Key result
Patients with IPAH and a severely reduced DLCO showed a similar response to PH-specific vasodilatory therapy in hemodynamics, cardiac function, and exercise capacity as those with higher DLCO.
Why the study?
Does PH-specific vasodilatory therapy improve hemodynamics and cardiac function in IPAH patients with severely reduced DLCO as effectively as in those with preserved DLCO?
Cohort (n=166)
No
Does PH-specific vasodilatory therapy improve hemodynamics and cardiac function in IPAH patients with severely reduced DLCO as effectively as in those with preserved DLCO?
IPAH patients with severely reduced DLCO respond similarly to PH-specific vasodilatory therapy as those with preserved DLCO, indicating that their known poorer survival is not due to treatment unresponsiveness.
Supports PH-specific therapy consideration in low-DLCO IPAH; leaves open survival determinants.
Patients with idiopathic pulmonary arterial hypertension (IPAH) and a reduced diffusion capacity of the lung for carbon monoxide (DLCO) have a worse survival compared to IPAH patients with a preserved DLCO. Whether this poor survival can be explained by unresponsiveness to pulmonary hypertension (PH)-specific vasodilatory therapy is unknown. Therefore, the aim of this study was to evaluate the hemodynamic and cardiac response to PH-specific vasodilatory therapy in patients with IPAH and a reduced DLCO. Retrospectively, we studied treatment naïve hereditary and IPAH patients diagnosed between January 1990 and May 2015 at the VU University Medical Center. After exclusion of participants without available baseline DLCO measurement or right heart catheterization data and participants carrying a BMPR2 mutation, 166 participants could be included in this study. Subsequently, hemodynamics, cardiac function, exercise capacity, and oxygenation at baseline and after PH-specific vasodilatory therapy were compared between IPAH patients with a preserved DLCO (DLCO >62%), IPAH patients with a moderately reduced DLCO (DLCO 43-62%), and IPAH patients with a severely reduced DLCO (DLCO <43%). Baseline hemodynamics and right ventricular function were not different between groups. Baseline oxygenation was worse in patients with IPAH and a severely reduced DLCO. Hemodynamics and cardiac function improved in all groups after PH-specific vasodilatory therapy without worsening of oxygenation at rest or during exercise. Patients with IPAH and a severely reduced DLCO show a similar response to PH-specific vasodilatory therapy in terms of hemodynamics, cardiac function, and exercise capacity as patients with IPAH and a moderately reduced or preserved DLCO.
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Bruggen et al. (2016) conducted a cohort in Idiopathic pulmonary arterial hypertension (IPAH) (n=166). Severely reduced DLCO (<43%) vs. Moderately reduced (43-62%) or preserved DLCO (>62%) was evaluated on Hemodynamics, cardiac function, exercise capacity, and oxygenation response to PH-specific vasodilatory therapy. Patients with IPAH and a severely reduced DLCO showed a similar response to PH-specific vasodilatory therapy in hemodynamics, cardiac function, and exercise capacity as those with higher DLCO.
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