Sequential treatment with riociguat and balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension

Abstract

Riociguat is the treatment of choice for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). We addressed here whether additional balloon pulmonary angioplasty (BPA) provides further benefits. A prospective series of 36 consecutive patients with inoperable CTEPH were treated with riociguat at least three months before BPA. All patients underwent diagnostic workup at baseline, before BPA treatments, and six months after final intervention. The main outcome measures were pulmonary hemodynamic parameters and World Health Organization (WHO) functional class (FC). Significant improvements in pulmonary hemodynamics and physical capacity were observed for riociguat treatment, and subsequent BPA interventions yielded further benefits. With targeted medication, WHO FC improved by at least one class in 13 (36.1%) patients ( P = 0.01). Hemodynamic assessment showed significant improvements in mean pulmonary arterial pressure (mPAP) (49 ± 12 mmHg vs. 43 ± 12 mmHg; P = 0.003) and PVR (956 ± 501 dyn·s·cm^-5 vs. 517 ± 279 dyn·s·cm^-5; P = 0.0001). Treatment with a combination of targeted medication and BPA resulted in WHO FC improvement in 34 (94.4%) patients. Hemodynamic assessment showed significant improvement in mPAP (43 ± 12 mmHg vs. 34 ± 14 mmHg; P = 0.0001) and PVR (517 ± 279 dyn·s·cm^-5 vs. 360 ± 175 dyn·s·cm^-5; P = 0.0001). These findings provide, for the first time, support for the therapeutic strategy recommended by current guidelines.

Keywords: balloon pulmonary angioplasty; chronic thromboembolic pulmonary hypertension; riociguat; targeted medication.

Fig. 1.

Flow chart describing the balloon pulmonary angioplasty program in Bad Nauheim showing patient selection.

Fig. 2.

Study flow chart for the 36 patients included in the study.

Fig. 3.

Effects of riociguat and BPA on (a) mPAP, (b) PVR, (c) WHO FC given in mean values, (d) NT-proBNP, (e) 6MWD, and (f) PAC. The asterisk indicates the significance level (*P < 0.05; **P < 0.001).