Myo-inositol trispyrophosphate prevents right ventricular failure and improves survival in monocrotaline-induced pulmonary hypertension in the rat.

BACKGROUND AND PURPOSE: Pulmonary hypertension (PH) results from pulmonary vasculopathy, initially leading to a compensatory right ventricular (RV) hypertrophy, and eventually to RV failure. Hypoxia can trigger both pulmonary vasculopathy and RV failure. Therefore, we tested if myo-inositol trispyrophosphate (ITPP), which facilitates oxygen dissociation from haemoglobin, can relieve pulmonary vasculopathy and RV hypoxia, and eventually prevent RV failure and mortality in the rat model of monocrotaline-induced PH. EXPERIMENTAL APPROACH: Rats were injected with monocrotaline (PH) or saline (control) and received ITPP or placebo for 5 weeks. Serial echocardiograms were obtained to monitor the disease, pressure-volume loops were recorded and evaluated, myocardial pO2 was measured using a fluorescent probe, and histological and molecular analyses were conducted at the conclusion of the experiment. KEY RESULTS AND CONCLUSIONS: ITPP reduced PH-related mortality. It had no effect on progressive increase in pulmonary vascular resistance, yet significantly relieved intramyocardial RV hypoxia, which was associated with improvement of RV function and reduction of RV wall stress. ITPP also tended to prevent increased hypoxia inducible factor-1α expression in RV cardiac myocytes but did not affect RV capillary density. IMPLICATIONS: Our study suggests that strategies aimed at increasing oxygen delivery to hypoxic RV in PH could potentially be used as adjuncts to other therapies that target pulmonary vessels, thus increasing the ability of the RV to withstand increased afterload and reducing mortality. ITPP may be one such potential therapy.

Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.

BACKGROUND: Sarcoidosis-associated pulmonary hypertension (SAPH) is listed in Group 5 of the clinical classification of pulmonary hypertension, due to its complex and multifactorial pathophysiology. The most common cause of SAPH development is advanced lung fibrosis with the associated destruction of the vascular bed, and/or alveolar hypoxia. However, a substantial proportion of SAPH patients (up to 30%) do not have significant fibrosis on chest imaging. In such cases, the development of pulmonary hypertension may be due to the lesions directly affecting the pulmonary vasculature, such as granulomatous angiitis, pulmonary veno-occlusive disease, chronic thromboembolism or external compression of vessels by enlarged lymph nodes. Based on the case of a 69-year-old female who developed SAPH due to pulmonary arteries stenosis, diagnostic difficulties and therapeutic management are discussed. CASE PRESENTATION: The patient, non-smoking female, diagnosed with stage II sarcoidosis twelve years earlier, presented with progressive dyspnoea on exertion, dry cough, minor haemoptysis and increasing oedema of the lower limbs. Computed tomography pulmonary angiography (CTPA) showed complete occlusion of the right upper lobe artery and narrowing of the left lower lobe artery, with post-stenotic dilatation of the arteries of the basal segments. The vascular pathology was caused by adjacent, enlarged lymph nodes with calcifications and fibrotic tissue surrounding the vessels. Pulmonary artery thrombi were not found. The patient was treated with systemic corticosteroid therapy and subsequently with balloon pulmonary angioplasty. Partial improvement in clinical status and hemodynamic parameters has been achieved. CONCLUSIONS: An appropriate screening strategy is required for early detection of pulmonary hypertension in sarcoidosis patients. Once SAPH diagnosis is confirmed, it is crucial to determine the appropriate phenotype of pulmonary hypertension and provide the most effective treatment plan. Although determining SAPH phenotype is challenging, one should remember about the possibility of pulmonary arteries occlusion.

Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension after the Removal of COVID-19 Pandemic Restrictions.

Background: There was increased risk of mental disturbances during the COVID-19 pandemic. Patients with chronic diseases, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), were particularly vulnerable. Our previous study showed high levels of fear of COVID-19 (FCV-19S), anxiety (HADS-A), and depression (HADS-D) in the second year of the pandemic among PAH/CTEPH patients. The aim of the present study was to assess changes in the levels of FCV-19S, HADS-A, and HADS-D after removing restrictions related to the COVID-19 pandemic. Methods: In this prospective, single-center study, 141 patients (62% females, 64% PAH) with a median age of 60 (range 42-72) years were included. Patients completed appropriate surveys in the second year of the pandemic, and then, after the restrictions were lifted in Poland (after 28 March 2022). Results: FVC-19S decreased significantly from 18 (12-23) to 14 (9-21), p < 0.001. The levels of anxiety (HADS-A ≥ 8 points) and depression (HADS-D ≥ 8 points) were abnormal in 26% and 16% of patients, respectively; these did not change at follow-up (p = 0.34 for HADS-A and p = 0.39 for HADS-D). Conclusions: Among PAH/CTEPH patients, fear of COVID-19 decreased significantly after the COVID-19 pandemic restrictions were removed, but anxiety and depression remained high, indicating that the COVID-19 pandemic was not a major factor in causing these disorders.

Mixed Reality Biopsy Navigation System Utilizing Markerless Needle Tracking and Imaging Data Superimposition.

Exact biopsy planning and careful execution of needle injection is crucial to ensure successful procedure completion as initially intended while minimizing the risk of complications. This study introduces a solution aimed at helping the operator navigate to precisely position the needle in a previously planned trajectory utilizing a mixed reality headset. A markerless needle tracking method was developed by integrating deep learning and deterministic computer vision techniques. The system is based on superimposing imaging data onto the patient's body in order to directly perceive the anatomy and determine a path from the selected injection site to the target location. Four types of tests were conducted to assess the system's performance: measuring the accuracy of needle pose estimation, determining the distance between injection sites and designated targets, evaluating the efficiency of material collection, and comparing procedure time and number of punctures required with and without the system. These tests, involving both phantoms and physician participation in the latter two, demonstrated the accuracy and usability of the proposed solution. The results showcased a significant improvement, with a reduction in number of punctures needed to reach the target location. The test was successfully completed on the first attempt in 70% of cases, as opposed to only 20% without the system. Additionally, there was a 53% reduction in procedure time, validating the effectiveness of the system.

The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia.

Background: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent pulmonary hypertension after pulmonary endarterectomy (PEA) include targeted medical therapy and balloon pulmonary angioplasty (BPA). BPA is an emerging treatment modality that has been reported to improve functional capacity, pulmonary hemodynamics, and right ventricular function. Reports from expert centers are promising, but more data are needed to make the results more generalizable. Materials and Methods: We conducted a prospective analysis of nine consecutive CTEPH patients who underwent balloon pulmonary angioplasty (BPA) sessions at Pauls Stradins Clinical University Hospital in Riga, Latvia between 1 April 2022 and 1 July 2023. We assessed World Health Organization (WHO) functional class, 6 min walk distance (6MWD), blood oxygen saturation (SpO2), brain natriuretic peptide (BNP) level at baseline and 3 months after the first BPA session. For two patients on whom repeated BPA sessions were performed, we additionally assessed cardiac output (CO), pulmonary vascular resistance (PVR), and mean pulmonary artery pressure (mPAP). Results: A total of 12 BPA procedures for nine patients were performed; repeated BPA sessions were performed for two patients. Our results show a reduction in BNP levels and improvement in WHO functional class, 6MWD, and SpO2 after the first BPA session. Improvement in 6MWD was statistically significant. Additionally, an improvement in pulmonary hemodynamic parameters was observed. Conclusions: Our data show that BPA is an effective interventional treatment modality, improving both the pulmonary hemodynamics and functional status. Moreover, BPA is safe and excellently tolerated.

Safety and Outcomes of Inferior Vena Cava Filter Placement in Oncology Patients: A Single-Centre Experience.

The risk of venous thromboembolism (VTE) in the oncology population is significantly higher than in non-cancer patients. Inferior vena cava (IVC) filters may, therefore, be an important part of VTE treatment. In this study, we address the outcomes of placing IVC filters in the oncology population. This single-centre, observational, retrospective study included 62 patients with active malignancy and acute VTE who underwent an IVC filter implantation due to contraindications to anticoagulation during the period 2012-2023. The control group consisted of 117 trauma patients. In both groups, an urgent surgical procedure requiring temporary cessation of anticoagulation was the most noted reason for IVC filter placement-76% in the oncology group vs. 100% in the non-oncology group (p < 0.001). No complications were reported during the IVC filter implantation procedures. There was no recurrence of pulmonary embolism or deep venous thrombosis in the oncology group after filter implantation. The rate of successful filter explantation, median time to retrieval, and abnormal findings during retrieval were not significantly different between both subgroups (64.3% vs. 76.5%, p = 0.334; 77 days vs. 84 days, p = 0.764; 61.5% vs. 54.2%, p = 0.672; respectively). The study showed that IVC filter placement is a safe and effective method of preventing PE in cancer patients with contraindications to anticoagulation. The complication rate following IVC filter implantation in cancer patients is low and similar to that in non-oncology patients.

Chronic thromboembolic disease among patients undergoing surgical pulmonary embolectomy for acute pulmonary embolism.

BACKGROUND: This study aimed to assess the prevalence of chronic thromboembolic lesions in the pulmonary arteries among patients undergoing pulmonary embolectomy for acute pulmonary embolism and their impact on treatment outcomes. METHODS: We conducted a retrospective, single-center analysis of consecutive patients undergoing emergency pulmonary embolectomy for acute pulmonary embolism between 2013 and August 2021. According to European Society of Cardiology guidelines, the diagnosis was based on clinical presentation, imaging studies and laboratory tests. Surgery was selected as the optimal treatment modality within the Pulmonary Embolism Response Team. Based on the intraoperatively identified chronic lesions patients were divided into two groups: acute only and acute/chronic. The analysis comprised history, laboratory and imaging studies, early and long-term mortality, and postoperative complications. We determined predictive factors for chronic thromboembolic lesions and risk factors for death. RESULTS: The analysis included 33 patients. Intraoperatively, 42% (14) of patients had chronic lesions. Predictive factors for these lesions are the duration of symptoms >1 week (OR=13.75), pulmonary artery dilatation >3.15 cm (OR=39.00) and right ventricle systolic pressure >52 mmHg (OR=29.33). No hospital deaths occurred in the acute only group and two in the acute/chronic group (0% vs. 14.3%; P=0.172). Risk factors for death are the duration of symptoms >3 weeks (HR=7.35) and postoperative use of extracorporeal membrane oxygenation (HR=7.04). CONCLUSIONS: Acute thromboembolic disease overlapping chronic clots is relatively common among patients undergoing pulmonary artery embolectomy. A detailed evaluation of the patient's medical history and imaging studies can identify these patients, as they require special attention when making treatment decisions. Surgical treatment in a center of expertise in pulmonary endarterectomy seems reasonable.

Role of Oxygen Starvation in Right Ventricular Decompensation and Failure in Pulmonary Arterial Hypertension.

Right ventricular (RV) function and eventually failure determine outcome in patients with pulmonary arterial hypertension (PAH). Initially, RV responds to an increased load caused by PAH with adaptive hypertrophy; however, eventually RV failure ensues. Unfortunately, it is unclear what causes the transition from compensated RV hypertrophy to decompensated RV failure. Moreover, at present, there are no therapies for RV failure; those for left ventricular (LV) failure are ineffective, and no therapies specifically targeting RV are available. Thus there is a clear need for understanding the biology of RV failure and differences in physiology and pathophysiology between RV and LV that can ultimately lead to development of such therapies. In this paper, we discuss RV adaptation and maladaptation in PAH, with a particular focus of oxygen delivery and hypoxia as the principal drivers of RV hypertrophy and failure, and attempt to pinpoint potential sites for therapy.

The Utility of a Resting Electrocardiogram (ECG-PH Index) in Evaluating the Efficacy of Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: The ECG-PH index (PH-ECG score) has been proposed as a valuable ECG-derived method of evaluating the effectiveness of balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary endarterectomy (PEA) is the main form of therapy for CTEPH with a proximal clot location. The objective of this study was to assess the clinical utility of a resting electrocardiogram (ECG-PH index) in assessing the effectiveness of PEA in CTEPH patients. METHODS: The retrospective analysis included 73 patients who underwent PEA. Their ECG-PH index values were calculated using four ECG parameters: R-wave amplitude V1 + S-wave amplitude V5/V6 > 10.5 mm, QRS-wave axis > 110 degrees, R-wave amplitude V1 > S-wave amplitude V1, and SIQIII pattern. PH-ECG scores were assessed after a median time of 13 months (IQR: 8-31 months) had passed since the PEA procedures. RESULTS: The current analysis documented that ECG-PH index = 0 is a good reflection of mPAP < 25mmHg (sensitivity 76.1%; specificity 66.7%; positive predictive value 79.5%; negative predictive value 62.1%) or mPAP ≤ 20 mmHg (sensitivity 69.6%; specificity 70.6%; positive predictive value 88.6%; negative predictive value 41.4%) after PEA. The values of the area under the ROC curve for ECG-PH index were 0.772 (95% CI: 0.676-0.867) and 0.743 (95% CI: 0.637-0.849) for the mPAP < 25 mmHg and mPAP ≤ 20 mmHg patient groups, respectively. CONCLUSION: The ECG-PH index may be useful for monitoring the haemodynamic effect of PEA in CTEPH patients.

Emerging Role of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease-Insights from the 2022 ESC Guidelines.

In this article, we discuss the topic of chronic thromboembolic pulmonary disease (CTEPD) and the growing role of balloon pulmonary angioplasty (BPA) in its treatment. We present the pathophysiology of CTEPD which arises from an incomplete resolution of thrombi in the pulmonary arteries and leads to stenosis and occlusion of the vessels. The article focuses mainly on the chronic thromboembolic pulmonary hypertension (CTEPH) subpopulation for which prognosis is very poor when left untreated. We describe a multimodal approach to treating CTEPH, including pulmonary endarterectomy (PEA), BPA, and pharmacological therapies. Additionally, the benefits of pharmacological pre-treatment before BPA and the technical aspects of the procedure itself are outlined. It is emphasized that BPA does not replace PEA but serves as a complementary treatment option for eligible patients. We summarized efficacy and treatment goals including an improvement in functional and biochemical parameters before and after BPA. Patients who received pre-treatment with riociguat prior to BPA exhibited a notable reduction in the occurrence of less severe complications. However, elderly patients are still perceived as an especially vulnerable group. It is shown that the prognosis of patients undergoing BPA is similar to PEA in the first years after the procedure but the long-term prognosis of BPA still remains unclear. The 2022 ESC/ERS guidelines highlight the significant role of BPA in the multimodal treatment of CTEPH, emphasizing its effectiveness and recommending its consideration as a therapeutic option for patients with CTEPD, both with and without pulmonary hypertension. This review summarizes the available evidence for BPA, patient selection, procedural details, and prognosis and discusses the potential future role of BPA in the management of CTEPH.

Case report: Transected Hickman catheter and its thrombotic occlusion in a patient with idiopathic pulmonary arterial hypertension-can a catheter replacement be avoided?

A 25-year-old female with idiopathic pulmonary arterial hypertension (PAH), who had a Hickman catheter implanted for continuous intravenous epoprostenol infusion, was admitted to the clinic after inadvertently cutting the catheter with nail scissors during a routine dressing change. Approximately 7 cm of the external segment of the Hickman catheter remained intact, with the distal end knotted by paramedics. A decision was made to repair the damaged Hickman catheter. However, it was discovered that its lumen was completely occluded by thrombosis. Therefore, catheter patency was mechanically restored using a 0.035-inch stiff guidewire in a sterile operating theatre setting, under fluoroscopy guidance. Successful aspiration and catheter flushing were achieved. Continuity of the Hickman catheter was then restored using a repair kit (Bard Access Systems) as per the manufacturer's instructions, with no visible leakage thereafter. Epoprostenol infusion through the Hickman catheter was resumed 24 h later, and the patient was discharged in good general condition two days afterward.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines.

The Diagnostic Approach to Pulmonary Hypertension.

The clinical presentation of pulmonary hypertension (PH) is nonspecific, resulting in significant delays in its detection. In the majority of cases, PH is a marker of the severity of other cardiopulmonary diseases. Differential diagnosis aimed at the early identification of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) who do require specific and complex therapies is as important as PH detection itself. Despite all efforts aimed at the noninvasive assessment of pulmonary arterial pressure, the formal confirmation of PH still requires catheterization of the right heart and pulmonary artery. The current document will give an overview of strategies aimed at the early diagnosis of PAH and CTEPH, while avoiding their overdiagnosis. It is not intended to be a replica of the recently published European Society of Cardiology (ESC) and European Respiratory Society (ERS) Guidelines on Diagnosis and Treatment of Pulmonary Hypertension, freely available at the Web sites of both societies. While promoting guidelines' recommendations, including those on new definitions of PH, we will try to bring them closer to everyday clinical practice, benefiting from our personal experience in managing patients with suspected PH.

Impact of diabetes mellitus on disease severity and patient survival in idiopathic pulmonary arterial hypertension: data from the Polish multicentre registry (BNP-PL).

BACKGROUND: Recent studies revealed that alterations in glucose and lipid metabolism in idiopathic pulmonary arterial hypertension (IPAH) are associated with disease severity and poor survival. However, data regarding the impact of diabetes mellitus (DM) on the prognosis of patients with IPAH remain scarce. The aim of our study was to determine that impact using data from a national multicentre prospective pulmonary hypertension registry. METHODS: We analysed data of adult patients with IPAH from the Database of Pulmonary Hypertension in the Polish population (BNP­PL) between March 1, 2018 and August 31, 2020. Upon admission, clinical, echocardiographic, and haemodynamic data were collected at 21 Polish IPAH reference centres. The all-cause mortality was assessed during a 30-month follow-up period. To adjust for differences in age, body mass index (BMI), and comorbidities between patients with and without DM, a 2-group propensity score matching was performed using a 1:1 pairing algorithm. RESULTS: A total of 532 patients with IPAH were included in the study and 25.6% were diagnosed with DM. Further matched analysis was performed in 136 patients with DM and 136 without DM. DM was associated with older age, higher BMI, more advanced exertional dyspnea, increased levels of N-terminal pro-brain natriuretic peptide, larger right atrial area, increased mean right atrial pressure, mean pulmonary artery pressure, pulmonary vascular resistance, and all-cause mortality compared with no DM. CONCLUSIONS: Patients with IPAH and DM present with more advanced pulmonary vascular disease and worse survival than counterparts without DM independently of age, BMI, and cardiovascular comorbidities.

Treatment of high- and intermediate-high-risk pulmonary embolism by the Pulmonary Embolism Response Team: Focus on catheter-directed therapies.

BACKGROUND: Multidisciplinary Pulmonary Embolism Response Teams (PERTs) were established to individualize the treatment of high-risk (HR) and intermediate-high-risk (IHR) pulmonary embolism (PE) patients, which pose a challenge in clinical practice. METHODS: We retrospectively collected the data of all HR and IHR acute PE patients consulted by PERT CELZAT between September 2017 and October 2022. The patient population was divided into four different treatment methods: anticoagulation alone (AC), systemic thrombolysis (ST), surgical embolectomy (SE), and catheter-directed therapies (CDTx). Baseline clinical characteristics, risk stratification, PE severity parameters, and treatment outcomes were compared between the four groups. RESULTS: Of the 110 patients with HR and IHR PE, 67 (61%) patients were treated with AC only, 11 (10%) with ST, 15 (14%) underwent SE, and 17 (15%) were treated with CTDx. The most common treatment option in the HR group was reperfusion therapy, used in 20/24 (83%) cases, including ST in 7 (29%) patients, SE in 5 (21%) patients, and CTDx in 8 (33%) patients. In contrast, IHR patients were treated with AC alone in 63/86 (73%) cases. The in-hospital mortality rate was 9/24 (37.5%) in the HR group and 4/86 (4.7%) in the IHR group. CONCLUSIONS: The number of advanced procedures aimed at reperfusion was substantially higher in the HR group than in the IHR PE group. Despite the common use of advanced reperfusion techniques in the HR group, patient mortality remained high. There is a need further to optimize the treatment of patients with HR PE to improve outcomes.

Catheter-directed mechanical aspiration thrombectomy in a real-world pulmonary embolism population: a multicenter registry.

AIMS: High- (HR) and intermediate-high risk (IHR) pulmonary embolisms (PEs) are related to high early mortality and long-term sequelae. We aimed to describe clinical outcomes and adverse events in IHR and HR pulmonary embolism (PE) treated with catheter-directed mechanical thrombectomy (CDMT) in a real-world population. METHODS AND RESULTS: This study is a multicenter, prospective registry enrolling 110 PE patients treated with CDMT between 2019 and 2022. The CDMT was performed using the 8F Indigo (Penumbra, Alameda, CA, USA) system bilaterally in pulmonary arteries (PAs). The primary safety endpoints included device or PE-related death during the 48-h after CDMT, procedure-related major bleeding, or other major adverse events. Secondary safety outcomes were all-cause mortality during hospitalization or the follow-up. The primary efficacy outcomes were the reduction of PA pressures and change in the right-to-left ventricular (RV/L) ratio assessed in the imaging 24-48 h after the CDMT.71.8% of patients had IHR PE and 28.2% HR PE. 11.8% of patients had a failure and 34.5% had contraindications to thrombolysis, and 2.7% had polytrauma. There was 0.9% intraprocedural death related to RV failure and 5.5% deaths within the first 48 h. CDMT was complicated by major bleeding in 1.8%, pulmonary artery injury in 1.8%, and ischaemic stroke in 0.9%. Immediate haemodynamic improvements included a 10.4 ± 7.8 mmHg (19.7%) drop in systolic PAP (P < 0.0001), a 6.1 ± 4.2 mmHg (18.8%) drop in mean PAP, and 0.48 ± 0.4 (36%) drop in RV/LV ratio (P < 0.0001). CONCLUSION: These observational findings suggest that CDMT may improve hemodynamics with an acceptable safety profile in patients with IHR and HR PE.

Long COVID syndrome after SARS-CoV-2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients have a more severe COVID-19 course than the general population. Many patients report different persistent symptoms after SARS-CoV-2 infection. The aim of our study is to analyze the prevalence of long COVID-19 symptoms and assess if COVID-19 affects pulmonary hypertension (PH) prognosis. PAH/CTEPH patients who survived COVID-19 for at least 3 months before visiting the PH centers were included in the study. The patients were assessed for symptoms in acute phase of SARS-CoV-2 infection and persisting in follow-up visit, WHO functional class, 6-min walk distance, NT-proBNP concentration. The COMPERA 2.0 model was used to calculate 1-year risk of death due to PH at baseline and at follow-up. Sixty-nine patients-54 (77.3%) with PAH and 15 (21.7%) with CTEPH, 68% women, with a median age of 47.5 years (IQR 37-68)-were enrolled in the study. About 17.1% of patients were hospitalized due to COVID-19 but none in an ICU. At follow-up (median: 155 days after onset of SARS-CoV-2 symptoms), 62% of patients reported at least 1 COVID-19-related symptom and 20% at least 5 symptoms. The most frequently reported symptoms were: fatigue (30%), joint pain (23%), muscle pain (17%), nasal congestion (17%), anosmia (13%), insomnia (13%), and dyspnea (12%). Seventy-two percent of PH patients had a low or intermediate-low risk of 1-year death due to PH at baseline, and 68% after COVID-19 at follow-up. Over 60% of PAH/CTEPH patients who survived COVID-19 suffered from long COVID-19 syndrome, but the calculated 1-year risk of death due to PH did not change significantly after surviving mild or moderate COVID-19.