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Initial chest radiography showed severe cardiomegaly. Fig. 3. Chest radiography showed improvement in cardiomegaly on follow-up after 2 months later.
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Pulmonary artery hypertension is a common cardiovascular complication in preterm infants with bronchopulmonary dysplasia which is associated with increased morbidity and mortality. Inhaled iloprost is used as a therapeutic option in pulmonary hypertension, especially in adults. There have been but a few reports on the use of iloprost for neonates a...
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... As to the population of premature newborns with BPD-PH, a few case reports have described clinical improvements when using either intravenous epoprostenol [120,121], subcutaneous treprostinil [122], or inhaled iloprost in premature infants with BPD-PH [123][124][125]. A retrospective study has been published describing the clinical experience at one institution for treating premature infants with BPD-PH with inhaled iloprost (35 patients), intravenous epoprostenol (12 patients), or subcutaneous treprostinil (9 patients) [91]. ...
Approximately 8–42% of premature infants with chronic lung disease of prematurity, bronchopulmonary dysplasia (BPD), develop pulmonary hypertension (PH). Infants with BPD-PH carry alarmingly high mortality rates of up to 47%. Effective PH-targeted pharmacotherapies are desperately needed for these infants. Although many PH-targeted pharmacotherapies are commonly used to treat BPD-PH, all current use is off-label. Moreover, all current recommendations for the use of any PH-targeted therapy in infants with BPD-PH are based on expert opinion and consensus statements. Randomized Control Trials (RCTs) are needed to determine the efficacy of PH-targeted treatments in premature infants with or at risk of BPD-PH. Prior to performing efficacy RCTs, studies need to be conducted to obtain pharmacokinetic, pharmacodynamic, and safety data for any pharmacotherapy used in this understudied and fragile patient population. This review will discuss current and needed treatment strategies, identify knowledge deficits, and delineate both challenges to be overcome and approaches to be taken to develop effective PH-targeted pharmacotherapies that will improve outcomes for premature infants with or at risk of developing BPD-PH.
... Boala pulmonară cronică, definită ca nevoia suplimentară de oxigen peste 28 de zile la un copil fost prematur cu VG sub 32 de săptămâni care este evaluat la 36 de săptămâni postmenstrual sau la 28 de zile postnatal dar înainte de 56 de zile de viaţă [103] apare mai ales în cazul prematurilor cu vârstă de gestaţie sub 28 de săptămâni dar poate să apară şi la vârste de gestaţie mai mari în cazul suferinţelor respiratorii perinatale severe. La apariţia bolii pulmonare cronice concură leziunile acute postnatale ale căilor aeriene şi alveolelor care afectează creşterea, structura şi funcţionalitatea structurilor pulmonare, inclusiv a vascularizaţiei pulmonare, structuri care continuă să se dezvolte postnatal [104,105] . Consecinţa acestor anomalii lezionale şi de dezvoltare este creşterea rezistenţelor vasculare pulmonare şi apariţia, în timp, a hipertensiunii pulmonare. ...
... The potential effect of inhaled iloprost for improving PH in the pediatric group has been established35). A case study had reported an improvement caused by inhaled iloprost in the BPD patient with severe PH36). For ensuring adequate improvement, inhalation should be performed 6-9 times a day because the half-life of the prostacyclin is extremely short. ...
An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type natriuretic peptide (BNP) or N-terminal-proBNP must be conducted in the BPD patients with greater than moderate degree to prevent PH and to ensure early treatment if PH is present. In the BPD patients with significant PH, multi-modality treatment, including treatment for correcting an underlying disease, oxygen supply, use of diverse selective pulmonary vasodilators (inhaled nitric oxide, inhaled prostacyclins, sildenafil, and endothelin-receptor antagonist) and other methods, is mandatory.
Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Up to 1/3 of children with BPD develop pulmonary hypertension (PH). PH increases mortality, the risk of adverse neurodevelopmental outcome and lacks effective treatment. Current vasodilator therapies address symptoms, but not the underlying arrested vascular development. Recent insights into placental biology and novel technological advances enabling the study of normal and impaired lung development at the single cell level support the concept of a vascular phenotype of BPD. Dysregulation of growth factor pathways results in depletion and dysfunction of putative distal pulmonary endothelial progenitor cells including Cap1, Cap2, and endothelial colony-forming cells (ECFCs), a subset of vascular progenitor cells with self-renewal and de novo angiogenic capacity. Preclinical data demonstrate effectiveness of ECFCs and ECFC-derived particles including extracellular vesicles (EVs) in promoting lung vascular growth and reversing PH, but the mechanism is unknown. The lack of engraftment suggests a paracrine mode of action mediated by EVs that contain miRNA. Aberrant miRNA signaling contributes to arrested pulmonary vascular development, hence using EV- and miRNA-based therapies is a promising strategy to prevent the development of BPD-PH. More needs to be learned about disrupted pathways, timing of intervention, and mode of delivery. IMPACT: Single-cell RNA sequencing studies provide new in-depth view of developmental endothelial depletion underlying BPD-PH. Aberrant miRNA expression is a major cause of arrested pulmonary development. EV- and miRNA-based therapies are very promising therapeutic strategies to improve prognosis in BPD-PH.
Premature infants are at risk of developing bronchopulmonary dysplasia and associated pulmonary hypertension. These infants make up a complex group of patients with unique considerations regarding development of lung and vascular disease, comorbidities, and care plans. They are high risk for many complications and poor outcomes due to the severity and complexity of disease. Because of this, a comprehensive approach to care with consideration for multiple organ systems and with an interdisciplinary team of experts is the preferred approach. Here we describe in detail the major considerations in care for these infants.
Background: Echocardiography has poor accuracy in grading the severity of pulmonary hypertension (PH) compared to cardiac catheterization. However, the relationship between degree of PH and prognostic outcomes remains uncertain. Our primary objective was to determine whether echocardiogram-assessed PH severity is associated with mortality and hospital readmission in the first year of life.
Methods: A retrospective cohort study of infants born less than 32 weeks of gestational age with BPD underwent echocardiography was performed. Echocardiograms were performed at 36-38 weeks postmenstrual age. Data during hospitalization and post-discharge collected at 1-year age were analyzed with cox regression models and logistic regression models to identify the association of PH severity with mortality and readmission. Area under curve (AUC) was calculated to examine the accuracy of these models to reflect the likelihood of outcomes.
Results: Fifty-six of 237 (23.6%) infants were diagnosed as pulmonary hypertension. Moderate and severe PH was significantly associated with mortality during the first one year of life (Moderate PH vs. None HR = 26.58, CI: 4.40-160.78, p < 0.001; Severe PH vs. None HR = 36.49, CI: 5.65-235.84, p < 0.001). Male, preeclampsia and inhaled nitric oxide were also associated with mortality. Mild PH was significantly associated with readmission (OR = 2.42, CI: 1.12-5.26, p = 0.025), but not associated with mortality (HR = 2.09, CI: 0.43-10.18, p = 0.36). The PH severity model based on echocardiography accurately informed mortality (AUC 0.79).
Conclusions: Echocardiogram-assessed PH severity is associated with prognostic outcomes, including mortality and readmission in very preterm infants with BPD. The severity of PH based on echocardiography is a potential predictor of mortality in the first year of life.
Purpose Persistent pulmonary hypertension of the newborn (PPHN) is a potentially fatal disease. Inhaled iloprost, a stable analogue of prostacyclin, has recently been used as a therapeutic option. However, there are no clinical guidelines on the use of iloprost, specifically for neonates. This study aimed to suggest the use of inhaled iloprost as a rescue therapy for PPHN based on our experience. Methods The efficacy and adverse events of inhaled iloprost were evaluated prospectively in nine full-term neonates with PPHN. We monitored the following parameters: fraction of inspired oxygen (FiO2), respiratory severity score (RSS), heart rate, and mean blood pressure. Results The inhalation dose was 1 to 2 μg/kg initially, and 4 to 8 inhalations per day were applied over 2 to 8 days, except in the case of one neonate who died 2 days after birth. Echocardiographic findings, changes in FiO2, and RSS improved within the next 7 days in eight of the nine patients. Severe side effects on heart rate and blood pressure were not observed. Conclusion Our experience suggests that inhaled iloprost can be used as a first-line treatment in newborn infants with PPHN when inhaled nitric oxide is not available. To the best of our knowledge, this report is the first prospective case series on the use of inhaled iloprost in PPHN.
This chapter describes important features of the fetal circulation, essential changes at birth and other age-specific developments of the cardiovascular system. The incidence, prevalence and basic pathophysiological principles of congenital heart defects are presented. Several special situations are discussed in more detail: the exercise physiology of patients with repaired congenital heart disease, the Fontan physiology and the transplanted heart. Indications and practical considerations for the use of various vasoactive and antiarrhythmic drugs are reviewed.
