Information, social support and coping in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension-A nationwide population-based study

Objective: 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. Methods: 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. Results: The response rate was 74% (n. =325), mean age 66. ±. 14. years, 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. Conclusion: Despite a high level of satisfaction with the received information, almost half of the patients wanted more information. Practice implications: Regular repeating or giving new information should be an ongoing process in the care of patients at the PAH-centres

Estimation of cardiac output in patients with congestive heart failure by analysis of right ventricular pressure waveforms

<p>Abstract</p> <p>Background</p> <p>Cardiac output (CO) is an important determinant of the hemodynamic state in patients with congestive heart failure (CHF). We tested the hypothesis that CO can be estimated from the right ventricular (RV) pressure waveform in CHF patients using a pulse contour cardiac output algorithm that considers constant but patient specific RV outflow tract characteristic impedance.</p> <p>Method</p> <p>In 12 patients with CHF, breath-by-breath Fick CO and RV pressure waveforms were recorded utilizing an implantable hemodynamic monitor during a bicycle exercise protocol. These data were analyzed retrospectively to assess changes in characteristic impedance of the RV outflow tract during exercise. Four patients that were implanted with an implantable cardiac defibrillator (ICD) implementing the algorithm were studied prospectively. During a two staged sub-maximal bicycle exercise test conducted at 4 and 16 weeks of implant, COs measured by direct Fick technique and estimated by the ICD were recorded and compared.</p> <p>Results</p> <p>At rest the total pulmonary arterial resistance and the characteristic impedance were 675 ± 345 and 48 ± 18 dyn.s.cm^-5, respectively. During sub-maximal exercise, the total pulmonary arterial resistance decreased (Δ 91 ± 159 dyn.s.cm^-5, p < 0.05) but the characteristic impedance was unaffected (Δ 3 ± 9 dyn.s.cm^-5, NS). The algorithm derived cardiac output estimates correlated with Fick CO (7.6 ± 2.5 L/min, R²= 0.92) with a limit of agreement of 1.7 L/min and tracked changes in Fick CO (R²= 0.73).</p> <p>Conclusions</p> <p>The analysis of right ventricular pressure waveforms continuously recorded by an implantable hemodynamic monitor provides an estimate of CO and may prove useful in guiding treatment in patients with CHF.</p

The usefulness of continuous hemodynamic monitoring to guide therapy in patients with cardiopulmonary disease

Introduction: Cardiovascular disease, whether secondary to myocardial injury, pulmonary hypertension or renal failure, have high morbidity and mortality. New treatments have improved quality of life and survival, but hospitalization rates remain high. Continuous hemodynamic monitoring allows for a new perspective in cardiovascular disease management allowing for treatment strategies based on measurements performed while the patient tends to normal daily activities. Feasibility: Hemodynamic monitoring by the means of an implanted pressure sensor has been shown earlier to be accurate in pressure measurement, safe to implant and stable in measurements over long-term. This thesis looked at acute and long-term stability of an oxygen sensor measuring mixed venous oxygen saturation from the right ventricle (Study I). The oxygen sensor was implanted in nine patients with a conventional pacemaker indication and showed a good correlation compared levels over six years. Study II established that one data point, the night-time minimum, from the 24-hour hemodynamic trend replicated hemodynamic values collected during a controlled changes over time. In addition to the fully implanted lead and memory device, the hemodynamic monitoring system includes remote monitoring, e.g. sending the data stored in the implantable hemodynamic monitor to a secured website for review by the treating clinician. Study III described this telemonitoring system and demonstrated that the transmission rate was acceptable and apparently independent of age and disease stage. Applicability: In patients with heart failure, peak VO2 has been shown to be a good predictor of outcome. However, maximal exercise tests are cumbersome to perform and involve risk for the patients. Submaximal tests, e.g. 6-minute walk tests are routinely used to evaluate patient status in the hospital clinic. Study IV compared hemodynamic response during maximal and submaximal exercise in 30 patients with heart failure. During submaximal exercise the pressures increased 70-80% and heart rate 90% of the change achieved during maximal exercise. Thus, submaximal exercise hemodynamic response could be a tool in patient assessment in patients inhaled prostacyclin analog, iloprost. The effect of the drug lasted shorter when the patients used the treatment at home than under supervision in the hospital. The treatment effect in both setting was shorter than previously demonstrated in other studies. The most probable explanation for this is that hemodynamic measurements occurred during normal, daily activities in Study V, while earlier measurements have been performed in stationary patients during invasive studies. Study VI found progressively increasing cardiac pressures between hemodialysis treatments in 16 patients with end stage renal disease. The pressure increase, especially after a weekend when hemodialysis treatment was withheld for an extra day, was in the same magnitude as seen in patients with heart failure before a volume overload event leading to hospitalization. These recurrent changes in cardiac pressures might result in myocardial damage. More frequent dialysis treatment might be Conclusion: The hemodynamic monitoring system and its components are feasible and mixed venous oxygen might add value to the system. One single data point could be extracted from the advise the clinician of possible changes in the hemodynamic trends. This thesis supports the use of implantable hemodynamic monitoring in patients with cardiovascular disease of different origin associated with compromised hemodynamics. These observations may help to evaluate disease progress and to make therapeutic decisions

Novel Insight into How Nurses Working at PH Specialist Clinics in Sweden Perceive Their Work

Outpatient pulmonary hypertension (PH) specialist centers have an important role in the optimal management of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the present study was to gain an understanding of the work facing nurses at the outpatient PH specialist centers in Sweden. All nurses (n = 14) working at the outpatient PH specialist centers in Sweden were included. Qualitative content analysis was employed to analyze the interviews, wherein an overarching theme emerged: "Build and maintain a relationship with the patient". Three categories described the nurses' experiences: "Ambiguous satisfaction regarding information and communication", "Acting as a coordinator" and "Professional and personal development". To provide good patient care, the nurses described the key components as the ability to give information on all aspects of the disease and their availability by phone for patients, their relatives, and other healthcare resources. This requires evidence-based, specialist knowledge about the disease, its care, and treatments as well as experience. In conclusion, working as a nurse at the outpatient PH specialist centers highlight the advantages, expectations, and difficulties in working with patients with a rare and life-threatening illness. The overall knowledge and skills were high, but the nurses expressed a need for in-depth and continued training

Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

Purpose of Review Pulmonary arterial hypertension (PAH) is a progressive disease with high mortality. A greater understanding of the physiology and function of the cardiovascular system in PAH will help improve survival. This review covers the latest advances within cardiovascular magnetic resonance imaging (CMR) regarding diagnosis, evaluation of treatment, and prognostication of patients with PAH. Recent Findings New CMR measures that have been proven relevant in PAH include measures of ventricular and atrial volumes and function, tissue characterization, pulmonary artery velocities, and arterio-ventricular coupling. Summary CMR markers carry prognostic information relevant for clinical care such as treatment response and thereby can affect survival. Future research should investigate if CMR, as a non-invasive method, can improve existing measures or even provide new and better measures in the diagnosis, evaluation of treatment, and determination of prognosis of PAH

The Odyssey from Symptom to Diagnosis of Pulmonary Hypertension from the Patients and Spouses Perspective

Introduction/objective: Diagnostic delays in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are related to increased morbidity and mortality. The risk of a delayed, or even a missed, diagnosis is high as the conditions are rare. The aim was to describe patients’ and spouses’ experiences of the journey from the first symptom to an established diagnosis. Methods: A secondary analysis of 31 transcripts, based on 2 primary datasets containing interviews with 17 patients and 14 spouses, was carried out and analyzed according to qualitative content analysis. Results: One overarching category was revealed from the content analysis; “The journey from doubt and hope to receive the diagnosis.” Five subcategories were identified as: overall experiences; ignoring symptoms; seeking primary care/hospital specialty care; blame and stigma; and finding a pulmonary hypertension specialist clinic. The main finding was that both patients and spouses experienced that waiting for a diagnosis and the deteriorating state of health led to anxiety and frustration. The knowledge about rare diseases among health professionals needs to be improved to enable a timelier diagnosis and initiation of treatment. Conclusion: Patients’ and spouses’ lives were negatively affected by having to search for a correct diagnosis. In order for health care to identify rare diseases earlier, a well-functioning and responsive health care system, in primary care as well as in specialist care, is needed. Symptoms like breathlessness and fatigue are often unspecific but should not be ignored. Keeping the patient and spouse in the loop, and providing information that the search for an answer might take time is essential for health care providers to create trust

Health-related quality of life, treatment adherence and psychosocial support in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) share similar quality of life impairment. The aim of the present study was to investigate health-related quality of life (HRQoL) and its relation to the perception of treatment and psychosocial support among PAH and CTEPH patients. All adult PAH or CTEPH patients in the Swedish Pulmonary Arterial Hypertension Register were invited to participate in a national cohort survey. The survey included the EuroQol 5-dimensions (EQ-5D) instrument that measures an individual's HRQoL; the Beliefs about Medicines Questionnaire-Specific Scale that assesses the perception of PAH-specific treatment; the Mastery scale that evaluates the feeling of control and ability to cope with the disease; and the Social Network and Support Scale that maps the social support network. Of the 440 invited patients, 74% responded. Mean age was 66 ± 14 years, 58% were female and 69% diagnosed with PAH. Patients with PAH were younger, more often female and had a lower EQ-5D index (0.67 ± 0.29 vs. 0.73 ± 0.25, p = 0.050) than patients with CTEPH. Patients with a low EQ-5D index had more concerns about treatment ( p = 0.004), lower coping ability ( p < 0.001), less emotional support ( p = 0.003) and less accessible social network ( p = 0.002). In conclusion, patients with an impaired HRQoL also reported negative effects on their social support network, ability to handle stressors and concerns about treatment