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PubMed1981

[Persistence of fetal circulation. Clinical and angiographic evaluation of two cases. Review of the literature (author's transl)].

Persistent fetal circulation ("PFC") is a condition characterized by perduring after birth of fetal hemodynamic pattern: persistence of high pulmonary vascular resistance then occurs. "PFC" is a high mortality syndrome recently recognized as a cause of severe cyanosis and tachypnea in full-term newborns. Perinatal hypoxia, hyperviscosity, acidosis etc, may be also responsible of a persistence of high pulmonary vascular resistance. The increased right heart pressures cause a right to left shunt across the ductus arteriosus and foramen ovale not yet completely closed at birth. In this paper the clinical and angiographic evaluation of two full-term newborns, males, 5 days and 5 months old, are described, who presented with tachypnea, cyanosis and heart failure. Moreover we want to make a critical review of medical literature about this subject. The electrocardiogram of both patients showed right ventricular hypertrophy and myocardial ischemia. The chest roentgenograms showed cardiac enlargement and increased pulmonary vascular markings. Lung and central nervous system disorders were excluded by clinical and instrumental exams. Both patients underwent cardiac catheterization and angiocardiography in order to exclude a congenital heart disease. There was just evidence of pulmonary vascular resistance and right to left shunt at ductal and atrial level. O2 100% administration in one patient determined a decrease in pulmonary resistance and the shunting abolition. Clinical and hemodynamic patterns of this syndrome are very different. There are "simple forms" with normal myocardial function and "complex" higher mortality forms with evidence of cardiac involvement. It is often difficult to distinguish such forms from the other causes of central cyanosis and/or cardiac failure in the newborn as transposition of the great arteries, total anomalous pulmonary venous connection: the individualization of the syndrome then may be impossible without cardiac catheterization. A correct diagnosis for a precocious and reasonable treatment are extremely important for the survival of such critical newborns.

Angiocardiography↗
PubMed2005

Utilization and outcomes of neonatal cardiac extracorporeal life support: 1996-2000.

OBJECTIVES: Extracorporeal life support for neonatal respiratory failure has decreased, but utilization and outcome of cardiac extracorporeal life support are not well characterized. Among neonates born 1996-2000, our objects were to evaluate changes in utilization and outcome of cardiac extracorporeal life support and characterize correlates of survival. DESIGN: Retrospective analysis of Extracorporeal Life Support Organization Registry data. SETTING: Intensive care units participating in the ELSO registry. PATIENTS: Patients placed on extracorporeal life support for center-specified "cardiac support" at </=30 days of age from 1996 to 2000. Patients with hypoplastic left heart syndrome were also analyzed separately. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patient characteristics and correlates of survival to discharge or transfer were analyzed by chi-square, Student's t-test, and logistic regression analysis. Neonates placed on cardiac extracorporeal life support increased from 112 in 1996 to 200 in 2000 (total n = 740). Overall survival was 34.2%: 28% for hypoplastic left heart syndrome and 35.4% for nonhypoplastic left heart syndrome. For the overall group, no significant correlations were found between survival and year on extracorporeal life support, multiple runs, or diagnosis of hypoplastic left heart syndrome. Diagnoses of transposition of the great arteries (p = .03) or persistent pulmonary hypertension of the neonate (p = .004) and extracorporeal life support at <3 days (p = .003) were associated with higher survival. Survivors had fewer mean extracorporeal life support hours (125.5 +/- 121.4 vs. 159.0 +/- 127.6, p = .0006). Logistic regression confirmed significant bivariate findings. A total of 118 hypoplastic left heart syndrome patients were reported from 1996 to 2000. Extracorporeal life support at >15 days was associated with improved survival among hypoplastic left heart syndrome patients (p = .03), and survivors had fewer mean extracorporeal life support hours (89.3 +/- 52.3 vs. 147.5 +/- 129.7, p = .015). Logistic regression showed that only greater number of hours on extracorporeal life support was independently associated with nonsurvival. CONCLUSIONS: Neonatal cardiac extracorporeal life support use increased substantially from 1996 to 2000, with survival to discharge or transfer in more than one third of patients. Hypoplastic left heart syndrome was not associated with nonsurvival. Fewer hours on extracorporeal life support, diagnoses of persistent pulmonary hypertension of the neonate and transposition of the great arteries, and extracorporeal life support at <3 days were associated with survival.

Extracorporeal Membrane Oxygenation↗
PubMed2001

International experience with trisomy 21 infants placed on extracorporeal membrane oxygenation.

OBJECTIVE: To characterize the international experience concerning neonates with trisomy 21 (T21) managed with extracorporeal membrane oxygenation (ECMO), and to compare and contrast this group of patients to the neonatal ECMO population as a whole. METHODS: Data from the Extracorporeal Life Support Organization for newborn infants placed on ECMO between January 1984 and June 1999 were analyzed. Infants with T21 were compared with the group of infants without T21. RESULTS: Fifteen thousand nine hundred forty-six infants, including 91 (n = 91) with the diagnosis of T21, were placed on ECMO for neonatal respiratory failure during the 14.5-year period. T21 infants were overrepresented in the ECMO population by several-fold when compared with the incidence of T21 in the general population. Eighty-seven of the 91 T21 infants were placed on ECMO after 1989. The distribution of primary diagnoses leading to ECMO differed between the groups (T21 vs non-T21): primary persistent pulmonary hypertension, 47.3% versus 13%; meconium aspiration syndrome, 23.1% versus 32.9%; sepsis, 7.7% versus 13.2%; congenital diaphragmatic hernia, 7.7% versus 19.9%; and respiratory distress syndrome, 3.3% versus 7.9%. Although survival to discontinuation of ECMO was similar in the 2 groups, likelihood of survival to discharge was decreased for T21 infants (65.9% vs 75.6%) because of increased post-ECMO mortality. CONCLUSIONS: Extracorporeal Life Support Organization registry data suggests that T21 infants are at a significantly higher risk of being placed on ECMO for neonatal respiratory failure than the general population, perhaps as a result of delayed extrauterine pulmonary vascular adaptation, as manifested in the high rate of primary persistent pulmonary hypertension as the primary diagnosis. There may have been a shift in attitude regarding the use of ECMO in the T21 patient after 1989. Although most T21 patients placed on ECMO will survive, the prognosis is more guarded in this population when compared with all infants so managed. The long-term neurodevelopmental outcome of this group of T21 ECMO survivors is currently unknown.

Down Syndrome↗
PubMed1980

Serum salicylate levels and right-to-left ductus shunts in newborn infants with persistent pulmonary hypertension.

Although a right-to-left shunt via a patent ductus arteriosus is one criterion for the diagnosis of persistent pulmonary hypertension of the newborn infant, it cannot be demonstrated by simultaneous pre- and postductus arteriosus blood oxygen tensions in many infants with the clinical syndrome. In animals, exposure of the fetal ductus arteriosus to salicylates causes contriction and results in pulmonary hypertension. We postulated that maternal ingestion of salicylates and premature closure of the ductus arteriosus may explain why some infants with PPHN do not have right-to-left ductus shunts. Therefore, we studied serum salicylate levels in six groups of infants: I, normal infants' cord blood (0.73 +/- 0.44 mg/dl, N = 20); Ia, normal infants at 24 to 36 hours of age (0.08 +/- 0.1 mg/dl, N = 5): II, other cardiopulmonary diseases with no right-to-left ductus shunt (2.08 +/- 1.74 mg/dl, N = 26); III, other cardiopulmonary diseases and right-to-left ductus shunt (2.34 +/- 1.70 mg/dl, delta Pao2 70 +/- 71 mm Hg, N = 6); IV, PPHN and right-to-left ductus shunt (1.86 +/- 1.51 mg/dl, delta Pao2 39.6 +/- 58.9 mm Hg, N = 5); V, PPHN without right-to-left ductus shunt (7.77 +/- 5.18 mg/dl, delta Pao2 2.2 +/- 1.5 mm Hg, N = 6). Serum salicylate levels were significantly greater (P less than 0.01) in infants with PPHN without right-to-left ductus shunt, indicating that the ductus arteriosus may have been closed prematurely. No other factor, including serum bilirubin, amikacin, ampicillin, or furosemide levels, could be found to account for the difference in serum salicylate levels. Premature closure of the ductus arteriosus secondary to maternal ingestion of salicylates may be one cause of PPHN and may explain the absence of right-to-left ductus shunting in some infants with the clinical syndrome.

Ductus Arteriosus↗
PubMed1983

[Value of 2-dimensional echocardiography in symptomatic heart diseases in the newborn infant and in early infancy].

From January 1980 to March 1983, 73 distressed newborns and young infants, in whom congenital cardiac defects were suspected, were investigated by two-dimensional echocardiography. The diagnosis in these infants could be re-established later by cardiac catheterization and cineangiocardiography, operation or autopsy. Correct diagnoses were set in 97% of the patients (71 out of 73). Specific echocardiographic criteria for the diagnosis of the individual defects are discussed.

Cardiac Catheterization↗
PubMed1988

Effects of prostaglandin D2 on pulmonary arterial pressure and oxygenation in newborn infants with persistent pulmonary hypertension.

We studied the effects of prostaglandin D2 (PGD2) in six newborn infants, 1 to 2 days of age, who had persistent pulmonary hypertension syndrome and a PaO2 less than 75 torr during mechanical hyperventilation with an inspired oxygen concentration of 100%. Tolazoline and dopamine were used to treat some of the patients. No patients had congenital heart disease or sepsis. Catheters were placed to measure pulmonary and systemic arterial blood pressures. PGD2 was infused intravenously at doses of 1 to 25 micrograms/kg/min. Pulmonary and systemic arterial blood pressures, heart rate, and descending aortic blood gas values were measured before each dose change. Only two of six patients had a transient increase in PaO2. All had an increase in heart rate. Two of six patients had an increase in pulmonary arterial blood pressure. No deleterious effects occurred during the infusion. Four of six patients subsequently died. Although PGD2 is a specific pulmonary vasodilator in fetal and newborn animals, it did not lower pulmonary arterial blood pressure nor improve oxygenation in newborn infants with persistent pulmonary hypertension syndrome.

Blood Pressure↗
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↗
PubMed2004

Clinical characteristics and follow up of Down syndrome infants without congenital heart disease who presented with persistent pulmonary hypertension of newborn.

We identified seventeen infants with Down syndrome without structural congenital heart disease who presented with persistent pulmonary hypertension in the newborn period. Respiratory distress with or without hypoxia was the presenting feature in these infants. Pulmonary hypertension resolved in the majority of the survivors. Two infants with refractory pulmonary hypertension benefited from patent ductus arteriosus ligation. Autopsies in two infants demonstrated structural lung immaturity. We suggest that infants with Down syndrome are at risk of developing persistent pulmonary hypertension even in the absence of structural heart disease and these infants should be followed up until resolution of the pulmonary hypertension.

Down Syndrome↗
PubMed1987

Umbilical cord compression produces pulmonary hypertension in newborn lambs: a model to study the pathophysiology of persistent pulmonary hypertension in the newborn.

We investigated the effects of chronic intrauterine hypoxaemia produced by prolonged partial umbilical cord compression on the circulation shortly after birth in lambs. Vascular catheters were inserted in 10 fetal sheep at 120 to 130 days gestation to measure descending aortic blood gases, arterial pH, and arterial O2 saturation. An inflatable silicone rubber balloon cuff was also placed around the umbilical cord. After recovery and the return of descending aortic blood gases to the normal range, the balloon was gradually inflated, decreasing the PaO2 from 21.2 +/- 3.6 to 17.5 +/- 1.3 mm Hg and the arterial O2 saturation from 57.1 +/- 9.2% to 37.2% +/- 5.2. After 14.3 +/- 3.7 days of partial umbilical cord compression, the lambs were delivered by Caesarean section, instrumented to measure systemic and pulmonary arterial, right atrial and pulmonary arterial wedge pressures, pulmonary and systemic blood flows, and mechanically ventilated. Five normal lambs were also studied. From 60 to 120 min after delivery, when compared to normal lambs, the umbilical compression lambs had an increased pulmonary arterial pressure (P less than 0.05) pulmonary vascular resistance (P less than 0.05), and right atrial pressure (P less than 0.05) with similar arterial blood gases. In both groups, hypoxic ventilation produced an increase in pulmonary arterial pressure (P less than 0.05) which on return to room air ventilation decreased to baseline in the normal lambs but not in the umbilical cord compression lambs (P less than 0.05). Prolonged partial umbilical cord compression produces chronic fetal hypoxaemia and pulmonary arterial hypertension after birth. This may represent a model to study the pathophysiology of persistent pulmonary hypertension syndrome.

Animals↗
PubMed2026

Severe Suprasystemic Refractory Pulmonary Hypertension in a Neonate with St&#xfc;ve-Wiedemann Syndrome Associated with Biallelic LIFR Variants: Molecular Insights and a Neonatal Case Report.

St&#xfc;ve-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↗
PubMed1986

Increased arterial pH, not decreased PaCO2, attenuates hypoxia-induced pulmonary vasoconstriction in newborn lambs.

Mechanically induced hyperventilation is used in the treatment of newborn infants with persistent pulmonary hypertension syndrome to induce respiratory alkalosis, which may attenuate their pulmonary vasoconstriction. Whether this treatment is effective because of the increase in arterial pH or the decrease in Paco2 was investigated in nine sedated, mechanically ventilated newborn lambs with hypoxia-induced pulmonary vasoconstriction. We found that respiratory alkalosis and metabolic alkalosis were equally effective in attenuating hypoxia-induced pulmonary vasoconstriction, but that hypocapnia (low Paco2 with a normal arterial pH) was ineffective. These results indicate that increased arterial pH, not decreased Paco2, attenuates hypoxia-induced pulmonary vasoconstriction in newborn lambs and possibly the pulmonary vasoconstriction in newborn infants with persistent pulmonary hypertension syndrome.

Alkalosis↗