The rise of methamphetamine-associated pulmonary arterial hypertension in the U.S. highlights a need for appropriate and effective management strategies. The complexities of Methamphetamine-Associated Pulmonary Arterial Hypertension are magnified by socioeconomic and health disparities associated with substance use disorders. This study asked: what are the experiences, barriers, and unmet needs of patients living with methamphetamine-associated pulmonary arterial hypertension? Structured interviews were conducted with 20 U.S. patients living with methamphetamine-associated pulmonary arterial hypertension. Interviews explored methamphetamine use patterns, circumstances of Pulmonary Arterial Hypertension diagnosis, treatment experiences, barriers to care, and perceptions of compassion and education during management. Participants were geographically diverse; most were female and White, with nearly half over 50 years old. Patients report having used methamphetamine almost daily, typically by inhalation; more than one-third reported use for over 10 years. At diagnosis, 60% of patients were still actively using, and half of these patients discontinued use within 1 year of diagnosis. Initial diagnosis commonly occurred in emergency departments, and delays in treatment were frequent, often related to lack of insurance or negative experiences with physicians. Patients described inadequate education and limited collaboration with substance use specialists. Compassionate care was perceived as lacking, particularly among those treated outside pulmonary hypertension specialty centers. This first-of-its-kind patient-centered study of methamphetamine-associated pulmonary arterial hypertension highlights major unmet needs in timely diagnosis, access to therapy, and provision of compassionate, informed care. Findings underscore the urgency of increasing awareness, strengthening education for both patients and providers, and integrating substance use support into pulmonary hypertension management.

BACKGROUND:Pulmonary vascular obstruction causes dyspnea in chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH). Conventional assessments, like 6-minute walk test (6MWT) and World Health Organization (WHO) functional class (WHO-FC), poorly discriminate the mechanism of dyspnea. Cardiopulmonary exercise testing (CPET) offers direct evaluation of ventilatory efficiency and gas-exchange abnormalities. OBJECTIVES:The purpose of this study was to evaluate the impact of balloon pulmonary angioplasty (BPA) and pulmonary thromboendarterectomy (PTE) on CPET-derived gas-exchange parameters. METHODS:In this prospective study, patients with CTEPD or CTEPH evaluated by a multidisciplinary team underwent outpatient point-of-care CPET (SHAPE-HF system) before and after BPA/PTE. The primary endpoint was change in ventilatory efficiency, assessed by the VE/VCO2 (minute ventilation/carbon dioxide production) slope. Secondary endpoints included changes in WHO-FC and 6MWT. RESULTS:Sixty patients were evaluated; 8 served as controls, and 52 underwent revascularization (20 PTE and 32 BPA). The VE/VCO2 slope improved from 43 to 31 (P < 0.001) after BPA and from 42 to 32 (P = 0.02) after PTE. WHO-FC improved from class III to I in both groups (P < 0.001). The mean 6MWT increased 38 m (381 ± 146 vs 419 ± 151 m; P = 0.03) in the BPA and PTE group (349 ± 162 vs 358 ± 140 m; P = 0.7). VE/VCO2 and Shape-HF Severity Score positively correlated (P < 0.001), but not with 6MWT (P = 0.83). Before and after revascularization, the Shape-HF score improved in the BPA (2.4 vs 1.5; P < 0.001) and the PTE (2.3 vs 1.6; P = 0.01) groups. Survival at 1-year follow-up was a 100%. CONCLUSIONS:Point-of-care CPET provides objective assessment of gas-exchange improvements after pulmonary revascularization in mild-to-moderate CTEPH/CTEPD.

Post-Splenectomy patients are at an increased risk of developing Chronic Thromboembolic Pulmonary Hypertension (CTEPH). We sought to characterize this population. Retrospective chart review of CTEPH patients was conducted and patients with splenectomy were identified and compared to those without. 529 patients with CTEPH were identified and 17 (3.2%) had a history of splenectomy. Of the splenectomy group, they were all White, 5 (29.4%) were female, mean age was 62 ± 12 years, and 7 (41.2%) had red blood cell dyscrasias (i.e. hereditary spherocytosis, thalassemia, and stomatocytosis). Direct oral anticoagulants were used in 15 (88.2%) of the splenectomy patients and 7 (41.2%) were on PH medications. Eight (47.1%) underwent Pulmonary Thromboendarterectomy (PTE), 7 (41.2%) underwent Balloon Pulmonary Angioplasty (BPA) and 1 (5.9%) underwent PTE with subsequent BPA. The mean pulmonary artery (mPA) pressure after intervention was the only significant difference amongst the group receiving BPA vs PTE. For the splenectomy patients who underwent PTE, 6 had segmental disease and 2 had subsegmental disease. Comparing patients with and without splenectomy, there was no significant difference in 6-minute walk (413 ± 147 vs 340 ± 159 meters, p=.08), mean PA (42.5 ± 12.6 vs 43.1 ± 11.8 mmHg, p=0.8), or pulmonary vascular resistance (7.2 ± 3.5 vs 8.2 ± 5, p=0.4). Our findings support the conclusion that post splenectomy patients develop segmental and distal disease.

Venous thromboembolism is significantly affected by hormonal and reproductive factors that pose unique challenges in women. Among various risk factors, the role of uterine fibroids, which are the most common benign tumors in women, is not well understood. The relationship between venous thromboembolism and fibroids is mainly attributed to the physical compression caused by large fibroids on pelvic veins, particularly the iliac veins, leading to venous stasis and thrombosis. This review explores the prevalence, pathogenesis, risk factors, possible racial influences, and management strategies of venous thromboembolism associated with fibroids. It highlights the need for better awareness, considering the asymptomatic nature of many fibroids and their potential to lead to serious thromboembolic complications. There is a clear need for screening methods, detailed guidelines, and treatments to prevent such complications and improve women’s health care.

Introduction Pulmonary hypertension (PH) typically presents with dyspnea and decreased functional status. We aimed to compare the traditional 6-minute walk distance (6MWD) test with a submaximal point of care shape-HFTM Cardiopulmonary Exercise Testing (CPET) System to better characterize patient's etiologies of symptoms and severity to better direct care. Methods Retrospective chart review of patients with PH, basic demographics, WHO functional class (FC) and group, PH medications, CPET parameters and 6MWD were obtained. Individual CPET parameters were obtained and integrated into organ-systemic specific ‘silo’ scores. The ‘silo’ scores incorporate multiple related variables specific to the organ system; i.e., heart silo utilizes VE efficiency linear slope, highest VO2/VE, O2 pulse to VO2 linear slope, and heart rate (HR) recovery. Chi Square and Anova tests were used for statistical analysis. Results Of the 24 patients with PH, 2(8.3%) were Black, 18(75%) were White, 4(16%) were Hispanic, 19(79%) were female. Regarding the WHO-FC, 2 (8.3%) patients were FC 1, 10 (41.7%) FC 2, 8 (33.3%) FC 3, 4 (16.7%) FC 4. There were 14 (58%) patients with WHO group 1, 6 (25%) WHO group 2, 2 (8%) WHO group 3, 1 (4.2%) WHO group 4, and 1 (4.2%) mixed WHO groups. The mean age was 61.9±14.6 years and the mean BMI was 31.8±7.2kg/m2. More than half of the cohort was not receiving PH medications 13 (54.2%). The mean 6MWD was 349.3±148.4 meters. The mean VE/VCO2 slope was 37.2±13.7, mean O2 efficiency slope was 1.6±0.6., mean peak gas capacitance was 431.4±278.1, mean peak VO2 was 34.4±24.3, mean HR at rest was 82.1±0.2bpm, mean PetCO2 was 31.0± 6.46-mmHg, mean SpO2 was 94.17 ±3.16. Shorter distance in the 6MWD was associated with worse FC (I= 485; II=431, III/IV=282 meters) (p=0.5). Better FC was associated with lower shape severity score (I=0.8; II=1; III=2.3; IV=2.2; p=0.2) and VE/VCO2 slope (I=29; II=34; III=37; IV=34; p=0.2). The mixed WHO group had elevated scores in all silos while group 3 had elevated scores in predominately lung silos. The figure demonstrates the different silos per WHO Group. Conclusion Point of care CPET done in 6 minutes can provide more detailed physiological information than the traditional 6MWD in PH patients with competing causes of dyspnea.

Background: Pulmonary thromboendarterectomy (PTE) remains the preferred treatment for surgical accessible thrombus in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, residual pulmonary hypertension (PH) can persist post-PTE. Methods: A retrospective single-center analysis of patients that underwent PTE between 2013 and 2023. At 3-month follow-up, we performed a qualitative Echo-Doppler (DE) assessment and applied a semi-quantitative DE scoring system (DESS), assigning point values for six DE parameters: right ventricle (RV) size, RV shape (systolic base–apex ratio), RV function, septal position, tricuspid regurgitation (TR) and RV outflow tract notching (RVOTN). Higher scores suggested a more significant residual PH syndrome. Results: A total of 188 subjects (80%) did not require further PH intervention at ≥3 months (Group A); 48 (20%) required ongoing PH treatment (Group B). The pre-PTE median DESS was 10 and the post-PTE median DESS was 3.00 (range 0–16). The maximum DESS was 17. Using ROC analysis, post-PTE DESS strongly discriminated between Group A and B (AUC 0.76; 95% CI 0.65–0.89; p < 0.001). A post-PTE DESS of >6.5 differentiated Group A and B. Evidence of TR (OR 0.191, CI 0.103–0.279; p < 0.0001) and RV enlargement (OR 0.242; CI 0.153–0.330; p < 0.0001) at follow-up was associated with a need for additional PH interventions. Conclusions: Serial DE examination is a viable, noninvasive method to assess significant residual PH post-PTE.

Pulmonary hypertension (PH) management requires thoughtful evaluation from the clinicians [...]

RATIONALE: Women of childbearing and childrearing age may face additional challenges and burden in pregnancy and motherhood when diagnosed with a progressive disease, such as pulmonary arterial hypertension (PAH). This study sought to capture the unique perceptions, attitudes, and behaviors in women with PAH related to their disease. METHODS: This is a quantitative, multi-country survey evaluating the impact of PAH on women of childbearing and childrearing age (21-50 years). Respondents completed a survey comprised of de novo questions derived from prior qualitative interviews. Perceptions, attitudes, and behaviors were summarized descriptively. Ethics approval/exemption was obtained in all included countries. Interim results are reported for the United States (US), and United Kingdom (UK). Final analysis which includes ∼120 more patients from Canada, Germany, France, and Italy will be available for presentation at the conference. RESULTS: 150 patients were included in interim analysis (US: n=120; UK: n=30). Patients were an average of 41 years old. The mean time from symptom onset to diagnosis was 2.4 years. Perceptions and attitudes: Nearly 1/4th of patients felt that certain healthcare providers had been dismissive of their symptoms due to their sex (24%) and lifestyle factors (25%; e.g. obesity). Sixteen-percent of patients reported their male physician could not relate to their situation. Overall, 63% of patients were advised not to have children following their PAH diagnosis. Feelings of extreme grief about the inability to have children naturally were reported by 17% (score of 6 or 7; 1-7 scale, 1= Received extremely inadequate/ unhelpful information), where 1= Experienced absolutely no grief; 7=Experienced extreme grief). Forty three percent of patients noted a moderate /very high financial burden of PAH on their family due to out-of-pocket expenses associated with PAH diagnosis and treatment. Behaviors: Patients reported having to self-research about the disease which involved gathering information about their disease on the internet (45%), social media (45%) and/or via patient advocacy groups (32%). 37% received information/resources from their health care provider; among them 38% (score of 7; 1-7 scale, 1= Received extremely inadequate/unhelpful information, 7= Received extremely helpful/adequate information) considered that the provided information was extremely helpful. CONCLUSIONS: This study presents novel findings on the unique perceptions and behaviors of women with PAH especially around challenging interactions with healthcare system and mechanisms to address these challenges, including the risks of childbearing. These findings underscore the need for improved disease awareness, individualized patient-healthcare provider communication and treatment.

Background: Sarcopenia, or loss of skeletal muscle mass, has been associated with poor outcomes (e.g., functional decline, increased mortality, and low quality of life), but its role in CTEPH remains unclear. The psoas muscle index (PMI) is a validated measure of sarcopenia. We investigated the incidence of sarcopenia using PMI in CTEPH. Methods: Retrospective analysis of a single-center cohort of patients with CTEPH with an available computed tomography of the abdomen and pelvis (CTAP). PMI was measured at the L3 level of the CTAP and was then calculated using the formula (left psoas area + right psoas area/height2). Patients in the first quartile of PMI were classified as sarcopenic. Results: We reviewed 558 patients with CTEPH, and 97 patients had an available CTAP before intervention. Sarcopenia was identified in 26 (24.8%) of the patients and was associated with worse baseline functional status (p = 0.008), higher mean pulmonary artery pressure (48 vs. 39 mmHg; p = 0.002), and higher pulmonary vascular resistance (9.9 vs. 6.8 WU; p = 0.013). Post-PTE, patients with sarcopenia exhibited longer intensive care unit (ICU) (9 vs. 4 days, p < 0.001) and overall hospital stays (24 vs. 11 days, p < 0.001), despite similar post-operative hemodynamics achieved compared to non-sarcopenic patients. Conclusions: CTEPH patients with sarcopenia have worse baseline functional class and hemodynamics. For those with sarcopenia requiring surgery, there is longer ICU and total hospitalization stays, but they achieve significant functional improvements and hemodynamics comparable to that of non-sarcopenic patients. Hence, the risk of longer perioperative hospitalization days is justified by the longer-term benefit of hemodynamic improvement. The use of PMI as part of routine pre-operative assessments could improve clinical decision-making in CTEPH patients undergoing surgical or medical intervention.

BACKGROUND:Pulmonary arterial hypertension (PAH) remains underrecognized and life-threatening due to limited awareness, nonspecific symptoms, and late referral to accredited pulmonary hypertension (PH) centers. The previously validated virtual echocardiography screening tool (VEST) predicts PAH hemodynamics. The objectives of the present study were to determine if the novel automated electronic medical record (EMR)-based algorithm could accurately calculate VEST scores to identify PAH hemodynamics and aid referral to PH specialty care. METHODS:This study is a retrospective analysis of 4,952 patients who underwent transthoracic echocardiogram (TTE) with tricuspid regurgitation velocity (TRV) ≥2.9 m/s in a hospital with an accredited PH Center of Comprehensive Care. Using the automated EMR-based algorithm, EMR-calculated VEST scores were calculated and compared to manually calculated VEST scores. Automated EMR VEST scores were used to identify those with highest risk for PAH (+3 score). Patients with +3 score were analyzed to determine whether they were evaluated within the accredited PH center or undergone right heart catheterization (RHC), the gold standard for PH diagnosis. RESULTS:Automated EMR VEST scores were validated with 100% correlation to 60 manual scores. Of 354 patients with +3 score, those that underwent RHC had severe PH, with mean pulmonary artery pressure 48 mm Hg and pulmonary vascular resistance 8.5 Wood units. One hundred and four patients (29.4%) were never referred for specialty PH care, and of these, only 37.5% underwent RHC. In the 250 patients referred to subspecialty PH care, 237 (94.8%) underwent RHC. CONCLUSIONS:This novel EMR-based automated VEST calculator is a powerful yet simple scoring tool that can capture patients at high risk for PAH, prompting earlier diagnosis and referrals to accredited PH centers to allow for earlier expert care and implementation of medical therapies.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive debilitating disease that disproportionately impacts young women. The aim of this study is to explore caregiver networks and support around these women. METHODS: This is an interim descriptive analysis of data from an online survey of women (age 21-50) with PAH living in the US and UK. De novo questions and validated instruments of social support and interpersonal support evaluation were included in the survey. Institutional Review Board/Ethics Committee exemption was obtained in all countries. Final analysis which includes ∼120 more patients from Canada, Germany, France, and Italy will be available for presentation at the conference. RESULTS: This analysis includes 150 women patients (US=120, UK=30). Regarding the make-up of informal caregiver networks, in the US patients most frequently reported spouses (58%), children (53%) and friend(s) (51%) as their caregivers, while in UK patients most frequently reported spouse (53%), relatives (30%) and parent(s) (23%). In the US patients required support from their spouse almost every day to provide financial support (49%), check in how they were doing (46%) and household duties (47%), while in the UK patients required support almost every day with household duties (25%), taking care of important people in life (31%) and making efforts to cheer up the patient (31%). Participants were asked to indicate the amount of support they needed versus the amount of support they receive. In the past four weeks patients reported ‘needing support' with household duties, 88% (US) and 100% (UK); caring for their parents and children, 64% (US) and 83% (UK); and finances, 72% (US) and 67% (UK). Patients reported ‘having reliable support’ with household duties, 88% (US) and 87% (UK); caring for their parents or children, 79% (US) and 90% (UK); and finances 72% (US) and 90% (UK). CONCLUSIONS: This study highlights that women with PAH require a strong network of caregivers and most burden of caregiving falls on family members. Despite these networks of care, there may be areas where more support is needed. Further research is warranted to investigate areas where patients don't have full coverage from their informal caregiver network and explore to what extent these patients are linked to formal support programs to cover those needs.

RATIONALE: Women diagnosed with pulmonary arterial hypertension (PAH), a rare and progressive disease, face an increased burden related to childbearing and childrearing across different regions. The aim of this study was to assess variations in the burden of disease in women with PAH across countries. METHODS: Women with self-reported PAH aged 21-50 years completed a 45-minute quantitative survey on the holistic impact of PAH. The survey included validated patient-reported outcome scales (WPAI, SF12, EQ5D-5L) and de novo questions derived from prior qualitative interviews. Survey responses were analyzed descriptively and stratified by country (US and UK). Exemption/approval was obtained from ethics committees for both countries, as applicable. Final analysis which includes ∼120 more patients from Canada, Germany, France, and Italy will be available for presentation at the conference. RESULTS: Interim quantitative survey data were collected from 150 respondents in the US (n=120), UK (n=30). The humanistic burden of PAH was reported to be high. Across both countries, PAH was reported to have a profound impact on quality of life (US=34%, UK = 30%, rated 6 or higher on a 7-point Likert scale where 1 is ‘absolutely no impact’ and 7 is ‘severely impacted’). Fear of worsening of PAH was higher in the US (71%) than in the UK (47%) and grief experienced by patients due to PAH was similar across both countries (US=47%, UK=43%). Forty-three-percent patients in the US and 77% patients in UK were employed part or full time. Patients reported missing 5.6 hours (mean) of work across both countries in the past 7 days due to PAH. Patients reported missing more work in the UK (mean 8.2 hours of 26 hours worked) as compared to the US (mean 4.5 hours of 27.9 hours worked). Patients reported receiving government/employer-sponsored disability benefits in both the US (58%) and UK (73%). Higher 12-month out-of-pocket costs >$1000 USD (e.g., medications, visits) were reported in patients in the UK (97%) as compared to US (50%). CONCLUSIONS: Findings from this study confirm that PAH exerts a substantial burden on women across both countries even though the level of impact differed between countries. The burden of PAH spanned clinical, humanistic, and economic domains, regardless of country. Moving forward, a holistic management approach is needed to address the high overall disease burden, as well as the unique challenges faced by women with PAH globally.

BackgroundCurrent clinical guidelines support use of parenteral prostacyclin therapy for patients with pulmonary arterial hypertension (PAH) at intermediate risk. The objective of this study was to assess parenteral prostacyclin therapy use among patients at intermediate risk according to the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) 2.0 four-strata risk assessment model.MethodsThis was a retrospective chart review and cross-sectional online survey of healthcare professionals (HCPs). Included patients were classified as intermediate-low or intermediate-high risk per COMPERA 2.0 between 2016 and 2020 (index visit), initiated on a parenteral prostacyclin any time following intermediate risk assessment, and had World Health Organization (WHO) Functional Class (FC), 6-minute walk distance (6MWD), and B-type natriuretic peptide/N-terminal pro B-type natriuretic peptide (BNP/NT-proBNP) assessments at index and first comprehensive follow-up visits (follow-up).ResultsA total of 139 HCPs (53% community-based, 47% Pulmonary Hypertension Care Center-based) participated in the survey and provided 350 patient records; among these, mean age (SD) was 54.1 (15.3) years and 52% were female. Median (IQR) time from parenteral prostacyclin initiation to follow-up was 3.0 months (2.0, 7.0). At parenteral prostacyclin initiation for the 280 patient records with available COMPERA 2.0 assessments, 62% of patients were intermediate-high risk, 33% were intermediate-low risk and 3% were low risk, improving to 38%, 53%, and 8%, respectively, at follow-up.ConclusionsImprovements were seen for the individual COMPERA 2.0 risk calculator parameters and for several other clinical parameters. Findings from this study substantiate recent guidelines suggesting earlier use of this treatment in intermediate-risk patients with PAH.Clinical trial numberNot applicable.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of incomplete resolution of acute pulmonary embolism. We hypothesize changes in CT Hounsfield Unit gradient (HU-Δ) created by the dispersion of IV contrast through the downstream blood pool correlate with cardiac index (CI). We sought to compare HU-Δ with invasively obtained CI. Methods: We completed a retrospective analysis of CTEPH patients in which individuals with low CI (<2.2-L/min/m2) were identified. Both absolute and fractional HU-Δ were derived from pulmonary CTA by subtracting the HU value of the left atrium (LA) and left ventricle (LV) from the main pulmonary artery (MPA) (absolute) and expressing them as a percentage of MPA-HU (fractional) on static axial images. These were compared between low and normal CI. Results: Of the 237 patients, 50.2% were female, 53.2% were White, 36.7% were Black. Hemodynamics were mean pulmonary artery (PA) pressure = 45.4 ± 11.2-mmHg, pulmonary vascular resistance = 9.2 ± 4.4-WU, CI = 2.05 ± 0.48-L/min/m2. There was a higher mean MPA-HU = 391.1 ± 113.6 than LA-HU = 251.6 ± 81. In patients with low CI, the HU-Δ was higher, HU-ΔMPA-LA was 148.9 ± 78.4 vs. 124.5 ± 77.2 (p = 0.02), and HU-ΔMPA-LV was 170.7 ± 87 vs. 140 ± 82 (p = 0.009). A HU-ΔMPA-LA = 118 had a sensitivity of 75.6% and specificity of 77% to detect low CI, AUC 0.61, p = 0.003. A HU-ΔPA-LV = 156 had a sensitivity of 77% and specificity of 53% to detect low CI, AUC = 0.62, p = 0.001. A fractional reduction HU-ΔMPA-LA of 35% had a sensitivity and specificity of 79% and 53%, respectively, to detect low CI (AUC 0.65, p < 0.001). A fractional reduction of the HU-ΔMPA-LV of 40% had a sensitivity and specificity of 80% and 55%, respectively, to detect low CI (AUC 0.65, p < 0.001). HU Δ were highly reproducible (Kappa = 0.9, p < 0.001, 95% CI 0.86–0.95). Conclusions: High HU Δ between MPA-LA and MPA-LV were associated with low CI in patients with CTEPH.

Cardiogenic shock (CS) is a heterogeneous clinical syndrome characterized by low cardiac output leading to end-organ hypoperfusion. Organ dysoxia ranging from transient organ injury to irreversible organ failure and death occurs across all CS etiologies but differing by incidence and type. Herein, we review the recognition and management of respiratory, renal and hepatic failure complicating CS. We also discuss unmet needs in the CS care pathway and future research priorities for generating evidence-based best practices for the management of extra-cardiac sequelae. The complexity of CS admitted to the contemporary cardiac intensive care unit demands a workforce skilled to care for these extra-cardiac critical illness complications with an appreciation for how cardio-systemic interactions influence critical illness outcomes in afflicted patients.

Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease.