Role of right atrial mechanics has not been studied in idiopathic pulmonary arterial hypertension (IPAH). We sought to investigate the correlation between the right sided, particularly right atrial mechanics, hemodynamic parameters, and functional capacity prior to initiation of treatment.
Twenty-five subjects (63 ± 17 years, 13 males) with newly diagnosed IPAH by right heart catheterization (RHC) underwent speckle tracking echocardiography (STE) of the right ventricle (RV) and right atrium (RA). All the patients had a six-minute walk test (6MWD) performed, functional class (WHO-FC) assessed, and plasma NT-proBNP estimated. STE strain imaging of RA and RV was performed offline. STE of the RA myocardium yielded characteristic strain (S%) and strain rate (SR) curves with clear delineation of its reservoir (RA-RS%), conduit (RA-SR E), and booster pump functions (RA-SR A). In IPAH, RHC-obtained pulmonary vascular resistance (PVR) and pulmonary artery systolic pressure (PASP), respectively, were 7.3 ± 5.6 Wood units and 66 ± 24 mmHg. RA-RS% was associated negatively with PVR (r = -0.46; P
The Swedish Pulmonary Arterial Hypertension Register (SPAHR) is an open continuous register, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients from 2000 and onwards. We hereby launch the first data from SPAHR, defining baseline characteristics and survival of Swedish PAH and CTEPH patients.
Incident PAH and CTEPH patients 2008-2014 from all seven Swedish PAH-centres were specifically reviewed.
There were 457 PAH (median age: 67 years, 64% female) and 183 CTEPH (median age: 70 years, 50% female) patients, whereof 77 and 81%, respectively, were in functional class III-IV at diagnosis. Systemic hypertension, diabetes, ischaemic heart disease and atrial fibrillation were common comorbidities, particularly in those >65 years. One-, 3- and 5-year survival was 85%, 71% and 59% for PAH patients. Corresponding numbers for CTEPH patients with versus without pulmonary endarterectomy were 96%, 89% and 86% versus 91%, 75% and 69%, respectively. In 2014, the incidence of IPAH/HPAH, associated PAH and CTEPH was 5, 3 and 2 per million inhabitants and year, and the prevalence was 25, 24 and 19 per million inhabitants.
The majority of the PAH and CTEPH patients were diagnosed at age >65 years, in functional class III-IV, and exhibiting several comorbidities. PAH survival in SPAHR was similar to other registers.
Lund University, Department of Clinical Sciences, Lund, Cardiothoracic Surgery, and Skåne University Hospital, and Medicine Services University Trust, Region Skåne, Lund, Sweden. Electronic address: firstname.lastname@example.org.
Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) decrease life expectancy for affected patients. The aim was to describe the perceptions of received information regarding diagnosis, treatment and management, as well as social support and coping perceived by patients with PAH or CTEPH.
A descriptive, national cohort survey that included patients from all PAH-centres in Sweden. A quantitative methodology was employed where all patients received three questionnaires, QLQ-INFO25, Mastery Scale and Social Network & Support Scale.
The response rate was 74% (n=325), mean age 66±14years, 71% were female and 69% were diagnosed with PAH and 31% with CTEPH. Sixty percent of respondents were satisfied with the received information and felt it was helpful. Best experiences were of information in the areas of medical tests (70%) and the disease (61%). Forty-five percent wanted more information. Men described their social network as more accessible than women (p=0.004). Those living alone had a lower coping ability.
Despite a high level of satisfaction with the received information, almost half of the patients wanted more information.
Regular repeating or giving new information should be an ongoing process in the care of patients at the PAH-centres.
To investigate survival, treatment escalation, effects of first-line single- and first-line combination therapy and prognostic markers in idiopathic- (IPAH), hereditary- (HPAH) and connective tissue disease-associated (CTD-PAH) pulmonary arterial hypertension (PAH).
Retrospective analysis of medical journals from PAH patients at Skåne University Hospital 2000-2011.
1-, 2- and 3-year survival was 87%, 67%, and 54%, respectively, for the entire population, but worse (p = 0.003) in CTD-PAH than IPAH/HPAH. After 1, 2 and 3 years, 58%, 41% and 24% of patients starting on single therapy were alive on single therapy. 37.5% of patients on first-line single therapy received escalated treatment at first follow-up. First-line combination therapy more greatly decreased pulmonary vascular resistance index (PVRI, p = 0.017) than first-line single therapy. Only first-line combination therapy improved (p = 0.042) cardiac index (CI). Higher mean right atrial pressure (MRAP, p = 0.018), MRAP/CI (p = 0.021) and WHO functional class (p
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.