Hypoxic pulmonary vasoconstriction is considered to be the primary cause of pulmonary hypertension and cor pulmonale in adult patients with cystic fibrosis (CF). The acute effect on pulmonary haemodynamics of a single sublingual dose of nifedipine 20 mg has been studied in 9 adult patients with CF.
Right ventricular performance and pulmonary haemodynamics in adolescent and adult patients with cystic fibrosis
✍ Scribed by O. C. Burghuber; U. Salzer-Muhar; H. Bergmann; M. Götz
- Publisher
- Springer
- Year
- 1988
- Tongue
- English
- Weight
- 606 KB
- Volume
- 148
- Category
- Article
- ISSN
- 0340-6997
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✦ Synopsis
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.
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