Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment

Cabral, João Guilherme G.; Belik, Jaques

doi:10.1016/j.jped.2012.11.009

Cited by 48 publications

(28 citation statements)

References 101 publications

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“…It also may be associated with respiratory distress syndrome, a disease most typically seen in the preterm neonate [21]. Although the etiology of PPHN is multifactorial and its pathogenesis is unclear [22], it is believed that disruptions to the NO-cyclic guanosine monophosphate and endothelial NO synthase signaling pathways play an important role in the vascular abnormalities associated with PH [23]. Regardless of GA, the primary goal of therapy for PPHN is to achieve selective pulmonary vasodilation, which makes iNO well suited for treatment of this condition [23].…”

Section: Discussionmentioning

confidence: 99%

Efficacy of inhaled nitric oxide in neonates with hypoxic respiratory failure and pulmonary hypertension: the Japanese experience

Suzuki

Togari

Potenziano

et al. 2018

Journal of Perinatal Medicine

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Objective: To analyze data from a registry of Japanese neonates with hypoxic respiratory failure associated with pulmonary hypertension (PH) to compare the effectiveness of inhaled nitric oxide (iNO) in neonates born <34 weeks vs. ≥34 weeks gestational age (GA). Materials and methods: iNO was administered according to approved Japanese product labeling. Study data were collected before iNO administration and at predefined intervals until discontinuation. Results: A total of 1,114 neonates were included (n = 431, <34 weeks GA; n = 675, ≥34 weeks GA; n = 8, missing age data). Mean decrease from baseline oxygenation index (OI) was similar in both age groups. OI reduction was more pronounced in the <34 weeks subgroups with baseline OI ≥25. Survival rates were similar in the <34 weeks GA and ≥34 weeks GA groups stratified by baseline OI (OI < 15, 89% vs. 93%; 15 ≤ OI < 25, 85% vs. 91%; 25 ≤ OI ≤ 40, 73% vs. 79%; OI > 40, 64% vs. 66%). Conclusion: iNO improved oxygenation in preterm neonates as effectively as in late preterm and term neonates, without negative impact on survival. If clinically significant PH is present, as measured by pulse oximetry or echocardiography, a therapeutic trial of iNO might be indicated for preterm neonates.

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Section: Discussionmentioning

confidence: 99%

Efficacy of inhaled nitric oxide in neonates with hypoxic respiratory failure and pulmonary hypertension: the Japanese experience

Suzuki

Togari

Potenziano

et al. 2018

Journal of Perinatal Medicine

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“…[8] Persistent pulmonary hypertension of the newborn is defined as the failure of the normal circulatory transition that occurs after birth, which is characterized by marked pulmonary hypertension that causes hypoxemia secondary to right-to-left shunting of blood. [9]…”

Section: Methodsmentioning

confidence: 99%

Analysis of In-hospital Neonatal Death in the Tertiary Neonatal Intensive Care Unit in China

Wang

et al. 2016

Chinese Medical Journal

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Background:Globally, the proportion of child deaths that occur in the neonatal period remains a high level of 37–41%. Differences of cause in neonate death exist in different regions as well as in different economic development countries. The specific aim of this study was to investigate the causes, characteristics, and differences of death in neonates during hospitalization in the tertiary Neonatal Intensive Care Unit (NICU) of China.Methods:All the dead neonates admitted to 26 NICUs were included between January l, 2011, and December 31, 2011. All the data were collected retrospectively from clinical records by a designed questionnaire. Data collected from each NICU were delivered to the leading institution where the results were analyzed.Results:A total of 744 newborns died during the 1-year survey, accounting for 1.2% of all the neonates admitted to 26 NICUs and 37.6% of all the deaths in children under 5 years of age in these hospitals. Preterm neonate death accounted for 59.3% of all the death. The leading causes of death in preterm and term infants were pulmonary disease and infection, respectively. In early neonate period, pulmonary diseases (56.5%) occupied the largest proportion of preterm deaths while infection (27%) and neurologic diseases (22%) were the two main causes of term deaths. In late neonate period, infection was the leading cause of both preterm and term neonate deaths. About two-thirds of neonate death occurred after medical care withdrawal. Of the cases who might survive if receiving continuing treatment, parents’ concern about the long-term outcomes was the main reason of medical care withdrawal.Conclusions:Neonate death still accounts for a high proportion of all the deaths in children under 5 years of age. Our study showed the majority of neonate death occurred in preterm infants. Cause of death varied with the age of death and gestational age. Accurate and prompt evaluation of the long-term outcomes should be carried out to guide the critical decision.

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“…The incidence of PPHN is about 1.9 per 1000 live births, and the mortality is reported to be 10%. The major findings of PPHN are elevated pulmonary artery pressure, right to left shunt at the foramen ovale or at the ductus level, and hypoxemia[21,22]. Recent studies have shown that PPHN can also be associated with severe neonatal anemia.…”

Section: Hematological Disorders and Phmentioning

confidence: 99%

Hematological disorders and pulmonary hypertension

Mathew

Huang

et al. 2016

WJC

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Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death. Hemolysis observed in hematological disorders leads to free hemoglobin which rapidly scavenges nitric oxide (NO), limiting its bioavailability, and leading to endothelial dysfunction. In addition, hemolysis releases arginase into the circulation which converts L-arginine to ornithine, thus bypassing NO production. Furthermore, treatments for hematological disorders such as immunosuppressive therapy, splenectomy, bone marrow transplantation, and radiation have been shown to contribute to the development of PH. Recent studies have shown deregulated iron homeostasis in patients with cardiopulmonary diseases including pulmonary arterial hypertension (PAH). Several studies have reported low iron levels in patients with idiopathic PAH, and iron deficiency is an important risk factor. This article reviews PH associated with hematological disorders and its mechanism; and iron homeostasis and its relevance to PH.

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Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment

Cited by 48 publications

References 101 publications

Efficacy of inhaled nitric oxide in neonates with hypoxic respiratory failure and pulmonary hypertension: the Japanese experience

Efficacy of inhaled nitric oxide in neonates with hypoxic respiratory failure and pulmonary hypertension: the Japanese experience

Analysis of In-hospital Neonatal Death in the Tertiary Neonatal Intensive Care Unit in China

Hematological disorders and pulmonary hypertension

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