scholarly journals Neonatal and Postneonatal Pulmonary Hypertension

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 131
Author(s):  
Satyan Lakshminrusimha

During transition at birth with ventilation of the lungs, pulmonary vascular resistance (PVR) decreases from high fetal values, leading to an 8 to 10-fold increase in pulmonary blood flow (Qp). In some infants, this transition does not occur, resulting in pulmonary hypertension (PH). In infants, PH can present as: (a) primary PH in term neonates (idiopathic), (b) PH secondary to lung disease or hypoplasia in term infants, (c) acute PH in preterm infants with respiratory distress syndrome (RDS), (d) chronic PH with bronchopulmonary dysplasia (BPD) in preterm infants and (e) post-neonatal PH. A hemodynamically significant patent ductus arteriosus (PDA) can exacerbate PH in preterm infants due to increased Qp. Pulmonary vein stenosis (PVS) can complicate BPD with PH. Diagnosis of PH is based on clinical features, echocardiography and, in some intractable cases, cardiac catheterization. Therapy of PH includes oxygen, invasive or non-invasive ventilation, correction of acidosis, surfactant and selective and non-selective pulmonary vasodilators such as inhaled nitric oxide and sildenafil, respectively. Early closure of a hemodynamically significant PDA has the potential to limit pulmonary vascular remodeling associated with BPD and PH. The role of thiamine in pathogenesis of PH is also discussed with the recent increase in thiamine-responsive acute pulmonary hypertension in early infancy. Recognition and prompt therapy of PH can prevent right ventricular dysfunction, uncoupling and failure.

2014 ◽  
Vol 10 (3) ◽  
pp. 216-237 ◽  
Author(s):  
Gian Maria Pacifici

Background: Ibuprofen and indomethacin are potent non-selective cyclo-oxygenase inhibitors and inhibit prostaglandin E2 synthesis. The patent ductus arteriosus (PDA) occurs in more than 70% of preterm infants weighing <1500 g. Prostaglandin E2 relaxes smooth muscle, tends to inhibit the closure of PDA, yields vasodilatation of the afferent renal arterioles and maintains glomerular filtration rate (GFR). Ibuprofen and indomethacin inhibiting prostaglandin E2 synthesis close PDA and reduce GFR with consequent decrease of urine output and increase of serum creatinine concentrations. Aims: The aims of this study are to give the definitive estimates of PDA closure rate following ibuprofen or indomethacin treatment and to evaluate the extent of renal side effects following the administration of these drugs to preterm infants. Other aims are to review the metabolism and the pharmacokinetics of ibuprofen and indomethacin in preterm infants with PDA. Methods: The bibliographic search was performed using PubMed and EMBASE databases as search engines, January 2013 was the cutoff point. Results: The %PDA closed by ibuprofen (n=24) and indomethacin (n=24) is 77.7±14.1 and 77.3±11.0, respectively. For ibuprofen, the gestational age of the infants included in the study ranged from 25.0 to 39.0 weeks (mean±SD=29.3±3.1 weeks). The %PDA did not correlate with the gestational age (p=0.2516). For indomethacin, the gestational age of infants included in the study ranged from 25.0 and 39.0 weeks (mean±SD=29.4±2.9 weeks). The %PDA did not correlate with the gestational age (p=0.3742). The treatment with ibuprofen reduces the urine output and increases the serum creatinine concentrations less extensively than indomethacin. The half-life (t1/2) of ibuprofen and indomethacin is lengthened and the clearance is reduced in preterm infants as compared with fullterm infants. Conclusions. Ibuprofen and indomethacin are equally effective in closing PDA. Treatment with ibuprofen decreases the risk of renal failure. Ibuprofen has the most favourable risk/benefit ratio. The rate of metabolism is reduced and t1/2 is lengthened in prematures as compared with term infants.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Maria Niccum ◽  
Fotios Spyropoulos ◽  
Jonathan Levin ◽  
Carter Petty ◽  
Mary P Mullen ◽  
...  

Introduction: Lower oxygen saturation targets in preterm infants have been associated with decreased incidence of bronchopulmonary dysplasia (BPD) but increased risk of pulmonary hypertension (PH). Studies have shown that targets of <90% are associated with higher incidence of PH, however data on the optimal saturation target >90% are lacking. In this study, we compared the rate of BPD and PH in two cohorts with saturation targets of 94-98% and 92-97%. We hypothesized that BPD rate would be lower and PH rate would be unchanged at the lower saturation target. Methods: We performed a retrospective cohort study comparing PH and BPD rates among two cohorts of infants born at ≤32 weeks gestation at Brigham and Women’s Hospital: cohort 1 with saturation target of 94-98% (n = 129, July 2017-July 2018), cohort 2 with saturation target of 92-97% (n = 124, July 2018-July 2019). PH was defined by echocardiographic evidence of systolic septal flattening or right ventricular pressure ≥35 mmHg (estimated by tricuspid regurgitant jet velocity or shunt velocity) at gestational age (GA) ≥36 weeks. Comparisons between groups were carried out by Chi-square test, t-test, and multivariable logistic regression. Results: Subjects had a GA of 23-32 weeks; 46% were female. Groups did not differ with respect to GA, sex, or birth weight. There was no difference in rate of PH (2.4% vs. 4.2%, p = 0.12) or BPD (25% vs. 20%, p = 0.31) between cohort 1 and cohort 2. Other clinical parameters were not different between groups, including presence of patent ductus arteriosus, presence of atrial septal defect, use of diuretics, or use of steroids. After controlling for GA, birth weight, sex, and diagnosis of BPD using logistic regression, there was no difference in rate of PH between groups (p = 0.47), but there was a positive association of BPD with PH (OR 3.45; 95% CI, 1.18-10.09; p = 0.02). Conclusions: A lower oxygen saturation target was not associated with a higher rate of PH or lower rate of BPD in preterm infants. The overall rate of PH was much lower than rates previously reported at saturation targets <90%. Given our low incidence of PH, and the lack of a significant difference in rate of PH between groups, a saturation target of 92-97% may be safe while also minimizing need for supplemental oxygen in this population.


Children ◽  
2020 ◽  
Vol 7 (9) ◽  
pp. 139
Author(s):  
Ranjit Philip ◽  
Vineet Lamba ◽  
Ajay Talati ◽  
Shyam Sathanandam

There continues to be a reluctance to close the patent ductus arteriosus (PDA) in premature infants. The debate on whether the short-term outcomes translate to a difference in long-term benefits remains. This article intends to review the pulmonary vasculature changes that can occur with a chronic hemodynamically significant PDA in a preterm infant. It also explains the rationale and decision-making involved in a diagnostic cardiac catheterization and transcatheter PDA closure in these preterm infants.


2007 ◽  
Vol 12 (3) ◽  
pp. 138-146 ◽  
Author(s):  
James E. Dice ◽  
Jatinder Bhatia

Patent ductus arteriosus (PDA) is one of the most common congenital heart defects, accounting for 5%–10% of all congenital heart disease in term infants. The occurrence of PDA is inversely related to gestational age and weight, with an even greater incidence in preterm infants. The maintenance of ductal patency is essential for the normal development of the fetus. In the neonate, however, persistent patency of the ductus arteriosus (DA) is associated with significant morbidity and mortality. Normally, at birth, the DA constricts, resulting in intraluminal ischemic hypoxia, which eventually leads to closure and remodeling of the ductus. PDA in term infants is usually associated with a functional defect, whereas in preterm infants it is associated with immaturity. Normal physiologic mechanisms contributing to closure - oxygen tension and decreased prostaglandins—are altered in prematurity. Clinical signs of ductal patency include murmur, tachycardia, bounding peripheral pulses, and congestive heart failure and associated symptoms. Symptoms are not always present; therefore, diagnostic imaging is critical if a PDA is suspected on clinical grounds. Three management strategies are currently available for PDA: fluid restriction and diuretics (as clinically appropriate), medical intervention, and surgical ligation. Pharmacologic closure can be achieved via administration of intravenous indomethacin or ibuprofen lysine. While both agents have shown similar efficacy, ibuprofen lysine has demonstrated an improved safety profile, particularly in terms of renal effects, compared to indomethacin.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Karen Lavie-Nevo ◽  
Kevin C. Harris ◽  
Joseph Y. Ting

Abstract Background Premature preterm rupture of membranes (PPROM) is reported to be associated with high rates of neonatal mortality and morbidity. Sildenafil has been used in infants with persistent pulmonary hypertension of newborn (PPHN) due to congenital diaphragmatic hernia (CDH) and bronchopulmonary dysplasia (BPD). Recently, Sildenafil has been evaluated as an alternative or adjunctive pulmonary vasodilator. This case report illustrates the use of early sildenafil for PPHN and right ventricular dysfunction in an unusual setting of lung and renal hypoplasia. Case presentation A male infant was born at 37 weeks with a birth weight of 2840 g. Rupture of membranes developed at approximately 24 weeks of gestational age (GA). Bilateral small kidneys (< 2 standard deviations below average) were detected on ultrasound (US) examination at 30 weeks of gestation. The baby developed pneumothorax and pulmonary hypertensive crisis towards the end of the first day. An echocardiogram showed a dilated right ventricle, moderate right ventricular systolic dysfunction, hypoplastic pulmonary arteries and a large patent ductus arteriosus with bidirectional flow. The patient was sedated, paralyzed, and inhaled nitric oxide was administered to decrease the pulmonary resistance. In anticipation of persistent pulmonary hypertension due to the hypoplastic lungs and small calibre of pulmonary arteries, sildenafil was started on day of life (DOL) 5 at a dosage of 0.25 mg/kg/dose Q8H and gradually increased to 2 mg/kg/dose Q8H on DOL 9. The patient was finally extubated on DOL 7 and weaned off of non-invasive respiratory support on DOL 26. Sildenafil was gradually weaned beginning on DOL 21 and discontinued on DOL 48. Repeat echocardiogram assessment at 3 months showed complete resolution of PHT and right ventricular dilatation. Conclusions We describe the early use of sildenafil in treating pulmonary hypertension associated with lung and renal hypoplasia in a non-CDH patient. Following this treatment the patient made a full recovery from right ventricular dysfunction.


2021 ◽  
Vol 11 (1) ◽  
pp. 204589402199144
Author(s):  
Hythem Nawaytou ◽  
Jeffrey R. Fineman ◽  
Shahin Moledina ◽  
Dunbar Ivy ◽  
Steven H. Abman ◽  
...  

Development of pulmonary hypertension (PH) in patients with left side heart disease (LHD) is a predictor of poor prognosis. The use of pulmonary vasodilators in PH associated with LHD (PH-LHD) is controversial. In this study, we describe the practice patterns regarding the use of pulmonary vasodilators in PH-LHD among a group of international pediatric PH specialists. A survey was distributed to the members of three pediatric PH networks: PPHNet, PVRI, and REHIPED. The survey queried participants on the rationale, indications, and contraindications of the use of pulmonary vasodilators in children with PH-LHD. Forty-seven PH specialists from 39 PH centers completed the survey. Participants included PH specialists from North America (57%), South America (15%), and Europe (19%). The majority of participants (74%) recommended the use of pulmonary vasodilators only in patients with combined pre-capillary and post-capillary pulmonary hypertension. Participants required the presence of clinical symptoms or signs of heart failure (68%) or right ventricular dysfunction by echocardiography (51%) in order to recommend pulmonary vasodilator therapy. There was no agreement regarding hemodynamic criteria used to recommend pulmonary vasodilators or the etiologies of LHD considered contraindications for using pulmonary vasodilators to manage PH-LHD. Of the available PH-targeted drugs, most participants preferred the use of phosphodiesterase-5-inhibitors for this indication. In conclusion, the practice of recommending pulmonary vasodilators in PH-LHD is highly variable among international pediatric PH specialists. Most specialists of those surveyed (57% in North America) would consider the use of pulmonary vasodilators in PH-LHD only if pre-capillary pulmonary hypertension and right ventricular dysfunction are present.


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