Outcomes in children with idiopathic pulmonary arterial hypertension

Yung, Delphine; Widlitz, Allison; Rosenzweig, E.B.; Kerstein, Diane; Maislin, Greg; Barst, RJ

doi:10.1016/j.accreview.2004.10.088

Cited by 60 publications

(109 citation statements)

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“…Longer term follow-up in 35 patients treated with i.v. epoprostenol revealed a 10-yr survival rate (including transplantation as a censoring event) of 61%, whilst 10-yr treatment success rate (defined as freedom from death, transplantation or atrial septostomy) was 37% for this group [16]. Maintenance doses of i.v.…”

Section: Intravenous Prostanoidsmentioning

confidence: 99%

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Paediatric pulmonary hypertension: monitoring progress and identifying unmet needs

Beghetti¹

2009

Eur Respir Rev

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Recent advances in the field of pulmonary hypertension (PH) have provided clinicians with a range of treatment options, but effective disease management in children presents a unique challenge. The present article will discuss the steps being taken to address unmet needs in paediatric PH.Understanding the epidemiology of paediatric PH is essential to guide management decisions, but such epidemiological data are scarce. The first international paediatric PH registry, Tracking Outcomes in Paediatric Pulmonary Hypertension (TOPP), promises to become a vital resource.Studies of PH therapies are rare in children, and treatment of paediatric PH is generally guided by the adult treatment algorithm, with some adaptations. However, invasive management options, such as continuous prostacyclin infusion, even if effective, are challenging in children, and further research is required to develop appropriate treatment strategies, formulations and doses for paediatric PH. Measures of treatment success must also be defined, and the applicability of endpoints from adult clinical studies remains an open question.In summary, further epidemiological and treatment data are needed for paediatric pulmonary hypertension. The international TOPP registry will provide a valuable insight, but this must be complemented by research and development of adapted paediatric therapies. Dedicated childhood pulmonary hypertension services would optimise the diagnosis and management of this life-threatening disease.

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Section: Intravenous Prostanoidsmentioning

confidence: 99%

“…Longer term follow-up revealed a 10-yr survival rate of 81% among the responders. However, careful follow-up is essential as some acute responders may become nonresponders with time and require additional treatment [16].…”

Section: Calcium Channel Blockersmentioning

confidence: 99%

Paediatric pulmonary hypertension: monitoring progress and identifying unmet needs

Beghetti¹

2009

Eur Respir Rev

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“…The long-term persistence of these beneficial effects, as reported by YUNG et al [33], who showed a 10-yr survival of 61% in IPAH patients, indicates that the use of epoprostenol in children is justified despite the invasive nature of its delivery system and its risk for associated complications, such as thromboembolic events, line sepsis, local infection or catheter displacement. In patients with PAH-CHD and right-to-left shunting, the additional risks of such complications, which also include cerebral emboli or abscesses, must be weighed against the expected therapeutic benefits on an individual basis.…”

Section: Epoprostenolmentioning

confidence: 65%

Treatment options for paediatric pulmonary arterial hypertension

Berger¹,

Bonnet²

2010

Eur Respir Rev

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Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult and paediatric PAH can, however, be distinguished based on underlying pathology and the presence of age-specific conditions, some of which are related to poor lung development in children. Improved knowledge of vascular biology has led to the development of several PAH-specific therapies, which have demonstrated clinical benefits in adults, including improved exercise capacity and prolonged survival. Treatment data in paediatric PAH are scarce. Although limited, the existing data indicate that current treatments for paediatric PAH are well tolerated and effective, at least in the short-and medium-term. Nevertheless, the current guidelines for clinicians, which recommend use of the adult treatment algorithm in paediatric patients, appear justified when judged according to the available evidence. However, further randomised, controlled trials are necessary to increase the evidence base for treatment of paediatric PAH, especially in relation to age-specific conditions. At present, early initiation of treatment and combination pharmacological therapy may offer the most promising courses of action to improve outcomes in paediatric PAH.

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“…(41) Children who were initially "acute responders", may become "non-responders" over time. (42) Improved survival has been observed in both adults and children who responded to an acute vasodilator challenge and were treated with nifedipine in addition to conventional anti-failure treatment with digoxin, diuretics, and supplemental oxygen. (43) Dihydropyridine calcium channel blockers, such as nifedipine and amlodipine that act on vascular smooth muscle are preferred.…”

Section: Calcium Channel Blockersmentioning

confidence: 99%