Comparing Diagnosis and Treatment of Pulmonary Hypertension Patients at a Pulmonary Hypertension Center versus Community Centers.

Once patients are diagnosed with pulmonary hypertension it is important to identify the correct diagnostic group as it will have implications on the disease state management. Pulmonary hypertension is increasingly diagnosed and treated in general medical practices; however, evidence-based guidelines recommend evaluation and treatment in pulmonary hypertension centers for accurate diagnosis and appropriate treatment recommendations. We conducted a retrospective cohort study of 509 random patients 18 years and older who were evaluated in our pulmonary hypertension clinic from January 2005 to December 2018. 68.4% (n = 348) had their diagnostic group clarified or changed. Pulmonary hypertension was deemed an incorrect diagnosis in 12.4% (n = 63). A total of 114 patients (22.4%) had been initiated on pulmonary hypertension specific treatment prior to presentation. Pulmonary hypertension specific medication was stopped in 57 (50.0%) cases. The estimated monthly saving of the stopped medication based on wholesale acquisition costs was USD 396,988.05-419,641.05, a monthly saving of USD 6964.70-7362.12 per patient. Evaluation outside of a pulmonary hypertension center may lead to misdiagnosis and inappropriate or inadequate treatment. Pulmonary arterial hypertension directed therapy improves median survival, but inappropriate therapy may cause harm; therefore, patients benefit from a specialized center with multiple resources to secure an accurate diagnosis and tailored treatment for their condition.

Facemasks and Walk Distance in Pulmonary Arterial Hypertension Patients.

Little is known about the effect of wearing a facemask on the physiological and perceptual responses to exercise in patients with pulmonary arterial hypertension (PAH). We performed a single-center retrospective study to evaluate whether facemask wearing impacted distanced covered, rating of perceived exertion (RPE), and arterial oxygen saturation (SpO2) during a 6-minute walk test (6MWT) in PAH patients. Forty-five patients being treated for group 1 PAH and who performed a 6MWT before and after implementation of a facemask mandate were included in the analysis. Each included patient performed a 6MWT without (test 1) and with (test 2) a facemask between October 1, 2019, and October 31, 2020. At both time points, all patients also underwent a submaximal cardiopulmonary exercise test, echocardiogram, and blood laboratory tests, with a Registry to Evaluate Early and Long-Term PAH Disease Management Lite 2.0 score calculated. The two 6MWTs were performed 81±51 days apart, and all patients were clinically stable at both testing timepoints. Six-minute walk test distance was not different between test 1 and test 2 (405±108 m vs 400±103 m, P=.81). Similarly, both end-test RPE and lowest SpO2 during the 6MWT were not different in test 1 and test 2 (RPE: 2.5±1.7 vs 2.5±2.1, P=.91; SpO2 nadir: 92.8±3.4% vs 93.3±3.3%, P=.55). Our findings show that wearing a facemask has no discernable impact on the arterial oxygen saturation and perceptual responses to exercise or exercise capacity in patients with moderate-to-severe PAH. This study reinforces the evidence that wearing a facemask is safe in PAH patients, even during exercise.

Psychosocial and Financial Burden of Therapy in USA Patients with Pulmonary Arterial Hypertension.

Abstract: Pulmonary arterial hypertension (PAH) is a devastating disease with significant morbidity and mortality. There are many psychosocial and financial implications of this disease; however, little is known how this affects the treatment of PAH patients. A questionnaire-based prospective cohort study was performed on 106 PAH patients from a Pulmonary Hypertension Center and the Pulmonary Hypertension Association national conference in 2018. The demographic, treatment, psychosocial, employment, financial impact on treatment data was obtained. The majority of patients had cardiopulmonary symptoms despite treatment. The symptoms affected their social and work lives, with about one in three applying for disability because of their PAH. The majority of PAH patients had insurance coverage, but still noted a significant financial burden of the disease, with nearly a half who needed financial assistance to pay for their PAH medications. Thirty (28.3%; 95% CI, 20.6-37.5%) patients mentioned they changed their medication regimen, with some skipping doses outright (28 [26.4%; 95% CI, 19-35.6%]) in order to save money. PAH continues to cause significant psychosocial and financial burden on patients despite advances in medications. This impact ranged from dissatisfaction with quality of life, to unemployment, to altering their medication regimen to save money.

Twelve-Year Survival in a Patient With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension on Nifedipine Monotherapy.

Pulmonary arterial hypertension is a progressive vascular disease with a high mortality rate without proper therapy. Identification of the appropriate treatment for each patient is critical in regard to adverse effects, health care costs, ease of treatment, and the potential for prognostication. Treatment strategies typically begin with acute vasoreactivity testing, which is performed during a right heart catherization. If positive, a calcium channel blocker may work; however, another pulmonary arterial hypertension-specific medication is necessary when testing is negative. Acute vasoreactivity testing is currently recommended to be performed only in certain subgroups of pulmonary arterial hypertension, but not when related to connective tissue disease. In this report, we describe a patient who had systemic sclerosis-related pulmonary arterial hypertension with a positive acute vasoreactivity test result. The patient was placed on calcium channel blocker monotherapy that has been well tolerated for 12 years, resulting in improved symptoms and exercise capacity. The long-term response to calcium channel blocker therapy in systemic sclerosis-associated pulmonary arterial hypertension has not been previously described. In addition, pulmonary artery pressures have been well controlled. The absence of genetic smooth muscle variants prevalent in vasoresponsive idiopathic pulmonary arterial hypertension is also unique.

Pulmonary arterial stiffness assessed by cardiovascular magnetic resonance imaging is a predictor of mild pulmonary arterial hypertension.

Early detection of mild pulmonary arterial hypertension (PAH) based on clinical evaluation and echocardiography remains quite challenging. In addition to enhanced right ventricular (RV) assessment, cardiac magnetic resonance (CMR) imaging may accurately reflect deleterious remodeling and increased stiffness of the central pulmonary arteries based on pulsatility, or percent change of the PA during the cardiac cycle. The purpose of this study is to assess the utility of measuring PA pulsatility by CMR as a potential early maker in PAH. We hypothesize that pulsatility may help discriminate mild PAH from normal control subjects. Consecutive patients with PAH (n = 51) were prospectively enrolled to receive same day CMR and right heart catheterization (RHC). PA stiffness indices including pulsatility, distensibility, compliance, and capacitance were calculated. Comparisons were made between patients with varying severities of PAH and normal controls (n = 18). Of the 51 subjects, 20 had mild PAH, and 31 moderate-severe based on hemodynamic criteria. PA pulsatility demonstrated a progressive decline from normal controls (53%), mild PAH (22%), to moderate-severe PAH (17%; p < 0.001). There was no difference in RV size, function or mass between mild PAH and normal controls. PA pulsatility below 40% had an excellent ability to discriminate between mild PAH and normal controls with a sensitivity of 95% and specificity of 94%. CMR assessment of PA stiffness may noninvasively detect adverse pulmonary vascular remodeling and mild PAH, and thus be a valuable tool for early detection of PAH. Trial Registration: ClinicalTrials.gov Identifier: NCT01451255; https://clinicaltrials.gov/ct2/show/NCT01451255 .

Prevalence of Sodium and Fluid Restriction Recommendations for Patients with Pulmonary Hypertension.

BACKGROUND: Patients with pulmonary hypertension (PH) are often afflicted with the consequences of right heart failure including volume overload. Counseling to assist the patient in the dietary restriction of sodium and fluid may be underutilized. METHODS: Consecutive patients seen in the PH Clinic at Mayo Clinic in Florida from June to November 2013. RESULTS: 100 patients were included; 70 were women and most had group 1 PH (n = 69). Patient characteristics using mean (±SD) were: Age 63 ± 13 years, functional class 3 ± 1, brain natriuretic peptide 302 ± 696 pg/mL, 6-min walk 337 ± 116 m, right atrial pressure 8 ± 5 mmHg, and mean pulmonary artery pressure 42 ± 13 mmHg. Overall, 79 had had complete (32) or partial instruction (47) and 21 had no prior counseling to restrict sodium or fluid. Of the 47 with partial instruction, 42 received complete education during the PH Clinic visit. Of the 21 without prior instruction, 19 received complete education during the PH visit. Seven patients with the opportunity to have their education enhanced or provided did not receive any additional counseling during the PH visit. CONCLUSION: Sodium and fluid restriction is an important but perhaps underutilized strategy to manage volume overload in patients with right heart failure. Focused questioning and education may permit an increase in the patients receiving instruction in this regard.

Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension.

BACKGROUND: Elevated mean right atrial pressure (RAP) measured by cardiac catheterization is an independent risk factor for mortality. Prior studies have demonstrated a modest correlation with invasive and noninvasive echocardiographic RAP, but the prognostic impact of estimated right atrial pressure (eRAP) has not been previously evaluated in patients with pulmonary arterial hypertension (PAH). METHODS: A retrospective analysis of 121 consecutive patients with PAH based on right-sided heart catheterization and echocardiography was performed. The eRAP was calculated by inferior vena cava diameter and collapse using 2005 and 2010 American Society of Echocardiography (ASE) definitions. Accuracy and correlation of eRAP to RAP was assessed. Kaplan-Meier survival analysis by eRAP, right atrial area, and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) risk criteria as well as univariate and multivariate analysis of echocardiographic findings was performed. RESULTS: Elevation of eRAP was associated with decreased survival time compared with lower eRAP (P < .001, relative risk = 7.94 for eRAP > 15 mm Hg vs eRAP ≤ 5 mm Hg). Univariate analysis of echocardiographic parameters including eRAP > 15 mm Hg, right atrial area > 18 cm², presence of pericardial effusion, right ventricular fractional area change < 35%, and at least moderate tricuspid regurgitation was predictive of poor survival. However, multivariate analysis revealed that eRAP > 15 mm Hg was the only echocardiographic risk factor that was predictive of mortality (hazard ratio = 2.28, P = .037). CONCLUSIONS: Elevation of eRAP by echocardiography at baseline assessment was strongly associated with increased risk of death or transplant in patients with PAH. This measurement may represent an important prognostic component in the comprehensive echocardiographic evaluation of PAH.