pulmonary artery flow...

OBJECTIVE: To obtain preliminary validity data for a hypoxemic respiratory failure/pulmonary hypertension (HRF/PH) score for classifying persistent pulmonary hypertension of the newborn (PPHN). STUDY DESIGN: Retrospective chart review of 100 consecutive neonates admitted to a Children's hospital from 2016-2021 with PPHN, gestational age ≥34 weeks, and echocardiograms in the first week. We assessed the correlation between HRF/PH score and short-term outcomes using linear and logistic regressions...

BACKGROUND: Transient tachypnea of the newborn (TTN) is the consequence of delayed resorption of lung fluid. When TTN develops, the infant may develop severe hypoxemia and progress to persistent pulmonary hypertension of the newborn (PPHN). OBJECTIVES: To examine factors associated with the development of PPHN in TTN infants. METHODS: This retrospective study comprised 23 infants in whom a diagnosis of TTN with PPHN (TTN-PPHN) was confirmed and 59 infants with severe TTN without PPHN who required mechanical ventilation between 2009 and 2018 at Songklanagarind Hospital, Thailand...

Meconium aspiration syndrome (MAS) is a complex respiratory disease that continues to be associated with significant morbidities and mortality. The pathophysiological mechanisms of MAS include airway obstruction, local and systemic inflammation, surfactant inactivation and persistent pulmonary hypertension of the newborn (PPHN). Supplemental oxygen and non-invasive respiratory support are the main therapies for many patients. The management of the patients requiring invasive mechanical ventilation could be challenging because of the combination of atelectasis and air trapping...

OBJECTIVE: To describe current treatment practices of preterm infants with early hypoxemic respiratory failure (HRF) and pulmonary hypertension (PH) and their association with patient outcomes. STUDY DESIGN: We developed a prospective, observational, multicenter clinical registry of preterm newborns <34 weeks' gestation with HRF and PH, based on either clinical or echocardiographic evidence during the first 72 hours of life, from 28 neonatal intensive care units in the US from 2017 through 2022...

Persistent pulmonary hypertension of the newborn (PPHN) is one of the diseases of the neonate with severe potential morbidity and mortality. Inhaled iloprost, a stable prostacyclin analog, has been suggested as an alternative treatment for inhaled nitric oxide (iNO). However, more data on neonates' dosing, setting, and effectiveness still needs to be solved. This study suggests using inhaled iloprost as rescue therapy for PPHN based on our experience. This was a retrospective study. The data from medical records of six newborns diagnosed with PPHN and had received inhaled iloprost from December 2019 to April 2022 were collected...

BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) is usually considered a consequence of impaired pulmonary circulation. However, little is known regarding the role of cardiac dysfunction in PPHN. In this study, we hypothesized that the tolerance for pulmonary hypertension in newborn infants depends on the biventricular function. The aim of this study is to evaluate biventricular cardiac performance by using Tissue Doppler Imaging (TDI) in an healthy newborn infants with asymptomatic pulmonary hypertension and in newborn infants with PPHN...

BACKGROUND: Persistent pulmonary hypertension of the newborn manifesting with refractory and severe cyanosis is the consequence of high pulmonary vascular resistance causing extrapulmonary right-to-left shunt. Acidosis and hypoxemia produce pulmonary vasoconstriction. Persistent pulmonary hypertension of the newborn occurs due to numerous disorders and has been rarely reported as a manifestation of methylmalonic acidemia. We report a newborn with methylmalonic acidemia who presented with persistent pulmonary hypertension of the newborn...

We report a case of double aneuploidy in a preterm male newborn with karyotype 48,XXY,+18 whose mother was of advanced age and infected with the SARS-CoV-2 virus during the early stages of her pregnancy. The clinical features observed in the newborn included intrauterine growth retardation, dysmorphic facial features, overlapping fingers on both hands, respiratory distress syndrome, ventricular septal defect, patent ductus arteriosus, persistent pulmonary hypertension, and bilateral clubfoot, a phenotype that mainly correlates with Edwards syndrome (trisomy 18)...

OBJECTIVE:  Terminating pregnancy appropriately before the intrauterine infection has progressed may have an improved prognosis for preterm infants. We evaluate how the combination of histological chorioamnionitis (hCAM) and clinical chorioamnionitis (cCAM) affects the short-term prognosis of infants. STUDY DESIGN:  This retrospective multicenter cohort study based on the Neonatal Research Network of Japan included extremely preterm infants born weighing <1,500 g between 2008 and 2018...

Trisomy 21, or Down syndrome (DS), is neonates' most common chromosomal abnormality. In addition, children born with DS have an increased risk of congenital anomalies such as congenital heart defects, gastrointestinal abnormalities, and, rarely, cleft palate. Cleft lip and palate are among the most common congenital anomalies associated with many congenital syndromes; however, Trisomy 21 is the least common congenital anomaly associated with orofacial clefts. We present a case of cleft palate, duodenal stenosis, persistent pulmonary hypertension of the newborn, patent ductus arteriosus, and atrial septal defect in a newborn with classical clinical features of Down syndrome...