ABSTRACT
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.
Subject(s)
Angioplasty, Balloon , Hypertension, Pulmonary , Pulmonary Embolism , Humans , Angioplasty, Balloon/methods , Latvia , Male , Hypertension, Pulmonary/therapy , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/surgery , Female , Middle Aged , Prospective Studies , Aged , Pulmonary Embolism/complications , Pulmonary Embolism/therapy , Pulmonary Embolism/physiopathology , Chronic Disease , Treatment Outcome , Adult , Vascular ResistanceABSTRACT
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive condition; however, the true characteristics of CTEPH are still unknown, as notable regional variations exist in terms of patients' age, baseline hemodynamic data, and management choices. This report aims to investigate the baseline clinical characteristics, incidence, and risk factors associated with CTEPH patients in Latvia from 2019 to 2020. Methods: The data were analyzed from a prospective, nationwide, Latvian pulmonary hypertension registry for incident CTEPH cases. The patients' clinical characteristics were assessed at the time of diagnosis. Results: During the course of this study, a cohort of 13 patients with CTEPH were included for analysis. Among the enrolled CTEPH patients, most exhibited low exercise and functional capacity, with a median (±IQR) 6 min walk distance of 300.0 (±150.0) m. The median values (±IQR) for mean pulmonary artery pressure and pulmonary vascular resistance were 40.0 ± 13.0 mmHg and 7.35 ± 2.82 Wood units, respectively. The most common risk factors for CTEPH were a history of acute pulmonary embolism and a blood group other than O. Conclusions: The findings of this report revealed the characteristics of the Latvian CTEPH population, indicating that a significant proportion of patients are elderly individuals with multiple comorbidities.
Subject(s)
Hypertension, Pulmonary , Aged , Humans , Incidence , Latvia/epidemiology , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/epidemiology , Prospective Studies , ExerciseABSTRACT
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.
Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Acute Disease , Aged , Anticoagulants , Chronic Disease , Computed Tomography Angiography , Female , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Pulmonary Embolism/diagnosisABSTRACT
Congenital diaphragm hernia (CDH) is a congenital disease that occurs during prenatal development. Although the morbidity and mortality rate is rather significant, the pathogenesis of CDH has been studied insignificantly due to the decreased accessibility of human pathological material. Therefore the aim of our work was to evaluate growth factors (transforming growth factor-beta (TGF-ß), basic fibroblast growth factor (bFGF), insulin-like growth factor 1 (IGF-1), hepatocyte growth factor (HGF)) and their receptors (fibroblast growth factor receptor 1 (FGFR1), insulin-like growth factor 1 (IGF-1R)), muscle (dystrophin, myosin, alpha actin) and nerve quality (nerve growth factor (NGF), nerve growth factor receptor (NGFR), neurofilaments (NF)) factors, local defense factors (ß-defensin 2, ß-defensin 4), programmed cell death (TUNEL), and separate gene (Wnt-1) expression in human pathological material to find immunohistochemical marker differences between the control and the CDH patient groups. A semi-quantitative counting method was used for the evaluation of the tissues and structures in the Biotin-Streptavidin-stained slides. Various statistically significant differences were found in immunoreactive expression between the patient and the control group tissue and the morphological structures as well as very strong, strong, and moderate correlations between immunoreactives in different diaphragm cells and structures. These significant changes and various correlations indicate that multiple morphopathogenetic pathways are affected in CDH pathogenesis. This work contains the evaluation of the causes for these changes and their potential involvement in CDH pathogenesis.
