Assessing Pulmonary Hypertensive Vascular Disease in Childhood. Data from the Spanish Registry

Marín, M.J. del Cerro; Sabaté-Rotés, Anna; Ogando, A. Rodríguez; Soto, Alberto Mendoza; Jiménez, M. Quero; Camacho, J. L.; Sonnenfeld, I. Raposo; Bonora, Adriana; Brotons, Dimpna Albert; Moreno‐Galdó, Antonio

doi:10.1164/rccm.201406-1052oc

Cited by 103 publications

(70 citation statements)

References 41 publications

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“…Although the true incidence and prevalence of PH in the pediatric population remain uncertain, recent epidemiologic data from national or international registries estimated an annual incidence of 64 cases per million children. 11–15 The incidence of idiopathic pulmonary arterial hypertension (IPAH) was 0.7 cases per million, which is slightly less than adult reports, and PAH associated with CHD was 2.2 cases per million. Past reports from pediatric registries have primarily emphasized that IPAH, heritable PAH (HPAH), and PAH associated with CHD constitute the majority of cases (WHO Group 1 disease).…”

Section: Insights Into Pediatric Pvd Gained From International Registmentioning

confidence: 98%

“…This study further showed that independent risk factors for mortality included etiology, age at diagnosis younger than 2 years, advanced functional class, and high right atrial pressure as assessed by right heart catheterization. 15 Although limited by its retrospective nature, this report provides a more accurate landscape that better reflects diverse causes of PH commonly seen in pediatric practice. However, as with data from the other registries, there is likely some selection bias of referral patients, as the number of newborns, infants, and children with BPD, DLD, and CF are likely under-represented.…”

Section: Insights Into Pediatric Pvd Gained From International Registmentioning

confidence: 99%

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Addressing the challenges of phenotyping pediatric pulmonary vascular disease

Goss¹,

Mourani

Baker

2017

Pulm. circ.

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Pediatric pulmonary vascular disease (PVD) and pulmonary hypertension (PH) represent phenotypically and pathophysiologically diverse disease categories, contributing substantial morbidity and mortality to a complex array of pediatric conditions. Here, we review the multifactorial nature of pediatric PVD, with an emphasis on improved recognition, phenotyping, and endotyping strategies for pediatric PH. Novel tailored approaches to diagnosis and treatment in pediatric PVD, as well as the implications for long-term outcomes, are highlighted.

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Section: Insights Into Pediatric Pvd Gained From International Registmentioning

confidence: 98%

Section: Insights Into Pediatric Pvd Gained From International Registmentioning

confidence: 99%

Addressing the challenges of phenotyping pediatric pulmonary vascular disease

Goss¹,

Mourani

Baker

2017

Pulm. circ.

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“…1 In our study, only around 33% of the CCB users had a record for right heart catheterization. The reported proportion of children with idiopathic PAH who received CCBs as monotherapy (and were acute responders) has been quite variable: 24% in a Dutch cohort, 23 11% in the French registry, 12 6% in the Spanish registry, 24 and 9% in the UK registry. 13 In the Reveal registry, 25 20% (43/211) of all PAH patients received CCBs as monotherapy (21/211) or in combination (22/211), and 42 of 43 patients had undergone acute vasoreactivity testing.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population

Jick

Breitenstein

et al. 2017

Pulm. circ.

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Pulmonary arterial hypertension (PAH) is rare in children and few data are available in a pediatric general population. This study aims to calculate the annual incidence and prevalence of PAH and to describe these children in a large US population of patients aged under 18 years. Using the US MarketScan claims database we identified 695 children with PAH in 2010–2013. We calculated annual incidence rates and prevalence overall, by age and PAH type (idiopathic and non-idiopathic) using Byar’s method. We also described characteristics, co-morbidities, treatment patterns, and diagnostic procedures for these children. In 2010–2013, the annual incidence rates of PAH per 1,000,000 children-years was in the range of 4.8–8.1; 0.5–0.9 for idiopathic PAH and 4.3–7.3 for non-idiopathic PAH. The annual prevalence of PAH was in the range of 25.7–32.6 per 1,000,000 children; 4.4–6.0 for idiopathic PAH and 21.3–27.0 for non-idiopathic PAH. Incidence rates and prevalence were highest in children under age 2 years. Around 36% of affected children were born prematurely. Most (75%) had some type of congenital heart defect and 13% had Down’s syndrome. Most patients received PAH monotherapy (83%), while 13% received dual therapy. Phosphodiesterase type 5 inhibitors were the most commonly used treatments. Around 92% had at least one echocardiogram and 37% a right heart catheterization. PAH is very rare in children especially in the absence of etiological factors such as congenital heart defects. A large proportion of diagnoses in children seem to be based on echocardiography rather than right heart catheterization.

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“…Although the true incidence and prevalence of PH in the pediatric population remain uncertain, recent epidemiologic data from national or international registries estimated an annual incidence of 64 cases per million children. (17–21) The incidence of idiopathic PAH was 0.7 cases per million and PAH associated with CHD was 2.2 cases per million. Past reports from pediatric registries have primarily emphasized that idiopathic pulmonary arterial hypertension (IPAH), heritable PAH (HPAH), and PAH associated with congenital heart disease (CHD) constitute the majority of cases (e.g., WHO Group 1 disease).…”

Section: Definition and Classification Of Pediatric Phmentioning

confidence: 99%

“…More recently, del Cerro and coworkers published data from the Spanish PH Registry, which has provided more extensive information on disease incidence, prevalence and the impact of functional class, WHO Group and other factors on survival (21). The Spanish Registry included many forms of PH and thereby provides a more accurate picture of the nature of PH-related disorders in children than past studies.…”

Section: Definition and Classification Of Pediatric Phmentioning

confidence: 99%

New guidelines for managing pulmonary hypertension: what the pediatrician needs to know

Abman

2016

Current Opinion in Pediatrics

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Purpose of the Review Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension. Recent Findings To address this issue, a group of clinical experts from several subspecialties, including pulmonology, cardiology, neonatology and others, were selected to form a task force to tackle this topic with support from the American Heart Association and American Thoracic Society. A joint guidelines paper presenting their findings was recently published. Summary This review highlights a few key topics underlying guidelines for the care of children with pulmonary hypertension that are especially important for the practicing pediatrician and others, and presents some of the major recommendations from the published guidelines report. Overall, the author emphasizes that these guidelines are based on the best current evidence and clinical experience of experts in the field, yet much more clinical research is needed to improve long term outcomes in pediatric pulmonary hypertension.

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Assessing Pulmonary Hypertensive Vascular Disease in Childhood. Data from the Spanish Registry

Cited by 103 publications

References 41 publications

Addressing the challenges of phenotyping pediatric pulmonary vascular disease

Addressing the challenges of phenotyping pediatric pulmonary vascular disease

Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population

New guidelines for managing pulmonary hypertension: what the pediatrician needs to know

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