Severe Persistent Pulmonary Hypertension Research Articles

High-Throughput Genomics Identify Novel FBN1/2 Variants in Severe Neonatal Marfan Syndrome and Congenital Heart Defects.

Fibrillin-1 and fibrillin-2, encoded by FBN1 and FBN2, respectively, play significant roles in elastic fiber assembly, with pathogenic variants causing a diverse group of connective tissue disorders such as Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCD). Different genomic variations may lead to heterogeneous phenotypic features and functional consequences. Recent high-throughput sequencing modalities have allowed detection of novel variants that may guide the care for patients and inform the genetic counseling for their families. We performed clinical phenotyping for two newborn infants with complex congenital heart defects. For genetic investigations, we employed next-generation sequencing strategies including whole-genome Single-Nucleotide Polymorphism (SNP) microarray for infant A with valvular insufficiency, aortic sinus dilatation, hydronephrosis, and dysmorphic features, and Trio whole-exome sequencing (WES) for infant B with dextro-transposition of the great arteries (D-TGA) and both parents. Infant A is a term male with neonatal marfanoid features, left-sided hydronephrosis, and complex congenital heart defects including tricuspid regurgitation, aortic sinus dilatation, patent foramen ovale, patent ductus arteriosus, mitral regurgitation, tricuspid regurgitation, aortic regurgitation, and pulmonary sinus dilatation. He developed severe persistent pulmonary hypertension and worsening acute hypercapnic hypoxemic respiratory failure, and subsequently expired on day of life (DOL) 10 after compassionate extubation. Cytogenomic whole-genome SNP microarray analysis revealed a deletion within the FBN1 gene spanning exons 7-30, which overlapped with the exon deletion hotspot region associated with neonatal Marfan syndrome. Infant B is a term male prenatally diagnosed with isolated D-TGA. He required balloon atrial septostomy on DOL 0 and subsequent atrial switch operation, atrial septal defect repair, and patent ductus arteriosus ligation on DOL 5. Trio-WES revealed compound heterozygous c.518C>T and c.8230T>G variants in the FBN2 gene. Zygosity analysis confirmed each of the variants was inherited from one of the parents who were healthy heterozygous carriers. Since his cardiac repair at birth, he has been growing and developing well without any further hospitalization. Our study highlights novel FBN1/FBN2 variants and signifies the phenotype-genotype association in two infants affected with complex congenital heart defects with and without dysmorphic features. These findings speak to the importance of next-generation high-throughput genomics for novel variant detection and the phenotypic variability associated with FBN1/FBN2 variants, particularly in the neonatal period, which may significantly impact clinical care and family counseling.

Risk Factors and Mortality in Newborns with Persistent Pulmonary Hypertension: A Six-Year Single-Center Experience.

Persistent pulmonary hypertension (PPHT) of the newborn is a disorder of circulatory transition resulting in high pulmonary vascular resistance with extrapulmonary right-to-left shunts causing hypoxemia. In this study, our aim was to evaluate the risk factors, administered treatments, and mortality of patients followed in our neonatal intensive care unit (NICU) due to PPHT over the past six years. Patients diagnosed with PPHT and followed in the NICU between January 2017 and November 2022 were included in the study. The sociodemographic characteristics, diagnoses that could lead to pulmonary hypertension, the presence of congenital anomalies, the duration of respiratory support treatment and hospital follow-up, treatments administered for PPHT, and mortality rates were evaluated. Out of 21 patients diagnosed with persistent pulmonary hypertension, 9 of them (42.9%) were male. The mean gestational age of the patients was 37.6±3.7 weeks, and their birth weight was 3006±819grams. The APGAR scores at 1 and 5 minutes were 4(2-7) and 6(3-8), respectively. Risk factors during the antenatal period included fetal distress (38.1%), oligohydramnios (23.8%), intrauterine growth restriction (23.8%), gestational diabetes (14.3%), preeclampsia (4.8%), and chorioamnionitis (4.8%). The median duration of invasive mechanical ventilation for cases requiring respiratory support was 20.1 days, while the median duration of non-invasive ventilation was 3.7 days. Patients with a diagnosis of persistent pulmonary hypertension were treated with inhaled nitric oxide (iNO) in 76.2% of cases, milrinone in 66.7% of cases, sildenafil in 52.4% of cases, and iloprost in 14.3% of cases. The length of hospital stay for patients was 38.4 days, and 9 (42.9%) patients died. The patients who died had severe PPHT along with fetal inflammatory response syndrome (FIRS), congenital heart disease, pulmonary hypoplasia, pneumothorax, hypoxic-ischemic encephalopathy (HIE), and congenital anomalies. Persistent pulmonary hypertension, characterized by severe hypoxemia, is a neonatal emergency that necessitates early intervention, effective treatment of the underlying cause to prevent potential short-term and long-term morbidities and mortality. Effective treatment of the underlying cause in patients diagnosed with PPHT could reduce morbidity and mortality. It is inevitable to avoid the loss of patients with major abnormalities, severe comorbidities, and unpreventable organ dysfunctions.

Can machine learning methods be used for identification of at-risk neonates in low-resource settings? A prospective cohort study

IntroductionTimely identification of at-risk neonates (ARNs) in the community is essential to reduce mortality in low-resource settings. Tools such as American Academy of Pediatrics pulse oximetry (POx) and WHO Young...

TO THE ISSUE OF THE DEVELOPMENT AN ALGORITHM FOR A DIFFERENTIATED APPROACH TO THE MANAGEMENT OF PERSISTENT PULMONARY HYPERTENSION IN PREMATURE INFANTS

The management of premature infants with persistent pulmonary hypertension (PPH) remains a major problem in modernneonatology. In recent years, scientifi c studies have identifi ed the role of oxidative stress (OS) in the development and course of PPH. The reserve for reducing mortality and optimizing the management of premature infants with respiratory distress syndrome (RDS) and asphyxia is the development of an algorithm for a diff erentiated approach to the management of PPH in premature infants, taking into account the severity and dynamics of OS, and its implementation into clinical practice.
 Aim of the study. To increase the eff ectiveness of management of premature infants with persistent pulmonary hypertension with asphyxia and respiratory distress syndrome based on the development of an algorithm for a diff erentiated approach to the management of pulmonary hypertension taking into account the levels of oxidative stress as determined by urinary 8-hydroxy-2-deoxyguanosine (8-OHdG).Material and methods. 100 premature infants between 26/1-34/6 weeks of gestation were included in the study: groupI consisted of 50 infants with RDS, group II – 50 newborns with RDS associated with perinatal asphyxia. The presence and severity of PPH was determined in all infants on the fi rst and third to fi fth day of life by echocardiography (EchoCG), and quantitative determination of 8-OHdG level (ng/ml) – in 44 infants on the fi rst day, and in dynamics – on the third to fi fth day of life by enzyme- linked immunosorbent assay (ELISA). For radiographic evaluation of PPH, all infants underwent chest radiography with determination of Moore’s, Schwedel’s and cardiothoracic index (CTI). The research was conducted in compliance with bioethical requirements as part of the planned scientifi c work of the Department (state registration number 0122U000025).Results. It was found that the characteristics of pulmonary hypertension in premature infants with RDS were signifi cantly lower levels of mean pressure in the pulmonary artery (mPAP) on the fi rst and 3-5 days of life than in children with perinatal asphyxia. It was noted that the factors with high diagnostic signifi cance determining the occurrence of persistent pulmonary hypertension are: birth weight <1500 g; presence of perinatal asphyxia; low Apgar score on the 1st (1-3 points) and on the 5th minute of life (<7 points); gestational age <30 weeks; non-appropriateness for gestational age; male sex. Furthermore, it was found that urinary 8-OHdG levels as a biomarker of OS in preterm infants with RDS and perinatal asphyxia correlated with mPAP on the fi rst and third to fi fth days of life, and that urinary 8-OHdG levels had a high diagnostic value for determining the risk of developing severe PPH on the third to fi fth days of life. The diagnostic signifi cance of the data of the comprehensive radiological assessment of PPH – the radiological indices of Moore, Schwedel, CTI for the development of severe PPH was analyzed and the correlations between the indices and mPAP and between the indices and the level of 8-OHdG were established. The Schwedel index showed the highest reliability in all cases.Conclusion. On the basis of scientifi cally established relationships between clinical, laboratory, radiological and genderaspects of premature infants with perinatal pathology and the identifi ed diagnostic and prognostic values of urinary 8-OHdG, an algorithm for a diff erentiated approach to the management of PPH was developed. Determination of the degree of OS and mPAP in premature infants allows us to adjust and individualize the tactics of respiratory support in the management of premature infants, thus improving the quality of medical care of premature infants with RDS and perinatal asphyxia. In prematurely born children in perinatal centers, additional determination of the severity of RDS based on the level of 8-OHdG in urine allows to predict the adverse course of PPH and the development of complications: bronchopulmonary dysplasia, intraventricular hemorrhage III-IV grade, retinopathy II-III grade, hearing impairment, hypoxic- ischemic lesions of the central nervous system II-III grade in prematurely born children.

Short-time mortality and severe complications of very premature infants-a multicenter retrospective cohort study from Jiangsu Province during 2019-2021.

The short-term and long-term severe complications of preterm infants have brought serious psychological and economic burdens to the society and family. Therefore, our study aimed to investigate the risk factors for the mortality and serious complications in very premature infants less than 32 weeks of gestational age (GA), so as to guide the antenatal and postnatal care of very premature. The very premature infants from 1 January 2019 to 31 December 2021 from 15 member hospitals of the Neonatal Intensive Care Unit (NICU) Multi-center Clinical Research Collaboration Group in Jiangsu Province were recruited. In accordance with the plan of the intensive care unit for unified management, recruitment of premature infants is carried out on the day of admission, and discharge or death is the outcome indicator in 1-2 months by telephone follow-up. The research content mainly includes three aspects: clinical information of mother and infant, outcomes and complications. According to the final outcomes, very premature infants were divided into two categories: survival without severe complications, survival with severe complications and death. Then, univariate and multivariate logistic regression model and receiver operating characteristic (ROC) analyses were used to analyze the independent risk factors. A total of 3,200 very premature infants with GA less than 32 weeks were recruited. The median GA is 30.00 (28.57, 31.14) weeks, the average birth weight is 1,350 (1,110, 1,590) g, among whom 375 premature infants survived with severe complications, and 2,391 premature infants survived without severe complications. Then, it was found that GA at birth was a protective factor for death and severe complications, whereas severe neonatal asphyxia and persistent pulmonary hypertension of the newborn (PPHN) were independent risk factors for death and severe complications in very premature infants born at less than 32 weeks of gestation. The prognosis of very premature infants in NICU treatment depends not only on GA, but also on various perinatal factors and their clinical management, such as preterm asphyxia and PPHN occurrence, so the next step is necessary for multicenter continuous quality improvement to improve outcomes in very preterm infants.

Use of Vasopressin as Rescue Therapy in Refractory Hypoxia and Refractory Systemic Hypotension in Term Neonates with Severe Persistent Pulmonary Hypertension-A Prospective Observational Study.

Persistent pulmonary hypertension of the newborn (PPHN) is a serious cardiorespiratory problem. PPHN is frequently associated with refractory hypoxia and hypotension, and optimal management has the potential to improve important clinical outcomes including mortality. The primary objective is to evaluate the efficacy and safety of rescue vasopressin (VP) therapy in the management of severe (refractory) hypoxia and refractory systemic hypotension in term neonates with severe PPHN. Neonates with refractory hypoxia and refractory hypotension due to severe PPHN needing VP were prospectively enrolled in the study. Refractory hypoxia was defined as oxygenation index (OI) ≥ 25 for at least 4 hours after the commencement of high-frequency oscillatory ventilation and nitric oxide at 20 ppm. Refractory hypotension was defined as mean blood pressure lesser than mean gestational age lasting for more than 15 minutes in spite of dopamine infusion at 10 µg/kg/min, adrenaline infusion at 0.3 µg/kg/min, and noradrenaline infusion at 0.1 µg/kg/min. Thirty-two neonates with PPHN were recruited. The baseline OI (mean ± standard deviation [SD]) before starting VP was 33.43 ± 16.54 which started decreasing significantly between 1 and 6 hours after the commencement of VP (p < 0.05). The mean blood pressure also increased concomitantly with a significant effect seen by 1 hour (p < 0.05). The vasoactive infusion score before the commencement of VP was mean 46.07 (SD = 25.72) and started decreasing after 12 to 24 hours of commencement of VP (p < 0.05). Lactate levels (mean ± SD) before starting VP were 7.8 ± 8.6 mmol/L and started decreasing between 6 and 12 hours (p < 0.05). Two neonates died due to refractory hypoxia and refractory hypotension (overall mortality 6.2%) CONCLUSION: Rescue VP therapy is a useful adjunct in the management of neonates with severe PPHN with refractory hypoxia and/or refractory hypotension. Improvement in oxygenation and hemodynamics with the use of VP results in reduced mortality. · Rescue vasopressin is a useful adjunct in the management of neonates with severe PPHN.. · Vasopressin helps reduce OI.. · Vasopressin reduces the vasoactive inotrope score..

Severe Persistent Pulmonary Hypertension of the Newborn is associated with Chorioamnionitis and Funisitis in Extremely Preterm Infants

Severe Persistent Pulmonary Hypertension of the Newborn is associated with Chorioamnionitis and Funisitis in Extremely Preterm Infants

Prostacyclins and pulmonary arterial hypertension in children.

Despite its low incidence, pulmonary hypertension in children places a substantial burden on families and society because survival can be shorter than 10 months and treatment options are limited and ineffective. Drugs to treat pulmonary hypertension include endothelin antagonists, phosphodiesterase type 5 inhibitors and prostacyclin, which is the most widely used to treat pediatric pulmonary hypertension. The main aim of this study was to provide a comprehensive overview of the advantages and disadvantages of prostacyclin and its analogs for treating pulmonary hypertension in children. To retrieve a thorough collection of studies, we performed a search in PubMed using the following combination of keywords: (Prostacyclins) or (Epoprostenol) or (Iloprost) or (Treprostinil) or (Beraprost), (children) and (pulmonary arterial hypertension). The time limits used for the search were December 1983 to May 2021. The search retrieved a total of 238 articles. Titles and abstracts of articles were screened for relevance, and all relevant articles published in English were included. Epoprostenol can be effective against severe pulmonary hypertension. Iloprost can treat severe persistent pulmonary hypertension in newborns and inhaled iloprost can be used in pulmonary vasoreactivity testing. Treprostinil is a long-acting prostacyclin analog, and it shows the highest antiproliferative activity among prostacyclins. Beraprost may be effective in premature infants, but available evidence comes from only one patient, so more clinical testing is needed.

Severe Congenital Syphilis in the Neonatal Intensive Care Unit: A Retrospective Case Series.

There has been a 291% relative increase in congenital syphilis (CS) cases in the United States from 2015 to 2019. Although the majority of affected fetuses/infants are stillborn or are asymptomatic, a subset is born with severe clinical illness. We describe a series of severe CS cases in the neonatal intensive care unit. Retrospective review of infants with CS, admitted to the Duke Intensive Care Nursery from June 2016 to February 2020. We recorded birthweight, gestational age, medications, procedures, diagnoses, laboratory data and outcomes. Severe symptoms included: birth depression, hypoxic ischemic encephalopathy (HIE), disseminated intravascular coagulopathy and/or persistent pulmonary hypertension (PPHN). Seven infants with CS were identified and 5 with severe presentations were included. Median gestational age was 35.1 weeks (range: 29-37 weeks, median: 35 weeks). All infants required intubation at birth, 2 required chest compressions and epinephrine in the delivery room. One had hydrops fetalis and died in the delivery room. All 4 surviving infants had HIE, severe PPHN, hepatitis and seizures. All infants had a positive rapid plasma reagin, and were treated with penicillin G. Maternal rapid plasma reagin was pending for 3 of 5 infants at delivery, and later returned positive; 2 were positive during pregnancy but not treated. Other infectious work-up was negative. Three infants survived to discharge. CS can be associated with HIE, PPHN and disseminated intravascular coagulopathy in affected infants. Clinicians should have a high index of suspicion and include CS in their differential diagnoses. This study also highlights the importance of adequate treatment of identified cases and screening during the third trimester and at delivery.

Large Left Ventricular Thrombus in a Neonate — A Rare Complication of Severe Persistent Pulmonary Hypertension of the Neonate

Large Left Ventricular Thrombus in a Neonate — A Rare Complication of Severe Persistent Pulmonary Hypertension of the Neonate

Neonatal Respiratory Distress Secondary to Meconium Aspiration Syndrome.

Infants born through meconium-stained amniotic fluid (MSAF) are 100 times more likely than infants born through clear amniotic fluid to develop respiratory distress in the neonatal period. Meconium aspiration syndrome (MAS) is a common cause of respiratory distress in term and post-mature neonates. MAS is defined as respiratory distress accompanied by a supplemental oxygen requirement in an infant born with MSAF, in the absence of any other identified etiology to explain the symptoms. Therapy for MAS is supportive, and should be tailored to each infant’s specific pathophysiology. In cases of MAS with severe persistent pulmonary hypertension of the newborn (PPHN), patients may remain hypoxic despite aggressive ventilation, and in these cases surfactant, inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO) can be life-saving. Long-term prognosis for MAS is more related to severity of initial hypoxemia and possible neurological insult than to the pulmonary pathology.

An unusual neonatal case of reverse flow in the aortic arch and persistent pulmonary hypertension

The reverse flow in the aortic arch in newborn is a rare finding due to congenital heart disease with blood "stealing" from the descending aorta or severe left ventricular outflow tract obstruction and to extracardiac abnormalities as systemic arteriovenous malformation. We describe a female newborn with unexpected reverse flow in the aortic arch due to severe persistent pulmonary hypertension (PPH). The presence of reverse flow in the aortic arch in PPH, secondary to compromised LV output, was rarely reported and investigated but can be a helpful sign of worse clinical course and poor prognosis.

Vein of galen malformation presenting as severe persistent pulmonary hypertension in neonate

Vein of Galen malformation (VOGM) is a rare type of arteriovenous malformation with majority presenting in neonatal period. The most common presentation is high output cardiac failure and association with persistent pulmonary hypertension (PPHN) is rarely reported. If left untreated mortality rate is very high. Endovascular embolization is the preferred treatment modality at present. We describe a full-term male baby presented at 1 h of life with respiratory distress, eventually diagnosed to have PPHN on echocardiography. Ultrasonography cranium done in view of cranial bruit revealed VOGM which later confirmed with magnetic resonance (MR) imaging brain and MR angio and venography. All supportive measures were provided. But because of hemodynamic instability endovascular embolization could not be attempted and baby succumbed due to severe refractory PPHN. We should consider the possibility of VOGM in all refractory cases of PPHN even though the association is very rare.

Oral sildenafil therapy for the management of mild persistent pulmonary hypertension of the newborn in term neonates

The use of sildenafil leads to an improvement in oxygenation and has the potential to reduce mortality in severe persistent pulmonary hypertension of neonates (PPHN). Mild PPHN in term neonates often prolongs the duration of oxygen requirement without other major morbidities, thereby increasing the length of hospital stay. We present a case series of eight term neonates with clinical and echocardiographic evidence of mild PPHN, in whom treatment with oral sildenafil resulted in early clinical improvement. It was observed that neonates could be weaned off from oxygen inhalation after a mean (SD) 53.1 (11.8) h of starting oral sildenafil as their oxygen saturation was maintained between 93 and 95% in room air. Infants could be discharged home after a median (IQR) 10 (7.5–12) days and oral sildenafil could be stopped after a median duration of 1.4 (1–1.8) months. All infants tolerated oral sildenafil well without any major adverse events.

Efficacy of Milrinone Plus Sildenafil in the Treatment of Neonates with Persistent Pulmonary Hypertension in Resource-Limited Settings: Results of a Randomized, Double-Blind Trial.

BackgroundThe management of severe persistent pulmonary hypertension (PPHN) can be very challenging in many resource-limited centers without access to inhaled nitric oxide or extracorporeal membrane oxygenation.ObjectivesThe current study aimed to investigate the efficacy of oral sildenafil and intravenous milrinone infusion and compare the effects of these drugs in combination versus as monotherapy in neonates with PPHN.MethodsA double-blind randomized controlled trial was conducted in which neonates with PPHN were divided into three groups of 20 patients each: group 1 received oral sildenafil starting at 0.5 mg/kg every 6 h to a target maintenance dose of 2 mg/kg every 6 h; group 2 received intravenous milrinone 0.5 μg/kg/min as a continuous infusion; and group 3 received both oral sildenafil and intravenous milrinone.ResultsPost-treatment pulmonary artery systolic pressure was significantly lower in group 3 than in groups 1 and 2, which both received monotherapy (p = 0.031). The oxygenation index also decreased significantly in the dual-therapy group (p = 0.002) compared with the monotherapy groups. Combined use of both drugs demonstrated a beneficial synergistic effect with better outcomes and reduced mortality.ConclusionDual therapy using sildenafil and milrinone was superior to monotherapy with either drug in neonates with severe PPHN and is recommended for use in resource-constrained settings.RegistrationPan African Clinical Trial Registry identifier number PACTR201902691230243.

Cost-Utility of Sildenafil for Persistent Pulmonary Hypertension of the Newborn.

While advanced therapies for severe persistent pulmonary hypertension of the newborn (PPHN) such as inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO) are standard treatments in high-income countries, these therapies are often unavailable in resource-limited settings such as middle-income countries. However, there are small clinical trials illustrating the efficacy of sildenafil at reducing mortality in PPHN. This analysis sought to determine the cost-utility of enteral sildenafil for the treatment of severe PPHN. A Markov-state transition model was constructed for the two clinical approaches to compare costs, clinical outcomes, and quality of life: (1) "conventional," (2) "sildenafil." The impact of sildenafil was modeled as a relative risk modifier of the conventional strategy's mortality risk. Transitional probabilities, costs, and utility metrics were extracted from the literature. Sensitivity analyses for each model input as well as 100-patient Monte Carlo simulations were used to test the durability of the model conclusion. The sildenafil strategy was cost-effective for upper but not lower middle-income countries with an incremental cost-effectiveness ratio of $2,339 per quality-adjusted life year. This conclusion was durable across a wide-range of model assumptions; the sildenafil strategy only failed to meet criteria for cost-effectiveness when sildenafil therapy had a mortality relative risk efficacy of >0.89, if life expectancy in that country is <40 years, or if the lifetime forecasted costs of a survivor's life was quite high. Enteral sildenafil is a cost-effective intervention for severe PPHN for upper middle-income countries where ECMO and iNO are not available. · PPHN is a common life-threatening condition in newborns.. · Sildenafil improves survival of PPHN.. · Sildenafil is cost-effective for upper-middle income countries..

Extracorporeal membrane oxygenation after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension.

Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) but can result in respiratory and cardiac complications that may require extracorporeal membrane oxygenation (ECMO). We reviewed our experience with ECMO in patients undergoing PEA. Between January 2012 and August 2015, 35 patients underwent PEA for CTEPH. In all, four patients (11%) required veno-arterial (V-A) ECMO support due to severe cardiac and respiratory failure, including severe reperfusion pulmonary edema and persistent pulmonary hypertension. No significant differences in preoperative characteristics were found between patients who required ECMO and those who did not require ECMO. ECMO support was associated with a significantly higher incidence of postoperative respiratory complications, a longer intensive care unit stay, increased in-hospital mortality, residual pulmonary hypertension, and postoperative balloon pulmonary angioplasty (BPA). The postoperative mean pulmonary artery pressure and pulmonary vascular resistance were significantly higher in patients requiring ECMO. All patients requiring ECMO were successfully weaned off ECMO support (100%), and three of them were discharged from the hospital alive (75%). Patients with CTEPH may benefit from ECMO after PEA for cardiac and respiratory complications. A prompt decision to use V-A ECMO is critical for a successful outcome in these patients.

PREVALENCE OF PULMONARY HYPERTENSION IN NEWBORN INFANTS

Background: Echocardiography is non-invasive technique that used to image the effects of pulmonary hypertension on the heart and estimate Pulmonary Artery Pressure from continuous wave Doppler measurements. Pulmonary hypertension prevalence of about 1 - 10% of the global population. Severe persistent pulmonary hypertension is about 2-6 / 1000 of all births. Female/Male ratio is about 2/1. The symptoms of pulmonary hypertension include respiratory distress, poor feeding, Cyanosis and others. Aim: The current study aimed to assess the incidence of pulmonary hypertension in newborn infants in NICU using Echocardiography. Patients and Methods: The current study is a case control study & aimed to estimate the prevalence of pulmonary Hypertension in newborn infants in the NICU in Al-HussainUniversityHospital during the period from May 2016 till April 2017. The newborn infants were subdivided into four groups according to the gestational age. Group (A): 15 apparent healthy babies who were collected from Obestetric and outpatient departments. Group (B): 15 babies who were admitted to NICU due to underlying problem. Group (C): 30 Near-term infants from 32 – 36 GA. Group (D): 30 Preterm infants less than 32 GA. Including all babies from both sexes, aged 0-14 days, any gestational age and evaluated with Echocardiography. Babies with congenital anomalies, weight less than 1000gm and GA less than 30Weeks were excluded. Results: The demographic data showed no differences between all groups regarding age and sex. Many risk factors were observed during the study as respiratory distress syndrome (16.7%), premature rupture of membrane (10%), maternal diabetes (5.6%) and meconium aspiration syndrome (8.9%). Pulmonary hypertension (P++) occurred commonly in females (60%), with RDS (33.3%) and with cesarean section (80%). All cases with P++ were in need to oxygen supplementation (100%) with O2 saturation ranging from 93-100% and the blood gases analysis tended to be metabolically acidosis. Finally we found the prevalence of pulmonary hypertension in our study were 5.6% of all cases with mean systolic artery pressure 51.80±10.73 mmHg and the mortality rate reached up to 20%.

Abstract P-327: OUTCOMES OF BABIES WITH CONGENITAL DIAPHRAGMATIC HERNIA IN A NON ECMO PEADIATRIC INTENSIVE CARE UNIT IN SOUTH AUSTRALIA. A RETROSPECTIVE OBSERVATIONAL STUDY OVER 16 YEARS.

Aims & Objectives: Introduction: Congenital diaphragmatic hernia (CDH) is a common birth defect which is associated with high morbidity and mortality. The cause for mortality is usually severe persistent pulmonary hypertension (PPHN) resulting from pulmonary hypoplasia. With Advances in intensive care and ECMO, mortality has reduced to < 20% in many centres. We report outcomes from Non ECMO tertiary PICU.1,2,3 Methods This retrospective observational study done over 16 years in a tertiary PICU without ECMO in South Australia. Results 62 babies born or referred to us with CDH had survival rate of 87%comparable to many ECMO centres. We noted severe PPHN in 30% (19/62) managed with inhaled Nitric oxide (iNO) of whom 36%(7/62) expired. Prostaglandin infusion was given to avoid right heart failure in 27 %(17/62) of which 50% received nitric and Prostaglandin infusion (8/62). Conclusions The outcome of CDH has improved with survival rates of 70–90% in centres even without ECMO. We report a survival rate of 87% with conventional management NO and PGE1 infusion. PGE1 results in ductal patency with reduction of right ventricular strain to manage transitional PPHN. We report increasing use of PGE1 over the past decade with improved outcomes.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register

To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.