Pulmonary vascular resistance in repaired congenital diaphragmatic hernia vs. age-matched controls

Zussman, Matthew; Bagby, Michelle C.; Benson, D. Woodrow; Gupta, Rahul; Hirsch, Russel

doi:10.1038/pr.2012.16

Cited by 25 publications

(20 citation statements)

References 18 publications

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“…Though we did not see a significant improvement in PaO2 in the CDH-Tadalafil in comparison to the CDH-Placebo group, we were able to show significant differences in total pulmonary blood flow (QPA). Patients with CDH have significantly elevated PAP, 14, 27 but pulmonary blood flow is significantly lower in CDH in comparison to age-matched controls, 27 which we were able to recapitulate in our fetal lamb model of diaphragmatic hernia. Tadalafil significantly improved QPA in fetal lambs with diaphragmatic hernia after maternal ewes were administered tadalafil orally.…”

Section: Discussionsupporting

confidence: 63%

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia

Shue

Schecter

Gong

et al. 2014

Journal of Pediatric Surgery

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Purpose Pulmonary hypertension (pHTN), a main determinant of survival in congenital diaphragmatic hernia (CDH), results from in utero vascular remodeling. Phosphodiesterase type 5 (PDE5) inhibitors have never been used antenatally to treat pHTN. The purpose of this study is to determine if antenatal PDE5 inhibitors can prevent pHTN in the fetal lamb model of CDH. Methods CDH were created in pregnant ewes. Postoperatively, pregnant ewes received oral placebo or tadalafil, a PDE5 inhibitor, until delivery. Near term gestation, lambs underwent resuscitations, and lung tissue was snap frozen for protein analysis. Results Mean cGMP levels were 0.53±0.11 in placebo-treated fetal lambs and 1.73±0.21 in tadalafil-treated fetal lambs (p=0.002). Normalized expression of eNOS was 82±12% in Normal-Placebo, 61±5% in CDH-Placebo, 116±6% in Normal-Tadalafil, and 86±8% in CDH-Tadalafil lambs. Normalized expression of β-sGC was 105±15% in Normal-Placebo, 82±3% in CDH-Placebo, 158±16% in Normal-Tadalafil, and 86±8% in CDH-Tadalafil lambs. Endothelial NOS and β-sGC were significantly decreased in CDH (p = 0.0007 and 0.01 for eNOS and β-sGC, respectively), and tadalafil significantly increased eNOS expression (p = 0.0002). Conclusions PDE5 inhibitors can cross the placental barrier. β-sGC and eNOS are downregulated in fetal lambs with CDH. Antenatal PDE5 inhibitors normalize eNOS and may prevent in utero vascular remodeling in CDH.

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Section: Discussionsupporting

confidence: 63%

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia

Shue

Schecter

Gong

et al. 2014

Journal of Pediatric Surgery

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“…Another prognostic factor for survival in CDH is the degree of PH. If PH is severe in the first weeks of life, it may continue into later childhood, and is associated with decreased survival rate . There are clinical and experimental data documenting the complicated events that occur in the pulmonary circulation during labor .…”

Section: Discussionmentioning

confidence: 99%

“…If PH is severe in the first weeks of life, it may continue into later childhood, and is associated with decreased survival rate. 4,15 There are clinical and experimental data documenting the complicated events that occur in the pulmonary circulation during labor. 16 Pulmonary vascular resistance decreases after birth, and in a healthy newborn 80% of this decline occurs within the first 24 h of life.…”

Section: Discussionmentioning

confidence: 99%

Prognostic markers in congenital diaphragmatic hernia: Left ventricular diameter and pulmonary hypertension

Karpuz

Giray

Çelik

et al. 2018

Pediatrics International

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“…A variety of stratifications have been employed, based on echocardiographic assessment of pulmonary arterial pressures (PAP). 27,[41][42][43][44][45] Whether the functional and structural abnormalities of the pulmonary vasculature at birth improve or deteriorate through childhood and beyond is unknown. Observational studies with small numbers of patients have assessed PAP and cardiac function in childhood survivors.…”

Section: Surveymentioning

confidence: 99%

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium

et al. 2018

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Treatment modalities for neonates born with congenital diaphragmatic hernia (CDH) have greatly improved in recent times with a concomitant increase in survival. In 2008, CDH EURO consortium, a collaboration of a large volume of CDH centers in Western Europe, was established with a goal to standardize management and facilitate multicenter research. However, limited knowledge on long-term outcomes restricts the identification of optimal care pathways for CDH survivors in adolescence and adulthood. This review aimed to evaluate the current practice of long-term follow-up within the CDH EURO consortium centers, and to review the literature on long-term outcomes published from 2000 onward. Apart from having disease-specific morbidities, children with CDH are at risk for impaired neurodevelopmental problems and failure of educational attainments which may affect participation in society and the quality of life in later years. Thus, there is every reason to offer them long-term multidisciplinary follow-up programs. We discuss a proposed collaborative project using standardized clinical assessment and management plan (SCAMP) methodology to obtain uniform and standardized follow-up of CDH patients at an international level.

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Pulmonary vascular resistance in repaired congenital diaphragmatic hernia vs. age-matched controls

Cited by 25 publications

References 18 publications

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia

Prognostic markers in congenital diaphragmatic hernia: Left ventricular diameter and pulmonary hypertension

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium

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