The Polish adaptation of the CAMbridge Pulmonary Hypertension Outcome Review (CAMPHOR).

BACKGROUND: Pulmonary hypertension (PH) results in severely impaired quality of life (QoL) in people with this condition. The CAMbridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the only questionnaire providing a disease-specific measurement of symptoms, functioning and QoL in PH patients. It has already been adapted for use in several countries. The aim of this study was to adapt and validate CAMPHOR for the Polish-speaking population. METHODS: Two panels (bilingual and lay) were conducted to translate CAMPHOR into Polish. This new version was then tested by cognitive debriefing interviews with 15 patients. Finally, a postal validation survey was conducted with 56 patients on two occasions 2 weeks apart to assess its psychometric properties. RESULTS: No problems were experienced in producing a Polish translation of CAMPHOR. Interviewees responded well to the Polish CAMPHOR, finding it relevant, comprehensible and easy to complete. For all three CAMPHOR scales (Symptoms, Activity, QoL), The Cronbach alpha coefficients were above 0.8 at both time points, indicating high internal consistency. Test-retest reliability for the three scales achieved a value above 0.80. Predicted correlations with the Nottingham Health Profile provided evidence of the construct validity of CAMPHOR scales. The Polish CAMPHOR could distinguish between patients who differed according to their perceived general health and perceived disease severity. No significant differences in scores were found between participants grouped by gender or age. CONCLUSIONS: The Polish version of CAMPHOR demonstrated good psychometric properties and is recommended for use in clinical practice.

Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Upregulation of the immune system is regarded to play an important role in the etiopathobiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). To the best of our knowledge, neopterin (NP) has never been investigated in patients with PAH and CTEPH. OBJECTIVES: The aim of the study was to evaluate the concentration of NP in blood in order to examine its impact on outcome and relationship with disease severity in that population. METHODS: Serum concentration of NP was analysed prospectively in 50 patients (36 with PAH and 14 with CTEPH vs. 31 healthy controls) and assessed in relation to clinical parameters and outcome. RESULTS: NP concentration in the PAH and CTEPH groups combined was significantly higher than in the control group (8.68, 6.39-15.03 vs. 5.14, 4.16-5.98 nmol/L, p < 0.0000001). During 9 months of follow-up, clinical deterioration occurred in 18 patients (including 8 deaths), and NP concentration in this group was higher when compared to stable patients (15.6, 8.52-25.13 vs. 7.87, 6.18-9.89, p = 0.002). The cutoff value of NP derived from ROC curve analysis was 15.3 nmol/L (p = 0.002, AUC 0.77, p = 0.0004, HR = 4.35, 95% CI 1.43-13.18, log-rank test). On Cox regression analysis, NP predicted clinical deterioration (p = 0.009, 95% CI 1.01-1.06). NP correlated positively with NT-proBNP (p < 0.001), red blood cell distribution width (p < 0.001), and right atrium area (p = 0.002) and inversely with 6-min walking test (p = 0.002) and peak oxygen consumption (p = 0.001). CONCLUSIONS: NP concentration is increased in patients with PAH and inoperable CTEPH. Elevated NP concentration is associated with adverse clinical outcomes and correlates with clinical parameters.

Red Blood Cells Distribution Width as a Potential Prognostic Biomarker in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Red blood cells distribution width (RDW) predicts survival in cardiovascular diseases. Little is known about the variability of RDW level over time among patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). To our knowledge, RDW has never been analysed as a marker of response to specific treatment. MATERIALS AND METHODS: We retrospectively analysed 77 patients for: i) RDW measured during the last hospitalisation before death or during the last follow-up (RDWlast); ii) mean RDW from all hospitalisations during the entire follow-up of the patient (RDWmean); iii) maximum RDW of all hospitalisations of each patient (RDWmax). In order to assess response to specific treatment and association with prognosis, we compared RDW levels (obtained from 56 patients) before and 3 to 6 months after introduction or intensification of treatment in both the alive and deceased group. RESULTS: Twenty-eight of 77 patients died, whereas in specific drugs treatment response analysis, 22 of 56 patients died during follow-up. The cut-off values derived from the ROC analysis and assessed using the log-rank test were significant for RDWlast (p<0.0001), RDWmean (p<0.001) and RDWmax (p=0.02). A decrease in RDW levels after introduction or intensification of specific treatment was significant (p=0.015) in survivors, whereas there was no significance (p=0.29) in decrease in RDW levels in non-survivors after change of therapy. CONCLUSIONS: Red blood cells distribution width might be a potential prognostic biomarker in patients with PAH and inoperable CTEPH. The decrease in RDW level after introduction or escalation of PAH-targeted and CTEPH-targeted drugs is associated with a good treatment response and better prognosis.

Early and long-term outcomes of pericardiotomy in the treatment of primary cardiac tamponade.

INTRODUCTION: Cardiac tamponade is a life-threatening clinical entity that requires emergent treatment. A variety of therapeutic methods have been applied. The purpose of this retrospective analysis was to study the efficacy of emergent surgical pericardiotomy in both the relief of cardiac tamponade and in the prevention of recurrence of pericardial effusion. MATERIAL AND METHODS: This study involved 90 consecutive patients (58 males and 32 females) with a mean age of 57.4 ± 14.1 years, who underwent emergent pericardiotomy in the years 2006 to 2011 due to symptomatic primary cardiac tamponade. At the end of the follow-up period all living subjects had control echocardiographic examination. Survival analysis was performed with the use of the Kaplan-Meier method. RESULTS: The mean duration time of surgery was 14.2 ± 4.5 minutes. All patients survived surgery but one died during in-hospital stay. During the post-discharge follow-up period (median 49 months) 32 patients died for any reason. One-year and four-year probability of survival was 0.68 ± 0.05 and 0.64 ± 0.05, respectively. Malignancy diagnosis was associated with significant negative impact on survival. One-year and four-year probability of survival was 0.56 ± 0.06 and 0.53 ± 0.06 for cancer patients while it was 0.93 ± 0.05 and 0.89 ± 0.06 for the others, respectively. In two cases, only cancer patients, re-intervention due to effusion recurrence was necessary. CONCLUSIONS: Pericardiotomy, although invasive, is a safe method to relieve cardiac tamponade. It is also very efficient in the prevention of recurrence of pericardial effusion, even in cancer patients.