Journal of Clinical Medicine

Research

11 pages, 1303 KiB

Open AccessArticle

The First Pulmonary Hypertension Registry in the United Arab Emirates (UAEPH): Clinical Characteristics, Hemodynamic Parameters with Focus on Treatment and Outcomes for Patients with Group 1-PH

by Khaled Saleh, Naureen Khan, Kelly Dougherty, Govinda Bodi, Miriam Michalickova, Samiuddin Mohammed, Theodora Kerenidi, Ziad Sadik, Jihad Mallat, Samar Farha and Hani Sabbour

J. Clin. Med. 2023, 12(5), 1996; https://doi.org/10.3390/jcm12051996 - 2 Mar 2023

Cited by 4 | Viewed by 2165

Abstract

Background: The aim of this study is to present the first United Arab Emirates pulmonary hypertension registry of patients’ clinical characteristics, hemodynamic parameters and treatment outcomes. Method: This is a retrospective study describing all the adult patients who underwent a right heart catheterization [...] Read more.

Background: The aim of this study is to present the first United Arab Emirates pulmonary hypertension registry of patients’ clinical characteristics, hemodynamic parameters and treatment outcomes. Method: This is a retrospective study describing all the adult patients who underwent a right heart catheterization for evaluation of pulmonary hypertension (PH) between January 2015 and December 2021 in a tertiary referral center in Abu Dhabi, United Arab Emirates. Results: A total of 164 consecutive patients were diagnosed with PH during the five years of the study. Eighty-three patients (50.6%) were World Symposium PH Group 1-PH; nineteen patients (11.6%) were Group 2-PH due to left heart disease; twenty-three patients (14.0%) were Group 3-PH due to chronic lung disease; thirty-four patients (20.7%) were Group 4-PH due to chronic thromboembolic lung disease, and five patients (3.0%) were Group 5-PH. Among Group 1-PH, twenty-five (30%) had idiopathic, twenty-seven (33%) had connective tissue disease, twenty-six (31%) had congenital heart disease, and five patients (6%) had porto-pulmonary hypertension. The median follow-up was 55.6 months. Most of the patients were started on dual then sequentially escalated to triple combination therapy. The 1-, 3- and 5-year cumulative probabilities of survival for Group 1-PH were 86% (95% CI, 75–92%), 69% (95% CI, 54–80%) and 69% (95% CI, 54–80%). Conclusions: This is the first registry of Group 1-PH from a single tertiary referral center in the UAE. Our cohort was younger with a higher percentage of patients with congenital heart disease compared to cohorts from Western countries but similar to registries from other Asian countries. Mortality is comparable to other major registries. Adopting the new guideline recommendations and improving the availability and adherence to medications are likely to play a significant role in improving outcomes in the future. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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11 pages, 1748 KiB

Open AccessArticle

The Influence of Methods for Cardiac Output Determination on the Diagnosis of Precapillary Pulmonary Hypertension: A Mathematical Model

by Léon Genecand, Gaëtan Simian, Roberto Desponds, Julie Wacker, Silvia Ulrich, Benoit Lechartier, Jean-Marc Fellrath, Olivier Sitbon, Maurice Beghetti and Frédéric Lador

J. Clin. Med. 2023, 12(2), 410; https://doi.org/10.3390/jcm12020410 - 4 Jan 2023

Cited by 2 | Viewed by 1616

Abstract

Background: precapillary pulmonary hypertension (PH, PcPH) is now defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg, a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 2 WU. For PVR calculation, the measurement of [...] Read more.

Background: precapillary pulmonary hypertension (PH, PcPH) is now defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg, a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 2 WU. For PVR calculation, the measurement of cardiac output (CO) is necessary. It is generally measured using thermodilution. However, recent data showed that the agreement with direct Fick method, historically the gold standard, is less than previously reported. We aimed to create a mathematical model that calculated the probability of being classified differently (PcPH or unclassified PH) if CO measured by direct Fick was used instead of thermodilution for any individual patients with a mPAP > 20 mmHg and a PAWP ≤ 15 mmHg. Methods: The model is based on Bland and Altman analysis with a normally distributed difference of cardiac output, fixed 1.96 standard deviation of bias, bias and physiological cardiac output limits. Results: Following a literature review of the studies comparing CO measured with direct Fick and thermodilution, we fixed the 1.96 standard deviation of bias at 2 L/min, bias at 0 L/min and physiological resting CO limits between 1.3 L/min and 10.2 L/min. Conclusions: This model can help the clinician to evaluate the potential benefit of measuring CO using direct Fick during the diagnostic work-up and its utility in confirming or ruling out a diagnosis of PcPH in any given patient with a mPAP > 20 mmHg and a PAWP ≤ 15 mmHg. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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16 pages, 1446 KiB

Open AccessArticle

Prognostic Implications of the Novel Pulmonary Hypertension Definition in Patients with Aortic Stenosis after Transcatheter Valve Replacement

by Dionysios Adamopoulos, Stamatia Pagoulatou, Georgios Rovas, Vasiliki Bikia, Hajo Müller, Georgios Giannakopoulos, Sarah Mauler-Wittwer, Marc-Joseph Licker, Nikolaos Stergiopulos, Frédéric Lador and Stéphane Noble

J. Clin. Med. 2022, 11(15), 4279; https://doi.org/10.3390/jcm11154279 - 22 Jul 2022

Cited by 3 | Viewed by 1924

Abstract

Introduction: Pulmonary hypertension (PH), traditionally defined as a mean pulmonary artery pressure (PAP) ≥ 25 mmHg, is associated with poor outcomes in patients undergoing a transcatheter aortic valve replacement (TAVR) for severe aortic stenosis (AS). Recently, a novel definition for PH has [...] Read more.

Introduction: Pulmonary hypertension (PH), traditionally defined as a mean pulmonary artery pressure (PAP) ≥ 25 mmHg, is associated with poor outcomes in patients undergoing a transcatheter aortic valve replacement (TAVR) for severe aortic stenosis (AS). Recently, a novel definition for PH has been proposed, placing the cut-off value of mean PAP at 20 mmHg, and introducing pulmonary vascular resistance as an exclusive indicator for the pre-capillary involvement. In light of the novel criteria, whether PH still preserves its prognostic significance remains unknown. Methods: The study population consisted of 380 patients with AS, who underwent a right heart catheterization before TAVR. The cohort was divided according to the presence of PH (n = 174, 45.7%) or not. Patients with PH were further divided into the following groups: (1) Pre-capillary PH ((Pre-capPH), n = 46, 12.1%); (2) Isolated post-capillary PH ((IpcPH), n = 78, 20.5%); (3) Combined pre and post-capillary PH ((CpcPH), n = 82, 21.6%). The primary endpoint was all-cause mortality at 1 year. Results: A total of 246 patients (64.7%) exhibited mean PAP > 20 mmHg. Overall, the presence of PH was associated with higher 1-year mortality rates (hazard ratio (HR) 2.8, 95% CI: 1.4–5.8, p = 0.004). Compared to patients with no PH, Pre-capPH and CpcPH (but not IpcPH) were related to higher 1-year mortality (HR 2.7, 95% CI: 1.0–7.2, p = 0.041 and HR 3.9, 95% CI: 1.8–8.5, p = 0.001, respectively). This remained significant even after the adjustment for baseline comorbidities. Conclusions: Pre-interventional PH according to the novel hemodynamic criteria, is linked with poor outcomes in patients undergoing TAVR for severe AS. However, this is mainly driven by patients with mean PAP ≥ 25 mmHg. Patients with a pre-capillary PH component as defined by increased PVR present an even worse prognosis as compared to patients with isolated post-capillary or no PH who present comparable 1-year mortality rates. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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12 pages, 1675 KiB

Open AccessArticle

Cardiorespiratory Adaptation to Short-Term Exposure to Altitude vs. Normobaric Hypoxia in Patients with Pulmonary Hypertension

by Simon R. Schneider, Mona Lichtblau, Michael Furian, Laura C. Mayer, Charlotte Berlier, Julian Müller, Stéphanie Saxer, Esther I. Schwarz, Konrad E. Bloch and Silvia Ulrich

J. Clin. Med. 2022, 11(10), 2769; https://doi.org/10.3390/jcm11102769 - 14 May 2022

Cited by 10 | Viewed by 2614

Abstract

Prediction of adverse health effects at altitude or during air travel is relevant, particularly in pre-existing cardiopulmonary disease such as pulmonary arterial or chronic thromboembolic pulmonary hypertension (PAH/CTEPH, PH). A total of 21 stable PH-patients (64 ± 15 y, 10 female, 12/9 PAH/CTEPH) [...] Read more.

Prediction of adverse health effects at altitude or during air travel is relevant, particularly in pre-existing cardiopulmonary disease such as pulmonary arterial or chronic thromboembolic pulmonary hypertension (PAH/CTEPH, PH). A total of 21 stable PH-patients (64 ± 15 y, 10 female, 12/9 PAH/CTEPH) were examined by pulse oximetry, arterial blood gas analysis and echocardiography during exposure to normobaric hypoxia (NH) (F_iO₂ 15% ≈ 2500 m simulated altitude, data partly published) at low altitude and, on a separate day, at hypobaric hypoxia (HH, 2500 m) within 20–30 min after arrival. We compared changes in blood oxygenation and estimated pulmonary artery pressure in lowlanders with PH during high altitude simulation testing (HAST, NH) with changes in response to HH. During NH, 4/21 desaturated to SpO₂ < 85% corresponding to a positive HAST according to BTS-recommendations and 12 qualified for oxygen at altitude according to low SpO₂ < 92% at baseline. At HH, 3/21 received oxygen due to safety criteria (SpO₂ < 80% for >30 min), of which two were HAST-negative. During HH vs. NH, patients had a (mean ± SE) significantly lower PaCO₂ 4.4 ± 0.1 vs. 4.9 ± 0.1 kPa, mean difference (95% CI) −0.5 kPa (−0.7 to −0.3), PaO₂ 6.7 ± 0.2 vs. 8.1 ± 0.2 kPa, −1.3 kPa (−1.9 to −0.8) and higher tricuspid regurgitation pressure gradient 55 ± 4 vs. 45 ± 4 mmHg, 10 mmHg (3 to 17), all p < 0.05. No serious adverse events occurred. In patients with PH, short-term exposure to altitude of 2500 m induced more pronounced hypoxemia, hypocapnia and pulmonary hemodynamic changes compared to NH during HAST despite similar exposure times and P_iO₂. Therefore, the use of HAST to predict physiological changes at altitude remains questionable. (ClinicalTrials.gov: NCT03592927 and NCT03637153). Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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13 pages, 4610 KiB

Open AccessArticle

Non-Invasive Cardiac Output Determination Using Magnetic Resonance Imaging and Thermodilution in Pulmonary Hypertension

by Lindsey A. Crowe, Léon Genecand, Anne-Lise Hachulla, Stéphane Noble, Maurice Beghetti, Jean-Paul Vallée and Frédéric Lador

J. Clin. Med. 2022, 11(10), 2717; https://doi.org/10.3390/jcm11102717 - 11 May 2022

Cited by 3 | Viewed by 1854

Abstract

Magnetic resonance imaging (MRI) can be used to measure cardiac output (CO) non-invasively, which is a paramount parameter in pulmonary hypertension (PH) patients. We retrospectively compared stroke volume (SV) obtained with MRI (SV_MRI) in six localisations against SV measured with thermodilution [...] Read more.

Magnetic resonance imaging (MRI) can be used to measure cardiac output (CO) non-invasively, which is a paramount parameter in pulmonary hypertension (PH) patients. We retrospectively compared stroke volume (SV) obtained with MRI (SV_MRI) in six localisations against SV measured with thermodilution (TD) (SV_TD) and against each other in 24 patients evaluated in our PH centre using Bland and Altman (BA) agreement analyses, linear correlation, and intraclass correlation (ICC). None of the six tested localisations for SV_MRI reached the predetermined criteria for interchangeability with SV_TD, with two standard deviations (2SD) of bias between 24.1 mL/beat and 31.1 mL/beat. The SV_MRI methods yielded better agreement when compared against each other than the comparison between SV_MRI and SV_TD, with the best 2SD of bias being 13.8 mL/beat. The inter-observer and intra-observer ICCs for CO_MRI were excellent (inter-observer ICC between 0.889 and 0.983 and intra-observer ICC between 0.991 and 0.999). We could not confirm the interchangeability of SV_MRI with SV_TD based on the predetermined interchangeability criteria. The lack of agreement between MRI and TD might be explained because TD is less precise than previously thought. We evaluated a new method to estimate CO through the pulmonary circulation (COp) in PH patients that may be more precise than the previously tested methods. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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Review

Jump to: Research, Other

21 pages, 1555 KiB

Open AccessReview

Pressure Overload and Right Ventricular Failure: From Pathophysiology to Treatment

by Nicolas Dayer, Zied Ltaief, Lucas Liaudet, Benoit Lechartier, John-David Aubert and Patrick Yerly

J. Clin. Med. 2023, 12(14), 4722; https://doi.org/10.3390/jcm12144722 - 17 Jul 2023

Cited by 2 | Viewed by 5376

Abstract

Right ventricular failure (RVF) is often caused by increased afterload and disrupted coupling between the right ventricle (RV) and the pulmonary arteries (PAs). After a phase of adaptive hypertrophy, pressure-overloaded RVs evolve towards maladaptive hypertrophy and finally ventricular dilatation, with reduced stroke volume [...] Read more.

Right ventricular failure (RVF) is often caused by increased afterload and disrupted coupling between the right ventricle (RV) and the pulmonary arteries (PAs). After a phase of adaptive hypertrophy, pressure-overloaded RVs evolve towards maladaptive hypertrophy and finally ventricular dilatation, with reduced stroke volume and systemic congestion. In this article, we review the concept of RV-PA coupling, which depicts the interaction between RV contractility and afterload, as well as the invasive and non-invasive techniques for its assessment. The current principles of RVF management based on pathophysiology and underlying etiology are subsequently discussed. Treatment strategies remain a challenge and range from fluid management and afterload reduction in moderate RVF to vasopressor therapy, inotropic support and, occasionally, mechanical circulatory support in severe RVF. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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14 pages, 335 KiB

Open AccessReview

Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives

by Argyro Vraka, Eleni Diamanti, Mithum Kularatne, Patrick Yerly, Frédéric Lador, John-David Aubert and Benoit Lechartier

J. Clin. Med. 2023, 12(13), 4349; https://doi.org/10.3390/jcm12134349 - 28 Jun 2023

Cited by 1 | Viewed by 3354

Abstract

Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. It serves a prominent role in everyday patient care and can be determined using several validated risk assessment scores worldwide. The recently published 2022 European Society of Cardiology (ESC)/European Respiratory [...] Read more.

Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. It serves a prominent role in everyday patient care and can be determined using several validated risk assessment scores worldwide. The recently published 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines underline the importance of risk stratification not only at baseline but also during follow-up. Achieving a low-risk status has now become the therapeutic goal, emphasising the importance of personalised therapy. The application of these guidelines is also important in determining the timing for lung transplantation referral. In this review, we summarise the most relevant prognostic factors of PAH as well as the parameters used in PAH risk scores and their evolution in the guidelines over the last decade. Finally, we describe the central role that risk stratification plays in the current guidelines not only in European countries but also in Asian countries. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

Other

Jump to: Research, Review

9 pages, 594 KiB

Open AccessBrief Report

Compression Stockings Improve Lower Legs Symptom in Patients with Pulmonary Artery Hypertension Treated by Pulmonary Vasodilators—A Pilot Study

by Naoko Nakashima, Nobuhiro Tahara, Yoichi Sugiyama, Munehisa Bekki, Shoko Maeda-Ogata, Akihiro Honda, Chidu Goto, Setsuko Tanaka and Yoshihiro Fukumoto

J. Clin. Med. 2023, 12(7), 2484; https://doi.org/10.3390/jcm12072484 - 24 Mar 2023

Cited by 1 | Viewed by 1375

Abstract

Pulmonary vasodilators have improved pulmonary arterial hypertension (PAH) symptoms and prognosis; however, the drugs cause some side effects, including lower legs pain, which impair quality of life (QOL). The present study examined if compression stockings improved lower extremity symptoms and QOL caused by [...] Read more.

Pulmonary vasodilators have improved pulmonary arterial hypertension (PAH) symptoms and prognosis; however, the drugs cause some side effects, including lower legs pain, which impair quality of life (QOL). The present study examined if compression stockings improved lower extremity symptoms and QOL caused by pulmonary vasodilators in PAH patients. We retrospectively enrolled consecutively ten patients with PAH treated by pulmonary vasodilators, who were regularly followed in Kurume University Hospital from January 2022 to June 2022. Oral questionnaire surveys, the Numeric Rating Scale for Pain (NRS) and the Pain Disability Assessment Scale (PDAS), were conducted regarding lower extremity symptoms before wearing elastic stockings and one month later, to evaluate how the lower extremity symptoms affected daily life. All ten patients were female, with a mean age of 50.2 ± 11.5 years, out of whom intravenous prostacyclin analogue (PGI₂) was administered in five patients. In no intravenous PGI₂ group, NRS score was significantly improved from 4.6 ± 2.3 to 2.8 ± 1.2 (p = 0.037), while from 9.4 ± 1.2 to 5.4 ± 1.6 (p = 0.002) in intravenous PGI₂ group. PDAS score was also significantly improved [no intravenous PGI₂ group; 18.0 (15.0–24.0) to 15.0 (10.0–19.0), intravenous PGI₂ group; 25.0 (17.0–37.0) to 17.0 (5.0–27.0)]. Lower extremity symptoms in patients using pulmonary vasodilators were improved by wearing compression stockings. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Pathophysiology, Innovative Exploration and Therapies)

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