Topical Collection "Novel Therapeutic Approaches for Pulmonary Hypertension"
Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology, Centre of Postgraduate Medical Education, European Health Center Otwock, Otwock, Poland
Interests: pulmonary hypertension; pulmonary embolism; pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; balloon pulmonary angioplasty; echocardiography
Topical Collection Information
Over the past 25 years, tremendous progress has been made in the therapeutic possibilities for pulmonary hypertension. This statement applies to both pharmacological and surgical or percutaneous interventions. The goals and methods of therapy differ significantly depending on the etiology of pulmonary hypertension. In group I, according to current ESC/ERS Guidelines (pulmonary arterial hypertension, PAH), the introduction of epoprostenol and other drugs from the targeted therapy at the turn of the 20th and 21st centuries changed the "primary pulmonary hypertension" from a clearly fatal condition to more chronic disease. The following years brought numerous studies of new drugs and their combinations to obtain the optimal therapeutic effect. Interventional methods such as atrial septostomy, Pott's anastomosis, and pulmonary artery denervation are also utilized to reduce right ventricular failure. Lung transplantation remains the ultimate treatment for PAH, but the timing of listing for this procedure is still not clearly defined. Drugs that proved successful in treating PAH were tested in patients with post-capillary pulmonary hypertension (group II) and pulmonary hypertension associated with chronic pulmonary diseases (group III). The results of these studies are inconclusive and do not clearly indicate which patients benefit from PAH-like therapy. In contrast, many interventions that control the underlying heart failure or chronic pulmonary disease have beneficial effects on pulmonary artery pressure and the degree of right ventricular failure. Balloon pulmonary angioplasty and modern pharmacological treatment have been added to pulmonary endarterectomy, the recognized treatment option for chronic thromboembolic pulmonary hypertension. It gave an unprecedented possibility of treatment which is tailored to the individual subject. Research on novel therapeutic approaches is still underway and goes beyond the well-known canons of management.
In the present Topical Collection, we want to gather a collection of original, interesting and innovative papers on new therapeutic options for pulmonary hypertension. We invite authors of papers in the field of clinical medicine in the adult and pediatric populations and basic science researchers who work on experimental studies facilitating new therapeutic options. Let this Topical Collection be a forum for the exchange of fresh ideas and innovative concepts!
Prof. Dr. Marcin Kurzyna
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- Pulmonary hypertension
- Pulmonary arterial hypertension
- Chronic thromboembolic pulmonary hypertension
- Congenital heart disease
- Congestive heart failure
- Balloon pulmonary angioplasty
- Atrial septostomy
- Pulmonary artery denervation
- Lung transplantation
Published Papers (5 papers)
Exercise Training as a Non-Pharmacological Therapy for Patients with Pulmonary Arterial Hypertension: Home-Based Rehabilitation Program and Training Recommendations
Cited by 2
| Viewed by 1168
Pulmonary arterial hypertension (PAH) is a chronic and progressive disorder with a poor prognosis associated with non-specific symptoms, including general weakness, shortness of breath on exertion, and decreased muscle strength and endurance. Despite recent significant progress in the field of PAH therapy, many
[...] Read more.
Pulmonary arterial hypertension (PAH) is a chronic and progressive disorder with a poor prognosis associated with non-specific symptoms, including general weakness, shortness of breath on exertion, and decreased muscle strength and endurance. Despite recent significant progress in the field of PAH therapy, many patients are still characterized by a dynamic course of the disease, a significant reduction in physical performance, a constantly deteriorating quality of life, and limited activity in everyday life. Thus, the main goal of PAH therapy is to ensure an acceptable level of quality of life as early as possible in the course of the disease, reduce the progression of symptoms and, if possible, improve the prognosis, which is still poor. The perception of the importance of activity and exercise has changed significantly in recent years, and rehabilitation dedicated to PAH patients is now considered to be one of the new adjuvant treatment options. Currently, there is insufficient data on what form, frequency, and intensity of exercise are required for the best results. Nevertheless, exercise training (ET) is necessary in order to reverse the accompanying PAH impairment of exercise capacity and, without additional clinical risk, to maximize the benefits of pharmacotherapy. This review summarizes the current state of knowledge on the rehabilitation of PAH patients and presents the available rehabilitation models. In addition, it includes a ready-to-use, illustrated, safe home rehabilitation program with recommendations for its use. Utilizing ET as an adjuvant treatment option to improve the functional capacity and quality of life of patients may enhance the clinical effectiveness of therapeutic management and contribute to the improvement of the quality of care for patients suffering from PAH. The beneficial effect of exercise training on the development of symptoms improves the clinical course of the disease, and a lower incidence of adverse events can lead to a reduction in health care expenditure.
Association of Electrocardiographic Signs of Right Ventricular Hypertrophy and Clot Localization in Chronic Thromboembolic Pulmonary Hypertension
Cited by 4
| Viewed by 1747
The role of electrocardiography (ECG) in chronic thromboembolic pulmonary hypertension (CTEPH) diagnosis and prognosticating has not been yet established. We aimed to assess the relationships of the recommended ECG criteria of right ventricular hypertrophy (RVH) with clot localization in CTEPH patients. ECG patterns
[...] Read more.
The role of electrocardiography (ECG) in chronic thromboembolic pulmonary hypertension (CTEPH) diagnosis and prognosticating has not been yet established. We aimed to assess the relationships of the recommended ECG criteria of right ventricular hypertrophy (RVH) with clot localization in CTEPH patients. ECG patterns of RVH according to the American College of Cardiology Foundation were assessed in patients with newly diagnosed CTEPH. We enrolled 58 (45.3%) patients with proximal and 70 (54.7%) with distal CTEPH. Receiver-operating characteristics curves analysis indicated that the following ECG abnormalities predicted proximal CTEPH localization: RV1
> 6 mm—AUC 0.75 (CI: 0.66–0.84, p
< 0.00001); SV6
> 3 mm—AUC 0.70 (CI: 0.60–0.79, p
< 0.00001); SI
wave—AUC 0.67 (CI: 0.58–0.77, p
= 0.0004); RV1
> 1.0—AUC 0.66 (CI: 0.56–0.76, p
= 0.0009); RV1
peak > 0.035 s (QRS < 120 ms)—AUC 0.66 (CI: 0.56–0.75, p
= 0.0016); RV1
AUC-0.65 (CI: 0.54–0.75, p
= 0.0081); RaVR
> 4 mm—AUC 0.62 (CI: 0.52–0.71, p
= 0.002) and PII
> 2.5 mm—AUC 0.62 (CI: 0.52–0.72, p
= 0.00162). Pulmonary vascular resistance significantly correlated with amplitudes of RV1
(r = 0.34, p
= 0.008), SV6
(r = 0.53, p
= 0.000027) and PII
(r = 0.44, p
= 0.00007). In patients with CTEPH, only 8 out of 23 ECG RVH criteria were useful for differentiating between proximal and distal CTEPH localization and we found that RV1
may contribute as potential discriminators.
Mean Velocity of the Pulmonary Artery as a Clinically Relevant Prognostic Indicator in Patients with Heart Failure with Preserved Ejection Fraction
Cited by 1
| Viewed by 1211
Background: Right ventricular (RV) to pulmonary circulation (PC) coupling can stratify prognosis in heart failure (HF). In this study, we assessed the prognostic role of the mean velocity of the pulmonary artery (mvPA) determined by cardiac magnetic resonance (CMR) in HF with preserved
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Background: Right ventricular (RV) to pulmonary circulation (PC) coupling can stratify prognosis in heart failure (HF). In this study, we assessed the prognostic role of the mean velocity of the pulmonary artery (mvPA) determined by cardiac magnetic resonance (CMR) in HF with preserved ejection fraction (HFpEF). Methods: Inclusion of 58 HFpEF outpatients that underwent CMR with measurement of RV–PC coupling parameters including mvPA between 2016 and 2019. The primary combined endpoint was a composite of HF readmissions and all-cause mortality. Results: Optimal cut-off value of mvPA calculated by receiver operating curve for the prediction of the primary endpoint was 9 cm/s. Over a median follow-up of 23 months (interquartile range: 24), 21 patients met the primary endpoint. The primary endpoint was more frequent in patients with mvPA ≤ 9 cm/s, as indicated by Kaplan–Meier survival curves; Log-Rank: 9.193, p
= 0.02, regardless of RV dysfunction. On Cox multivariate analysis, mvPA ≤ 9 cm/s emerged as an independent prognostic predictor of the primary endpoint (HR: 4.11, 95% CI: 1.28–13.19, p
= 0.017), together with left atrial area by CMR (HR: 1.08, 95% CI: 1.01–1.24, p
= 0.034). Conclusions: In our HFpEF cohort, mvPA was associated with a higher rate of the primary endpoint, regardless of RV function, thus enabling identification of patients at higher risk of cardiovascular events before structural damage onset.
Selective Segmental Pulmonary Angiography: Anatomical, Technical and Safety Aspects of a Must-Learn Technique in Times of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension
Cited by 2
| Viewed by 7570
With the advent of balloon pulmonary angioplasty (BPA) for non-surgical chronic thromboembolic pulmonary hypertension (CTEPH) patients, there is renewed interest in the pulmonary angiography technique. This technique is still the standard imaging modality to confirm CTEPH, which, in addition, helps to determine the
[...] Read more.
With the advent of balloon pulmonary angioplasty (BPA) for non-surgical chronic thromboembolic pulmonary hypertension (CTEPH) patients, there is renewed interest in the pulmonary angiography technique. This technique is still the standard imaging modality to confirm CTEPH, which, in addition, helps to determine the most appropriate treatment. Furthermore, learning this technique fulfills two main purposes: to identify BPA candidates and to provide the operator with the catheter handling needed to perform BPA. Operators interested in performing BPA must learn not only the pulmonary arteries’ anatomy, but also which are the best angiographic projections and the most suitable catheters to canalize and display each segmental branch. Unfortunately, this information is scarce in the literature. With this goal, learning the diagnostic pulmonary angiography technique can be a first step on the way to perform BPA. Although there are descriptions on how to perform a pulmonary angiography with balloon-tipped catheters and the digital subtraction technique, this technique does not provide operators with the catheter knowledge and manual skill needed to cannulate each segmental branch. In contrast, learning the conventional selective segmental pulmonary angiography (SSPA) technique provides the operator with this knowledge and skills. In this review, based on the experience of the authors, we describe the pulmonary arteries’ anatomy and detail the practical aspects of the SSPA procedure, with the aim of providing operators with the anatomical and technical knowledge needed to perform BPA. We also summarize the contemporary complications of SSPA in CTEPH patients at a reference center.
Balloon Pulmonary Angioplasty in Technically Operable and Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension
Cited by 12
| Viewed by 2016
Background: In this study, we aimed to assess the efficacy and safety of balloon pulmonary angioplasty (BPA) in patients with technically inoperable distal-type chronic thromboembolic pulmonary hypertension (d-CTEPH) and technically operable proximal-type disease (p-CTEPH) by analyzing the results of BPA treatment in two
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Background: In this study, we aimed to assess the efficacy and safety of balloon pulmonary angioplasty (BPA) in patients with technically inoperable distal-type chronic thromboembolic pulmonary hypertension (d-CTEPH) and technically operable proximal-type disease (p-CTEPH) by analyzing the results of BPA treatment in two collaborating CTEPH referral centers. Methods and results: We assessed hemodynamic results, functional efficacy, complication and survival rate after BPA treatment in 70 CTEPH patients (median age 64 years; (interquartile range (IQR): 52–73 years)), of whom 16 (median age 73 years; (QR 62–82 years)) were in the p-CTEPH subgroup. Altogether, 377 BPA procedures were performed, resulting in significant (p
< 0.001) improvement in mean pulmonary artery pressure (mPAP 48.6 ± 10 vs. 31.3 ± 8.6 mmHg), pulmonary vascular resistance (694 ± 296 vs. 333 ± 162 dynes*s*cm−5
), six-minute walk test (365 ± 142 vs. 433 ± 120 metres) and N-terminal pro B-type natriuretic peptide (1307 (510–3294) vs. 206 (83–531) pg/mL). The rate of improvement did not differ between the sub-groups. Lung injury episodes and severe hemoptysis were similarly infrequent in d-CTEPH and p-CTEPH (6.4% vs. 5%; p
= 0.55 and 1.0% vs. 2.5; p
= 0.24, respectively). There was no significant difference between the sub-groups regarding survival (p
= 0.53 by log-rank test). Conclusion: BPA may be beneficial in patients with p-CTEPH who cannot undergo pulmonary endarterectomy (PEA). Larger long-term studies are needed to better define the efficacy, safety, and optimal BPA procedural standards in this population.