Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Abstract

Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies. Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry. Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment. Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH. Clinical Trial Registration: clinicaltrials.gov/ct2/show/NCT03959748

Fear of COVID-19, Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension during the Pandemic

“…Pulmonary arterial hypertension (PAH) is a rare disease [ 4 ] characterized by pre-capillary pulmonary hypertension, which is defined by mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg in right heart catheterization, elevated pulmonary vascular resistance (PVR) ≥ 3 Wood units, and normal pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, in the absence of other causes of pre-capillary pulmonary hypertension [ 5 ]. Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease [ 6 ] characterized by pre-capillary pulmonary hypertension due to obstruction and remodeling of the pulmonary artery by major vessel thromboembolism [ 5 ]. Untreated PAH and CTEPH lead to progressive right heart failure and death [ 5 ].…”

Extended Precordial T Wave Inversions Are Associated with Right Ventricular Enlargement and Poor Prognosis in Pulmonary Hypertension

“…We have shown for the first time the role of TWI in left-sided precordial leads in the prognosis of PH patients and in the monitoring of treatment efficacy. Second, we enrolled a large cohort of PAH and CTEPH patients despite the rarity of these conditions [ 21 , 22 , 23 ]. Third, we described the correlation between TWI and changes in RV and LV morphology in patients with precapillary PH.…”

Role of the Immune System Elements in Pulmonary Arterial Hypertension

“…CD4+ CD45RA− FoxP3high Tregs are activated Tregs and have strong immunosuppressive properties. CD4+ CD45RA− FoxP3low Tregs, however, do not have immunosuppressive properties, rather they produce the pro-inflammatory cytokines IL-2 and interferon gamma [31,32].…”