European respiratory review : an official journal of the European Respiratory Society
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Review Comparative Study
Issues related to the management and therapy of paediatric pulmonary hypertension.
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 far from paediatric registries, national cohorts and clinical trials and discuss the best approach for developing a treatment algorithm designed for children with different types of PH. The many unanswered questions, challenges and issues relating to the PH in the paediatric population will also be discussed.
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Pulmonary arterial hypertension (PAH) is a life-threatening complication of systemic sclerosis (SSc). However, PAH-specific treatments are available and can significantly improve survival of patients, especially those diagnosed in World Health Organization (WHO) functional class (FC) II. Registry data have shown that without screening, more than two-thirds of PAH-SSc patients are in WHO FC III or IV when diagnosed. ⋯ Data from bosentan trials have shown that nearly one-quarter of patients in WHO FC III at baseline can attain WHO FC II status with monotherapy. Maintenance of PAH-SSc patients in WHO FC II with monotherapy is unrealistic, and sequential goal-directed combination therapy is now becoming an accepted treatment strategy. It is hoped that earlier diagnosis, coupled both with regular assessments to ensure treatment goals are being met and timely, appropriate treatment, will further improve the survival rates of those PAH-SSc patients.