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

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878399 ◽  
Author(s):  
Christoph B. Wiedenroth ◽  
H. Ardeschir Ghofrani ◽  
Miriam S.D. Adameit ◽  
Andreas Breithecker ◽  
Moritz Haas ◽  
...  

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.

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321775312 ◽  
Author(s):  
Christoph B. Wiedenroth ◽  
Karen M. Olsson ◽  
Stefan Guth ◽  
Andreas Breithecker ◽  
Moritz Haas ◽  
...  

Symptomatic patients with residual pulmonary perfusion defects or vascular lesions but no pulmonary hypertension at rest are diagnosed with chronic thromboembolic disease (CTED). Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but data regarding the safety and efficacy of BPA in patients with CTED are lacking. We report a prospective series of ten consecutive patients with CTED who underwent 35 BPA interventions (median of four per patient) at two German institutions. All patients underwent a comprehensive diagnostic workup at baseline and 24 weeks after their last intervention. BPA was safe, with one pulmonary vascular injury and subsequent self-limiting pulmonary bleeding as the only complication (2.9% of the interventions, 10% of the patients). After the procedures, World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, and pulmonary arterial compliance improved, and NT-proBNP concentrations declined in 9/10 patients. BPA may be a new treatment option for carefully selected patients with CTED. A larger, prospective, international registry is required to confirm these results.


Open Heart ◽  
2020 ◽  
Vol 7 (1) ◽  
pp. e001144 ◽  
Author(s):  
Stephen P Hoole ◽  
John G Coghlan ◽  
John E Cannon ◽  
Dolores Taboada ◽  
Mark Toshner ◽  
...  

ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK opened in October 2015 and we now describe the treatment of our initial patient cohort.MethodsThirty consecutive, inoperable, anatomically suitable, symptomatic patients on stable medical therapy for CTEPH were identified and offered BPA. They initially underwent baseline investigations including Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) quality of life (QoL) questionnaire, cardiopulmonary exercise test, 6 min walk distance (6MWD), transthoracic echocardiography, N-terminal probrain natriuretic peptide (NT pro-BNP) and right heart catheterisation. Serial BPA sessions were then performed and after completion, the treatment effect was gauged by comparing the same investigations at 3 months follow-up.ResultsA median of 3 (IQR 1–6) BPA sessions per patient resulted in a significant improvement in functional status (WHO functional class ≥3: 24 vs 4, p<0.0001) and QoL (CAMPHOR symptom score: 8.7±5.4 vs 5.6±6.1, p=0.0005) with reductions in pulmonary pressures (mean pulmonary artery pressure: 44.7±11.0 vs 34.4±8.3 mm Hg, p<0.0001) and resistance (pulmonary vascular resistance: 663±281 vs 436±196 dyn.s.cm-5, p<0.0001). Exercise capacity improved (minute ventilation/carbon dioxide production: 55.3±12.2 vs 45.0±7.8, p=0.03 and 6MWD: 366±107 vs 440±94 m, p<0.0001) and there was reduction in right ventricular (RV) stretch (NT pro-BNP: 442 (IQR 168–1607) vs 202 (IQR 105–447) pg/mL, p<0.0001) and dimensions (mid RV diameter: 4.4±1.0 vs 3.8±0.7 cm, p=0.002). There were no deaths or life-threatening complications and the mild-moderate per-procedure complication rate was 10.5%.ConclusionsBPA is safe and improves the functional status, QoL, pulmonary haemodynamics and RV dimensions of patients with inoperable CTEPH.


2020 ◽  
Vol 10 (3) ◽  
pp. 204589402091788
Author(s):  
Christoph B. Wiedenroth, MD ◽  
Andreas J. Rieth, MD ◽  
Steffen Kriechbaum, MD ◽  
H.-Ardeschir Ghofrani, MD ◽  
Andreas Breithecker, MD ◽  
...  

Background * These authors contributed equally as last authors. Balloon pulmonary angioplasty is an evolving, interventional treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary hypertension at rest as well as exercise capacity is considered to be relevant outcome parameters. The aim of the present study was to determine whether measurement of pulmonary hemodynamics during exercise before and six months after balloon pulmonary angioplasty have an added value. Methods From March 2014 to July 2018, 172 consecutive patients underwent balloon pulmonary angioplasty. Of these, 64 consecutive patients with inoperable CTEPH underwent a comprehensive diagnostic workup that included right heart catheterization at rest and during exercise before balloon pulmonary angioplasty treatments and six months after the last intervention. Results Improvements in pulmonary hemodynamics at rest and during exercise, in quality of life, and in exercise capacity were observed six months after balloon pulmonary angioplasty: WHO functional class improved in 78% of patients. The mean pulmonary arterial pressure (mPAP) at rest was reduced from 41 ± 9 to 31 ± 9 mmHg (p < 0.0001). The mPAP/cardiac output slope decreased after balloon pulmonary angioplasty (11.2 ± 25.6 WU to 7.7 ± 4.1 WU; p < 0.0001), and correlated with N-terminal fragment of pro-brain natriuretic peptide (p = 0.035) and 6-minute walking distance (p = 0.01). Conclusions Exercise right heart catheterization provides valuable information on the changes of pulmonary hemodynamics after balloon pulmonary angioplasty in inoperable CTEPH patients that are not obtainable by measuring resting hemodynamics.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Shunsuke Tatebe ◽  
Koichiro Sugimura ◽  
Kotaro Nochioka ◽  
Masanobu Miura ◽  
Saori Yamamoto ◽  
...  

Background: Insulin resistance, dyslipidemia and renal dysfunction have been regarded as poor prognostic factors for pulmonary hypertension. We and others have recently demonstrated that percutaneous transluminal pulmonary angioplasty (PTPA) markedly improves pulmonary hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH). In this study, we examined whether PTPA also improves metabolic and renal impairments in CTEPH patients. Methods and Results: From April 2012 to May 2013, we examined serum levels of lipids and fatty acids fractions and plasma levels of glucose and immunoreactive insulin in 68 consecutive patients with CTEPH (64±14[SD] years, M/F 13/55) and calculated the homeostatic model assessment of insulin resistance (HOMA-IR). Renal function was assessed by estimated GFR (eGFR) and urinary albumin-to-creatinine (U-A/C) ratio. Vascular stiffness was evaluated by cardio-ankle vascular index (CAVI). The measurements were repeated after PTPA in 49 patients. Among the 68 patients, we noted NYHA functional class ≥III in 17, 6 min-walk distance <300m in 15, and cardiac index <2.0l/min/m 2 in 18, and regarding metabolic disorders, hypertension in 41, diabetes in 6 and dyslipidemia in 24. Insulin resistance (defined as HOMA-IR >2.0) was noted in 29 out of 63 (58%). Regarding renal function, eGFR was 61.6±17.8 ml/min/m 2 , U-A/C ratio 80.8±214.7mg/gCre, and chronic kidney disease in stage ≥3 was noted in 32 patients (47%). We performed PTPA in 49 patients (mean 3.3 essions/patient), which markedly improved NYHA functional class (P=0.008), 6min-walk distance (102±25 m, P<0.0001) and mean pulmonary arterial pressure (-9.9±1.3 mmHg, P<0.0001). Furthermore, PTPA significantly improved metabolic profiles such as HDL-chol (6.2 ±2.2 mg/dl, P=0.01), EPA (18.3±6.2 mg/dl, P=0.006), fasting blood sugar (-8.0±3.5 mg/dl, P=0.04), HbA1c (-0.3±0.1 %, P<0.0001) and CAVI (-0.44±0.23, P=0.02) . PTPA also significantly improved eGFR (5.2±1.2 ml/min/m 2 , P <0.001) and U-A/C ratio (-47.1±18.9 mg/gCre, P=0.004). Conclusions: These results indicate that metabolic and renal dysfunctions are commonly present in CTEPH patients and that PTPA markedly improves those disorders in addition to pulmonary hemodynamics.


2020 ◽  
Vol 9 (11) ◽  
pp. 3608
Author(s):  
Pavel Jansa ◽  
Samuel Heller ◽  
Michal Svoboda ◽  
Michal Pad’our ◽  
David Ambrož ◽  
...  

Balloon pulmonary angioplasty (BPA) is a novel treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not eligible for pulmonary endarterectomy (PEA) or suffer from persistent pulmonary hypertension after PEA. The aim of this study was to evaluate the real-life efficacy and safety of BPA in a consecutive group of patients who were diagnosed and treated in the national referral center for CTEPH in the Czech Republic. Here we report data from 160 BPA procedures performed in 64 patients. Efficacy analysis was performed in the subgroup of 25 patients who completed BPA series. Significant improvements were observed in New York Heart Association functional class (4% to 79% in I/II, p < 0.001), 6 min walking test distance (+54.3 m, p < 0.001), risk profile (15.8% to 68.5% with presence of 2/3 low risk criteria, p < 0.001), pulmonary artery mean pressure (−18%, p < 0.001), pulmonary vascular resistance (−32%, p < 0.001), stroke volume (+17%, p = 0.011) and quality of life (+37% in assessment of overall health status by a patient, p < 0.001). We observed 1 fatal periprocedural complication (1.6% of all 64 patients) and 19 BPA-related non-fatal complications (11.9% of all 160 interventions) that predominantly included hemoptysis (10.0% of all sessions). Overall survival at 12 months was 94.6%.


2019 ◽  
Vol 91 (4) ◽  
pp. 43-47
Author(s):  
N M Danilov ◽  
Yu G Matchin ◽  
A M Chernyavsky ◽  
A G Edemsky ◽  
D S Grankin ◽  
...  

Aim. To evaluate the effectiveness of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. Forty patients with inoperable CTEPH were enrolled in this study. The indications were determined by multidisciplinary team. The average age of patients was 53.5 [43; 63] years. In 65% of cases patients had functional class III (according to WHO); the distance in the 6-minute walk test (6MWD) was 327 [280; 400] m; catheterization of the right heart revealed systolic pulmonary artery pressure (SPAP) 82 [64; 100] mm Hg, mean pulmonary artery (mPAP) 48.5 [38; 56] mm Hg, pulmonary vascular resistance (PVR) 784 [525; 1257] dyn·s/cm-5. Each patient underwent 6 BPA. Results and discussion. The effectiveness of BPA was assessed 2 months after the last session. According to the data of right heart catheterization SPAP decreased by 27.3%, mPAP by 26%, PVR by 34.5% from baseline. After all series of BPA echocardiography and magnetic resonance imaging demonstrated reverse remodeling of the right heart. Also significant decrease in the level of BNP by 62%, increasing in 6MWD distance by 39% and improvement of the functional class up to I in 60% cases and up to II in 40% cases were noted. Conclusion. The results of the present study demonstrated a high efficacy of BPA allowing to normalize hemodynamic and clinical parameters, increasing the physical activity. Balloon pulmonary angioplasty is a new highly effective, safe method for treating patients with inoperable CTEPH.


2017 ◽  
Vol 49 (6) ◽  
pp. 1602409 ◽  
Author(s):  
Karen M. Olsson ◽  
Christoph B. Wiedenroth ◽  
Jan-Christopher Kamp ◽  
Andreas Breithecker ◽  
Jan Fuge ◽  
...  

Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention.BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%.BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk–benefit assessments.


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