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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. The primary end point was mortality among those who did or did not receive PH-targeted treatment, and the secondary end points included comparisons of major morbidities. Variables were compared using t tests, Wilcoxon rank-sum tests, Fisher exact tests, and χ² tests. RESULTS: We analyzed the results of 224 preterm infants enrolled in the registry. Of which, 84% (188/224) received PH-targeted treatment, most commonly inhaled nitric oxide (iNO). Early mortality in this cohort was high, as 33% (71/224) of this sample died in the first month of life, and 77% of survivors (105/137) developed bronchopulmonary dysplasia. Infants who received PH-targeted treatment had higher oxygenation indices at the time of enrollment (28.16 [IQR: 13.94, 42.5] vs 15.46 [IQR: 11.94, 26.15]; P = .0064). Patient outcomes did not differ between those who did or did not receive PH-targeted therapy. CONCLUSIONS: Early-onset HRF with PH in preterm infants is associated with a high early mortality and a high risk of developing bronchopulmonary dysplasia. iNO is commonly used to treat early-onset PH in preterm infants with HRF. In comparison with untreated infants with lower oxygenation indices, iNO treatment in severe PH may prevent poorer outcomes.

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OBJECTIVES: To assess the management and outcomes of neonatal arteriovenous brain malformations (mostly vein of Galen malformations) complicated by cardiac failure in the era of prenatal diagnosis and endovascular treatment in a tertiary referral center. STUDY DESIGN: This observational study included 77 living newborn infants with arteriovenous brain malformations with cardiac failure, admitted to our referral center from 2001 to 2017. All infants underwent cardiovascular evaluation including echocardiogram and brain magnetic resonance imaging. Long-term survivors had standard neurocognitive assessments. RESULTS: Infants were admitted to the neonatal intensive care unit at a median of 5 days of age (including 18 inborn patients since 2009). Sixty transarterial shunt embolizations were performed in 46 patients during their first month (at a median age of 7.5 days) or postponed beyond the first month in another 10 long-term survivors. Embolization was not performed in 21 infants, including 19 nonsurvivors with severe brain injury, uncontrolled cardiac failure, or multiple organ failure. Cardiac failure requiring vasopressor infusion occurred in 48 patients (64%) during the hospitalization. Infants who survived the first month underwent a median of 3 embolization sessions. Among the 51 survivors, 21 had a good outcome and 19 had a poor outcome at follow-up (median age, 5.3 years); 11 children were lost to follow-up. CONCLUSIONS: In the era of multidisciplinary prenatal diagnosis, using a standardized care protocol, 47% of liveborn infants with an arteriovenous shunt malformation with cardiac failure experienced a favorable outcome.

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Resumen La hipertensión pulmonar persistente del recién nacido (HPPRN) es una entidad frecuente en las unidades de cuidados intensivos neonatales (UCIN), sin embargo, algunas de sus causas pasan desapercibidas, debido a su baja frecuencia y poca asociación con esta patología. A propósito de un caso en nuestra UCIN, en un neonato a término que presentó HPPRN secundario a una malformación pulmonar, raramente asociada con esta patología en este grupo etareo, ya que se encontró mayor disposición a factores propios del nacimiento, y noxas maternas. El paciente presentó dificultad respiratoria horas después de su nacimiento, con evolución y persistencia de su sintomatología, por lo que se necesitó de diversos medios diagnósticos para establecer su etiología. Es importante destacar que el primer ecocardiograma doppler no mostró ninguna alteración anatómica o funcional, reportado normal; con posterior empeoramiento clínico del neonato, por lo que se ordenó un control ecocardiografico, y se evidenció una presión pulmonar estimada de 71 mmHg; se continuó el estudio etiológico con imágenes complementarias, en esta caso una tomografia de tórax contrastada, y finalmente fue corroborado con un cateterismo cardiaco, lo cual permitió a su vez, manejo terapéutico y corrección de anomalía estructural de vasos pulmonares.

Abstract Persistent pulmonary hypertension of the newborn (PPHN) is a common feature in neonatal intensive care units (NICUs), but some of its causes go unnoticed due to their low frequency and low association with this pathology. A case in our NICU, in a term neonate who presented PPHN secondary to a pulmonary malformation, rarely associated with this pathology in this age group, since it was found more disposition to factors of the birth, and maternal noxas. The patient presented respiratory difficulty hours after birth, with evolution and persistence of their symptoms, which required different diagnostic methods to establish their etiology. It is important to note that the first Doppler echocardiogram did not show any anatomical or functional alterations, which were normal; With subsequent clinical worsening of the neonate, so that an echocardiographic control is ordered, evidencing an estimated pulmonary pressure of 71 mmHg; The etiological study was followed with complementary images, in this case, a contrast-enhanced chest tomography, and finally it was corroborated with cardiac catheterization, allowing, in turn, therapeutic management and correction of structural abnormality of pulmonary vessels.

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OBJECTIVE: To compare echocardiography (ECHO) findings of patients with congenital diaphragmatic hernia (CDH) who required extracorporeal membrane oxygenation (ECMO) to non-ECMO treated patients. STUDY DESIGN: We reviewed clinical and ECHO data of newborns with CDH born between 2009 and 2016. Exclusions included major anomalies, genetic syndromes, or no ECHO prior to ECMO. Pulmonary hypertension was assessed by ductal shunting and tricuspid regurgitant jet. Speckle tracking echocardiography (STE) assessed function by quantifying deformation. RESULTS: Patients with CDH (15 ECMO and 29 with no ECMO) were analyzed. Most patients had a left CDH (88.6%). Age at ECHO was similar between groups. Outborn status (P = .009) and liver position (P = .009) were associated with need for ECMO. Compared with non-ECMO patients, patients who required ECMO had significantly decreased left and right ventricular function by both conventional and STE measures, as well as decreased right and left ventricular output. The right ventricular eccentricity index was higher in ECMO vs non-ECMO patients (2.2 vs 1.8, P = .02). There was no difference in pulmonary hypertension between CDH groups. CONCLUSIONS: Need for ECMO was associated with decreased left and right ventricular function, as assessed by standard and STE measures. There was no difference in pulmonary hypertension between non ECMO and ECMO patients. Abnormal cardiac function may explain nonresponse to pulmonary vasodilators in patients with CDH. Management strategies to improve cardiac function may reduce the need for ECMO in newborns with CDH.

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Alveolar capillary dysplasia (ACD) with misalignment of pulmonary veins (MPV) is a lethal neonatal lung disease. Death from ACD/MPV is caused by hypoxia, but the role of the MPV is unknown. Using 3-dimensional reconstruction of ACD/MPV lung tissue, we report that the veins in MPV are intrapulmonary shunt vessels, and speculate that MPV contributes to the poor prognosis.

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OBJECTIVES: To compare proportions of infants at different gestational ages discharged from the neonatal intensive care unit (NICU) on home oxygen, to determine how many were classified with chronic lung disease based on timing of discharge on home oxygen, and to determine the percentage discharged on home oxygen who received mechanical ventilation. STUDY DESIGN: We evaluated a retrospective cohort of infants of 23-43 weeks' gestational age discharged from 228 NICUs in 2009, using the Pediatrix Clinical Data Warehouse. Multilevel logistic regression analysis identified predictors of home oxygen use among extremely preterm, early-moderate preterm, late preterm, and term infants. Duration of mechanical ventilation and median length of stay were calculated for infants discharged on home oxygen. RESULTS: For the 48877 infants studied, the rate of home oxygen use ranged from 28% (722 of 2621) in extremely preterm infants to 0.7% (246 of 34 934) in late preterm and term infants. Extremely preterm infants composed 56% (722 of 1286) of the infants discharged on home oxygen; late preterm and term infants, 19% (246 of 1286). After gestational age, mechanical ventilation was the main predictor of home oxygen use; however, 61% of the late preterm and term infants discharged on home oxygen did not receive ventilation. The median length of hospital stay was 95 days (IQR, 76-114 days) for extremely preterm infants discharged on home oxygen, but only 15 days (IQR, 10-22 days) for late preterm and term ventilated infants discharged on home oxygen. CONCLUSION: Although home oxygen use is uncommon in later-gestation infants, the greater overall numbers of later-gestation infants contribute significantly to the increased need for home oxygen for infants at NICU discharge. Neither respiratory failure nor lengthy hospitalization is a prerequisite for home oxygen use at later gestational age.

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