New Trend in Pediatric Cardiology: Pulmonary Hypertension

A special issue of Children (ISSN 2227-9067).

Deadline for manuscript submissions: closed (31 May 2017) | Viewed by 84340

Special Issue Editor


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Guest Editor
Division of Pediatric Cardiology, University of Illinois College of Medicine at Chicago, 840 S. Wood St., Chicago, IL 60612-7324, USA
Interests: children obesity; children hypertension; children pulmonary hypertension; children congenital heart disease; children exercise

Special Issue Information

Dear Colleagues,

Pulmonary hypertension is a hot issue in paediatric cardiology affecting all ages, from neonates to adolescents, and significantly impacting morbidity and mortality.  Even though our knowledge and understanding of this pathology has increased in the last few years, many aspects need further clarification.  Thus, there is a need for additional investigation.

This special issue of Children will act as a forum to increase understanding of this syndrome, by sharing knowledge about incompletely understood and controversial aspects.

Both original research articles and literature reviews will be considered for publication.

Prof. Maria Serratto
Guest Editor

Manuscript Submission Information

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Keywords

  • congenital heart disease
  • persistent pulmonary hypertension of the newborn
  • bronchopulmonary dysplasia
  • sickle cell disease
  • sleep-disordered breathing
  • congenital diaphragmatic hernia
  • genetics, idiopathic

Published Papers (7 papers)

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Review

28 pages, 15537 KiB  
Review
Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children
by Benjamin S. Frank and D. Dunbar Ivy
Children 2018, 5(4), 44; https://doi.org/10.3390/children5040044 - 23 Mar 2018
Cited by 24 | Viewed by 8781
Abstract
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the [...] Read more.
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure. In recent years, a number of pulmonary arterial hypertension (PAH)-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome. A growing body of evidence in both the adult and pediatric literature demonstrates enhanced quality of life, functional status, and survival among treated patients. This review provides a description of select etiologies of PH seen in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH/PAH. The available evidence for specific classes of PAH-targeted therapies in pediatrics is additionally discussed. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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390 KiB  
Review
Pediatric Perioperative Pulmonary Arterial Hypertension: A Case-Based Primer
by Shilpa Shah and Jacqueline R. Szmuszkovicz
Children 2017, 4(10), 92; https://doi.org/10.3390/children4100092 - 24 Oct 2017
Cited by 6 | Viewed by 6385
Abstract
The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this [...] Read more.
The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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Review
Diagnostic Approach to Pulmonary Hypertension in Premature Neonates
by Vasantha H.S. Kumar
Children 2017, 4(9), 75; https://doi.org/10.3390/children4090075 - 24 Aug 2017
Cited by 17 | Viewed by 12228
Abstract
Bronchopulmonary dysplasia (BPD) is a form of chronic lung disease in premature infants following respiratory distress at birth. With increasing survival of extremely low birth weight infants, alveolar simplification is the defining lung characteristic of infants with BPD, and along with pulmonary hypertension, [...] Read more.
Bronchopulmonary dysplasia (BPD) is a form of chronic lung disease in premature infants following respiratory distress at birth. With increasing survival of extremely low birth weight infants, alveolar simplification is the defining lung characteristic of infants with BPD, and along with pulmonary hypertension, increasingly contributes to both respiratory morbidity and mortality in these infants. Growth restricted infants, infants born to mothers with oligohydramnios or following prolonged preterm rupture of membranes are at particular risk for early onset pulmonary hypertension. Altered vascular and alveolar growth particularly in canalicular and early saccular stages of lung development following mechanical ventilation and oxygen therapy, results in developmental lung arrest leading to BPD with pulmonary hypertension (PH). Early recognition of PH in infants with risk factors is important for optimal management of these infants. Screening tools for early diagnosis of PH are evolving; however, echocardiography is the mainstay for non-invasive diagnosis of PH in infants. Cardiac computed tomography (CT) and magnetic resonance are being used as imaging modalities, however their role in improving outcomes in these patients is uncertain. Follow-up of infants at risk for PH will help not only in early diagnosis, but also in appropriate management of these infants. Aggressive management of lung disease, avoidance of hypoxemic episodes, and optimal nutrition determine the progression of PH, as epigenetic factors may have significant effects, particularly in growth-restricted infants. Infants with diagnosis of PH are managed with pulmonary vasodilators and those resistant to therapy need to be worked up for the presence of cardio-vascular anomalies. The management of infants and toddlers with PH, especially following premature birth is an emerging field. Nonetheless, combination therapies in a multi-disciplinary setting improves outcomes for these infants. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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200 KiB  
Review
The Pulmonary Circulation in the Single Ventricle Patient
by Amanda Hauck, Nicolas Porta, Steven Lestrud and Stuart Berger
Children 2017, 4(8), 71; https://doi.org/10.3390/children4080071 - 7 Aug 2017
Cited by 26 | Viewed by 7013
Abstract
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in ‘single ventricle’ physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many [...] Read more.
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in ‘single ventricle’ physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan. Yet there is still a fair amount of knowledge to be gained, specifically as it relates to the pulmonary circulation in this group of patients. Knowledge gaps relate not only to the pulmonary circulation after Fontan operation, but also at each stage of the single ventricle surgical palliation, including the native physiology prior to any intervention. The pulmonary circulation is affected by multiple issues related to the single ventricle, including specific details of the anatomy unique to each patient, any intervention(s) undertaken, and potential complications such as aortopulmonary collaterals, protein losing enteropathy, plastic bronchitis, venovenous collaterals, pulmonary arteriovenous fistulae, ventricular dysfunction, pulmonary venous stenosis, and more. This chapter will review the current knowledge with regard to the pulmonary circulation in the single ventricle patient, primarily after the Fontan operation. Additionally, it is our hope to help the practitioner assess the pulmonary circulation in the single ventricle patient; we will also discuss the evidence behind and approach to treatment strategies in order to optimize the pulmonary circulation in this complex group of patients. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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Review
The Fetus Can Teach Us: Oxygen and the Pulmonary Vasculature
by Payam Vali and Satyan Lakshminrusimha
Children 2017, 4(8), 67; https://doi.org/10.3390/children4080067 - 3 Aug 2017
Cited by 39 | Viewed by 14186
Abstract
Neonates suffering from pulmonary hypertension of the newborn (PPHN) continue to represent an important proportion of patients requiring intensive neonatal care, and have an increased risk of morbidity and mortality. The human fetus has evolved to maintain a high pulmonary vascular resistance (PVR) [...] Read more.
Neonates suffering from pulmonary hypertension of the newborn (PPHN) continue to represent an important proportion of patients requiring intensive neonatal care, and have an increased risk of morbidity and mortality. The human fetus has evolved to maintain a high pulmonary vascular resistance (PVR) in utero to allow the majority of the fetal circulation to bypass the lungs, which do not participate in gas exchange, towards the low resistance placenta. At birth, oxygen plays a major role in decreasing PVR to enhance pulmonary blood flow and establish the lungs as the organ of gas exchange. The failure of PVR to fall following birth results in PPHN, and oxygen remains the mainstay therapeutic intervention in the management of PPHN. Knowledge gaps on what constitutes the optimal oxygenation target leads to a wide variation in practices, and often leads to excessive oxygen use. Owing to the risk of oxygen toxicity, avoiding hyperoxemia is as important as avoiding hypoxemia in the management of PPHN. Current evidence supports maintaining arterial oxygen tension in the range of 50–80 mm Hg, and oxygen saturation between 90–97% in term infants with hypoxemic respiratory failure. Clinical studies evaluating the optimal oxygenation in the treatment of PPHN will be enthusiastically awaited. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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Review
Persistent Pulmonary Hypertension in the Newborn
by Bobby Mathew and Satyan Lakshminrusimha
Children 2017, 4(8), 63; https://doi.org/10.3390/children4080063 - 28 Jul 2017
Cited by 49 | Viewed by 28205
Abstract
Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth due to delay or impairment in the normal fall in pulmonary vascular resistance (PVR) that occurs following birth. The fetus is in a state of physiological pulmonary [...] Read more.
Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth due to delay or impairment in the normal fall in pulmonary vascular resistance (PVR) that occurs following birth. The fetus is in a state of physiological pulmonary hypertension. In utero, the fetus receives oxygenated blood from the placenta through the umbilical vein. At birth, following initiation of respiration, there is a sudden precipitous fall in the PVR and an increase of systemic vascular resistance (SVR) due to the removal of the placenta from circulation. There is dramatic increase in pulmonary blood flow with a decrease in, and later reversal of shunts at the foramen ovale and ductus arteriosus. The failure of this normal physiological pulmonary transition leads to the syndrome of PPHN. PPHN presents with varying degrees of hypoxemic respiratory failure. Survival of infants with PPHN has significantly improved with the use of gentle ventilation, surfactant and inhaled nitric oxide (iNO). PPHN is associated with significant mortality and morbidity among survivors. Newer agents that target different enzymatic pathways in the vascular smooth muscle are in different stages of development and testing. Further research using these agents is likely to further reduce morbidity and mortality associated with PPHN. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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231 KiB  
Review
Sildenafil in Infants and Children
by Larisa Simonca and Robert Tulloh
Children 2017, 4(7), 60; https://doi.org/10.3390/children4070060 - 24 Jul 2017
Cited by 17 | Viewed by 6401
Abstract
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to [...] Read more.
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension. Although recent data regarding long-term mortality and the repeal of Food and Drug Administration (FDA) approval has complicated the issue, Sildenafil continues to be the major treatment option for paediatric PH for patients in a variety of contexts, and this does not seem likely to change in the foreseeable future. In this review, we provide a summary of pulmonary hypertension in infants and children and the use of Sildenafil for such diseases. Full article
(This article belongs to the Special Issue New Trend in Pediatric Cardiology: Pulmonary Hypertension)
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