Right ventricular performance and pulmonary haemodynamics in adolescent and adult patients with cystic fibrosis.
A combined haemodynamic and radionuclide approach was used to evaluate right ventricular performance in 16 adolescent and adult patients with cystic fibrosis ( CF). There were nine patients with mild arterial hypoxaemia (PaO2 greater than 80% of predicted) and normal resting pulmonary artery pressure and seven patients with severe arterial hypoxaemia (PaO2 less than 70% of predicted) and resting pulmonary arterial hypertension (PH). The right ventricular ejection fraction (RVEF) by equilibrium angiocardiography using krypton 81m as a tracer and stroke volume index (SVI) by thermodilution techniques were measured simultaneously and right ventricular end-diastolic and end-systolic volumes were derived. RVEF was normal in CF patients without PH (58.9 +/- 7.2%) but was reduced in those with PH (45.4 +/- 2.6%). There was a statistically significant inverse linear correlation between RVEF and afterload as assessed by mean pulmonary artery pressure (Pap: r = -0.76) and pulmonary vascular resistance ( PVR: r = -0.78), indicating that RVEF ist afterload-dependent. Right ventricular function, however, as assessed by right ventricular end-systolic pressure-volume relations was even higher in CF patients with PH, indicating preserved or even increased right ventricular function in the face of an increased afterload stress.[1]References
- Right ventricular performance and pulmonary haemodynamics in adolescent and adult patients with cystic fibrosis. Burghuber, O.C., Salzer-Muhar, U., Bergmann, H., Götz, M. Eur. J. Pediatr. (1988) [Pubmed]
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