Sildenafil Use in Children with Pulmonary Hypertension

Cohen, Jennifer L; Nees, Shannon N.; Valencia, Gerson A.; Rosenzweig, Erika B.; Krishnan, Usha

doi:10.1016/j.jpeds.2018.09.067

Cited by 58 publications

(44 citation statements)

References 28 publications

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“…Similar to previous studies, 35% of the cohort of BPD infants had PH with resolution of PH in 44% of neonates with BPD-PH [10,11]. Additionally, BPD infants with PH had lower gestational age, lower birthweight, increased BPD severity, longer hospital LOS, duration of respiratory support, and increased level of respiratory support at hospital discharge compared to infants without PH consistent with prior studies in this population [4][5][6]27].…”

Section: Discussionsupporting

confidence: 87%

“…Alterations in lung development in BPD infants are associated with pulmonary vascular disease and pulmonary hypertension (PH). BPD associated PH (BPD-PH) is associated with increased BPD severity, short and late term respiratory outcomes and mortality, and PH secondary to BPD is associated with increased mortality compared to other etiologies of PH [2,[4][5][6][7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

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Cardiovascular magnetic resonance imaging derived septal curvature in neonates with bronchopulmonary dysplasia associated pulmonary hypertension

Critser

Higano

Lang

et al. 2020

Journal of Cardiovascular Magnetic Resonance

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Background: Bronchopulmonary dysplasia (BPD) associated with pulmonary hypertension (PH) is a significant source of morbidity and mortality in premature infants. Recent advances have allowed the use of cardiovascular magnetic resonance (CMR) in the assessment of respiratory and cardiac disease in infants with BPD. In adults and older pediatric patients, decreased CMR interventricular septal curvature correlates with increased mean pulmonary artery pressure and pulmonary vascular resistance. The current study sought to determine the relationship of CMR derived septal curvature in neonates with BPD and BPD-PH with a need for PH therapy. Methods: Forty moderate or severe BPD and 12 mild BPD or control infants were imaged without contrast between 38 and 47 weeks post-menstrual age on a neonatal-sized, neonatal intensive care unit-sited 1.5 T CMR scanner. CMR indices including eccentricity index (CMR-EI) and septal curvature were measured and compared to BPD severity and clinical outcomes including hospital length of stay (LOS), duration of respiratory support, respiratory support level at discharge and PH therapy. Results: CMR-EI was directly associated and septal curvature was inversely associated with BPD severity. In a univariate analysis, CMR-EI and septal curvature were associated with increased hospital LOS, duration of respiratory support, respiratory support at hospital discharge, and need for PH therapy. In multivariable analysis CMR-EI was associated with hospital LOS and duration of respiratory support and septal curvature was associated with respiratory support at hospital discharge. Septal curvature was the only clinical or CMR variable associated with need for PH therapy (R 2 = 0.66, p = 0.0014) in multivariable analysis demonstrating improved discrimination beyond CMR-EI.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Cardiovascular magnetic resonance imaging derived septal curvature in neonates with bronchopulmonary dysplasia associated pulmonary hypertension

Critser

Higano

Lang

et al. 2020

Journal of Cardiovascular Magnetic Resonance

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“…A recent meta-analysis studied the effectiveness and safety of chronic use sildenafil in preterm infants with BPD-PH and found an overall mortality of 29.7% per year and demonstrated improvement in estimated pulmonary arterial pressure and respiratory severity scores in patients on sildenafil [ 36 ]. Our group previously reported on our experience with sildenafil in children with PH including those with BPD-PH and demonstrated that PH improved over time in these patients and sildenafil was well-tolerated [ 37 ].…”

Section: Discussionmentioning

confidence: 99%

Targeted Therapy for Pulmonary Hypertension in Premature Infants

et al. 2020

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Pulmonary hypertension (PH) is common in premature infants with bronchopulmonary dysplasia (BPD) and is associated with significant mortality. Despite expert consensus suggesting the use of targeted therapies such as phosphodiesterase inhibitors, endothelin receptor antagonists, and prostanoids, there is little data on safety and outcomes in infants with BPD-associated PH (BPD-PH) treated with these medications. We sought to describe the pharmacologic management of BPD-PH and to report outcomes at our institution. Premature infants with BPD-PH born between 2005 and 2016 were included. Follow-up data were obtained through January 2020. A total of 101 patients (61 male, 40 female) were included. Of these, 99 (98.0%) patients were treated with sildenafil, 13 (12.9%) with bosentan, 35 (34.7%) with inhaled iloprost, 12 (11.9%) with intravenous epoprostenol, and nine (8.9%) with subcutaneous treprostinil. A total of 33 (32.7%) patients died during the study period and 10 (9.9%) were secondary to severe to pulmonary hypertension. Of the surviving patients, 57 (83.8%) had follow-up data at a median of 5.1 (range 0.38–12.65) years and 44 (77.2%) were weaned off PH medications at a median 2.0 (range 0–8) years. Mortality for BPD-PH remains high mostly due to co-morbid conditions. However, for those patients that survive to discharge, PH therapies can frequently be discontinued in the first few years of life.

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“…However, longitudinal assessment of NT-proBNP in combination with echocardiography is very useful to assess disease progression and/or response to treatment. [41][42][43][44] Meanwhile, additional biomarkers are under consideration and may contribute to future PH management. 12,40 TREATMENT Supportive measures Supplemental oxygen should be supplied when target oxygen saturations are >93% for infants with suspected and >95% for infants with proven PH (Fig.…”

Section: Diagnosis Of Bpd and Bpd-phmentioning

confidence: 99%

Pulmonary hypertension in bronchopulmonary dysplasia

et al. 2020

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Bronchopulmonary dysplasia (BPD) is a major complication in prematurely born infants. Pulmonary hypertension (PH) associated with BPD (BPD-PH) is characterized by alveolar diffusion impairment, abnormal vascular remodeling, and rarefication of pulmonary vessels (vascular growth arrest), which lead to increased pulmonary vascular resistance and right heart failure. About 25% of infants with moderate to severe BPD develop BPD-PH that is associated with high morbidity and mortality. The recent evolution of broader PH-targeted pharmacotherapy in adults has opened up new treatment options for infants with BPD-PH. Sildenafil became the mainstay of contemporary BPD-PH therapy. Additional medications, such as endothelin receptor antagonists and prostacyclin analogs/mimetics, are increasingly being investigated in infants with PH. However, pediatric data from prospective or randomized controlled trials are still sparse. We discuss comprehensive diagnostic and therapeutic strategies for BPD-PH and briefly review the relevant differential diagnoses of parenchymal and interstitial developmental lung diseases. In addition, we provide a practical framework for the management of children with BPD-PH, incorporating the modified definition and classification of pediatric PH from the 2018 World Symposium on Pulmonary Hypertension, and the 2019 EPPVDN consensus recommendations on established and newly developed therapeutic strategies. Finally, current gaps of knowledge and future research directions are discussed.

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Sildenafil Use in Children with Pulmonary Hypertension

Cited by 58 publications

References 28 publications

Cardiovascular magnetic resonance imaging derived septal curvature in neonates with bronchopulmonary dysplasia associated pulmonary hypertension

Cardiovascular magnetic resonance imaging derived septal curvature in neonates with bronchopulmonary dysplasia associated pulmonary hypertension

Targeted Therapy for Pulmonary Hypertension in Premature Infants

Pulmonary hypertension in bronchopulmonary dysplasia

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