Risk Assessment and Monitoring of Chronic Pulmonary Hypertension in Premature Infants

Levy, Paul C.; Jain, Amish; Nawaytou, Hythem; Teitel, David; Keller, Roberta L.; Fineman, Jeffery R.; Steinhorn, Robin H.; McNamara, Patrick J.

doi:10.1016/j.jpeds.2019.10.034

Cited by 39 publications

(24 citation statements)

References 97 publications

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“…Collaboration across national and international clinical PH sites will be necessary to yield sufficient sample sizes due to the extremely small number of pediatric PAH patients at single PH sites, heterogeneity of risk genes for PAH, and need for ancestral diversity. PPHNet is an example of a pediatric-specific PAH consortium with ongoing recruitment across 13 North American clinical sites [ 74 , 75 , 76 , 77 , 78 ]. PVDomics [ 79 ], a US multicenter study launched in 2014, and PAH-ICON (pahicon.com), a new international effort, represent additional large-scale PAH cohorts.…”

Section: A Genomics First Approach Towards Better Understanding Ofmentioning

confidence: 99%

Genetics and Genomics of Pediatric Pulmonary Arterial Hypertension

Welch

Chung

2020

Genes

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Pulmonary arterial hypertension (PAH) is a rare disease with high mortality despite recent therapeutic advances. The disease is caused by both genetic and environmental factors and likely gene–environment interactions. While PAH can manifest across the lifespan, pediatric-onset disease is particularly challenging because it is frequently associated with a more severe clinical course and comorbidities including lung/heart developmental anomalies. In light of these differences, it is perhaps not surprising that emerging data from genetic studies of pediatric-onset PAH indicate that the genetic basis is different than that of adults. There is a greater genetic burden in children, with rare genetic factors contributing to ~42% of pediatric-onset PAH compared to ~12.5% of adult-onset PAH. De novo variants are frequently associated with PAH in children and contribute to at least 15% of all pediatric cases. The standard of medical care for pediatric PAH patients is based on extrapolations from adult data. However, increased etiologic heterogeneity, poorer prognosis, and increased genetic burden for pediatric-onset PAH calls for a dedicated pediatric research agenda to improve molecular diagnosis and clinical management. A genomics-first approach will improve the understanding of pediatric PAH and how it is related to other rare pediatric genetic disorders.

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Section: A Genomics First Approach Towards Better Understanding Ofmentioning

confidence: 99%

Genetics and Genomics of Pediatric Pulmonary Arterial Hypertension

Welch

Chung

2020

Genes

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“…Recently, a report of a dedicated PPHNet workshop summarised the challenges of obtaining comprehensive information regarding disease severity and the relative contributions of pulmonary vascular disease, cardiac dysfunction and lung disease in preterm infants. 16 According to the current World Symposium on Pulmonary Hypertension clinical classification of PH, PH-BPD is classified as group 3 associated with developmental lung diseases, but concomitant comorbidities such as cardiac shunts, collaterals and pulmonary vein stenosis may obscure the type of PH. 30 The use of PAH-targeted treatment, including 5-phosphodiesterase inhibitors, endothelin-receptor antagonists and prostacyclin analogues, in these extremely premature born infants is still controversial.…”

Section: Discussionmentioning

confidence: 99%

Fate of pulmonary hypertension associated with bronchopulmonary dysplasia beyond 36 weeks postmenstrual age

Arjaans

Haarman

Roofthooft

et al. 2020

Arch Dis Child Fetal Neonatal Ed

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ObjectiveTo determine the survival and evolution of pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD) in extremely premature born infants beyond 36 weeks postmenstrual age (PMA).DesignA single-centre retrospective cohort study from a university hospital.PatientsExtremely preterm (gestational age <30 weeks and/or birth weight <1000 g) infants, born between 2012 and 2017, in the University Medical Center Groningen with confirmed PH at/beyond 36 weeks PMA.Main outcome measuresSurvival, mortality rate and PH resolution. Patient characteristics, treatment, presence and evolution of PH were collected from patient charts.ResultsTwenty-eight infants were included. All had BPD, while 23 (82%) had severe BPD and 11 infants (39%) died. Survival rates at 1, 3 and 7 months from 36 weeks PMA were 89%, 70% and 58%, respectively. In 16 of the 17 surviving infants, PH resolved over time, with a resolution rate at 1 and 2 years corrected age of 47% and 79%, respectively. At 2.5 years corrected age, the resolution rate was 94%.ConclusionsThese extremely preterm born infants with PH-BPD had a survival rate of 58% at 6 months corrected age. Suprasystemic pulmonary artery pressure was associated with poor outcome. In the current study, infants surviving beyond the corrected age of 6 months showed excellent survival and resolution of PH in almost all cases. Prospective follow-up studies should investigate whether resolution of PH in these infants can be improved by multi-modal therapies, including respiratory, nutritional and cardiovascular treatments.

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“…Abnormal echocardiographic findings consistent with elevation of right‐sided pressure or resistance are often described as being diagnostic of PH in infants with developmental lung disease; Table 1 describes various published schema for this determination in these patients. However, published echocardiography data suggest that agreement and accuracy for diagnosis and severity of PH due to PVD in children is variable when compared to the gold standard of cardiac catheterization 34‐36 . The preponderance of pressure assessments resulting from conventional echocardiography, as well as the dynamic conditions affecting systemic vascular resistance and pressure in children during both echocardiography and cardiac catheterization are limitations in these comparisons.…”

Section: Echocardiography For Assessment Of Phmentioning

confidence: 99%

Pulmonary hypertension is an important co‐morbidity in developmental lung diseases of infancy: Bronchopulmonary dysplasia and congenital diaphragmatic hernia

Varghese

Tillman

Keller

2021

Pediatric Pulmonology

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Bronchopulmonary dysplasia (BPD) following preterm birth and congenital diaphragmatic hernia (CDH) are both forms of developmental lung disease that may result in persistent pulmonary and pulmonary vascular morbidity in childhood. The pulmonary vascular disease (PVD) which accompanies BPD and CDH is due to developmental abnormalities and ongoing perinatal insults. This may be accompanied by evidence of elevated right heart pressures and pulmonary vascular resistance, leading to diagnosis of pulmonary hypertension (PH). The development of PH in these conditions is associated with increased morbidity and mortality in the vulnerable BPD and CDH populations. We present a review of PVD pathogenesis and evaluation in BPD and CDH and discuss management of related sequelae of PH co‐morbidity for affected infants.

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Risk Assessment and Monitoring of Chronic Pulmonary Hypertension in Premature Infants

Cited by 39 publications

References 97 publications

Genetics and Genomics of Pediatric Pulmonary Arterial Hypertension

Genetics and Genomics of Pediatric Pulmonary Arterial Hypertension

Fate of pulmonary hypertension associated with bronchopulmonary dysplasia beyond 36 weeks postmenstrual age

Pulmonary hypertension is an important co‐morbidity in developmental lung diseases of infancy: Bronchopulmonary dysplasia and congenital diaphragmatic hernia

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