Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006

Haworth, Sheila G.; Hislop, Alison A.

doi:10.1136/hrt.2008.150086

Cited by 266 publications

(237 citation statements)

References 33 publications

(27 reference statements)

Supporting

Mentioning

224

Contrasting

Unclassified

Order By: Relevance

“…52 For every 1-mm decrease in TAPSE, the risk of death increases by 17% for adult PAH patients. 51 A significant amount of PH in childhood is CHD associated, 53 be it likely causally linked (large systemic left-to-right shunt) or out of proportion (e.g., severe PH in the setting of a small secundum atrial septal defect or left heart lesions). With sustained PH over years in children and young adults, TAPSE values decrease, progressively diverging from agematched control values.…”

Section: Global and Regional Functional Assessment Of The Rv Rv Longimentioning

confidence: 99%

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

et al. 2016

View full text Add to dashboard Cite

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.

show abstract

Section: Global and Regional Functional Assessment Of The Rv Rv Longimentioning

confidence: 99%

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

et al. 2016

View full text Add to dashboard Cite

show abstract

“…6 The hemodynamic presented by this group is very similar to idiopathic PAH and thus prognosis seems to be poor based on recent data. 13 This underscores the importance of accurate decision for operability and survival could even be better with an open shunt and ES than with a closed shunt and right ventricular failure. 31 EISENMENGER SYNDROME MANAGEMENT ES represents the most advanced form of PAH associated with CHD.…”

Section: Non-operable Preeisenmengermentioning

confidence: 99%

“…Almost one quarter of these children died (11/47). 13 Children with ES had a greater cumulative survival time by 1.3 years indicating that surgical repair is not necessarily always the best option.…”

Section: Abbreviationsmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Hypertension in Congenital Shunts

Beghetti¹,

Tissot

2010

Revista Española de Cardiología (English Edition)

View full text Add to dashboard Cite

“…4,5 While a large proportion of iPAH patients present with, or rapidly develop, symptomatic heart failure, 5,6 PS patients are often asymptomatic or present with only mild exercise intolerance. 4 Moreover, mortality vastly differs between the groupshigh in iPAH and very low in PS.…”

Section: Introductionmentioning

confidence: 99%

Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

et al. 2017

View full text Add to dashboard Cite

Background Right ventricular (RV) pressure overload in the context of pulmonary stenosis (PS) has a much better prognosis than in the context of idiopathic pulmonary arterial hypertension (iPAH), which may be related to differences in global and regional RV remodeling and systolic function. We compared RV mechanics in children with PS to those with iPAH, aiming to identify mechanisms and markers of RV dysfunction. Methods Eighteen controls, 18 iPAH and 16 PS patients were retrospectively studied. Age, BSA, and sex distribution were comparable. Two‐dimensional echocardiography, blood flow and tissue Doppler, and longitudinal RV deformation were analyzed. ANCOVA—including RV systolic pressure (RVSP) and length as covariates—was used to compare patient groups. Results RV systolic pressure was higher in iPAH vs PS (96.8±25.4 vs 75.4±18.9 mm Hg, P=.011). Compared to controls, PS patients showed mild dilation (P<.01) and decreased longitudinal deformation (P<.001) at the RV apex. Compared to both PS and controls, iPAH patients showed marked spherical RV dilation (P<.001), reduced global, RV free wall and septal longitudinal deformation (iPAH −22.07%±4.35% vs controls −28.18%±1.69%; −9.98%±4.30% vs −17.45%±2.52%; P<.001) and RV postsystolic shortening (P<.001). RV transverse shortening (radial performance) was increased in PS (31.75%±10.35%; P<.001) but reduced in iPAH (−1.62%±11.11% vs controls 12.00%±7.74%; P<.001). Conclusion Children with iPAH demonstrate adverse global and regional RV remodeling and mechanics compared to those with PS. Mechanisms of RV systolic dysfunction in iPAH include decreased longitudinal deformation, decreased or absent transverse shortening, and postsystolic shortening. These markers may be useful to identify children at risk of RV failure.

show abstract

Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006

Abstract: New pulmonary hypertension-specific medicines have improved survival in children as in adults. Outcome in this series compares favourably with international outcome data.

Cited by 266 publications

References 33 publications

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

Pulmonary Hypertension in Congenital Shunts

Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

Contact Info

Product

Resources

About