Key result
Precapillary PH linked to ~34% lower diaphragm velocity, but RV function drives exercise intolerance.
Why the study?
Determinants of exercise intolerance in precapillary pulmonary hypertension, including diaphragm dysfunction, have rarely been evaluated in a multimodal manner.
Does diaphragm dysfunction independently predict exercise intolerance in patients with precapillary pulmonary hypertension?
Case-Control (n=86)
No
Does diaphragm dysfunction independently predict exercise intolerance in patients with precapillary pulmonary hypertension?
Absolute Event Rate: 4.5% vs 6.8%
p-value: p=<0.01
Diaphragm dysfunction is prevalent in precapillary pulmonary hypertension but does not independently predict exercise intolerance when accounting for right ventricular systolic dysfunction.
Diaphragm dysfunction does not independently predict exercise intolerance in precapillary PH; leaves open its role beyond RV systolic dysfunction.
Background: Several determinants of exercise intolerance in patients with precapillary pulmonary hypertension (PH) due to pulmonary arterial hypertension and/or chronic thromboembolic PH (CTEPH) have been suggested, including diaphragm dysfunction. However, these have rarely been evaluated in a multimodal manner. Methods: Forty-three patients with PH (age 58 ± 17 years, 30% male) and 43 age- and gender-matched controls (age 54 ± 13 years, 30% male) underwent diaphragm function (excursion and thickening) assessment by ultrasound, standard spirometry, arterial blood gas analysis, echocardiographic assessment of pulmonary artery pressure (PAP), assay of amino-terminal pro-brain natriuretic peptide (NT-proBNP) levels, and cardiac magnetic resonance (CMR) imaging to evaluate right ventricular systolic ejection fraction (RVEF). Exercise capacity was determined using the 6-min walk distance (6MWD). Results: Excursion velocity during a sniff maneuver (SniffV, 4.5 ± 1.7 vs. 6.8 ± 2.3 cm/s, P<0.01) and diaphragm thickening ratio (DTR, 1.7 ± 0.5 vs. 2.8 ± 0.8, P<0.01) were significantly lower in PH patients versus controls. PH patients with worse exercise tolerance (6MWD <377 vs. ≥377 m) were characterized by worse SniffV, worse DTR, and higher NT-pro-BNP levels as well as by lower arterial carbon dioxide levels and RVEF, which were all univariate predictors of exercise limitation. On multivariate analysis, the only independent predictors of exercise limitation were RVEF (r = 0.47, P=0.001) and NT-proBNP (r = −0.27, P=0.047). Conclusion: Patients with PH showed diaphragm dysfunction, especially as exercise intolerance progressed. However, diaphragm dysfunction does not independently contribute to exercise intolerance, beyond what can be explained from right heart failure.
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Spießhoefer et al. (2019) conducted a case-control in Precapillary pulmonary hypertension (n=86). Precapillary pulmonary hypertension vs. Healthy age- and gender-matched volunteers was evaluated on Excursion velocity during a sniff maneuver (SniffV, cm/s) (p=<0.01). Patients with precapillary pulmonary hypertension had significantly lower diaphragm excursion velocity during a sniff maneuver than controls (4.5 vs 6.8 cm/s, p<0.01), but right ventricular function, not diaphragm metrics, independently predicted exercise intolerance.
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