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.

Individually tailored home-based physiotherapy program makes sustainable improvement in exercise capacity and daily physical activity in patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, chronic, progressive, and life-threatening cardiopulmonary disease. This study investigated the impact of an individually tailored 12 weeks home-based physiotherapy program in PAH patients, with the aim to evaluate change in exercise capacity and daily physical activity level. METHODS: This was an analysis of secondary outcomes from a prospective, randomized, controlled intervention study. Twenty-one participants were recruited from the Latvian PH registry based on inclusion criteria and randomized in a training group (TG) and control group (CG). Both groups continued a medical target therapy, but for TG, the individually tailored home-based physiotherapy program was added including physical exercises, relaxation, self-control, education, and supervision with telehealth elements. Outcomes included a 6-min walk test (6MWT) (m) and daily physical activity based on accelerometry results assessed at baseline, after 12 weeks, and at follow-up 6 months after commencement of intervention. RESULTS: 6MWT distance significantly (p < 0.05) and clinically (>33 m) increased within TG after 12 weeks (51.8 m, 95% CI = 25.7-77.9 m, Cohen's d = 1.7) and at follow-up (75.5 m, 95% CI = 46.1-104.8 m, Cohen's d = 2.1). A significant difference in 6MWT results between the groups at 12 weeks and follow-up was approved. In TG, low-intensity activities significantly (p < 0.05) increased both after 12 weeks (Cohen's d = 1.6) and at follow-up (Cohen's d = 1.2), moderate-intensity activities significantly (p < 0.05) increased at follow-up (Cohen's d = 1.3), and no significant improvements were present in CG. CONCLUSION: The individually tailored 12 weeks home-based physiotherapy program comprising comprehensive physical exercise training, relaxation, self-control skills training, and education, added to stable medical target therapy and supervised by physiotherapist through telehealth elements, is effective in improving exercise capacity and increasing daily time in low or moderate physical activities 6 months after commencement of the intervention in patients with PAH.

Chronic Thromboembolic Pulmonary Hypertension Mimicking Acute Pulmonary Embolism: A Case Report.

BACKGROUND Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension which is often caused by recurrent emboli. The reported prevalence in Latvia is 15.7 cases per million inhabitants. Several risk factors predispose patients to develop chronic thromboembolic pulmonary hypertension, including the presence of chronic myeloproliferative diseases and splenectomy. CASE REPORT We present a case of a 68-year-old woman with a variant of chronic myeloproliferative disease, essential thrombocythemia, splenectomy, and chronic thromboembolic pulmonary hypertension, in whom chronic thromboembolic pulmonary hypertension was mimicking acute pulmonary embolism. On admission, the patient had progressive dyspnea, elevated right ventricular systolic pressure (RVSP) 60-70 mmHg, and elevated thrombocytes, C-reactive protein, BNP, and d-dimer levels. These results, as well as the results of thoracic computed tomography angiography with contrast, supported the diagnosis of acute pulmonary embolism. During the sequent follow-up visit after 3 months of effective anticoagulant therapy, the patient had elevated RVSP: 55-60 mmHg. Therefore, right heart catheterization was performed, in which it was found that mPAP was 37 mmHg with PCWP 5 mm Hg and PVR 8.9 Wood units, confirming the CTEPH diagnosis. CONCLUSIONS Patients who are at high risk of thrombosis need an increased level of monitoring to be properly evaluated. An easy solution to misdiagnosis of CTEPH with an acute pulmonary embolism could be taking scrupulous patient history, which can reveal multiple risk factors of CTEPH development. The subsequent assessment of risk factors can lead to a more appropriate consideration of CTEPH diagnosis vs acute pulmonary embolism.

Long-Term Response to Vasoactive Treatment in a Case of Kyphoscoliosis-Associated Pulmonary Hypertension.

BACKGROUND Kyphoscoliosis is an anatomical deformity of the spine often accompanied by an array of respiratory complications, pulmonary hypertension being among the most severe ones. At present, evidence-based treatment options for kyphoscoliosis-related pulmonary hypertension remain limited to the correction of hypoxemia through ventilatory support and long-term oxygenation. CASE REPORT We report a case of a 61-year-old female with severe kyphoscoliosis-related pulmonary hypertension who was admitted to a university hospital in September 2018 due to progressive dyspnea and respiratory failure. She was diagnosed with pulmonary hypertension in 2016 and had been on endothelin receptor antagonist (ambrisentan) and oxygen therapy ever since. Upon admission, the patient presented with severe depression of peripheral oxygen saturation (SpO2 at 75%). The patient declined further treatment hours after hospitalization, despite optimized supportive oxygen therapy. Ambrisentan was discontinued and replaced by inhaled iloprost. Over the course of the next 4 days, the patient showed symptomatic improvement and was discharged on Day 5. Right heart catheterization follow-up in February 2019 showed no worsening in pulmonary hemodynamic parameters compared to the time of initial diagnosis. CONCLUSIONS Managing the respiratory decline in kyphoscoliosis-related pulmonary hypertension can be challenging since these patients tend to deteriorate despite current treatment options. Our case reports on the use of vasoactive agents as a safe and effective treatment option in addition to established therapeutic regimen.

Pulmonary Endarterectomy in Latvia: A National Experience.

Background and objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a hemodynamic state characterized by chronic obstruction in pulmonary circulation. The treatment of choice is pulmonary endarterectomy (PEA). The aim of our study was to compile and analyze the data of a small, national center, which has not yet been done in the Baltic states. Materials and methods: The data of Latvian CTEPH registry in timeframe from 1 September 2007 to 31 December 2016 was retrospectively analyzed and all patients who underwent PEA were included. Results: PEA was done for 7 patients. The in-hospital mortality was 14%. The 3-year survival rate was 86%. The procedure restored pulmonary blood pressure to normal values for three of the patients (42%). The remaining four patients (57%) had persistent pulmonary hypertension (mPAP > 30 mmHg), which required continuous therapy. There was a comparable decline in mean mPAP compared to baseline, 53.4 ± 14.4 mmHg to 44.3 ± 30 mmHg, respectively. At 12-month follow-up, there was a significant improvement in functional capacity, as seen by increased 6-min walk test distance and shifts in New York Heart Association functional class. Conclusions: Only 16% of all prevalent Latvian CTEPH patients have underwent PEA in the course of nine years, despite it being the treatment of choice for CTEPH. As PEA and other emerging treatment options, such as balloon pulmonary angioplasty, can only be done in expert centers, numerous organizational, logistical, and economic issues arise for patients of smaller countries, where such centers have not yet been created due to lack of experience and limited amount of patients.

Chronic Thromboembolic Pulmonary Hypertension and Antiphospholipid Syndrome with Immune Thrombocytopenia: A Case Report.

BACKGROUND Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state associated with circulating antiphospholipid antibodies. The presence of antiphospholipid antibodies can result in a variety of clinical symptoms, such as thrombocytopenia, stillbirth, endocardial pathologies, and recurrent pulmonary embolism. CASE REPORT We present the case of a 23-year-old man with antiphospholipid syndrome and chronic thromboembolic pulmonary hypertension who developed severe thrombocytopenia. The patient died from right heart failure before the thrombocytopenia could be managed, preventing performance of a pulmonary endarterectomy procedure. CONCLUSIONS Managing platelet counts in patients with antiphospholipid syndrome prior to major surgery is very problematic, and requires similar treatment strategy as in patients with immune thrombocytic thrombocytopenia. Platelet transfusions may further decrease platelet count, as it can trigger formation of new antibodies.

Pulmonary arterial hypertension in a patient treated with dasatinib: a case report.

BACKGROUND: There have been several reports on dasatinib-induced reversible pulmonary hypertension. This is the first reported case in Latvia; the patient did not discontinue the drug after the first adverse effects in the form of pleural effusions, which we speculate led only to partial reversion of the disease. CASE PRESENTATION: A 67-year-old white man with chronic myelogenous leukemia was treated with the dual Src and BCR-ABL tyrosine kinase inhibitor dasatinib. After treatment with dasatinib he had multiple pleural effusions which were suspected to be caused by congestive heart failure. Later a transthoracic Doppler echocardiography and right-sided heart catheterization revealed severe pulmonary hypertension with pulmonary vascular resistance of 12 Wood units and mean pulmonary artery pressure of 53 mmHg. Computed tomography ruled out a possible pulmonary embolism; laboratory specific tests for human immunodeficiency virus, rheumatoid factor, and anti-nuclear antibodies were negative, and dasatinib-induced pulmonary arterial hypertension was diagnosed. A follow-up right-sided heart catheterization and 6-minute walk test done a month after the discontinuation of dasatinib showed significant improvement: mean pulmonary artery pressure of 34 mmHg and pulmonary vascular resistance of 4 Wood units. CONCLUSIONS: Patients should always be closely monitored when using dasatinib for a prolonged time. Dasatinib-induced pulmonary hypertension may be fully reversible after the therapy is suspended, but the key factors involved are still unclear and need to be further studied.