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 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.
Highlights
Pulmonary hypertension (PH) is a chronic pathology with a progressive nature that, without treatment, can cause various complications, such as right-sided heart failure and death [1]
Five groups of conditions have been classified as leading to PH: (1) pulmonary arterial hypertension (PAH); (2) a reaction to left-sided heart disease; (3) illnesses that emerge from either lung diseases or hypoxia; (4) chronic thromboembolism and (5) a grouping for unclear, unknown, multifactorial mechanisms [5]
The guidelines of the European Society of Cardiology (ESC) [1] for PH recommend Sildenafil therapy in children, for those aged 1–17 years old. These recommendations state that once diagnosis is confirmed of Group 1 PH (PAH), those children that fit the criteria for World Health Organisation
Summary
Pulmonary hypertension (PH) is a chronic pathology with a progressive nature that, without treatment, can cause various complications, such as right-sided heart failure and death [1]. Often screened by means of echocardiography, a mean pulmonary arterial pressure over 25 mm Hg at rest, observed at the catheterization of the right heart, defines this condition [2]. Five groups of conditions have been classified as leading to PH: (1) pulmonary arterial hypertension (PAH); (2) a reaction to left-sided heart disease; (3) illnesses that emerge from either lung diseases or hypoxia; (4) chronic thromboembolism and (5) a grouping for unclear, unknown, multifactorial mechanisms [5]. The natural evolution of the condition leads to right-sided heart dysfunction, failure and death [6]. Recent studies have shown promising results for the use of phosphodiesterase type 5 inhibitors (PDE5 inhibitors), of which Sildenafil has emerged as a top potential therapy
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