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Medicina
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Diagnosis and Treatment of Pulmonary Hypertension
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Diagnosis and Treatment of Pulmonary Hypertension

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (31 August 2022) | Viewed by 20697

Special Issue Editors

Dr. Pavel Jansa

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Guest Editor
1st Faculty of Medicine, 2nd Department of Medicine-Department of Cardiovascular Medicine, General University Hospital, Charles University, Prague, Czech Republic
Interests: epidemiology of pulmonary arterial hypertension; coagulation disorders in pulmonary hypertension; small-vessel disease in chronic thromboembolic pulmonary hypertension; pulmonary hypertension
Prof. Dr. Iveta Simkova

E-Mail Website
Guest Editor
Cardiology and Angiology Clinic, School of Medicine, Slovak Medical University and National Institute of Cardiovascular Diseases, 83348 Bratislava, Slovakia
Interests: valvular and congenital heart disease; pulmonary circulation-pulmonary hypertension- pulmonary embolism-right heart failure

Special Issue Information

Dear Colleagues,

Pulmonary hypertension includes conditions characterized by an increase in mean pulmonary artery pressure, which is most common in heart or lung disease. Pulmonary hypertension is infrequently caused by primary pulmonary vascular involvement, particularly pulmonary arterial hypertension (PAH) in pulmonary arteriolar involvement and chronic thromboembolic pulmonary hypertension (CTEPH) associated with insufficient reperfusion after acute pulmonary embolism.

PAH is a chronic progressive disease leading to right heart failure and ultimately death if untreated. It is characterized by the presence of precapillary pulmonary hypertension, defined by an increase in mean pulmonary artery pressure of ≥25 mm, pulmonary artery wedge pressure of ≤15 mmHg, and a pulmonary vascular resistance of ˃3 Wood units. A revision of this definition was recently proposed, recommending the lowering of the mean pulmonary artery pressure threshold from ≥25 mmHg to >20 mmHg. The reason for this proposed change is that accumulating evidence has taught us that patients with a mean pulmonary artery pressure between 21 and 24 mmHg have an increased risk to progress to ≥25 mmHg and have a higher mortality than patients with a mean pulmonary artery pressure of ≤20 mmHg.

Pharmacotherapy dominates the treatment of PAH. These therapies individually target three signaling pathways known to be involved in the pathogenesis of PAH: the endothelin (ET), nitric oxide, and prostacyclin pathways. The progressive nature of PAH provides a rationale for early and intensive management and the use of combination therapy to target multiple pathways simultaneously. Combination therapy is an important component of current PAH management strategies, which aim for patients to reach or maintain a low risk for mortality, the defined treatment goal in PAH.

CTEPH is a chronic complication of acute pulmonary embolism caused by the formation of persistent thrombotic obstructions in pulmonary vessels and concurrent peripheral vascular remodeling. Surgical pulmonary endarterectomy (PEA) is an established standard treatment that is indicated for 50–70% of patients with confirmed CTEPH. In inoperable patients and in patients with residual pulmonary hypertension after PEA, pharmacological therapy with riociguat (a soluble guanylate cyclase stimulator) and recently approved subcutaneous treprostinil (a prostacycline analogue) is successful if peripheral vascular remodeling is the predominant cause. Balloon pulmonary angioplasty (BPA) is an interventional treatment option for CTEPH patients not eligible for PEA or with residual pulmonary hypertension after PEA. Multimodal therapy with the combination of PEA, BPA, and/or pharmacological therapy is considered in an increasing number of patients to completely cure CTEPH.

Despite success in the understanding of pulmonary hypertension pathophysiology and treatment development over the last few decades, many questions are still unresolved—our understanding of pulmonary hypertension remains incomplete, specific therapy of the most common types of pulmonary hypertension (in heart or lung disease) is not available, PAH remains a life-limiting illness with no prospect of cure, and curative treatment of CTEPH is not available in many countries. The aim of this Special Issue of Medicina is to collect papers reflecting further progress in this field.

Dr. Pavel Jansa
Prof. Dr. Iveta Simkova
Guest Editors

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Keywords

  • pulmonary hypertension
  • pulmonary arterial hypertension
  • chronic thromboembolic pulmonary hypertension
  • pharmacotherapy
  • combination therapy
  • pulmonary endarterectomy
  • balloon pulmonary angioplasty

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Published Papers (7 papers)

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10 pages, 1172 KiB  
Article
Central versus Peripheral CTEPH—Clinical and Hemodynamic Specifications
by Monika Kaldararova, Iveta Simkova, Marcela Bohacekova, Adriana Reptova, Tereza Hlavata, Jozef Pacak, Jaroslav Lindner and Pavel Jansa
Medicina 2022, 58(11), 1538; https://doi.org/10.3390/medicina58111538 - 27 Oct 2022
Cited by 3 | Viewed by 1931
Abstract
Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic progressive disease, resulting from persistent arterial obstruction combined with small-vessel remodeling. Central and peripheral CTEPH are distinguished, according to the dominant lesion’s location. This is important for surgical or percutaneous interventional assessment [...] Read more.
Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic progressive disease, resulting from persistent arterial obstruction combined with small-vessel remodeling. Central and peripheral CTEPH are distinguished, according to the dominant lesion’s location. This is important for surgical or percutaneous interventional assessment or for medical treatment. Material and Methods: Eighty-one patients (51 male/30 female) with confirmed CTEPH were analyzed, while the CENTRAL type included 51 patients (63%) and the PERIPHERAL type 30 patients (37%). Results: A significant difference in CENTRAL type vs. PERIPHERAL type was determined in gender (male 72.5% vs. 46.7%; p = 0.0198). No difference was found in age, functional status, or echocardiographic parameters. Invasive hemodynamic parameters showed a significant difference in mean pulmonary arterial pressure (46 vs. 58 mmHg; p = 0.0002), transpulmonary gradient (34 vs. 47 mmHg; p = 0.0005), and cardiac index (2.04 vs. 2.5 L.min.m2; p = 0.02) but not in pulmonary vascular resistance. Risk factors showed a significant difference only in acute pulmonary embolism (93.8% vs. 60%; p = 0.0002) and malignancy (2% vs. 13.3%; p = 0.0426). Conclusions: Our study showed hemodynamic differences between CENTRAL type vs. PERIPHERAL type CTEPH with a worse hemodynamic picture in CENTRAL form. This may indicate a different pathophysiological response and/or possible additional influences contributing especially to the peripheral pulmonary bed affection. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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13 pages, 1334 KiB  
Article
Chronic Thromboembolic Pulmonary Hypertension: An Observational Study
by Barbara Ruaro, Paola Confalonieri, Gaetano Caforio, Elisa Baratella, Riccardo Pozzan, Stefano Tavano, Chiara Bozzi, Selene Lerda, Pietro Geri, Marco Biolo, Maurizio Cortale, Marco Confalonieri and Francesco Salton
Medicina 2022, 58(8), 1094; https://doi.org/10.3390/medicina58081094 - 13 Aug 2022
Cited by 16 | Viewed by 2896
Abstract
Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) has a high mortality. The treatment of CTEPH could be balloon pulmonary angioplasty (BPA), medical (MT) or pulmonary endarterectomy (PEA). This study aims to assess the clinical characteristics of CTEPH patients, surgically or medically treated, [...] Read more.
Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) has a high mortality. The treatment of CTEPH could be balloon pulmonary angioplasty (BPA), medical (MT) or pulmonary endarterectomy (PEA). This study aims to assess the clinical characteristics of CTEPH patients, surgically or medically treated, in a pulmonology referral center. Materials and Methods: A total of 124 patients with PH with suspected CTEPH (53 male subjects and 71 female subjects; mean age at diagnosis 67 ± 6) were asked to give informed consent and then were evaluated. The presence of CTEPH was ascertained by medical evaluations, radiology and laboratory tests. Results: After the evaluation of all clinical data, 65 patients met the inclusion criteria for CTEPH and they were therefore enrolled (22 males and 43 females; mean age at diagnosis was 69 ± 8). 26 CTEPH patients were treated with PEA, 32 with MT and 7 with BPA. There was a statistically significant age difference between the PEA and MT groups, at the time of diagnosis, the PEA patients were younger than the MT patients, whereas there was no statistically significant difference in other clinical characteristics (e.g., smoking habit, thrombophilia predisposition), as well as functional and hemodynamic parameters (e.g., 6-min walk test, right heart catheterization). During three years of follow-up, no patients in the PEA groups died; conversely, eleven patients in the MT group died during the same period (p < 0.05). Furthermore, a significant decrease in plasma BNP values and an increase in a meter at the six-minute walk test, 1 and 3 years after surgery, were observed in the PEA group (p < 0.05). Conclusions: This study seems to confirm that pulmonary endarterectomy (PEA) can provide an improvement in functional tests in CTEPH. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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13 pages, 336 KiB  
Article
Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals
by Virginija Rudienė, Lina Kaplerienė, Dovilė Jančauskaitė, Emilija Meškėnė, Eglė Palevičiūtė, Monika Laukytė-Slėnienė, Diana Gasiūnaitė, Diana Ramašauskaitė, Elena Jurevičienė and Lina Gumbienė
Medicina 2022, 58(4), 476; https://doi.org/10.3390/medicina58040476 - 25 Mar 2022
Cited by 4 | Viewed by 3261
Abstract
Background and Objectives: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available [...] Read more.
Background and Objectives: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients’ well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. Materials and Methods: The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. Results: Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. Conclusions: On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
14 pages, 1491 KiB  
Article
Effects of Mean Artery Pressure and Blood pH on Survival Rate of Patients with Acute Kidney Injury Combined with Acute Hypoxic Respiratory Failure: A Retrospective Study
by Chi-Hua Ko, Ying-Wei Lan, Ying-Chou Chen, Tien-Tsai Cheng, Shan-Fu Yu, Abdulkadir Cidem, Yu-Hsien Liu, Chia-Wen Kuo, Chih-Ching Yen, Wei Chen and Chuan-Mu Chen
Medicina 2021, 57(11), 1243; https://doi.org/10.3390/medicina57111243 - 14 Nov 2021
Cited by 8 | Viewed by 3072
Abstract
Background and Objectives: In the intensive care unit (ICU), renal failure and respiratory failure are two of the most common organ failures in patients with systemic inflammatory response syndrome (SIRS). These clinical symptoms usually result from sepsis, trauma, hypermetabolism or shock. If this [...] Read more.
Background and Objectives: In the intensive care unit (ICU), renal failure and respiratory failure are two of the most common organ failures in patients with systemic inflammatory response syndrome (SIRS). These clinical symptoms usually result from sepsis, trauma, hypermetabolism or shock. If this syndrome is caused by septic shock, the Surviving Sepsis Campaign Bundle suggests that vasopressin be given to maintain mean arterial pressure (MAP) > 65 mmHg if the patient is hypotensive after fluid resuscitation. Nevertheless, it is important to note that some studies found an effect of various mean arterial pressures on organ function; for example, a MAP of less than 75 mmHg was associated with the risk of acute kidney injury (AKI). However, no published study has evaluated the risk factors of mortality in the subgroup of acute kidney injury with respiratory failure, and little is known of the impact of general risk factors that may increase the mortality rate. Materials and Methods: The objective of this study was to determine the risk factors that might directly affect survival in critically ill patients with multiple organ failure in this subgroup. We retrospectively constructed a cohort study of patients who were admitted to the ICUs, including medical, surgical, and neurological, over 24 months (2015.1 to 2016.12) at Chiayi Chang Gung Memorial Hospital. We only considered patients who met the criteria of acute renal injury according to the Acute Kidney Injury Network (AKIN) and were undergoing mechanical ventilator support due to acute respiratory failure at admission. Results: Data showed that the overall ICU and hospital mortality rate was 63.5%. The most common cause of ICU admission in this cohort study was cardiovascular disease (31.7%) followed by respiratory disease (28.6%). Most patients (73%) suffered sepsis during their ICU admission and the mean length of hospital stay was 24.32 ± 25.73 days. In general, the factors independently associated with in-hospital mortality were lactate > 51.8 mg/dL, MAP ≤ 77.16 mmHg, and pH ≤ 7.22. The risk of in-patient mortality was analyzed using a multivariable Cox regression survival model. Adjusting for other covariates, MAP ≤ 77.16 mmHg was associated with higher probability of in-hospital death [OR = 3.06 (1.374–6.853), p = 0.006]. The other independent outcome predictor of mortality was pH ≤ 7.22 [OR = 2.40 (1.122–5.147), p = 0.024]. Kaplan-Meier survival curves were calculated and the log rank statistic was highly significant. Conclusions: Acute kidney injury combined with respiratory failure is associated with high mortality. High mean arterial pressure and normal blood pH might improve these outcomes. Therefore, the acid–base status and MAP should be considered when attempting to predict outcome. Moreover, the blood pressure targets for acute kidney injury in critical care should not be similar to those recommended for the general population and might prevent mortality. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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11 pages, 3305 KiB  
Case Report
Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series
by Tereza Hlavata, Monika Kaldararova, Filip Klauco, Erika Drangova, Adriana Reptova and Iveta Simkova
Medicina 2022, 58(10), 1484; https://doi.org/10.3390/medicina58101484 - 19 Oct 2022
Cited by 2 | Viewed by 2871
Abstract
Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and [...] Read more.
Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and causes an imbalance between vasodilators and vasoconstrictors in the pulmonary circulation, which, again, might lead to the development of secondary portopulmonary hypertension (PoPH). Establishing the exact morphology of the splanchnic venous system is important when evaluating possible therapeutic options (differentiating type I and II CAPV), because some variants enable the closure of the shunt, and this represents a potential cure for pulmonary arterial hypertension (PAH). Once PoPH is diagnosed, complex care in a specialized expert centre is necessary. If possible, CPSS closure is recommended. For long-term successful patient management, specific targeted PAH therapy administration is crucial. Significant morbidity and mortality in these patients may result not only from PAH itself but also due to specific PoPH complications, such as compression of the left main coronary artery by pulmonary artery aneurysm. Case Report. We report on two patients with PoPH due to CAPV and CPSS (without any liver disease) who presented as severe PAH and who, before admission to our expert centre, were misdiagnosed as idiopathic PAH. The case reports also represent our experience with respect to the long-term follow-up and PAH-specific medical treatment of these patients, as well as the possible (even fatal) complications of these rare and complex patients. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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8 pages, 3589 KiB  
Case Report
The Treatment Strategy for the Atrial Septal Defect in the Presence of Severe Pulmonary Hypertension
by Evelina Zarambaitė, Grytė Ramantauskaitė, Aušra Krivickienė, Adakrius Siudikas, Skaidrius Miliauskas and Eglė Ereminienė
Medicina 2022, 58(7), 892; https://doi.org/10.3390/medicina58070892 - 2 Jul 2022
Cited by 2 | Viewed by 3264
Abstract
Atrial septal defect is one of the most common congenital heart diseases in adults. The defect often leads to volume overload in the right heart coupled with the potential risk of right heart failure and pulmonary arterial hypertension. These conditions lead to worsening [...] Read more.
Atrial septal defect is one of the most common congenital heart diseases in adults. The defect often leads to volume overload in the right heart coupled with the potential risk of right heart failure and pulmonary arterial hypertension. These conditions lead to worsening in quality of life, decrease in physical capacity, and even to fatal outcomes. The main strategy for treatment of atrial septal defect is a transcatheter or surgical closure of the defect, but in patients with severe pulmonary arterial hypertension, it is recommended to manage pulmonary arterial hypertension and after that treat the defect invasively. This strategy is called “treat and repair” strategy. We present an illustrative case report of management and treatment of atrial septal defect, complicated with severe pulmonary arterial hypertension. In this case, surgical closure of the defect was contraindicated because of the high pulmonary vascular resistance. Therefore, the “treat and repair” strategy was approached. After specific medical treatment of pulmonary arterial hypertension, surgical closure of the defect was chosen and proven successful. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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4 pages, 584 KiB  
Case Report
Giant Pulmonary Artery Thrombotic Material, Due to Chronic Thromboembolic Pulmonary Hypertension, Mimics Pulmonary Artery Sarcoma
by Helen Triantafyllidi, Dimitrios Iordanidis, Aikaterini Mpahara, Maria Mademli, Dionyssia Birmpa, Stylianos Argentos, Dimitrios Benas, Paraskevi Trivilou, Konstantinos Anagnostopoulos and Eckhard Mayer
Medicina 2021, 57(9), 992; https://doi.org/10.3390/medicina57090992 - 20 Sep 2021
Cited by 1 | Viewed by 2262
Abstract
In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery [...] Read more.
In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery sarcoma (PAS) due to extensive filling defects and extraluminal findings. Although surgery was postponed for nine months due to the COVID-19 pandemic, CTEPH diagnosis, due to a high-thrombus burden, was finally confirmed after pulmonary endarterectomy (PEA). Conclusively, imaging findings of rare cases of CTEPH might mimic PAS and the surgical removal of the lesion are both needed for a final diagnosis. What is Already Known about This Topic? Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy, which originates from the intimal layer of the pulmonary artery (PA); Chronic thromboembolic pulmonary hypertension (CTEPH) is based on chronic, organized flow-limiting thrombi inside PA circulation and subsequent pulmonary hypertension. What Does This Study Contribute? Since radiological findings of CTEPH cases might rarely mimic PAS, pulmonary artery endarterectomy and subsequent histopathologic study are needed for a final diagnosis. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Pulmonary Hypertension)
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