Heart rate variability predicts outcome in children with pulmonary arterial hypertension

Lammers, Astrid E.; Munnery, Elizabeth; Hislop, Alison A.; Haworth, Sheila G.

doi:10.1016/j.ijcard.2008.12.087

Cited by 49 publications

(34 citation statements)

References 30 publications

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“…In children, heart rate and heart rate variability at time of diagnosis have been shown to predict survival [17]. Our study confirms the predictive value of heart rate but a significant treatment-induced change could not be demonstrated, arguing against a role in defining treatment goals.…”

Section: Echocardiographysupporting

confidence: 68%

Identification of treatment goals in paediatric pulmonary arterial hypertension

et al. 2014

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To be able to design goal-oriented treatment strategies in paediatric pulmonary arterial hypertension (PAH), we aimed to identify treatment goals by investigating the prognostic value of treatment-induced changes in noninvasive predictors of transplant-free survival.66 consecutive, treatment-naïve paediatric PAH patients in the Dutch National Network for Paediatric Pulmonary Hypertension who started taking PAH-targeted drugs between January 2000 and April 2013 underwent prospective, standardised follow-up. Clinical, biochemical and echocardiographic measures were longitudinally collected at treatment initiation and follow-up, and their respective predictive values for transplant-free survival were assessed. Furthermore, the predictive values of treatment-induced changes were assessed.From the identified set of baseline predictors, the variables World Health Organization functional class (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP) and tricuspid annular plane systolic excursion (TAPSE) were identified as follow-up predictors in which treatment-induced changes were associated with survival. Patients in whom these variables improved after treatment showed better survival (p<0.002).Therefore, WHO-FC, NT-proBNP and TAPSE are not only predictors of transplant-free survival in paediatric PAH but can also be used as treatment goals, as treatment-induced improvements in these variables are associated with improved survival. The identification of these variables allows for the introduction of goal-oriented treatment strategies in paediatric PAH.

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Section: Echocardiographysupporting

confidence: 68%

Identification of treatment goals in paediatric pulmonary arterial hypertension

et al. 2014

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“…HR = hazard ratio; CI = 95% confidence interval; APAH = associated pulmonary arterial hypertension; IPAH = idiopathic pulmonary arterial hypertension; WHO-FC = WHO functional class; (NT-pro)BNP = (N-terminal-pro) brain natriuretic peptide; mRAP = mean right atrial pressure; mPAP = mean pulmonary arterial pressure; PVRi = (indexed) pulmonary vascular resistance; WU = wood units. patient inclusion [26,27]. Combining the remaining 4 non-overlapping cohorts representing 254 patients yielded a HR (CI) of 1.18 (0.99-1.40) per mm Hg increase (Fig.…”

Section: Identified Candidate Prognostic Factorsmentioning

confidence: 97%

“…4). An additional 2 studies were omitted to prevent duplicate patient inclusion [26,27]. Combining the remaining 3 non-overlapping Notes to Table 2: '✓' = significant association with survival; '✗' = no significant association with survival; shaded indicates that sufficient survival analysis results were provided in the paper to be included in meta-analysis; HR = hazard ratio; WHO-FC = WHO functional class; 6MWT = 6-minute walk test; RR = blood pressure; BSA = body surface area; VO2 = oxygen consumption; VE/VCO2 = ventilatory-efficiency slope; BMPR2 = bone morphogenetic protein receptor type II; (NT-pro)BNP = (N-terminal pro) brain natriuretic peptide; Hb = hemoglobin; Apo-A1 = apolipoprotein-A-1; TIMP-1 = metallopeptidase-inhibitor-1; sST2 = soluble ST2; mRAP = mean right atrial pressure; mPAP = mean pulmonary artery pressure; mPAP/mSAP = pulmonary-to-systemic arterial pressure ratio; PVRi = indexed pulmonary vascular resistance; Qp(i) = pulmonary blood flow (index); SvO2 = mixed venous oxygen saturation; PAC(i) = pulmonary arterial capacitance (index); PVR/SVR = pulmonary-to-systemic vascular resistance ratio; VRT = vasoreactivity testing; PFR = pulmonary flow reserve; PSVi = pulmonary stroke volume index; CMR = cardiac magnetic resonance imaging; CT = computed tomography.…”

Section: Identified Candidate Prognostic Factorsmentioning

confidence: 99%

Prognostic factors in pediatric pulmonary arterial hypertension: A systematic review and meta-analysis

Ploegstra

Zijlstra

Douwes

et al. 2015

International Journal of Cardiology

101

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“…Specific haemodynamic variables, such as systemic cardiac index ,2.5 L?min -1 ?m -2 , mean pulmonary arterial pressure/mean systemic arterial pressure .0.75 mmHg, right atrial pressure .10 mmHg or PVR index .20 Wood Unit?m -2 , are associated with worse prognosis in paediatric patients [18]. There are conflicting reports regarding the predictive value of the 6-min walk test in children with PAH [7,16,19,20]. Emerging evidence indicates that the serum level of B-type natriuretic peptide or N-terminal pro-brain natriuretic peptide (NT-proBNP) is associated with exercise capacity [21][22][23] and predicts prognosis [24].…”

Section: Prognostic Factors In Paediatric Pah Patientsmentioning

confidence: 99%

The challenges in paediatric pulmonary arterial hypertension

Beghetti

Berger

2014

Eur Respir Rev

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Pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting both adults and children. While overall survival has improved in recent years, the need for improved therapeutic approaches remains. Treatments for paediatric PAH have not yet been sufficiently examined, particularly regarding potential toxicities and optimal dosing, and there is a lack of appropriate clinical trial end-points and validated treatment goals that might enable a goal-oriented therapeutic approach. Adult randomised controlled trials in PAH are demonstrating a shift towards more long-term designs, focusing on mortality and morbidity end-points rather than changes in 6-min walking distance. However, such trial designs may not be feasible within the paediatric setting due to challenges such as sufficient recruitment and retention of paediatric patients. Consideration should, therefore, be given towards identifying optimal end-points for the paediatric population, allowing sufficient duration to evaluate efficacy and safety of potential treatments.Herein we consider some of the complexities involved in the management of paediatric PAH, specifically presenting diagnostic challenges as well as reflecting on the lack of evidence currently available to support various therapeutic approaches within the paediatric population. @ERSpublications Paediatric pulmonary arterial hypertension: towards consistency in management and improved patient outcomes http://ow.ly/Cydpv

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Heart rate variability predicts outcome in children with pulmonary arterial hypertension

Cited by 49 publications

References 30 publications

Identification of treatment goals in paediatric pulmonary arterial hypertension

Identification of treatment goals in paediatric pulmonary arterial hypertension

Prognostic factors in pediatric pulmonary arterial hypertension: A systematic review and meta-analysis

The challenges in paediatric pulmonary arterial hypertension

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