Palliative care referrals in patients with pulmonary arterial hypertension: The Pulmonary Hypertension Association Registry.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a life limiting disease with substantial symptom burden and healthcare utilization. Palliative care alleviates physical and emotional symptoms for patients with serious illness, and has been underutilized for these patients. OBJECTIVE: To characterize patients with PAH referred to palliative care and identify predictors of referral. METHODS: We conducted an observational study of adult patients enrolled in the Pulmonary Hypertension Association Registry from January 2015 through June 2021, performing descriptive statistics on patient characteristics at baseline for all patients and the subset referred to palliative care. These characteristics were modeled in a backwards elimination Cox regression with time to referral to palliative care as the primary outcome. RESULTS: 92 of 1,578 patients were referred to palliative care (5.8%); 43% were referred at their last visit prior to death. Referrals were associated with increasing age per decade (hazard ratio 1.35 [95% confidence interval 1.16-1.58]), lower body mass index (hazard ratio 0.97 [95% confidence interval 0.94-0.998]), supplemental oxygen use (hazard ratio 2.01 [95% confidence interval 1.28-3.16]), parenteral prostanoid use (hazard ratio 2.88 [95% confidence interval 1.84-4.51]), and worse quality of life, measured via lower physical (hazard ratio 0.97 [95% confidence interval 0.95-0.99]) and mental (hazard ratio 0.98 [95% confidence interval 0.96-0.995]) scores on the 12-item Short Form Health Survey. CONCLUSION: Patients with PAH are infrequently referred to palliative care, even at centers of excellence. Referrals occur in sicker patients with lower quality of life scores, often close to the end of life.

ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery.

Pulmonary hypertension (PH) is a risk factor for morbidity and mortality in patients undergoing surgery and anesthesia. This document represents the first international consensus statement for the perioperative management of patients with pulmonary hypertension and right heart failure. It includes recommendations for managing patients with PH being considered for surgery, including preoperative risk assessment, planning, intra- and postoperative monitoring and management strategies that can improve outcomes in this vulnerable population. This is a comprehensive document that includes common perioperative patient populations and surgical procedures with unique considerations.

A 52-Year-Old Woman Presenting With a Right Breast Mass: A Case Report.

We present the case of a 52-year-old female who presented with a rapidly enlarging right breast mass. An initial breast ultrasound showed a solid mass with cystic components and posterior acoustical enhancement and mammography showed a mass in the same area without calcifications. Biopsy of the lesion revealed spindle cell carcinoma of the breast. To date, the patient has undergone radiation therapy and simple mastectomy and will receive post-operative radiotherapy in the coming months.

Revision reverse total shoulder arthroplasty in patients 65 years old and younger: outcome comparison with older patients.

BACKGROUND: Reverse total shoulder arthroplasty (RTSA) is a procedure growing in prevalence among younger populations. Consequently, its use in revision arthroplasty is growing in this demographic. However, studies examining the functional outcomes of revision RTSA in younger populations compared with older populations are lacking. The primary purpose of this study is to evaluate the functional outcomes of revision RTSA in patients 65 years old and younger compared with older patients who underwent revision RTSA. We hypothesized that younger patients would have similar outcomes to older patients and both groups would demonstrate improvement in outcomes. METHODS: A retrospective review was conducted on a prospectively collected research database at a single tertiary referral center of all patients who underwent RTSA between 2007 and 2018. Patients 65 years old or younger who underwent a revision RTSA and had minimum 2-year follow-up were evaluated. A control group of patients ≥70 years old who underwent revision RTSA were also evaluated. Demographics, surgical factors, active range of motion (ROM), and patient-reported outcomes (PROMs) were compared. The ROM parameters measured were forward elevation, abduction, external rotation, and level of internal rotation. The PROMs collected included American Shoulder and Elbow Surgeons score, Simple Shoulder Test score, University of California-Los Angeles score, Constant score, normalized Constant, and Shoulder Pain and Disability Index 130. The differences in outcomes were compared against the minimal clinically important difference and substantial clinical benefit reported for primary reverse shoulder arthroplasty. RESULTS: A total of 81 patients undergoing revision RTSA were evaluated at a mean follow-up of 4.5 years with 42 patients in the study group and 39 patients in the control group. Both groups demonstrated similar demographics and rates of prior surgeries. Preoperative outcome scores were lower in the study group (≤65 years old) than those in the older control group with American Shoulder and Elbow Surgeons score, Simple Shoulder Test score, and Shoulder Pain and Disability Index 130 remaining worse postoperatively. Both groups experienced statistically significant improvements in ROM from before operation to after operation, with slightly higher improvements in overhead motion in the younger cohort. Both the study group and the control group demonstrated statistically significant improvements in all PROMs with improvement above the substantial clinical benefit for the Constant and Simple Shoulder Test scores. Despite lower functional outcomes reported in the study group postoperatively, the improvement from before operation to after operation in all PROMs was similar between groups. CONCLUSION: Revision RTSA is a viable option for patients ≤65 years old with a poorly functioning shoulder arthroplasty. ROM and outcome improvements are similar compared with older patients undergoing revision RTSA, but the preoperative and postoperative functional outcomes are worse in the younger patients.

Heart-After-Liver Transplantation Attenuates Rejection of Cardiac Allografts in Sensitized Patients.

BACKGROUND: In patients undergoing heart transplantation, significant allosensitization limits access to organs, resulting in longer wait times and high waitlist mortality. Current desensitization strategies are limited in enabling successful transplantation. OBJECTIVES: The purpose of this study was to describe the cumulative experience of combined heart-liver transplantation using a novel heart-after-liver transplant (HALT) protocol resulting in profound immunologic protection. METHODS: Reported are the results of a clinical protocol that was instituted to transplant highly sensitized patients requiring combined heart and liver transplantation at a single institution. Patients were dual-organ listed with perceived elevated risk of rejection or markedly prolonged waitlist time due to high levels of allo-antibodies. Detailed immunological data and long-term patient and graft outcomes were obtained. RESULTS: A total of 7 patients (age 43 ± 7 years, 86% women) with high allosensitization (median calculated panel reactive antibody = 77%) underwent HALT. All had significant, unacceptable donor specific antibodies (DSA) (>4,000 mean fluorescence antibody). Prospective pre-operative flow cytometric T-cell crossmatch was positive in all, and B-cell crossmatch was positive in 5 of 7. After HALT, retrospective crossmatch (B- and T-cell) became negative in all. DSA fell dramatically; at last follow-up, all pre-formed or de novo DSA levels were insignificant at <2,000 mean fluorescence antibody. No patients experienced >1R rejection over a median follow-up of 48 months (interquartile range: 25 to 68 months). There was 1 death due to metastatic cancer and no significant graft dysfunction. CONCLUSIONS: A heart-after-liver transplantation protocol enables successful transplantation via near-elimination of DSA and is effective in preventing adverse immunological outcomes in highly sensitized patients listed for combined heart-liver transplantation.

EmPHasis-10 as a measure of health-related quality of life in pulmonary arterial hypertension: data from PHAR.

INTRODUCTION: While the performance of the emPHasis-10 (e10) score has been evaluated against limited patient characteristics within the United Kingdom, there is an unmet need for exploring the performance of the e10 score among pulmonary arterial hypertension (PAH) patients in the United States. METHODS: Using the Pulmonary Hypertension Association Registry, we evaluated relationships between the e10 score and demographic, functional, haemodynamic and additional clinical characteristics at baseline and over time. Furthermore, we derived a minimally important difference (MID) estimate for the e10 score. RESULTS: We analysed data from 565 PAH (75% female) adults aged mean±sd 55.6±16.0 years. At baseline, the e10 score had notable correlation with factors expected to impact quality of life in the general population, including age, education level, income, smoking status and body mass index. Clinically important parameters including 6-min walk distance and B-type natriuretic peptide (BNP)/N-terminal proBNP were also significantly associated with e10 score at baseline and over time. We generated a MID estimate for the e10 score of -6.0 points (range -5.0--7.6 points). CONCLUSIONS: The e10 score was associated with demographic and clinical patient characteristics, suggesting that health-related quality of life in PAH is influenced by both social factors and indicators of disease severity. Future studies are needed to demonstrate the impact of the e10 score on clinical decision-making and its potential utility for assessing clinically important interventions.

Inpatient Palliative Care Use in Patients With Pulmonary Arterial Hypertension: Temporal Trends, Predictors, and Outcomes.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease associated with significant morbidity and mortality. Despite the negative impact of PAH on quality of life and survival, data on use of specialty palliative care services (PCS) is scarce. RESEARCH QUESTION: We sought to evaluate the inpatient use of PCS in patients with PAH. STUDY DESIGN AND METHODS: Using the National (Nationwide) Inpatient Sample, 30,495 admissions with a primary diagnosis of PAH were identified from 2001 through 2017. The primary outcome of interest was temporal trends and predictors of inpatient PCS use in patients with PAH. RESULTS: The inpatient use of PCS was low (2.2%), but increased during the study period from 0.5% in 2001 to 7.6% in 2017, with a significant increase starting in 2009. White race, private insurance, higher socioeconomic status, hospital-specific factors, higher comorbidity burden (Charlson Comorbidity Index), cardiac and noncardiac organ failure, and use of extracorporeal membrane oxygenation and noninvasive mechanical ventilation were independent predictors of increased PCS use. PCS use was associated with a higher prevalence of do-not-resuscitate status, a longer length of stay, higher hospitalization costs, and increased in-hospital mortality with less frequent discharges to home, likely because these patients were also sicker (higher comorbidity index and illness acuity). INTERPRETATION: The inpatient use of PCS in patients with PAH is low, but has been increasing over recent years. Despite increased PCS use over time, patient- and hospital-specific disparities in PCS use continue. Further studies evaluating these disparities and the role of PCS in the comprehensive care of PAH patients are warranted.

Outcomes After Noncardiac Surgery for Patients with Pulmonary Hypertension: A Historical Cohort Study.

OBJECTIVE: Pulmonary hypertension (PH) is a substantial preoperative risk factor. For this study, morbidity and mortality were examined after noncardiac surgery in patients with precapillary PH. DESIGN: A retrospective cohort study. SETTING: Quaternary medical center in Rochester, MN. PARTICIPANTS: Adults with PH undergoing noncardiac surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The PH and surgical databases were reviewed from 2010 to 2017. Patients were excluded if PH was attributable to left-sided heart disease or they had undergone cardiac or transplantation surgeries. To assess whether PH-specific diagnostic or cardiopulmonary testing parameters were predictive of perioperative complications, generalized estimating equations were used. Of 196 patients with PH, 53 (27%) experienced 1 or more complications, including 5 deaths (3%) within 30 days. After adjustment for age and PH type, there were more complications in those undergoing moderate- to high-risk versus low-risk procedures (odds ratio [OR] 4.17 [95% confidence interval {CI} 2.07-8.40]; p < 0.001). After adjustment for age, surgical risk, and PH type, the complication risk was greater for patients with worse functional status (OR 2.39 [95% CI 1.19-4.78]; p = 0.01 for classes III/IV v classes I/II) and elevated serum N-terminal fragment of the prohormone brain natriuretic peptide (NT-proBNP) (OR 2.28 [95% CI 1.05-4.96]; p = 0.04 for ≥300 v <300 pg/mL). After adjusting for age, surgical risk, and functional status, elevated NT-proBNP remained associated with increased risk (OR 2.23 [95% CI 1.05-4.76]; p = 0.04). CONCLUSION: PH patients undergoing noncardiac surgery have a high frequency of complications. Worse functional status, elevated serum NT-proBNP, and higher-risk surgery are predictive of worse outcome.

Predictors and Clinical Outcomes of Vasoplegia in Patients Bridged to Heart Transplantation With Continuous-Flow Left Ventricular Assist Devices.

Background The presence of a durable left ventricular assist device (LVAD) is associated with increased risk of vasoplegia in the early postoperative period following heart transplantation (HT). However, preoperative predictors of vasoplegia and its impact on survival after HT are unknown. We sought to examine predictors and outcomes of patients who develop vasoplegia after HT following bridging therapy with an LVAD. Methods and Results We identified 94 patients who underwent HT after bridging with continuous-flow LVAD from 2008 to 2018 at a single institution. Vasoplegia was defined as persistent low vascular resistance requiring ≥2 intravenous vasopressors within 48 hours after HT for >24 hours to maintain mean arterial pressure >70 mm Hg. Overall, 44 patients (46.8%) developed vasoplegia after HT. Patients with and without vasoplegia had similar preoperative LVAD, echocardiographic, and hemodynamic parameters. Patients with vasoplegia were significantly older; had longer LVAD support, higher preoperative creatinine, longer cardiopulmonary bypass time, and higher Charlson comorbidity index; and more often underwent combined organ transplantation. In a multivariate logistic regression model, older age (odds ratio: 1.08 per year; P=0.010), longer LVAD support (odds ratio: 1.06 per month; P=0.007), higher creatinine (odds ratio: 3.9 per 1 mg/dL; P=0.039), and longer cardiopulmonary bypass time (odds ratio: 1.83 per hour; P=0.044) were independent predictors of vasoplegia. After mean follow-up of 4.0 years after HT, vasoplegia was associated with increased risk of all-cause mortality (hazard ratio: 5.20; 95% CI, 1.71-19.28; P=0.003). Conclusions Older age, longer LVAD support, impaired renal function, and prolonged intraoperative CPB time are independent predictors of vasoplegia in patients undergoing HT after LVAD bridging. Vasoplegia is associated with worse prognosis; therefore, detailed assessment of these predictors can be clinically important.

Global Pulmonary Vascular Remodeling in Pulmonary Hypertension Associated With Heart Failure and Preserved or Reduced Ejection Fraction.

BACKGROUND: We hypothesized that pulmonary venous hypertension in heart failure (HF) leads to predominate remodeling of pulmonary veins and that the severity of venous remodeling is associated with the severity of pulmonary hypertension (PH) in HF. METHODS: Patients with HF (n=108; 53 preserved and 55 reduced ejection fraction) with PH (HF-PH; pulmonary artery systolic pressure [PASP] ≥40 mm Hg) were compared to normal controls (n=12) and patients with primary pulmonary veno-occlusive disease (PVOD; n=17). In lung specimens from autopsy (control, HF-PH, and 7 PVOD) or surgery (10 PVOD), quantitative histomorphometry was performed in all analyzable arteries (n=4949), veins (n=7630), and small indeterminate vessels (IV; n=2168) to define percent medial thickness (arteries) and percent intimal thickness (%IT) (arteries, veins, and IV) relative to external diameter. RESULTS: The average arterial percent medial thickness (control, 6.9; HF-PH, 11.0; PVOD, 15.0), arterial %IT (control, 4.9; HF-PH, 14.9; PVOD, 31.1), venous %IT (control, 14.0; HF-PH, 24.9; PVOD, 43.9), and IV %IT (control, 10.6; HF-PH, 25.8; PVOD, 50.0) in HF-PH were higher than controls (P<0.0001 for all) but lower than PVOD (P≤0.005 for all). PASP (mm Hg) was lower in HF-PH (median, 59 [interquartile range, 50-70]) than in PVOD (median, 91 [interquartile range, 82-103]). PASP correlated with arterial percent medial thickness (r=0.41) and arterial %IT (r=0.35) but more strongly with venous %IT (r=0.49) and IV %IT (r=0.55) (P<0.0001 for all). Associations between PASP and venous or IV %IT remained significant after adjusting for arterial percent medial thickness and %IT and did not vary by HF type. In patients with right heart catheterization (30 HF-PH, 14 PVOD), similar associations between the transpulmonary gradient and pulmonary vascular remodeling existed, with numerically stronger associations for venous and IV %IT. Although the PASP was slightly higher in patients with HF-PH with right ventricular dysfunction, pulmonary vascular remodeling was not more severe. Pulmonary vascular remodeling severity was associated with reductions in the diffusing capacity of the lungs. CONCLUSIONS: In HF, PH is associated with global pulmonary vascular remodeling, but the severity of PH correlates most strongly with venous and small IV intimal thickening, similar to the pattern observed in PVOD. These findings expand our understanding of the pathobiology of PH in HF.

Early Gains in Renal Function Following Implantation of HeartMate II Left Ventricular Assist Devices May Not Persist to One Year.

Renal function improves early after left ventricular assist device (LVAD) implantation but later decline has been observed. We sought to determine the occurrence and evaluate possible causes for this decline. In 62 consecutive patients with HeartMateII LVAD with available calculated glomerular filtration rate (GFR, ml/min/1.73 m) 1 year after implant, GFR was assessed repeatedly and possible predictors for decline from 3 to 12 months were investigated. Post-mortem renal specimens for patients supported with an LVAD were evaluated. GFR 54.5 ± 19.5 at admission increased to 66.4 ± 22.3 preoperatively and to 79.2 ± 30.1 ~1 month after implantation. Subsequently at ~3 months GFR declined to 74.7 ± 25.4, at ~6 months to 68.8 ± 23.1, and ~1 year after implant to 63.9 ± 17.7. Glomerular filtration rate at 1 year was significantly lower (p < 0.0001, p < 0.0001, p = 0.005) than GFR 1, 3, and 6 months after implant. Early rise in GFR after surgery was not associated with late decline. Shorter bypass time (ß = -0.09, p = 0.048) and higher albumin 3 months after LVAD (ß = 14.4, p = 0.025) were significantly associated with less later decline in GFR. Arteriosclerosis was identified in autopsy renal specimens. In conclusion, early gains in renal function after LVAD implant are not sustained in many patients. Patient, device, and operative factors may influence long-term renal function in these patients.

Long term outcomes of cardiac transplant for immunoglobulin light chain amyloidosis: The Mayo Clinic experience.

AIM: To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis. METHODS: The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement. Baseline characteristics including age, gender, organ involvement, and New York Heart Association functional class were recorded. Laboratory data, waiting time until heart transplant, and type of treatment of the underlying plasma cell disorder were recorded. Survival from the time of OHT was calculated using Kaplan-Meier survival curves. Survival of patients undergoing OHT for AL was compared to that of non-amyloid patients undergoing OHT during the same time period. RESULTS: Twenty-three patients (median age 53 years) with AL received OHT. There were no deaths in the immediate perioperative period. Twenty patients have died post OHT. For the entire cohort, the median overall survival was 3.5 years (95%CI: 1.2, 8.2 years). The 1-year survival post OHT was 77%, the 2-year survival 65%, and the 5-year survival 43%. The 5-year survival for non-amyloid patients undergoing OHT during the same era was 85%. Progressive amyloidosis contributed to death in twelve patients. Of those without evidence of progressive amyloidosis, the cause of death included complications of autologous hematopoietic stem cell transplantation for 3 patients, post-transplant lymphoproliferative disorder for 2 patients; and for the remaining one death was related to each of the following causes: acute rejection; cardiac vasculopathy; metastatic melanoma; myelodysplastic syndrome; and unknown. Eight patients had rejection at a median of 1.8 mo post OHT (range 0.4 to 4.9 mo); only one patient died of rejection. Median survival of seven patients who achieved a complete hematologic response to either chemotherapy or autologous hematopoietic stem cell transplantation was 10.8 years. CONCLUSION: Our data demonstrate that long term survival after heart transplant is feasible in AL patients with limited extra-cardiac involvement who achieve complete hematologic response.

Submaximal Exercise Pulmonary Gas Exchange in Left Heart Disease Patients With Different Forms of Pulmonary Hypertension.

BACKGROUND: We determined whether pulmonary gas exchange indices during submaximal exercise are different in heart failure (HF) patients with combined post- and pre-capillary pulmonary hypertension (PPC-PH) versus HF patients with isolated post-capillary PH (IPC-PH) or no PH. METHODS AND RESULTS: Pulmonary hemodynamics and pulmonary gas exchange were assessed during rest and submaximal exercise in 39 HF patients undergoing right heart catheterization. After hemodynamic evaluation, patients were classified as having no PH (n = 11), IPC-PH (n = 12), or PPC-PH (n = 16). At an equivalent oxygen consumption, end-tidal CO2 (PETCO2) and arterial oxygen saturation (SaO2) were greater in no-PH and IPC-PH versus PPC-PH patients (36.1 ± 3.2 vs. 31.7 ± 4.5 vs. 26.2 ± 4.7 mm Hg and 97 ± 2 vs. 96 ± 3 vs. 91 ± 1%, respectively). Conversely, dead-space ventilation (VD/VT) and the ventilatory equivalent for carbon dioxide (V˙(E)/V˙CO2 ratio) were lower in no-PH and IPC-PH versus PPC-PH patients (0.37 ± 0.05 vs. 0.38 ± 0.04 vs. 0.47 ± 0.03 and 38 ± 5 vs. 42 ± 8 vs. 51 ± 8, respectively). The exercise-induced change in V(D)/V(T), V˙(E)/V˙CO2 ratio, and PETCO2 correlated significantly with the change in mean pulmonary arterial pressure, diastolic pressure difference, and transpulmonary pressure gradient in PPC-PH patients only. CONCLUSIONS: Noninvasive pulmonary gas exchange indices during submaximal exercise are different in HF patients with combined post- and pre-capillary PH compared with patients with isolated post-capillary PH or no PH.

Early trends in N-terminal pro-brain natriuretic peptide values after left ventricular assist device implantation for chronic heart failure.

Left ventricular assist devices (LVADs) acutely decrease left ventricular wall stress. Thus, early postoperative levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) should decrease. This study investigated postoperative changes in NT-proBNP levels, the parameters related to changes, and the possible association with complications by performing a retrospective analysis of changes in daily NT-proBNP (pg/ml) levels from admission to discharge both before and after LVAD implantation in a tertiary referral center. For 72 patients implanted with HeartMate II LVADs, baseline NT-proBNP levels were elevated at 3,943 ng/ml (interquartile range 1,956 to 12,964). Preoperative stabilization led to marked decreases in NT-proBNP. Levels peaked 3 days after surgery and subsequently decreased. Patients with complicated postoperative courses had higher early postoperative elevations. By discharge, NT-proBNP decreased markedly but was still 2.83 (1.60 to 5.76) times the age-based upper limit of normal. The 26% reduction in NT-proBNP between admission and discharge was due mostly to the preoperative reductions and not those induced by the LVAD itself. The decrease was not associated with decreases in LV volume. In conclusion, preoperative treatment reduces NT-proBNP values. The magnitude of early postoperative changes is related to the clinical course. Levels at discharge remain markedly elevated and similar to values after preoperative stabilization despite presumptive acute LV unloading.

Imatinib in pulmonary arterial hypertension: c-Kit inhibition.

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by severe remodeling of the pulmonary artery resulting in increased pulmonary artery pressure and right ventricular hypertrophy and, ultimately, failure. Bone marrow-derived progenitor cells play a critical role in vascular homeostasis and have been shown to be involved in the pathogenesis of PAH. A proliferation of c-Kit(+) hematopoietic progenitors and mast cells has been noted in the remodeled vessels in PAH. Imatinib, a tyrosine kinase inhibitor that targets c-Kit, has been shown to be beneficial for patients with PAH. Here we hypothesize that the clinical benefit of imatinib in PAH could be related to c-Kit inhibition of progenitor cell mobilization and maturation into mast cells. As a corollary to the phase 3 study using imatinib in PAH, blood samples were collected from 12 patients prior to starting study drug (baseline) and while on treatment at weeks 4 and 24. Eight were randomized to imatinib and 4 to placebo. Circulating c-Kit(+) and CD34(+)CD133(+) hematopoietic progenitors as well as biomarkers of mast cell numbers and activation were measured. Circulating CD34(+)CD133(+) and c-Kit(+) progenitor cells as well as c-Kit(+)/CD34(+)CD133(+) decreased with imatinib therapy (all P < 0.05). In addition, total tryptase, a marker of mast cell load, dropped with imatinib therapy (P = 0.02) and was related to pulmonary vascular resistance (R = 0.7, P = 0.02). The findings support c-Kit inhibition as a potential mechanism of action of imatinib in PAH and suggest that tryptase is a potential biomarker of response to therapy.

Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: results from a cross-sectional patient survey.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a complex disease with variable clinical manifestations; nevertheless, morbidity and mortality associated with PAH are considerable. This study examined quality of life (QOL) in PAH patients and assessed use of palliative care (PC) for addressing QOL issues and what barriers might exist regarding early PC implementation for patients with PAH. METHODS: An Internet-based survey was distributed to Pulmonary Hypertension Association patient-related listservs. Symptom burden and QOL were assessed using Linear Analog Self Assessment (LASA) QOL items and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). RESULTS: Of 774 eligible patients with active e-mail addresses, 315 returned surveys (41% overall response), and 276 (88%) contained analyzable responses. Responders (mean age, 48.9 years ± 16.0) were predominantly white (85%), female (86%), and with idiopathic PAH (42%). Profound deficiency in overall QOL (40%), fatigue (57%), physical well-being (56%), social activity (49%), emotional well-being (49%), and pain (38%) were reported. Most patients believed their PAH physician had excellent understanding of PAH progression/plan of care (92%), but less were satisfied with care regarding QOL management (77%). Few patients considered PC (8%), or had pain management (4%) or PC involved (1%). Most common reasons were beliefs that patients were doing well/not sick (63%) or that PC had not been suggested (22%). CONCLUSIONS: PAH may result in symptoms or QOL impairment persisting despite optimal PAH therapy. However, PC awareness or use by PAH patients and providers is low. Opportunities may exist to integrate PC into care for PAH patients.

Diagnostic dilemmas in pulmonary hypertension.

Dilemmas persist in the screening, assessment, and follow-up of patients with pulmonary hypertension, relating to issues of whom and how to screen, how to resolve ambiguities in the clinical classification of patients with multiple potential substrates of pulmonary vascular disease, how to interpret test results, how to integrate multiple clinical parameters into a global diagnosis, how to use ambiguous test results, how to determine disease severity and prognosis, and how to monitor patients on treatment. This article describes how to incorporate available information into the diagnostic process, and where lack of concrete data should impose caution in patient management.

A pulmonary hypertension gas exchange severity (PH-GXS) score to assist with the assessment and monitoring of pulmonary arterial hypertension.

Submaximal exercise gas analysis may be a useful method to assess and track pulmonary arterial hypertension (PAH) severity. The aim of the present study was to develop an algorithm, using exercise gas exchange data, to assess and monitor PAH severity. Forty patients with PAH participated in the study, completing a range of clinical tests and a novel submaximal exercise step test, which lasted 6 minutes and incorporated rest (2 minutes), exercise (3 minutes), and recovery (1 minute) ventilatory gas analysis. Using gas exchange data, including breathing efficiency, end-tidal carbon dioxide, oxygen saturation, and oxygen pulse, a pulmonary hypertension gas exchange severity (PH-GXS) score was developed. Patients were retested after about 6 months. There was significant separation between healthy controls and patients with moderate PAH (World Health Organization [WHO] class I/II) and those with more severe PAH (WHO class III/IV) for breathing efficiency, end-tidal carbon dioxide, oxygen saturation, and oxygen pulse. The PH-GXS score was significantly correlated with WHO class (r = 0.51), 6-minute walking distance (r = -0.59), right ventricular systolic pressure (r = 0.49), log N-terminal pro-B-type natriuretic peptide (r = 0.54), and pulmonary vascular resistance (r = 0.71). The PH-GXS score remained unchanged in 22 patients retested (1.50 ± 0.92 vs 1.48 ± 0.94), as did WHO class (2.3 ± 0.8 vs 2.3 ± 0.8) and 6-minute walking distance (455 ± 120 vs 456 ± 103 m). Small individual changes were observed in the PH-GXS score, with 8 patients improving and 8 deteriorating. In conclusion, the PH-GXS score differentiated between patients with PAH and was correlated with traditional clinical measures. The PH-GXS score was unchanged in our cohort after 6 months, consistent with traditional clinical metrics, but individual differences were evident. A PH-GXS score may be a useful way to track patient responses to therapy.