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Key pathways involved in the pathophysiology of pulmonary hypertension. Three main pathways are known to be involved in pulmonary vasodilation and, to
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An increasing number of medical services dedicated to the diagnosis, treatment and follow-up of pulmonary hypertension (PH) in children are being established. This has, in turn, increased the need to adapt current guidelines for the treatment of PH to be more relevant to paediatric patients with PH. This article will summarise the data obtained so...
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... is a disease characterised by vasoconstriction, and hyper- trophy of the vascular smooth muscle cells in the pulmonary arteries, resulting in reduced individual vascular areas and total perfusion areas. Cardiac output is maintained by right ventricular hypertrophy and dilatation, and pulmonary arterial pressure increases. Right heart hypertrophy and dilatation then lead to right heart failure and, eventually, death. Current pulmonary arterial hypertension (PAH)-specific therapies, therefore, primarily re-establish pulmonary vasodilation and, to some extent, reduce smooth muscle proliferation and hypertrophy in the pulmonary arterial vessels. Three main pathways known to be involved in these processes in PH, the prostacyclin pathway, the endothelin pathway and the nitric oxide pathway, are targeted using prostanoids, ERAs and PDE-5 inhibitors, respectively ( fig. 1) ...
Citations
... There is a lack of randomized trials in paediatrics, making it difficult to deliver strong guidelines. 291,292 A specific treatment algorithm, similar to that used in adults (Figure 2), has been recommended. Determinants of risk and risk stratification have also been suggested. ...
... 4,7 PH associated with CHD (PH-CHD) is more frequent in children (36%) than in adults (10%), 4 while other associated forms are uncommon in children. 8 For the initial assessment and follow-up of children with suspected or confirmed PH, transthoracic echocardiography (TTE) is the noninvasive diagnostic method of choice. 5,[9][10][11] TTE allows a detailed description of cardiovascular anatomy, ventricular function, and flow determination and carries low risk and wider availability, compared with cardiac magnetic resonance imaging (MRI). ...
Transthoracic echocardiography (TTE) is the most accessible noninvasive diagnostic procedure for the initial assessment of pediatric pulmonary hypertension (PH). This review focuses on principles and use of TTE to determine morphologic and functional parameters that are also useful for follow-up investigations in pediatric PH patients. A basic echocardiographic study of a patient with PH commonly includes the hemodynamic calculation of the systolic pulmonary artery pressure (PAP), the mean and diastolic PAP, the pulmonary artery acceleration time, and the presence of a pericardial effusion. A more detailed TTE investigation of the right ventricle (RV) includes assessment of its size and function. RV function can be evaluated by RV longitudinal systolic performance (e.g., tricuspid annular plane systolic excursion), the tricuspid regurgitation velocity/right ventricular outflow tract velocity time integral ratio, the fractional area change, tissue Doppler imaging-derived parameters, strain measurements, the systolic-to-diastolic duration ratio, the myocardial performance (Tei) index, the RV/left ventricle (LV) diameter ratio, the LV eccentricity index, determination of an enlarged right atrium and RV size, and RV volume determination by 3-dimensional echocardiography. Here, we discuss the potential use and limitations of TTE techniques in children with PH and/or ventricular dysfunction. We suggest a protocol for TTE assessment of PH and myocardial function that helps to identify PH patients and their response to pharmacotherapy. The outlined protocol focuses on the detailed assessment of the hypertensive RV; RV-LV crosstalk must be analyzed separately in the evaluation of different pathologies that account for pediatric PH.
... There is a lack of randomized trials in paediatrics, making it difficult to deliver strong guidelines [291,292]. A specific treatment algorithm, similar to that used in adults (Figure 2), has been recommended. ...
Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.
2015 ESC/ERS pulmonary hypertension guidelines incorporate changes and adaptations focusing on clinical management
... There is a lack of randomized trials in paediatrics, making it difficult to deliver strong guidelines. 291,292 A specific treatment algorithm, similar to that used in adults (Figure 2), has been recommended. Determinants of risk and risk stratification have also been suggested. ...
Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk-benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.
... The international TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry shows that, of 362 children with PAH, 57% had idiopathic PAH (IPAH) or familial PAH, a proportion similar to that observed in adult registries [5,11,12]. PAH associated with congenital heart disease (CHD) is relatively more frequent in children (36%) than adults (10-11%) [5], while PAH associated with connective tissue disease and other associated forms are very rare in children compared with the adult population [4,5,13]. ...
Pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting both adults and children. While overall survival has improved in recent years, the need for improved therapeutic approaches remains. Treatments for paediatric PAH have not yet been sufficiently examined, particularly regarding potential toxicities and optimal dosing, and there is a lack of appropriate clinical trial end-points and validated treatment goals that might enable a goal-oriented therapeutic approach. Adult randomised controlled trials in PAH are demonstrating a shift towards more long-term designs, focusing on mortality and morbidity end-points rather than changes in 6-min walking distance. However, such trial designs may not be feasible within the paediatric setting due to challenges such as sufficient recruitment and retention of paediatric patients. Consideration should, therefore, be given towards identifying optimal end-points for the paediatric population, allowing sufficient duration to evaluate efficacy and safety of potential treatments. Herein we consider some of the complexities involved in the management of paediatric PAH, specifically presenting diagnostic challenges as well as reflecting on the lack of evidence currently available to support various therapeutic approaches within the paediatric population.
©ERS 2014.
... Despite the paucity of data, sildenafil appears to be more widely and variably used in pediatrics than other oral treatments. 43 Factors that may explain this include the recognition of the therapeutic potential of sildenafil prior to its approval for PAH, the broad availability partly due to the initial indication of erectile dysfunction, its favorable side-effect profile, and lastly, the ease of dosing for infants and young children in compounded form. Reported uses in pediatrics reflects the spectrum of etiology of PAH in children, such as chronic lung disease, 44 congenital heart disease, 45 and cardiomyopathy. ...
The number of approved therapies for pulmonary hypertension continues to increase. This article reviews the literature related to pediatric use of these therapies from the past 5 years. The evidence-based algorithm for pulmonary hypertension treatment in adults is discussed with respect to children.
Meconium aspiration syndrome (MAS) is a complex respiratory disease that continues to be associated with significant morbidities and mortality. The pathophysiological mechanisms of MAS include airway obstruction, local and systemic inflammation, surfactant inactivation and persistent pulmonary hypertension of the newborn (PPHN). Supplemental oxygen and non-invasive respiratory support are the main therapies for many patients. The management of the patients requiring invasive mechanical ventilation could be challenging because of the combination of atelectasis and air trapping. While studies have explored various ventilatory modalities, evidence to date does not clearly support any singular modality as superior. Patient's pathophysiology, symptom severity, and clinician/unit expertise should guide the respiratory management. Early identification and concomitant management of PPHN is critically important as it contributes significantly to mortality and morbidities.
This chapter deals with frail children suffering from a chronic respiratory disease. In the first part of the chapter, we analyzed especially primitive and secondary disorders that could affect the respiratory system, determining chronic frailty in children. In the second part, we focused on different long-term different treatments in order to give to the reader a comprehensive overview of the management of a child with a chronic respiratory disease.KeywordsLong-term ventilationRespiratory failureOxygenPhysiotherapyTracheostomy
This article describes the use of supplemental oxygen in the acute and chronic situations in children. It goes through the common causes of hypoxia in children and explains some of the pathology of the underlying illness and how oxygen therapy may help this disease process. High flow oxygen is discussed but further methods of delivering oxygenation and ventilation are outside the remit of this article.
Pulmonary hypertension (PH) is a rare, incurable disease. In children, its most common form is the pulmonary arterial hypertension (PAH), caused by congenital heart diseases or occurring as the idiopathic/hereditary type. Pathological alterations are localised in peripheral pulmonary arteries and the mechanism responsible for their initiation is not entirely known. Pulmonary hypertension remains asymptomatic or oligosymptomatic for a long period of time. Its diagnosis and the determination of its aetiology require detailed diagnostically procedures, including: physical examination, transthoracic echocardiography, electrocardiography, chest X-ray, and finally, catheterisation of the "right heart" as a test necessary for the final confirmation of the diagnosis. Expanded diagnostic tests help to establish the causes of PAH. To determine the advancement stage and progress of the disease not only the clinical examination is vital, but also the results of a 6-minute walk test, echocardiography, haemodynamics and biochemical markers. Based on the advancement stage of the disease and the reactivity of pulmonary arteries, an appropriate therapeutic approach involving general guidelines, pharmacotherapy and possible interventional treatment is implemented. Currently applied pharmacotherapy does not allow the patient's complete recuperation. However, due to the early determination of the diagnosis and faster implementation of specific therapy, the quality of life may be markedly improved. This may also decelerate the occurrence of complications, progress of the disease as well as decreased mortality. If pharmacological approach does not yield satisfactory results, palliative invasive treatment in the form of balloon atrioseptostomy or, ultimately, lung or lung and heart transplant should be considered.
