Aims This study investigated the relationship between right ventricular (RV) structure and function and survival in idiopathic pulmonary arterial hypertension (IPAH). Methods and resultsIn 64 patients, cardiac magnetic resonance, right heart catheterization, and the six-minute walk test (6MWT) were performed at baseline and after 1-year follow-up. RV structure and function were analysed as predictors of mortality. During a mean follow-up of 32 months, 19 patients died. A low stroke volume (SV), RV dilatation, and impaired left ventricular (LV) filling independently predicted mortality. In addition, a further decrease in SV, progressive RV dilatation, and further decrease in LV end-diastolic volume (LVEDV) at 1-year follow-up were the strongest predictors of mortality. According to Kaplan-Meier survival curves, survival was lower in patients with an inframedian SV index 25 mL/m 2 , a supramedian RV end-diastolic volume index !84 mL/m 2 , and an inframedian LVEDV 40 mL/m 2 . Conclusions The RV contains prognostic information in IPAH. A large RV volume, low SV, and a reduced LV volume are strong independent predictors of mortality and treatment failure.

Although in controls, RCA flow is similar in systole and diastole, in PH there is systolic flow impediment, which is proportional to RV pressure and mass. In patients with severe RV hypertrophy total mean flow is reduced.

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The aim of the current study was to investigate whether alterations in N-terminal pro brain natriuretic peptide (NT-proBNP) reflect changes in right ventricular structure and function in pulmonary hypertension patients during treatment.The study consisted of 30 pulmonary hypertension patients; 15 newly diagnosed and 15 on long-term treatment. NT-proBNP, right heart catheterisation and cardiac magnetic resonance imaging measurements were performed, at baseline and follow-up.There were no significant differences between newly diagnosed patients and those on treatment at baseline or follow-up with respect to NT-proBNP, haemodynamics and right ventricular parameters. Relative changes in NT-proBNP during treatment were correlated to the relative changes in right ventricular end-diastolic volume index (r50.59), right ventricular mass index (r50.62) and right ventricular ejection fraction (r5 -0.81).N-terminal pro brain natriuretic peptide measurements reflect changes in magnetic resonance imaging-measured right ventricular structure and function in pulmonary hypertension patients. An increase in N-terminal pro brain natriuretic peptide over time reflects right ventricular dilatation concomitant to hypertrophy and deterioration of systolic function.KEYWORDS: Magnetic resonance imaging, N-terminal pro brain natriuretic peptide, pulmonary hypertension, right ventricle P ulmonary hypertension (PH) is a disease characterised by increased pulmonary vascular resistance leading to chronic right ventricular (RV) pressure overload. Without treatment, patients have a poor prognosis and die of right heart failure. Right atrial pressure, measured by right heart catheterisation, has been shown to be an important prognostic parameter in pulmonary arterial hypertension (PAH) [1]. Recently, the search for a less invasive prognostic marker was successful with the detection of the cardiac hormone brain natriuretic peptide (BNP) and its biologically inactive alternative N-terminal pro brain natriuretic peptide (NT-proBNP). Research performed in patients with a diseased left ventricle, i.e. ischemia, pressure or volume overload, showed the clinical and prognostic value of this hormone [2][3][4][5]. NAGAYA et al. [6] showed in idiopathic PAH patients that a high BNP level at baseline and a further increase in BNP at follow-up was associated with a poor prognosis. In patients with increased RV load it has been shown that BNP is related to: functional status, initial haemodynamics, RV systolic function and structure [7][8][9]. In PAH patients, BNP parallels alterations in haemodynamics and functional status during treatment [10]. Although these findings emphasise the clinical significance of the change in BNP after long-term therapy in PAH patients, insight into changes in RV structure and systolic function in relation to BNP are lacking. Furthermore, in previous reports BNP was measured, however, it is unclear whether measurements of NT-proBNP show similar relations. Therefore, the aim of the current study was to assess the tempo...

In pulmonary arterial hypertension (PAH) an increased pulmonary vascular resistance results in chronic pressure overload on the right ventricle and induces pathological right ventricular (RV) remodelling and RV failure. This causes limited exercise capacity, fatigue and increased mortality. Several treatment options have become available for PAH, and in patients given monotherapy, a second drug of a different class is given to improve symptoms and exercise capacity. Whether the addition of a second treatment reverses RV remodelling has not been described. In this study, the phosphodiesterase 5 inhibitor sildenafil was added to treatment with bosentan, an endothelin receptor antagonist. The objective of this study was to investigate whether the addition of sildenafil reverses RV remodelling and further improves RV function in patients with PAH treated with bosentan. METHODSIn 15 patients with PAH receiving bosentan for one year, sildenafil was added for three months. Sildenafil was started at 50 mg twice daily and increased to 50 mg thrice daily after four weeks. At the start of the study and again after one year of bosentan, right-heart catheterisation with vasoreactivity testing, cardiac magnetic resonance (CMR) and 6 min walk test (6MWT) were performed. N-terminal pro-brain natriuretic peptide (NT-proBNP) was determined after one year of bosentan and after three months of combination therapy. After three months of combination therapy, the effects of the addition of sildenafil were evaluated with CMR, 6MWT and NT-proBNP. CMR was performed according to a previously described protocol and analysed by one observer blinded to clinical aspects of the study.1 The 6MWT was performed according to American Thoracic Society guidelines.2 Blood was analysed for NT-proBNP plasma concentrations (ECLIA, Roche Inc, Mannheim, Germany). Blood was sampled with the patients at rest within 24 h of CMR measurements. The institutional ethics review committee approved the study, and all patients gave informed consent.Data are presented as mean (SD). Changes in parameters were analysed with analysis of variance for multiple assessment. Changes in NT-proBNP were analysed with the Wilcoxon signed rank test. Correlation coefficients were determined by linear regression analysis. RESULTSOf the 15 patients, 12 were women, and the age was 45 (15) years. Causes of PAH were idiopathic PAH (nine patients), HIV (one patient), collagen vascular disease (three patients) and congenital systemic to pulmonary shunt (two patients). Thirteen patients were in New York Heart Association functional class III and two patients were in class II. Baseline pulmonary artery pressure was 54 (13) mm Hg and pulmonary vascular resistance was 866 (450) dyn?s?cm 25 . Side effects after addition of sildenafil were headache (one patient), heartburn (one patient) and epistaxis (one patient). None of the 15 patients had documented liver function abnormalities or changes in haematological or biochemical measurements. Table 1 summarises cardiac parameters assessed by CMR. ...