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Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives

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A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (15 April 2021) | Viewed by 35191

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Dr. Grzegorz Kopec

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Jagiellonian University Medical College, Faculty of Medicine, Institute of Cardiology, Department of Cardiac and Vascular Diseases, John Paul II Hospital in Krakow, Pradnicka 80, 31-349 Krakow, Poland
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

I have a privilege to invite you to the special issue of the Journal of Clinical Medicine titled Pulmonary Arterial Hypertension and beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives. We dedicate this issue mainly but not solely to pulmonary arterial hypertension (PAH) which means that papers presenting the newest achievements in other forms of pulmonary hypertension are also welcome.

The definition of pulmonary hypertension has been revised recently but how this change can affect epidemiology of this disease, the approach to its management and patients’ outcome is not well known. Therefore, in the present issue we are going to prioritize studies which enroll patients with pulmonary hypertension diagnosed based on the novel definition.

Clinical characteristics of PAH population has been changing during the last decades. Currently, significant number of patients have more than 65 years at diagnosis and have a number of different diseases when PAH is diagnosed. Thus of special interest is the differential diagnosis between PAH and other forms of pulmonary hypertension including those due to left heart diseases and due to diseases of the respiratory system. We are also interested in papers presenting how different comorbidities can affect patients’ outcome.

PAH is very rare in pediatric population and there is a paucity of data from clinical studies on optimal therapy of PAH in this population. Therefore, epidemiological data on PAH in children, cohort studies analyzing clinical outcomes as well as clinical trials in this field are of scope in the present issue of the Journal of Clinical Medicine.

Despite an increasing knowledge on PAH mechanisms still the etiology of this disease is not well understood. Accordingly, studies presenting new data from preclinical studies on the pathophysiology of human PAH especially with use of the emerging omic technologies will be considered in the present issue.

Furthermore, we are also interested in good quality papers on novel diagnostic modalities and new concepts of PAH therapies.

Dr. Grzegorz Kopec
Guest Editor

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Keywords

Pulmonary arterial hypertension
Exercise pulmonary hypertension
Omic techniques
Patomechanisms
Epidemiology
Outcome studies
Clinical trials
Phenotypes of pulmonary hypertension
Subpopulations
Classification

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Published Papers (11 papers)

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13 pages, 1571 KiB

Open AccessArticle

Peripheral Arterial Stiffness in Acute Pulmonary Embolism and Pulmonary Hypertension at Short-Term Follow-Up

by Silvia Papa, Cristiano Miotti, Giovanna Manzi, Gianmarco Scoccia, Federico Luongo, Federica Toto, Claudia Malerba, Nadia Cedrone, Elena Sofia Canuti, Annalisa Caputo, Giulia Manguso, Serena Valentini, Susanna Sciomer, Francesco Ciciarello, Giulia Benedetti, Francesco Fedele, Carmine Dario Vizza and Roberto Badagliacca

J. Clin. Med. 2021, 10(14), 3008; https://doi.org/10.3390/jcm10143008 - 6 Jul 2021

Cited by 2 | Viewed by 2560

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and under-recognized complication of acute pulmonary embolism (PE). Forty consecutive patients with acute PE (Group 1), predominantly female (22, 55%) with a mean age of 69 ± 15 years, were matched for demographic data with 40 healthy subjects (Group 2), 40 systemic hypertension patients (Group 3) and 45 prevalent idiopathic pulmonary arterial hypertension (IPAH) patients (Group 4). The baseline evaluation included physical examination, NYHA/WHO functional class, right heart catheterization (RHC) limited to IPAH patients, echocardiographic assessment and systemic arterial stiffness measurement by cardio-ankle vascular index (CAVI). Patients with PE underwent an echocardiographic evaluation within 1 month from hospital discharge (median 27 days; IQR 21–30) to assess the echo-derived probability of PH. The CAVI values were significantly higher in the PE and IPAH groups compared with the others (Group 1 vs. Group 2, p < 0.001; Group 1 vs. Group 3, p < 0.001; Group 1 vs. Group 4, p = ns; Group 4 vs. Group 2, p < 0.001; Group 4 vs. Group 3, p < 0.001; Group 2 vs. Group 3, p = ns). The predicted probability of echocardiography-derived high-risk criteria of PH increases for any unit increase of CAVI (OR 9.0; C.I.3.9–20.5; p = 0.0001). The PE patients with CAVI ≥ 9.0 at the time of hospital discharge presented an increased probability of PH. This study highlights a possible positive predictive role of CAVI as an early marker for the development of CTEPH. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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12 pages, 3036 KiB

Open AccessArticle

Extended Precordial T Wave Inversions Are Associated with Right Ventricular Enlargement and Poor Prognosis in Pulmonary Hypertension

by Marcin Waligóra, Matylda Gliniak, Jan Bylica, Paweł Pasieka, Patrycja Łączak, Piotr Podolec and Grzegorz Kopeć

J. Clin. Med. 2021, 10(10), 2147; https://doi.org/10.3390/jcm10102147 - 16 May 2021

Cited by 4 | Viewed by 2535

Abstract

In pulmonary hypertension (PH), T wave inversions (TWI) are typically observed in precordial leads V1–V3 but can also extend further to the left-sided leads. To date, the cause and prognostic significance of this extension have not yet been assessed. Therefore, we aimed to assess the relationship between heart morphology and precordial TWI range, and the role of TWI in monitoring treatment efficacy and predicting survival. We retrospectively analyzed patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) treated in a reference pulmonary hypertension center. Patients were enrolled if they had a cardiac magnetic resonance (cMR) and 12-lead surface ECG performed at the time of assessment. They were followed from October 2008 until March 2021. We enrolled 77 patients with PAH and 56 patients with inoperable CTEPH. They were followed for a mean of 51 ± 33.5 months, and during this time 47 patients died (35.3%). Precordial TWI in V1–V6 were present in 42 (31.6%) patients, while no precordial TWI were observed only in 9 (6.8%) patients. The precordial TWI range correlated with markers of PH severity, including right ventricle to left ventricle volume

\frac{R V E D V}{L V E D V}

(R = 0.76, p < 0.0001). The presence of TWI in consecutive leads from V1 to at least V5 predicted severe RV dilatation (

\frac{R V E D V}{L V E D V}

≥ 2.3) with a sensitivity of 88.9% and specificity of 84.1% (AUC of 0.90, 95% CI = 0.83–0.94, p < 0.0001). Presence of TWI from V1 to at least V5 was also a predictor of mortality in Kaplan–Meier estimation (p = 0.02). Presence of TWI from V1 to at least V5 had a specificity of 64.3%, sensitivity of 58.1%, negative predictive value of 75%, and positive predictive value of 45.5% as a mortality predictor. In patients showing a reduction in TWI range of at least one lead after treatment compared with patients without this reduction, we observed a significant improvement in RV-EDV and

\frac{RV - EDV}{LV - EDV}

. We concluded that the extension of TWI to left-sided precordial leads reflects significant pathological alterations in heart geometry represented by an increase in RV/LV volume and predicts poor survival in patients with PAH and CTEPH. Additionally, we found that analysis of precordial TWI range can be used to monitor the effectiveness of hemodynamic response to treatment of pulmonary hypertension. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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12 pages, 8191 KiB

Open AccessArticle

The Impact of COVID-19 Pandemic on Children with Pulmonary Arterial Hypertension. Parental Anxiety and Attitudes. Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

by Joanna Kwiatkowska, Jaroslaw Meyer-Szary, Anna Mazurek-Kula, Malgorzata Zuk, Anna Migdal, Jacek Kusa, Elzbieta Skiba, Karolina Zygielo, Kinga Przetocka, Zbigniew Kordon, Pawel Banaszak, Agata Michalczyk, Alina Rzeznik-Bieniaszewska, Rafal Surmacz, Waldemar Bobkowski, Barbara Wojcicka-Urbanska, Bozena Werner, Joanna Pluzanska, Katarzyna Ostrowska, Magdalena Bazgier and Grzegorz Kopec Show full author list Hide full author list

J. Clin. Med. 2021, 10(8), 1640; https://doi.org/10.3390/jcm10081640 - 12 Apr 2021

Cited by 7 | Viewed by 3039

Abstract

The COVID-19 pandemic has impacted healthcare systems worldwide. Little is known about the impact of the pandemic on medical and psycho-social aspects of children with rare diseases such as pulmonary arterial hypertension and their parents. The study is based on children registered in The Database of Pulmonary Hypertension in the Polish Population and a parent-reported survey deployed during the first 6 months of the pandemic. The questionnaire consisted of six question panels: demographic data, fear of COVID-19, General Anxiety Disorder-7 (GAD-7), social impact of pandemic, patients’ medical status, and alarming symptoms (appearance or exacerbation). Out of 80 children registered, we collected 58 responses (72.5% response rate). Responders (parents) were mostly female (n = 55; 94.8%) at a mean age of 40.6 ± 6.9 years. Patients (children) were both females (n = 32; 55%) and males with a mean age of 10.0 ± 5.1 years. Eleven (19%) children had symptoms of potential disease exacerbation. Eight parents (72.7%) decided for watchful waiting while others contacted their GPs or cardiologists (n = 6; 54.5%). Three children had to be hospitalized (27.3%). Most planned hospitalizations (27/48; 56.2%) and out-patient visits (20/35; 57.1%) were cancelled, delayed, or substituted by telehealth services. Among the participating parents, the study shows very high levels of anxiety (n = 20; 34.5%) and concern (n = 55; 94.8%) and the need for detailed information (52; 89.6%) regarding COVID-19 and medical service preparedness during the pandemic. The COVID-19 pandemic has influenced child healthcare and caused high levels of anxiety among parents. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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21 pages, 717 KiB

Open AccessArticle

High CD200 Expression on T CD4+ and T CD8+ Lymphocytes as a Non-Invasive Marker of Idiopathic Pulmonary Hypertension–Preliminary Study

by Michał Tomaszewski, Ewelina Grywalska, Weronika Topyła-Putowska, Piotr Błaszczak, Marcin Kurzyna, Jacek Roliński and Grzegorz Kopeć

J. Clin. Med. 2021, 10(5), 950; https://doi.org/10.3390/jcm10050950 - 1 Mar 2021

Cited by 4 | Viewed by 3132

Abstract

Pulmonary arterial hypertension (PAH) can develop subsequently to disorganized endothelial cell proliferation within the pulmonary arteriolar layers that provide mechanical limits to the pulmonary vascular bed. Although the actual factor triggering vascular endothelial proliferation remains unknown to date, genetic susceptibility, hypoxia, inflammation, as well as response to drugs and toxins have been proposed as possible contributors. Since inflammation contributes to vascular remodeling, the changed immune response is increasingly considered a plausible cause of this cardiovascular disease. The interaction of a membrane glycoprotein cluster of differentiation 200 (CD200) and its structurally similar receptor (CD200R) plays a crucial role in the modulation of the inflammatory response. Our previous studies have shown that the overexpression of the other negative co-stimulatory molecule (programmed death cell-PD-1) and its ligand-1 (PD-L1) is closely related to iPAH and the presence of Epstein-Barr virus (EBV) reactivation markers. Therefore, we considered it necessary to analyze the different types of PAH in terms of CD200 and CD200R expression and to correlate CD200/CD200R pathway expression with important clinical and laboratory parameters. The CD200/C200R-signaling pathway has not been subject to much research. We included 70 treatment-naïve, newly diagnosed patients with PAH in our study. They were further divided into subsets according to the pulmonary hypertension classification: chronic thromboembolic pulmonary hypertension (CTEPH) subset, pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), pulmonary arterial hypertension associated with connective tissue disease (CTD-PAH), and idiopathic pulmonary arterial hypertension (iPAH). The control group consisted of 20 healthy volunteers matched for sex and age. The highest percentages of T CD200+CD4+ and T CD200+CD8+ lymphocytes were observed in the group of patients with iPAH and this finding was associated with the presence of EBV DNA in the peripheral blood. Our assessment of the peripheral blood lymphocytes expression of CD200 and CD200R indicates that these molecules act as negative co-stimulators in the induction and persistence of PAH-associated inflammation, especially that of iPAH. Similar results imply that the dysregulation of the CD200/CD200R axis may be involved in the pathogenesis of several immune diseases. Our work suggests that CD200 and CD200R expression may serve to distinguish between PAH cases. Thus, CD200 and CD200R might be useful as markers in managing PAH and should be further investigated. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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13 pages, 1359 KiB

Open AccessArticle

Overexpression of PD-1 on Peripheral Blood Lymphocytes in Patients with Idiopathic Pulmonary Arterial Hypertension and Its Association with High Viral Loads of Epstein-Barr Virus and Poor Clinical Parameters

by Michał Tomaszewski, Ewelina Grywalska, Andrzej Tomaszewski, Piotr Błaszczak, Marcin Kurzyna, Jacek Roliński and Grzegorz Kopeć

J. Clin. Med. 2020, 9(6), 1966; https://doi.org/10.3390/jcm9061966 - 24 Jun 2020

Cited by 13 | Viewed by 2932

Abstract

Idiopathic pulmonary arterial hypertension (IPAH) is a rare but severe disease with the elevated blood pressure in the pulmonary arteries without a known trigger of vascular remodelling. It leads to the right heart failure with reduced survival. Changes in the immunological landscape of the lungs and the periphery are common in IPAH patients, suggesting an immune system dysfunction. A cohort of 25 IPAH patients was enrolled in our study to investigate a link between the patient’s clinical status, immune parameters of the blood, and the Epstein–Barr virus (EBV) infection. We found significant alterations of the patients’ peripheral blood parameters. Therein, T lymphocytes and NK cell counts were decreased in the IPAH patients’ blood, while the proportion of regulatory T cells was increased. Additionally, levels of proinflammatory cytokines interleukin-6 (IL-6), IL-2, and interferon-gamma (IFN-γ) were elevated. We identified a weak correlation between EBV loads and IPAH patients’ clinical state (r = 0.54) and between EBV loads and overexpression of PD-1 on helper T cells (r = 0.56). We speculate that a significant dysregulation of the immune system homeostasis observed in IPAH patients may contribute to increased susceptibility of those patients to EBV infection, yet further longitudinal studies are required to characterize this relation in detail. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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17 pages, 1285 KiB

Open AccessArticle

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

by Joanna Kwiatkowska, Malgorzata Zuk, Anna Migdal, Jacek Kusa, Elzbieta Skiba, Karolina Zygielo, Kinga Przetocka, Piotr Werynski, Pawel Banaszak, Alina Rzeznik-Bieniaszewska, Rafal Surmacz, Waldemar Bobkowski, Barbara Wojcicka-Urbanska, Bozena Werner, Joanna Pluzanska, Katarzyna Ostrowska, Anna Waldoch and Grzegorz Kopec

J. Clin. Med. 2020, 9(6), 1717; https://doi.org/10.3390/jcm9061717 - 3 Jun 2020

Cited by 20 | Viewed by 4533

Abstract

We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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13 pages, 1472 KiB

Open AccessArticle

Relationships between Pulmonary Hypertension Risk, Clinical Profiles, and Outcomes in Dilated Cardiomyopathy

by Ewa Dziewięcka, Sylwia Wiśniowska-Śmiałek, Aleksandra Karabinowska, Katarzyna Holcman, Matylda Gliniak, Mateusz Winiarczyk, Arman Karapetyan, Monika Kaciczak, Piotr Podolec, Magdalena Kostkiewicz, Marta Hlawaty, Agata Leśniak-Sobelga and Paweł Rubiś

J. Clin. Med. 2020, 9(6), 1660; https://doi.org/10.3390/jcm9061660 - 1 Jun 2020

Cited by 9 | Viewed by 3446

Abstract

Pulmonary hypertension (PH) in patients with heart failure (HF) contributes to a poorer prognosis. However, in those with dilated cardiomyopathy (DCM), the true prevalence and role of PH is unclear. Therefore, this study aimed to analyze the profile of DCM patients at various levels of PH risk, determined via echocardiography, and its impact on outcomes. The 502 DCM in- and out-patient records were retrospectively analyzed. Information on patient status was gathered after 45.9 ± 31.3 months. Patients were divided into 3 PH-risk groups based on results from echocardiography measurements: low (L, n = 239, 47.6%), intermediate (I, n = 153, 30.5%), and high (H, n = 110, 21.9%). Symptom duration, atrial fibrillation, ventricular tachyarrhythmia, ejection fraction, right atrial area, and moderate or severe mitral regurgitation were found to be independently associated with PH risk. During the follow-up period, 83 (16.5%) DCM patients died: 29 (12.1%) in L, 31 (20.3%) in I, and 23 (20.9%) in H. L-patients had a significantly lower risk of all-cause death (L to H: HR 0.55 (95%CI 0.32–0.98), p = 0.01), while no differences in prognosis were found between I and H. In conclusion, over one in five DCM patients had a high PH risk, and low PH risk was associated with better prognoses. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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12 pages, 1100 KiB

Open AccessArticle

Virtual Histology to Evaluate Mechanisms of Pulmonary Artery Lumen Enlargement in Response to Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension

by Wojciech Magoń, Jakub Stępniewski, Marcin Waligóra, Kamil Jonas, Roman Przybylski, Martyna Sikorska, Piotr Podolec and Grzegorz Kopeć

J. Clin. Med. 2020, 9(6), 1655; https://doi.org/10.3390/jcm9061655 - 1 Jun 2020

Cited by 10 | Viewed by 2286

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) results from an obstruction of pulmonary arteries (PAs) by organized thrombi. The stenosed PAs are targeted during balloon pulmonary angioplasty (BPA). We aimed to evaluate the mechanism of BPA in inoperable patients with CTEPH. We analyzed stenosed PAs with intravascular grey-scale ultrasound (IVUS) to determine the cross-sectional area (CSA) of arterial lumen and of organized thrombi. The composition of organized thrombi was assessed using virtual histology. We distinguished two mechanisms of BPA: Type A with dominant vessel stretching, and type B with dominant thrombus compression. PAs were assessed before (n = 159) and after (n = 98) BPA in 20 consecutive patients. Organized thrombi were composed of dark-green (57.1 (48.0–64.0)%), light-green (34.0 (21.4–46.4)%), red (6.4 (2.9–11.7)%;) and white (0.2 (0.0–0.9)%) components. The mechanism type depended on vessel diameter (OR = 1.09(1.01–1.17); p = 0.03). In type B mechanism, decrease in the amount of light-green component positively correlated with an increase in lumen area after BPA (r = 0.50; p = 0.001). The mechanism of BPA depends on the diameter of the vessel. Dilation of more proximal PAs depends mainly on stretching of the vessel wall while dilation of smaller PAs depends on compression of the organized thrombi. The composition of the organized thrombi contributes to the effect of BPA. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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Review

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23 pages, 1787 KiB

Open AccessReview

Role of the Immune System Elements in Pulmonary Arterial Hypertension

by Michał Tomaszewski, Dominika Bębnowska, Rafał Hrynkiewicz, Jakub Dworzyński, Paulina Niedźwiedzka-Rystwej, Grzegorz Kopeć and Ewelina Grywalska

J. Clin. Med. 2021, 10(16), 3757; https://doi.org/10.3390/jcm10163757 - 23 Aug 2021

Cited by 25 | Viewed by 3398

Abstract

Pulmonary arterial hypertension (PAH) is a relatively rare disease, but, today, its incidence tends to increase. The severe course of the disease and poor patient survival rate make PAH a major diagnostic and therapeutic challenge. For this reason, a thorough understanding of the pathogenesis of the disease is essential to facilitate the development of more effective therapeutic targets. Research shows that the development of PAH is characterized by a number of abnormalities within the immune system that greatly affect the progression of the disease. In this review, we present key data on the regulated function of immune cells, released cytokines and immunoregulatory molecules in the development of PAH, to help improve diagnosis and targeted immunotherapy. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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11 pages, 768 KiB

Open AccessReview

High Right Ventricular Afterload during Exercise in Patients with Pulmonary Arterial Hypertension

by Mari Nishizaki, Aiko Ogawa and Hiromi Matsubara

J. Clin. Med. 2021, 10(9), 2024; https://doi.org/10.3390/jcm10092024 - 9 May 2021

Cited by 4 | Viewed by 2787

Abstract

The right ventricle (RV) is more sensitive to an increase in afterload than the left ventricle (LV), and RV afterload during exercise increases more easily than LV afterload. Pulmonary arterial hypertension (PAH)-specific therapy has improved pulmonary hemodynamics at rest; however, the pulmonary hemodynamic response to exercise is still abnormal in most patients with PAH. In these patients, RV afterload during exercise could be higher, resulting in a greater increase in RV wall stress. Recently, an increasing number of studies have indicated the short-term efficacy of exercise training. However, considering the potential risk of promoting myocardial maladaptive remodeling, even low-intensity repetitive exercise training could lead to long-term clinical deterioration. Further studies investigating the long-term effects on the RV and pulmonary vasculature are warranted. Although the indications for exercise training for patients with PAH have been expanding, exercise training may be associated with various risks. Training programs along with risk stratification based on the pulmonary hemodynamic response to exercise may enhance the safety of patients with PAH. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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9 pages, 240 KiB

Open AccessReview

The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing Piece

by Cristiano Miotti, Silvia Papa, Giovanna Manzi, Gianmarco Scoccia, Federico Luongo, Federica Toto, Claudia Malerba, Nadia Cedrone, Susanna Sciomer, Francesco Ciciarello, Francesco Fedele, Carmine Dario Vizza and Roberto Badagliacca

J. Clin. Med. 2021, 10(4), 619; https://doi.org/10.3390/jcm10040619 - 6 Feb 2021

Cited by 17 | Viewed by 3229

Abstract

Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. The pathophysiologic model is mainly characterized by an afterload mismatch in which an increased right ventricle afterload, driven by increased pulmonary vascular resistance (PVR), leads to right heart failure. International guidelines recommend optimization of treatment based on regular risk assessments to achieve or maintain a low-risk status. Current risk scores are based on a multi-modality approach, including demographic, clinical, functional, exercise, laboratory, and hemodynamic parameters, which lack significant echocardiographic parameters. The originality of echocardiography relies on the opportunity to assess in a non-invasive way a physiologically meaningful combination of easy to measure variables tightly related to right ventricle adaptation/maladaptation to increased afterload, the main determinant of a patient’s prognosis. Echo-derived morphological and functional parameters have been investigated in PAH, proving to have prognostic relevance. Different therapeutic strategies proved to have different effects in reducing PVR. An upfront combination of drugs, including a parenteral prostacyclin, has shown to be associated with right heart reverse remodeling in a greater proportion of patients than other treatment strategies as a function of PVR reduction. Adding echocardiographic data to current risk scores would allow better identification of right ventricle (RV) adaptation in PAH patients’ follow-up. This additional information would allow better stratification of the patient, leading to optimized and personalized therapeutic management. Full article

(This article belongs to the Special Issue Pulmonary Arterial Hypertension and Beyond: Risk Assessment, Prognosis and Emerging Clinical Perspectives)

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