Pediatric Pulmonary Hypertension

Journal of the American College of Cardiology (Impact Factor: 16.5). 12/2013; 62(25 Suppl):D117-26. DOI: 10.1016/j.jacc.2013.10.028
Source: PubMed


Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

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Available from: David Dunbar Ivy, Feb 01, 2014
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    • "The use of multiple biomarker panels assessed in large multi-center collaborations to increase cohort sizes will change the current paradigm of 100s of studies on a few kids to a few studies on 100s of kids. We recently summarized the current approach and diagnostic classification of PAH in children, as based on discussions and recommendations from the Pediatric Task Force of the fifth World Symposium on Pulmonary Hypertension (WSPH) in Nice, France (147). We outline our perspective on the use of biomarkers with regard to the classification, etiology, epidemiology and survival, diagnosis, treatment goals, and treatment of pediatric PH. "
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    ABSTRACT: Therapeutic approaches in pediatric pulmonary arterial hypertension (PAH) are based primarily on clinician experience, in contrast to the evidence-based approach in adults with pulmonary hypertension. There is a clear and present need for non-invasive and objective biomarkers to guide the accurate diagnosis, treatment, and prognosis of this disease in children. The multifaceted spectrum of disease, clinical presentation, and association with other diseases makes this a formidable challenge. However, as more progress is being made in the understanding and management of adult PAH, the potential to apply this knowledge to children has never been greater. This review explores the state of the art with regard to non-invasive biomarkers in PAH, with an eye toward those adult PAH biomarkers potentially suitable for application in pediatric PAH.
    Full-text · Article · Feb 2014 · Frontiers in Pediatrics
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    • "APCs supply systemic arterial blood to the distal pulmonary vasculature where it competes with blood flow from the pulmonary arterial system. Theoretically, chronic elevated shunt flow to the lung creates areas of high-pressure and increased pulmonary vascular resistance [28,29] and therefore has the potential to contribute to adverse clinical outcomes. On the other side, APC flow promotes pulsatile flow to the pulmonary vessels, thereby increasing shear stress on the endothelium that consecutively releases more nitric oxide, a potent vasodilator, that subsequently lowers pulmonary vascular resistance. "
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    ABSTRACT: Aortopulmonary collaterals (APCs) are frequently found in patients with a single-ventricle (SV) circulation. However, knowledge about the clinical significance of the systemic-to-pulmonary shunt flow in patients after the modified Fontan procedure and its potential causes is limited. Accordingly, the aim of our study was to detect and quantify APC flow using cardiovascular magnetic resonance (CMR) and assess its impact on SV volume and function as well as to evaluate the role of the size of the pulmonary arteries in regard to the development of APCs. 60 patients (mean age 13.3 ± 6.8 years) after the Fontan procedure without patent tunnel fenestration underwent CMR as part of their routine clinical assessment that included ventricular functional analysis and flow measurements in the inferior vena cava (IVC), superior vena cava (SVC) and ascending aorta (Ao). APC flow was quantified using the systemic flow estimator: (Ao) - (IVC + SVC). Pulmonary artery index (Nakata index) was calculated as RPA + LPA area/body surface area using contrast enhanced MR angiography. The patient cohort was divided into two groups according to the median APC flow: group 1 < 0.495 l/min/m(2) and group 2 > 0.495 l/min/m(2). Group 1 patients had significant smaller SV enddiastolic (71 ± 16 vs 87 ± 25 ml/m(2); p=0.004) and endsystolic volumes (29 ± 11 vs 40 ± 21 ml/m(2); p=0.02) whereas ejection fraction (59 ± 9 vs 56 ± 13%; p=0.38) differed not significantly. Interestingly, pulmonary artery size showed a significant inverse correlation with APC flow (r=-0.50, p=0.002). Volume load due to APC flow in Fontan patients affected SV dimensions, but did not result in an impairment of SV function. APC flow was related to small pulmonary artery size, suggesting that small pulmonary arteries represent a potential stimulus for the development of APCs.
    Full-text · Article · Oct 2013 · PLoS ONE
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    ABSTRACT: The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations on the diagnosis of pulmonary hypertension (PH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the treatment of pulmonary arterial hypertension (PAH). This commentary describes in detail the results and recommendations of the working group on treatment of PAH which were last updated in October 2011.
    Full-text · Article · Dec 2011 · International Journal of Cardiology
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