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• W616192470 abstract "Pulmonary hypertension (PHT) is a progressive and ultimately fatal disease. Thecondition is poorly understood in children. This thesis evaluated non-invasiveassessment techniques, experience with epoprostenol, atrial septostomy and lungtransplantation in children. As therapy is guided by patients’ functional capacity,studies focused on 6-minute walk test distance (6MWTd) and cardiopulmonaryexercise testing (CPET). Age-related normal values of 6MWTd were determined,and the relationship between 6MWTd and CPET was assessed. Echocardiographicmarkers of impaired myocardial function were investigated using tissue Doppler(TDI) and potential risk-factors of mortality were sought using heart rate variability(HRV) and B-natriuretic peptide (BNP).Parameters of CPET correlated with 6MWTd in highly compromised children but inless impaired children the 6MWT represented a sub-maximal test, indicating that aCPET is required when 6MWTd exceeds 300m. BNP and HRV were related toprognosis but sensitivity and specificity are limited. TDI demonstrated thatbiventricular function is impaired in children with PHT and that left ventricularimpairment cannot be evaluated adequately using conventional echocardiographictechniques. Reviewing our experience with intravenous epoprostenol, it improvedfunctional capacity, survival and could be used safely in children with acceptablemorbidity. Atrial septostomy abolished syncope and improved right ventricularfunction, at the expense of mild systemic arterial oxygen desaturation. Septostomies,with and without a fenestrated device, can close spontaneously with time, stressingthe need for a more reliable device. When medical therapies were exhausted,transplantation dramatically improved quality of life and survival. Listing criteriawere shown to have been appropriate but more objective criteria are needed. Thesestudies evaluating assessment techniques, prognosticators, and therapies have givenfurther insight into the management of children with PHT. Future work shouldinclude assessment of non-invasive magnetic resonance imaging, exploring acombined prognosticator, combination therapy and improving classification ofclinical status to optimise therapy and facilitate trials of new, urgently neededmedicines." @default.
• W616192470 created "2016-06-24" @default.
• W616192470 creator A5054893611 @default.
• W616192470 date "2011-09-28" @default.
• W616192470 modified "2023-09-28" @default.
• W616192470 title "Non-invasive evaluation, therapy and transplantation in children with pulmonary arterial hypertension" @default.
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