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Persistent Pulmonary hypertension of the newborn

Satyan Lakshminrusimha, Seetha Shankaran, Abbot Laptook, Scott McDonald, Martin Keszler, Krisa Van Meurs, Ronnie Guillet, Sanjay Chawla, Beena G Sood, Sonia Bonifacio, Abhik Das, Rosemary D Higgins
OBJECTIVE: To determine the characteristics of term infants with persistent pulmonary hypertension of the newborn (PPHN) associated with moderate or severe hypoxic ischemic encephalopathy (HIE). METHODS: We compared infants with and without PPHN enrolled in 2 randomized trials of therapeutic hypothermia: the induced hypothermia trial of cooling to 33.5°C for 72 hours vs normothermia, and the "usual-care" arm (33.5°C for 72 hours) of the optimizing cooling trial...
March 1, 2018: Journal of Pediatrics
Aamisha Gupta, Dennis VanLoozen, Anastasios C Polimenakos, Kenneth A Murdison
Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario...
March 2, 2018: Pediatric Cardiology
Allyson Kayton, Paula Timoney, Lyn Vargo, Jose A Perez
BACKGROUND: Excessive supplemental oxygen exposure in the neonatal intensive care unit (NICU) can be associated with oxygen-related toxicities, which can lead to negative clinical consequences. Use of inhaled nitric oxide (iNO) can be a successful strategy for avoiding hyperoxia in the NICU. iNO selectively produces pulmonary vasodilation and has been shown to improve oxygenation parameters across the spectrum of disease severity, from mild to very severe, in neonates with hypoxic respiratory failure associated with persistent pulmonary hypertension of the newborn...
February 20, 2018: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
N Katz, Y Bar-Or, A Raucher-Sterrnfeld, A Tamir, D Kohelet
Persistent pulmonary hypertension (PPHN) of the newborn is one of the most challenging acute disorders of postnatal transition with substantial morbidity and mortality. The aim of the study was to find if there is an association between persistent pulmonary hypertension and histologic chorioamnionitis in preterm infants. 27 preterm infants with echocardiographic evidence of PPHN within the first 3 days of life were eligible for the study. A matched control group of 27 patients was chosen according to gestational age, date of birth, and gender...
February 16, 2018: Pediatric Cardiology
Chung-Ting Hsu, Chao-Huei Chen, Ming-Chih Lin, Teh-Ming Wang, Ya-Chi Hsu
BACKGROUND: Premature infants are at high risk for developmental delay and cognitive dysfunction. Besides medical conditions, growth restriction is regarded as an important risk factor for cognitive and neurodevelopmental dysfunction throughout childhood and adolescence and even into adulthood. In this study, we analyzed the relationship between post-discharge body weight and psychomotor development using a nationwide dataset. MATERIALS AND METHODS: This was a nationwide cohort study conducted in Taiwan...
2018: PloS One
Daphne S Mous, Heleen M Kool, Rene Wijnen, Dick Tibboel, Robbert J Rottier
Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation...
March 31, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
Hidehiko Nakanishi, Shunichi Morikawa, Shuji Kitahara, Asuka Yoshii, Atsushi Uchiyama, Satoshi Kusuda, Taichi Ezaki
PURPOSE: Pulmonary microvascular injury is associated with the pathogenesis of bronchopulmonary dysplasia (BPD). To characterize the mechanisms of pulmonary vascular disease resulting from BPD, we studied the ultrastructural changes affecting pulmonary microvasculature. METHODS: Newborn ICR mice were exposed to 85% hyperoxia or normoxia for 14 days, and then normal air replacement conditions for the following 7 days. At postnatal day (P)14 and P21, lungs were harvested for ultrastructural examination and assessment of pulmonary hypertension...
January 23, 2018: Medical Molecular Morphology
Philippe Gaudard, Claudio Barbanti, Bertrand Rozec, Philippe Mauriat, Mimoun M'rini, Gilles Cambonie, Jean Michel Liet, Claude Girard, Pierre Louis Leger, Ziad Assaf, Pierre Damas, Gauthier Loron, Laurent Lecourt, Julien Amour, Philippe Pouard
BACKGROUND: Nitric oxide (NO) has a well-known efficacy in pulmonary hypertension (PH), with wide use for 20 years in many countries. The objective of this study was to describe the current use of NO in real life and the gap with the guidelines. METHODS: This is a multicenter, prospective, observational study on inhaled NO administered through an integrated delivery and monitoring device and indicated for PH according to the market authorizations. The characteristics of NO therapy and ventilation modes were observed...
January 16, 2018: Anesthesia and Analgesia
Ali Shabbir Hussain, Rehan Ali, Shakeel Ahmed, Farah Naz, Anila Haroon
BACKGROUND: The prevalence of PPHN has been estimated at 1.9 per 1000 live births. After the discovery of iNO's, its efficacy and benefit in PPHN is well established. Even in the best of centers equipped with iNo and ECMO the mortality is around 20%. Also, iNO is expensive and difficult to administer and monitor which makes it difficult choice in our part of the world. Furthermore About 40% of patients do not respond or have rebound pulmonary hypertension after discontinuation. Owing to these reasons, other treatment modalities like phosphodiesterase inhibitors such as Sildenafil need to be evaluated...
October 2017: Journal of Ayub Medical College, Abbottabad: JAMC
Rachel L León, Nicholas B Zaban, Marcus S Schamberger, Chang Y Ho, Ulrike Mietzsch
Small remnants of the right valve of the sinus venosus are commonly found in adults, but the incidence and risk associated with these embryonic remnants in neonates are not well studied. The following report describes a cyanotic neonate with a large Eustachian valve remnant creating a functional cor triatriatum dexter who was initially diagnosed with persistent pulmonary hypertension of the newborn. The cyanosis in this infant improved over the first postnatal week with conservative management, but she suffered multifocal subcortical stroke, likely related to her intracardiac shunt...
January 10, 2018: Neonatology
Ai Ito, Hideshi Fujinaga, Sachiko Matsui, Kumiko Tago, Yuka Iwasaki, Shuhei Fujino, Junko Nagasawa, Shoichiro Amari, Masao Kaneshige, Yuka Wada, Shigehiro Takahashi, Keiko Tsukamoto, Osamu Miyazaki, Takako Yoshioka, Akira Ishiguro, Yushi Ito
Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care...
October 2017: American Journal of Perinatology Reports
Hidehiko Nakanishi, Hideyo Suenaga, Atsushi Uchiyama, Satoshi Kusuda
OBJECTIVE: To investigate the characteristics of persistent pulmonary hypertension of the newborn (PPHN) in extremely preterm infants and its impact on neurodevelopmental outcomes at 3 years of age. DESIGN: A retrospective multicentre cohort study. SETTINGS: 202 tertiary perinatal centres registered in the Neonatal Research Network of Japan (NRNJ). PATIENTS: Infants born at <28 weeks of gestational age (GA), between 2003 and 2012, were extracted from tertiary perinatal centres participating in NRNJ...
January 3, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
David J McCulley, Mark D Wienhold, Elizabeth A Hines, Timothy A Hacker, Allison Rogers, Ryan J Pewowaruk, Rediet Zewdu, Naomi C Chesler, Licia Selleri, Xin Sun
A critical event in the adaptation to extrauterine life is relaxation of the pulmonary vasculature at birth, allowing for a rapid increase in pulmonary blood flow that is essential for efficient gas exchange. Failure of this transition leads to pulmonary hypertension (PH), a major cause of newborn mortality associated with preterm birth, infection, hypoxia, and malformations including congenital diaphragmatic hernia (CDH). While individual vasoconstrictor and dilator genes have been identified, the coordination of their expression is not well understood...
December 18, 2017: Journal of Clinical Investigation
Aakash Pandita, Srinivas Murki, Tejo Pratap Oleti, Baswaraj Tandur, Sai Kiran, Sachin Narkhede, Amrut Prajapati
Importance: Nasal continuous positive airway pressure (NCPAP) as a primary respiratory therapy in meconium aspiration syndrome (MAS) has not been studied extensively. Nasal continuous positive airway pressure, when applied in newborns with MAS, may resolve atelectasis by sufficiently expanding partially obstructed small airways and stabilizing the collapsing terminal airways to enhance oxygen exchange. Objective: To compare NCPAP vs standard care in neonates with moderate to severe respiratory failure due to MAS in reducing the need for invasive ventilation...
February 1, 2018: JAMA Pediatrics
Merrill McHoney, Philip Hammond
Congenital diaphragmatic hernia (CDH) is typified morphologically by failure of diaphragmatic development with accompanying lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). Patients who have labile physiology and low preductal saturations despite optimal ventilatory and inotropic support may be considered for extracorporeal membrane oxygenation (ECMO). Systematic reviews into the benefits of ECMO in CDH concluded that any benefit is unclear. Few randomised trials exist to demonstrate clear benefit and guide management...
March 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
Salwa Khedr, Anna Piskorski, Adrienne R Bingham, Justin Goldstein, Abbot R Laptook, Monique E De Paepe
Therapeutic hypothermia (head or whole-body cooling) improves survival and neurodevelopmental outcome in term newborns with moderate-to-severe encephalopathy. Hypothermia treatment is well tolerated; the most common side effect is thrombocytopenia. In about 1% of infants, focal subcutaneous fat necrosis has been reported. We describe a case of clinically unsuspected massive visceral fat necrosis in a term infant with Apgar score 0 at 1 min ("resuscitated apparently stillborn" infant) who was treated with therapeutic hypothermia for 72 h and expired on the 25th day of life following a neonatal course complicated by severe encephalopathy, pulmonary artery hypertension, persistent thrombocytopenia, hypoglycemia, and severe basal ganglia-thalamic abnormalities on magnetic resonance imaging...
January 1, 2017: Pediatric and Developmental Pathology
Carlo Dani, Iuri Corsini, Jessica Cangemi, Venturella Vangi, Simone Pratesi
BACKGROUND: Inhaled nitric oxide (iNO) cannot be recommended for the routine treatment of respiratory failure in premature neonates, but it has been suggested that the effectiveness of iNO therapy should be further studied in more select preterm infants, such as those with persistent pulmonary hypertension of the newborn (PPHN). OBJECTIVE: To evaluate the frequency of PPHN in very preterm infants with severe respiratory distress syndrome (RDS), to assess the effectiveness of iNO in these patients, and to individuate possible predictive factors for the response to iNO in preterm infants with RDS...
November 2017: Pediatric Pulmonology
Niralee Babooa, Wen-Jing Shi, Chao Chen
BACKGROUND: Several studies have clearly demonstrated a significantly higher incidence of persistent pulmonary hypertension of the newborn (PPHN) in neonates delivered by caesarean section (CS) compared to those delivered vaginally. The pathophysiological factors underlying the link between CS and PPHN are still poorly understood. In this review, we describe the mechanisms that could explain the association between CS delivery and subsequent PPHN, as well as potential preventive measures...
December 2017: World Journal of Pediatrics: WJP
Megha Sharma, Adeleye J Afolayan
Reactive oxygen species (ROS) are redox-signaling molecules that are critically involved in regulating endothelial cell functions, host defense, aging, and cellular adaptation. Mitochondria are the major sources of ROS and important sources of redox signaling in pulmonary circulation. It is becoming increasingly evident that increased mitochondrial oxidative stress and aberrant signaling through redox-sensitive pathways play a direct causative role in the pathogenesis of many cardiopulmonary disorders including persistent pulmonary hypertension of the newborn (PPHN)...
2017: Advances in Experimental Medicine and Biology
M Dehdashtian, M Aletayeb, A Malakian, M R Aramesh, H Malvandi
BACKGROUND: Transient tachypnea of the newborn (TTN) is a respiratory disorder secondary to inadequate or delayed clearance of lung fluids. Early symptoms of the disease are indistinguishable from neonatal respiratory distress syndrome, pneumonia, and persistent pulmonary hypertension. Therefore, these newborns, in addition to receiving conservative management, receive antibiotics until blood cultures provide definite results. In this study, we assessed the clinical course of neonates diagnosed with TTN who received conventional versus conservative management...
February 2018: Journal of the Chinese Medical Association: JCMA
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