Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of =25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of =15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months. Up to now, our country has had no guidelines for the diagnosis and treatment of this rare severe disease that, when appropriately untreated, has an extremely poor prognosis. The main task in the preparation of this document was to generalize and analyze the data of current registries, multicenter randomized clinical trials, national and international guidelines, and consensus documents recently published on this problem in order to optimize a diagnostic process and treatment in this category of patients. Part 1 gives a definition of CTEPH, its place in the clinical classification, epidemiology and prognosis, risk factors, pathogenesis and morphology, diagnostic approaches and determination of operability in patients, and specific features of differential diagnosis.
The paper gives current approaches to treating chronic thromboembolic pulmonary hypertension (CTEPH) from the document «Federal Guidelines for the Diagnosis and Treatment of CTEPH» approved at the Third Russian Congress on Pulmonary Hypertension on December 11, 2015. The guidelines had been elaborated to optimize the treatment of patients with CTEPH on the basis of an analysis of the data of the present-day registries and multicenter randomized clinical trials, national and international guidelines and consensus documents, and documents published in recent years. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The paper presents indications for and contraindications to pulmonary thromboendartectomy; preparation for surgery; operating room facilities; the specific features of postoperative management and possible complications; and long-term RESULTS: In terms of therapy, in addition to non-pharmacological measures, the authors discuss maintenance and specific treatment options for CTEPH, balloon pulmonary angioplasty, and lung/heart-lung transplantation. In conclusion they propose a management algorithm in patients with CTEPH and requirements for its problem to the center of experts.
The aim of current study was to estimate the economic burden of the chronic thromboembolic pulmonary hypertension (CTEPH) in Russia based on patient registry.
Cost of illness study was based on data derived from CTEPH patient registry that was developed at the Division of hypertension of FSBI "National Medical Research Center of Cardiology". Demographic and clinical patient characteristics were analyzed with descriptive statistic methods. Cost of illness study was performed from the state perspective and with bottom-up approach. Bootstrapping was used for calculation of average costs per patient/year. Within the study direct costs (medical costs: outpatient, inpatient, emergency, PAH-specific therapy, concomitant therapy; non-medical costs: pension due to disability status, payments for patients on sick-leave) and indirect costs (loss in GDP) were estimated.
Overall, 113 CTEPH patients (67 women and 46 men) from 33 Russian regions were included, mean age of patients with CTEPH was 54.6±13.95 years. Most of the patients (55%) were in able-bodied age. It was found that about half of patients with diagnosed CTEPH had a disability. Average duration of disease at the time of analysis was 6.88±11.41 years. Period from the first occurrence of symptoms to the confirmation of diagnosis of CTEPH was 2.58±5.21 years on average. More than 70% of patients had III and IV FC (WHO) at the time of diagnosis. Mean number of outpatient visits was 1.97±1.65 per patient/year, and inpatient visits were reported for 59% of patients. About 54% of patients used PAH-specific therapy, moreover 46% patients had interruptions of PAH-specific therapy (58.4±66.3 days). The total costs of CTEPH per patient/year were calculated as 805,901 RUB. The overall burden of CTEPH in Russia for total CTEPH population (470 patients) was 379 million RUB per year.
CTEPH is the rare disease that is characterized with later diagnosis due to absence of disease-specific symptoms. Therefore economic burden of the CTEPH is significantly low in comparison to widespread cardiovascular diseases. Development of network of expert PH-centers and increase of the access for PAH-specific therapy will help to increase the quality of health care for patients with CTEPH.
Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry.
In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug- and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017.
Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug- and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years.
The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.
Pulmonary hypertension (PH) is a progressive disease which is characterized with the increase of pulmonary artery pressure and pulmonary vascular resistance. Such condition leads to right ventricular heart failure and premature death of patients. Pulmonary arterial hypertension (PAH) has the status of an orphan disease. However in Russia only idiopathic PH is included in the list of 24 life-threatening and chronic progressive rare diseases, while other forms of PH are not in it. Inclusion in this list guarantees drug provision for patients at the expense of the regional budget, while patients with other forms of PH can rely on free medication only if they have a disability. The lack of criteria for revising this list as well as the imperfection of legal regulation in the field of drug support for orphan diseases leads to high disability, a significant decrease in the duration and quality of life of patients with PH. As part of a multicriteria approach, a clinical and economic analysis of the disease burden can be one of the tools for policy development and decision-making on the distribution of funding in the healthcare. The article provides a review of the economic burden of various forms of PH in the world.