Development of the Pulmonary Hypertension Functional Classification Self-Report: a patient version adapted from the World Health Organization Functional Classification measure.

BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by progressive limitations on physical activity, right heart failure, and premature death. The World Health Organization functional classification (WHO-FC) is a clinician-rated assessment used widely to assess PAH severity and functioning, but no equivalent patient-reported version of PAH symptoms and activity limitations exists. We developed a version of the WHO-FC for self-completion by patients: the Pulmonary Hypertension Functional Classification Self-Report (PH-FC-SR). METHODS: Semistructured interviews were conducted with three health care providers (HCPs) via telephone to inform development of the draft PH-FC-SR. Two rounds of semi-structured interviews were conducted with 14 US patients with a self-reported PAH diagnosis via telephone/online to elicit concepts and iteratively refine the PH-FC-SR. RESULTS: HCPs reported that the WHO-FC was a useful tool for evaluating patients' PAH severity over time and for making treatment decisions but acknowledged that use of the measure is subjective. Patients in round 1 interviews (n = 6) reported PAH symptoms, including shortness of breath (n = 6), fatigue (n = 5), syncope (n = 5), chest pains (n = 3), and dizziness (n = 3). Round 1 patients identified challenges with the original WHO-FC, including comprehensibility of clinical terms and overlapping descriptions of class II and III, and preferred the Draft 1 PH-FC-SR over the original WHO-FC. After minor changes were made to Draft 2, round 2 interviews (n = 8) confirmed patients understood the PH-FC-SR class descriptions, interpreting them consistently. CONCLUSIONS: The HCP and patient interviews identified and confirmed certain limitations inherent within the clinician-rated WHO-FC, including subjective assessment and overlapping definitions for class II and III. The PH-FC-SR includes patient-appropriate language, symptoms, and physical activity impacts relevant to patients with PAH. Future research is recommended to validate the PH-FC-SR and explore its correlation with the physician-assessed WHO-FC and other outcomes.

Navigating the Sotatercept landscape: A meta-analysis of clinical outcomes.

Pulmonary arterial hypertension (PAH) is a widespread condition that affects around 1% of the global population, with a higher prevalence among older individuals. The approach to managing PAH has undergone significant changes, requiring extensive treatment strategies. Sotatercept, an FDA-approved medication, has recently attracted attention for its potential role in PAH therapy. However, information on its safety and effectiveness is scarce. In this study, we performed a meta-analysis of existing randomized clinical trials to assess the impact of Sotatercept on PAH patients. Our findings revealed that those treated with Sotatercept showed greater improvement in pulmonary vascular resistance and World Health Organization functional class compared with placebo recipients. The occurrence of adverse events was similar between both groups. Importantly, the Sotatercept group displayed a considerably higher number of cases with an increase in hemoglobin levels. Considering that about 33% of PAH patients experience anemia and both anemia and polycythemia can adversely affect disease prognosis, additional research is necessary to establish the potential advantages and disadvantages of Sotatercept as a treatment choice, specifically regarding its erythropoietic properties.

Atrial flutter and fibrillation in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension in the ASPIRE registry: Comparison of rate versus rhythm control approaches.

BACKGROUND: The development of atrial flutter and fibrillation (AFL/AF) in patients with pre-capillary pulmonary hypertension has been associated with an increased risk of morbidity and mortality. Rate and rhythm control strategies have not been directly compared. METHODS: Eighty-four patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) with new-onset AFL/AF were identified in the ASPIRE registry. First, baseline characteristics and rates of sinus rhythm (SR) restoration of 3 arrhythmia management strategies (rate control, medical rhythm control and DC cardioversion, DCCV) in an early (2009-13) and later (2014-19) cohort were compared. Longer-term outcomes in patients who achieved SR versus those who did not were then explored. RESULTS: Sixty (71%) patients had AFL and 24 (29%) AF. Eighteen (22%) patients underwent rate control, 22 (26%) medical rhythm control and 44 (52%) DCCV. SR was restored in 33% treated by rate control, 59% medical rhythm control and 95% DCCV (p < 0.001). Restoration of SR was associated with greater improvement in functional class (FC) and Incremental Shuttle Walk Distance (p both <0.05). It also independently predicted superior survival (3-year survival 62% vs 23% in those remaining in AFL/AF, p < 0.0001). In addition, FC III/IV independently predicted higher mortality (HR 2.86, p = 0.007). Right atrial area independently predicted AFL/AF recurrence (OR 1.08, p = 0.01). DCCV was generally well tolerated with no immediate major complications. CONCLUSIONS: Restoration of SR is associated with superior functional improvement and survival in PAH/CTEPH compared with rate control. DCCV is generally safe and is more effective than medical therapy at achieving SR.

Preprocedural frailty is strongly associated with symptoms after balloon pulmonary angioplasty.

Balloon pulmonary angioplasty (BPA) has improved the survival rate of patients with chronic thromboembolic pulmonary hypertension (CTEPH). The resolution of symptoms is one of the remaining goals of BPA. Frailty affects the outcome of cardiovascular diseases or treatments. The aim of this study is to assess the association between frailty and outcome of BPA. The resolution of symptoms is evaluated by the post-BPA World Health Organization functional class (WHO-FC). A total of 54 patients with CTEPH were divided into 2 groups by post-BPA WHO-FC (WHO-FC I group; n = 34 vs. WHO-FC ≥ II group; n = 20). Frailty was assessed by physicians using the clinical frailty scale (CFS) at the point of patient admission for their first BPA sessions. Compared to the WHO-FC ≥ II group, the WHO-FC I group was younger (65.6 ± 13.9 years vs. 74.3 ± 8.0 years) and had a lower CFS (3 [3, 4] vs. 4 [4, 6]) (median [25th, 75th percentiles]). The WHO-FC I achievement rates for each CFS score were CFS 3: 82.8%; 4: 53.8%; 5: 25.0%; 6: 33.3%; and 7: 20.0%. Logistic regression analysis showed that CFS was an independent predictor of WHO-FC I achievement (odds ratio 0.50, p = 0.012), but pre-BPA hemodynamic parameters and age were not independent predictors. Whether WHO-FC I can be achieved is predicted by pre-BPA patient frailty but not by pre-BPA hemodynamic parameters and age.

The Utility of Brain Natriuretic Peptide in Patients Undergoing an Initial Evaluation for Pulmonary Hypertension.

Introduction: Brain natriuretic peptide (BNP) is a polypeptide released from the cardiac ventricles and has been used as a diagnostic marker in cardiovascular diseases. Some patients with pulmonary hypertension have significant increases in BNP levels. This study wanted to determine whether the BNP levels in patients referred for evaluation of possible pulmonary hypertension were associated with a particular functional class or diagnostic group. Methods: Data were collected on patients from the Pulmonary Vascular Disease clinic undergoing right heart catheterization between 1/1/2019 and 5/20/2020. Clinical information, laboratory results including BNP, and hemodynamic parameters were recorded. Results: This study included 117 patients referred for evaluation for PH with measured BNP levels. The mean age was 63; the female to male ratio was 2:1, 25.4% of the patients were Hispanic. The average BNP level for the entire cohort was 4127.1 ± 11761.98 pg/ml. Patients in higher WHO functional classes tended to have higher levels of BNP, but statistical analysis BNP showed no differences between the functional classes. Patients in WHO Group 4 had significantly higher BNP levels than other WHO groups. Hemodynamic group classification demonstrated significant differences in BNP values between the low, intermediate, and high composite score patients. Conclusions: Patients undergoing evaluation for pulmonary hypertension had a wide range of BNP values. Patients with more abnormal composite hemodynamic scores higher BNP levels. Measurement of BNP provides an independent test to help interpret patients' descriptions of their functional limitations and to identify patients with more abnormal hemodynamic parameters.

Using a knowledge translation program to facilitate guideline- and evidence-based patient management: the PAH-QuERI Extension Program.

The Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative Extension Program was designed to support physicians' adherence to pulmonary arterial hypertension (PAH) guidelines. Guidelines were followed in >95% of patients with functional class (FC) II/III, but for only 28.6% of FC IV patients (Month 36). Low adherence was driven by FC IV patients' preference to avoid parenteral treatment.

Incremental Shuttle Walking Test Distance Is Reduced in Patients With Pulmonary Hypertension in World Health Organisation Functional Class I.

Background: There is increasing interest in screening for and diagnosing pulmonary hypertension earlier in the course of disease. However, there is limited data on cardiopulmonary abnormalities in patients with pulmonary hypertension newly diagnosed in World Health Organization Function Class (WHO FC) I. Methods: Data were retrieved from the ASPIRE registry (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral center) for consecutive treatment naïve patients diagnosed with pulmonary hypertension by cardiac catheterization between 2001 and 2010 who underwent incremental shuttle walk exercise testing. Results: Eight hundred and ninety-five patients were diagnosed with Group 1-5 pulmonary hypertension. Despite the absence of symptoms, patients in WHO FC I (n = 9) had a significant reduction in exercise capacity (Incremental shuttle walk distance percent predicted (ISWD%pred) 65 ± 13%, Z score -1.77 ± 1.05), and modest pulmonary hypertension with a median (interquartile range) pulmonary artery pressure 31(20) mmHg and pulmonary vascular resistance 2.1(8.2) Wood Units, despite a normal diffusion of carbon monoxide adjusted for age and sex (DLco)%pred 99 ± 40%. Compared to patients in WHO FC I, patients in WHO FC II (n = 162) had a lower ISWD%pred 43 ± 22 and lower DLco%pred 65 ± 21%. Conclusion: Our results demonstrate that patients with newly diagnosed pulmonary hypertension with no or minimal symptomatic limitation have a significant reduction of exercise capacity.