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PubMed2024

Metagenomic Analysis of Gut Microbiome of Persistent Pulmonary Hypertension of the Newborn.

Persistent pulmonary hypertension of the newborn (PPHN) is one of the most common diseases in the neonatal intensive care unit which severely affects neonatal survival. Gut microbes play an increasingly important role in human health, but there are rarely reported how gut microbiota contribute to PPHN. In our study, the metagenomic sequencing of feces from 12 PPHN's neonates and 8 controls were performed to expose the relation between neonatal gut microbes and PPHN disease. Firstly, we found that the abundance of Actinobacteria, Proteobacteria, Bacteroidetes were significantly increased in PPHN compared with controls, but the Firmicutes components was reduced. And some pathogenic strains (like Vibrio metschnikovii) were significantly enriched in the PPHN compared with controls. Secondly, functional annotation of genes found that PPHN up-regulated transmembrane transport, but down-regulated ribosome and ATP binding. Lastly, microbial metabolic pathway enrichment analysis indicated that some metabolic pathway in PPHN were conflicting and contradictory, showed that an abnormally increased metabolism, disturbed protein synthesis and genomic instability in the PPHN neonate. Our results contribute to understanding the changes in the species and function of gut microbiota in PPHN, thus providing a theoretical basis for the explanation and treatment of PPHN.

Gastrointestinal Microbiome↗

PubMed1992

Persistent pulmonary hypertension complicating cystic adenomatoid malformation in neonates.

Neonates with congenital diaphragmatic hernia (CDH) are known to be susceptible to stress-induced persistent pulmonary hypertension (PPHN). Congenital cystic adenomatoid malformations (CCAMs) may also present as respiratory distress in the newborn. Intubation and mechanical ventilation cause clinical deterioration because of air trapping within cystic spaces; these patients require prompt lobectomy. PPHN has not been commonly associated with CCAM. Three patients with CCAM were encountered who developed PPHN postlobectomy. Three newborns, 36 to 38 weeks' gestation, presented with respiratory distress. Two had diagnosis of thoracic tumors on fetal ultrasound (22 and 33 weeks). Chest x-ray at birth confirmed cystic intrathoracic tumors in all and they underwent immediate thoracotomy and lobectomy (1 right upper, 1 left lower, 1 left upper). The patients were stable for 4 hours to 5 days postoperatively and then developed findings consistent with PPHN by cardiac echocardiography and required extracorporeal membrane oxygen (ECMO) support. ECMO was required for 66.5 to 120 hours. Each patient was successfully weaned to conventional ventilatory support. The clinical course of these patients was similar to those with CDH who undergo immediate surgery. The stress of surgical intervention combined with hypoxia and hypercarbia stimulates a hyperactive pulmonary vasculature and the development of PPHN. ECMO provides an effective adjunct to support patients with PPHN on the basis of congenital cystic adenomatoid malformations.

Cystic Adenomatoid Malformation of Lung, Congenita↗

PubMed1990

Circulating plasma platelet activating factor in persistent pulmonary hypertension of the newborn.

Platelet activating factor (PAF) is an endogenous phospholipid mediator that causes pulmonary hypertension and thrombocytopenia in experimental animal models. To investigate circulating PAF in persistent pulmonary hypertension of the newborn (PPHN), we studied PAF and its degradative enzyme, acetylhydrolase. Thirteen neonates with PPHN, diagnosed by routine clinical methods including echocardiography, were compared to six age-matched control patients with respiratory distress. Overall, plasma PAF levels were elevated in patients with PPHN compared to control patients (20.1 +/- 3.9 versus 1.6 +/- 0.7 ng/ml, p less than 0.01). In addition, plasma PAF concentrations in patients with PPHN correlated with the severity of disease as defined by the delta AaPO2 (r = 0.65, p = 0.015). In three patients with elevated PAF levels, as the clinical status improved, the plasma PAF values decreased. Acetylhydrolase activity was similar in both groups (3.96 +/- 0.90 versus 3.78 +/- 1.44 nmol/ml/min, p = NS). We conclude that PAF production is increased in PPHN and that abnormal production of PAF may be associated with pulmonary hypertension.

1-Alkyl-2-acetylglycerophosphocholine Esterase↗

PubMed1991

Profile and stability of sensorineural hearing loss in persistent pulmonary hypertension of the newborn.

The purpose of this study was to examine the incidence, profile, and stability of sensorineural hearing loss (SNHL) in infants diagnosed with persistent pulmonary hypertension of the newborn (PPHN). Over a five-year period, 19 of 51 infants (37%) with PPHN were diagnosed with SNHL: 16 with bilateral and 3 with unilateral impairment. This incidence of SNHL is approximately 25 times greater than observed in graduates of our intensive care nursery. The profile of SNHL in the group of 19 children was typically downward sloping. However, there was considerable variation with respect to degree of loss. In addition, progressive SNHL was identified in 5 children whose hearing worsened an average of 55 dB at 2 to 4 kHz. On retrospective analysis, the perinatal variables associated with PPHN were comparable between hearing-impaired and normal-hearing infants. In contrast, the two groups were significantly different when treatment variables, such as the duration of mechanical ventilation or amikacin, were compared. Children with SNHL were treated for longer durations as compared to normal-hearing infants. Children with SNHL were subdivided into two groups, mild and severe, based on degree of loss and treatment variables, and compared again to the normal-hearing group. Two findings emerged from this analysis. First, the duration of hyperventilation was now the only variable significantly different between children with mild SNHL and children with normal hearing. Second, comparison of the mild with the severe SNHL groups showed that the duration of hyperventilation was similar. The pathophysiology and underlying mechanisms resulting in SNHL in PPHN survivors are discussed.

Amikacin↗

PubMed1990

[Persistent pulmonary hypertension of newborn. The PFC syndrome].

Persistent pulmonary hypertension of the newborn (PPHN), initially described by Gersony et al as persistent foetal circulation (PFC syndrome), results from a flawed transition from foetal to extrauterine pulmonary circulation. It is characterised by the maintenance of a high pulmonary vascular resistance and right-to-left shunting through the ductus arteriosus and foramen ovale. Infants with a wide variety of underlying clinical conditions develop PPHN. According to Rudolph three main anatomic types of PPHN can be identified: normal pulmonary vascular development increased pulmonary vascular smooth muscle development decreased cross-sectional area of pulmonary vascular bed. It is important to realize that several pathophysiologic mechanisms may coexist and interact. Besides metabolic and respiratory acidosis, hypercapnia and hypoxaemia some other factors induce pulmonary vasoconstriction. Thromboxane, leukotrienes and prostaglandins play a decisive role. Since PPHN can be associated with a broad spectrum of clinical conditions, a specific clinical picture is lacking. The baby is usually term or post-term, cyanotic immediately after birth or some hours later. Birth asphyxia, hyperviscosity, sepsis and aspiration of meconium have been recognized as predisposing factors. The diagnosis can be confirmed by echocardiography. Contrast echo will indicate right-to-left shunting with normal anatomy. Currently hyperventilation, tolazolin, chlorpromazin and dopamine/dobutamine have been advocated as central foci for clinical therapy. Recently prostacyclin was introduced as a specific pulmonary vasodilatator.(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiography↗

PubMed1992

Vasodilators in persistent pulmonary hypertension of the newborn: a need for optimal appraisal of efficacy.

Tolazoline hydrochloride is usually the first choice pulmonary vasodilator in persistent pulmonary hypertension of the neonate (PPHN). The analysis of 26 articles including 467 tolazoline-treated infants has been hindered by many methodological drawbacks. Tolazoline has always been administered to infants suffering refractory hypoxemia, but, unfortunately, pulmonary hypertension has not usually been investigated. Moreover, 80% of the tolazoline-treated neonates had an underlying pulmonary parenchymal disease as a potential cause of severe hypoxemia. Noteworthy is that similar comments apply to all studies dealing with the use of other pulmonary vasodilators in PPHN. Pulsed Doppler echocardiography (PDE) should allow a qualitative and quantitative approach for PPHN and an analysis of both success and failure of vasodilator therapeutics. In the meantime, the use of PDE requires more intense investigation prior to wide application in PPHN.

Epoprostenol↗

PubMed1991

Histological study of pulmonary vasculature in fatal cases of persistent pulmonary hypertension of the newborn.

Using our own morphometric technique, we examined pulmonary vasculature in eight neonates who died of persistent pulmonary hypertension of the newborn (PPHN). PPHN was judged to be idiopathic in three neonates and was associated with fatal meconium aspiration in the other five. Two characteristic structural features of PPHN were revealed in this study. First, medial thickening of the normally fully-muscularized pulmonary vessels occurred in the smaller and more peripherally located arteries. Second, fully-muscularized vessels extended as far as the intraacinar arteries, which are normally non-muscular vessels at this age. This structural abnormality appears not to merely represent a failure of the fetal pattern to regress, and it is suggested that PPHN is due to a structural abnormality of the pulmonary microcirculation in fatal cases.

Humans↗

PubMed1991

Effects of indomethacin in utero on the pulmonary vasculature of the newborn lambs.

Exposure of the fetus to indomethacin and the resultant closure of the ductus arteriosus in utero has been suggested to be one possible cause of persistent pulmonary hypertension of the newborn (PPHN). We examined whether or not closing the ductus arteriosus of fetal lambs by the administration of in-domethacin to the fetus and ewe could produce the functional and structural changes of PPHN. Three treated and four non-treated lambs were delivered by cesarean section. Postnatal hemodynamics were studied in two of the treated lambs. Pulmonary arterial pressure gradually decreased after delivery and did not reveal features compatible with PPHN, while the blood gases remained within the physiological range. However, acute hypoxemia produced a re-elevation in pulmonary arterial pressure. Morphometric analysis showed the medial thickening of normally fully-muscularized pulmonary arteries, but there was no abnormal extension of fully-muscularized arteries to the intraacinar level in the treated lambs. In conclusion, we could not reproduce functional and structural changes of PPHN in newborn lambs by the administration of indomethacin in utero. However, our experimental animal model with excessive muscularization of the pulmonary vasculature appears to be useful for studying vasoconstriction in response to hypoxemia.

Animals↗

PubMed2026

Severe Suprasystemic Refractory Pulmonary Hypertension in a Neonate with Stüve-Wiedemann Syndrome Associated with Biallelic LIFR Variants: Molecular Insights and a Neonatal Case Report.

Stüve-Wiedemann syndrome (SWS) is an ultra-rare autosomal recessive skeletal dysplasia caused by loss-of-function variants in the leukemia inhibitory factor receptor (LIFR) gene. While characterized by bone deformities and dysautonomia, severe persistent pulmonary hypertension of the newborn (PPHN) significantly contributes to high early mortality. We report a neonate with genetically confirmed SWS who presented with severe, suprasystemic PPHN refractory to standard pulmonary vasodilators, including inhaled nitric oxide. This case provides a detailed longitudinal hemodynamic characterization of severe suprasystemic PPHN in genetically confirmed SWS, including serial assessment of pulmonary pressures, shunt direction, and right ventricular function during treatment. Rather than identifying PPHN as a novel manifestation of SWS, it extends the phenotypic and hemodynamic characterization of pulmonary vascular involvement in this rare disorder.

Humans↗

PubMedDate not supplied

[Prostacyclin in the treatment of persistent fetal circulation syndrome].

Persistent pulmonary hypertension of the neonate (PPHN), described initially by Gersony in 1969 as persistent foetal circulation (PFC syndrome), results from a flawed transition from foetal to extrauterine pulmonary circulation. It is primarily characterised by persistence of, or return to, the suprasystemic pulmonary vascular resistance and pressure normally found in the foetus. The increased pulmonary pressure causes right to left shunting through the ductus arteriosus or the foramen ovale, or both. The resulting hypoxaemia and acidosis may produce further pulmonary vasoconstriction and lead to a vicious cycle of shunting, hypoxia and acidosis. Infants with a wide variety of underlying clinical conditions develop PPHN. This condition is reversible, but can cause very severe and unrelenting respiratory failure and ultimate death when uncontrolled. Although vasodilating agents, such as tolazoline, have been used with variable success in the treatment of PPHN, a generally acceptable therapy is still lacking. We report here the use of prostacyclin (epoprostenol, PGI2) in two infants with severe and refractory hypoxaemia secondary to pulmonary vasoconstriction.

Epoprostenol↗

PubMed1992

High frequency oscillatory ventilation and extracorporeal membrane oxygenation in severe persistent pulmonary hypertension of the newborn.

We report on 50 term and near-term neonates (birth weight greater than 1800 g, gestational age greater than 33 weeks) with severe persistent pulmonary hypertension of the newborn (PPHN), referred to us from January 1987 to July 1991 after failure of maximum conventional treatment. All infants had paO2 less than 45 mm Hg when ventilated with peak inspiratory pressure greater than 38 cm H2O and FiO2 = 1.0, hence meeting entry criteria for extracorporeal membrane oxygenation (ECMO). High frequency oscillatory ventilation (HFOV) was tried in all patients. If sufficient oxygenation could not be achieved (paO2 less than 40 mm Hg for at least 2 h), ECMO therapy was begun, which was the case in 25 children. Neonates responding to HFOV (n = 25) were of a slightly younger gestational age (37.0 weeks vs 38.8 weeks, P less than 0.05), had higher Apgar scores and were less hypoxaemic before HFOV (paO2 36.6 mm Hg vs 28.8 mm Hg, P less than 0.01); during HFOV there was a significant rise in paO2 (greater than 150 mm Hg; P less than 0.001) and a fall in pCO2 to 21.6 mm Hg (P less than 0.001). Due to air leaks, which was the main complication of HFOV (52%), ECMO therapy had to be begun in two additional infants after an initial positive effect. HFOV tended to be successful in cases of primary PPHN, meconium aspiration and sepsis, but not in infants with lung hypoplasia as a result of diaphragmatic hernia or other reasons. Success or failure of HFOV could not be reliably predicted by any parameter.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Gas Analysis↗

PubMedDate not supplied

Persistent pulmonary arterial hypertension of the newborn.

Persistent pulmonary hypertension of the newborn (PPHN) characterised by right to left shunting with intense cyanosis is difficult to manage, and in the best of centres carries a 40-60 percent mortality. We report our one year's experience of managing six neonates with PPHN. There were 5 males and 1 female with mean birth weight of 2.59 +/- 0.487 kg and gestation period 39 +/- 2.0 wks and 1 minute Apgar score 2.8 +/- 2.1. Four to six babies were born by cesarean section and 3-6 babies had aspiration pneumonia. All babies presented within 12 hours of age (mean 5.08 +/- 5 hrs) with intense cyanosis and respiratory distress. Diagnosis were confirmed in all by (a) hyperoxia test, (b) simultaneous determination of preductal and postductal paO2 (c) contrast echocardiography and (d) hyperoxia-hyperventilation test. Babies were managed with hyperventilation using mean ventilatory rates of 100 +/- 45 per minute, an inspired oxygen concentration of 100%, peak inspiratory pressures 27 +/- 9 cm of H2O, and expiratory pressures 5 +/- 1.6 cms of H2O, and mean air way pressures of 10.4 +/- 2.7 cms H2O. Alkali therapy was used in 3 of the six babies whereas low dose dopamine was infused in all six babies. Inspite of aggressive ventilatory therapy, only 3 out of 6 babies could be salvaged.

Birth Weight↗

PubMed1990

Extracorporeal membrane oxygenation and congenital diaphragmatic hernia: should any infant be excluded?

Mortality in infants with congenital diaphragmatic hernia (CDH) remains high despite improvements in neonatal and surgical care because many infants develop persistent pulmonary hypertension of the newborn (PPHN) following repair. Since 1984, extracorporeal membrane oxygenation (ECMO) has been used as rescue therapy in all infants (n = 25) with PPHN following CDH repair when conventional management failed, with an overall survival of 60%. Repair was performed in this hospital on 12 infants and in other hospitals in 13 infants transferred for consideration of ECMO after repair. Mortality was the same in the group repaired here and those transferred for ECMO. Although complications were frequent in the surviving group, they were successfully managed with nonoperative or operative therapy. Selective use of ECMO has been advocated in CDH patients based on various predictors of high mortality such as "best" PO2 postrepair less than 100 mm Hg, oxygenation index greater than 40, and ventilation index greater than 1,000 with PCO2 greater than 40. Seven surviving infants following ECMO would have been classified as unsalvageable by at least one parameter if selection criteria based on these parameters had been used. We conclude from this series that current predictors of high mortality in CDH patients are unreliable when ECMO is used. Surgeons caring for infants with CDH should consider the use of ECMO in all infants.

Extracorporeal Membrane Oxygenation↗

PubMed1991

Extracorporeal membrane oxygenation and neonatal respiratory failure: experience from the extracorporeal life support organization.

Extracorporeal membrane oxygenation (ECMO) has rescued moribund infants with respiratory failure from a variety of causes. We report the experience from 58 United States and 7 overseas ECMO centers between 1980 and 1989. Voluntarily submitted data forms provided details of diagnosis, clinical condition, ECMO indications, morbidity, and mortality. Of 3,528 infants with a predicted mortality greater than 80% treated with ECMO, 83% survived. Entry diagnoses and aggregate survival were: meconium aspiration syndrome (MAS) 1,356 (93%), persistent pulmonary hypertension of the newborn (PPHN) 480 (83%); congenital diaphragmatic hernia (CDH) 585 (62%); hyaline membrane disease (HMD) 532 (84%); sepsis 416 (77%); and other 185 (77%). ECMO indications were a-AdO2 greater than 600 for 6 to 8 hours (22%), oxygenation index greater than 40 for 4 hours (18%), acute deterioration (14%), maximal therapy failure (34%), and barotrauma (1%). Annual survival improved over 9 years except for CDH, which decreased from 70% (1987) to 56% (1989) P less than .01). Survivors differed from non-survivors (P less than .05) by birth weight (greater than 2 kg), gestational age (greater than 37 weeks), entry diagnosis (MAS, PPHN, HMD, sepsis v CDH), inborn versus outborn, pre-ECMO pH, and ECMO duration. Technical complications in 25% of patients and medical complications in 75% adversely affected survival. Annual sepsis survival improved to 75% (1989) but had significantly greater complication rates (P less than .05) than other diagnoses. Multicenter data yield information not available from single institution experience. Although entry criteria and conventional therapy continue to evolve, ECMO currently improves survival from an estimated 20% to 83% overall. Individual prognosis depends on entry diagnosis, clinical condition, and complications.

Equipment Failure↗

PubMed1992

Inhaled nitric oxide in persistent pulmonary hypertension of the newborn.

Nitric oxide (NO) has vasodilatory effects on the pulmonary vasculature in adults and animals. We examined the effects on systemic oxygenation and blood pressure of inhaling up to 80 parts per million by volume of NO at FiO2 0.9 for up to 30 minutes by 6 infants with persistent pulmonary hypertension of the newborn (PPHN). In all infants this treatment rapidly and significantly increased preductal oxygen saturation (SpO2); in 5 infants postductal SpO2 and oxygen tensions also increased. Inhalation of NO did not cause systemic hypotension or raise methaemoglobin. These data suggest that low levels of inhaled NO have an important role in the reversal of hypoxaemia due to PPHN.

Administration, Inhalation↗

PubMed1991

Possible causes linking asphyxia, thick meconium and respiratory distress.

The cause of fetal distress and neonatal respiratory distress (RD) in association with meconium-stained liquor is not always clear. To clarify this, a prospective study was undertaken in a tertiary referral maternity hospital for 1 year. In all infants born after meconium-stained liquor who developed RD, evidence was sought for 1) fetal distress (from the cardiotocograph (CTG), the cord blood pH, the Apgar score and the asphyxial complications in the neonate) 2) causes of fetal distress (including maternal risk factors, fetal infection and fetal malnutrition) 3) causes of respiratory distress (including meconium aspiration syndrome (MAS), persistent pulmonary hypertension of the newborn (PPHN) and infection). Of 4,026 livebirths, 717 (17.8%) had meconium-stained liquor and 44 term and 5 preterm infants developed RD. In the 44 term infants, there was frequent evidence of fetal distress possibly caused by previously unrecognized factors such as fetal malnutrition with reduced neonatal skinfold thickness in 35% triceps and 41% subscapular measurements, and histological chorioamnionitis (CA) in 74%. The cause for respiratory distress was identified in only 48% of infants, and included clinical evidence of PPHN (41%), MAS (16%) and infection (2%). However in preterm infants, 80% had definite or suspected infection. The findings indicate that fetal distress is common in infants who develop respiratory distress after meconium-stained liquor. A role for histological CA and reduced nutrition in the fetus, as factors contributing to the vulnerability of the term infant to intrapartum fetal distress, is suggested.

Apgar Score↗

PubMed1992

Treatment of severe persistent pulmonary hypertension of the newborn with magnesium sulphate.

Eight of nine newborn infants with severe persistent pulmonary hypertension of the newborn (PPHN), and a predicted mortality of 100%, and one infant with a predicted mortality greater than 94% based on alveolar-arterial oxygen tension difference [A-a)DO2) were treated with magnesium sulphate (MgSO4) as a life saving therapy after they failed to improve with conventional treatment. Magnesium at high serum concentrations decreases pulmonary pressures and is a muscle relaxant and sedative. Diluted MgSO4.7H2O solution (200 mg/kg) was given intravenously over 20-30 minutes. No changes in the treatment were made after MgSO4. Mean serum magnesium concentration was maintained between 2.88 and 5.67 mmol/l by continuous intravenous infusion (six infants). Baseline arterial oxygen tension (PaO2) and haemoglobin oxygen saturation had mean (SD) values of 4.66 (1.8) kPa and 60.4 (29.7)% respectively, which started to increase one hour after MgSO4 infusion, and increased significantly at six hours to 12.04 (7.07) kPa and 91.8 (10.88)% respectively. Arterial carbon dioxide tension (PaCO2) decreased and pH increased significantly after one hour compared with the baseline value. PaO2 increases are probably secondary to a decrease in pulmonary vascular resistance and pressure, decrease in a right to left shunt, better ventilation:perfusion ratio, and PaCO2 decrease and pH rise. Seven infants survived (77.8%). These results demonstrate the beneficial effect of magnesium in the management of PPHN when other accepted treatment fails, is contraindicated, or not available.

Humans↗

PubMed2026

Umbilical Cord-Derived Cell-Based Interventions for Bronchopulmonary Dysplasia and Related Complications in Preterm Infants: A Bayesian Sparse-Data Meta-Analysis.

Bronchopulmonary dysplasia (BPD) is a major complication of prematurity with limited disease-modifying therapies. We evaluated umbilical cord-derived cell-based interventions for BPD and related complications in preterm infants. This Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020-based systematic review and meta-analysis were registered in PROSPERO. PubMed, Cochrane Library, Web of Science, CNKI, and Wanfang were searched from inception to June 14, 2026. Comparative clinical studies of umbilical cord-derived cell-based interventions in preterm infants at risk of or diagnosed with BPD were included. Outcomes included BPD, BPD severity, death, persistent pulmonary hypertension of the newborn (PPHN), patent ductus arteriosus (PDA), intraventricular hemorrhage (IVH), necrotizing enterocolitis (NEC), retinopathy of prematurity (ROP), late-onset sepsis (LOS), and adverse events (AEs). Bayesian random-effects meta-analysis used a binomial-normal hierarchical model to estimate pooled odds ratios (ORs), 95% credible intervals (CrIs), prediction intervals, and heterogeneity. Twelve studies were included. Umbilical cord-derived cell-based interventions showed a possible protective effect on overall BPD (OR, 0.48; 95% CrI, 0.14-1.20). Stronger associations were observed for severe BPD (OR, 0.17; 95% CrI, 0.01-0.85), moderate or severe BPD (OR, 0.28; 95% CrI, 0.09-0.70), and ROP stage ≥3 (OR, 0.17; 95% CrI, 0.02-0.65). No conclusive benefit or harm was observed for death, PPHN, PDA, IVH, NEC, or LOS. No treatment-related serious AEs were identified. However, prediction intervals were generally wide, and the certainty of evidence was low to very low for most outcomes. Umbilical cord-derived cell-based interventions may reduce the risk of moderate or severe BPD in preterm infants, with an additional potential benefit for ROP stage ≥3. Current evidence remains limited, and larger randomized trials with standardized outcomes and long-term follow-up are needed.

Humans↗