Pulmonary hypertension (PH) is a disorder involving a heterogeneous group of medical conditions encompassing several cardiopulmonary illnesses. Implementing new diagnostic criteria for PH in conjunction with multimodality diagnostic tools is crucial for accurate and early recognition of this life-threatening form of right heart failure. This should streamline early referrals to accredited PH centers, with a goal to rapidly institute targeted therapy in order to optimize prognosis.

In patients with right heart failure (RHF) and pulmonary hypertension (PH), classical teaching often advises cautious diuresis in the setting of ‘preload dependence’ to avoid renal injury and hemodynamic compromise. However, while this physiology may hold true in some clinical settings, such as acute ischemia with right ventricular infarction, it cannot necessarily be extended to PH-related RHF. Rather, in patients with precapillary PH and decompensated RHF, diuresis aimed to decongest the right heart and systemic venous system may be directly beneficial. This study aimed to evaluate the effects of diuresis on renal function and blood pressure in patients with severe precapillary PH. A retrospective chart review was conducted on 62 patients with severe precapillary PH admitted for decompensated RHF. The hemodynamic phenotype of these patients was characterized by invasive hemodynamics and echocardiographic data. Laboratory and hemodynamic data were collected at both admission and discharge. After large-volume diuresis in this patient population, there was an improvement in both glomerular filtration rate and creatinine. While there was a decline in blood pressure after diuresis, this was not clinically significant, given the blood pressure remained in a normal range with improvement in renal function. In conclusion, this study demonstrated that despite concern for preload dependence, significant diuresis in patients with acute decompensated RHF from precapillary PH is not only safe but beneficial.

Background: Invasive cardiopulmonary exercise testing (iCPET) provides valuable insight into dyspnea in patients with chronic thromboembolic pulmonary disease, in part through an increased relationship of minute ventilation to CO₂ production (V_E/VCO₂). Obesity lowers the V_E/VCO₂ in patients without cardiopulmonary disease; however, whether this holds true in obese subjects with chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary disease (CTEPD) is unknown. Objective: Report on the iCPET findings of patients with CTEPH and CTEPD and investigate the relationship between obesity and gas exchange parameters, especially V_E/VCO₂ in these patients. Methods: Retrospective analysis of CTEPH and CTEPD patients undergoing iCPET. Results: We studied 60 patients; 34 (56.7%) had CTEPH and 26 (43.3%) had CTEPD. The mean age was 61.2 ± 14 years and the mean BMI was 31.8 ± 8.3 mg/kg². A higher V_E/VCO₂ (41.9 ± 10.2 vs. 36.8 ± 8.9; p = 0.045) was observed in CTEPH vs. CTEPD. There was an inverse relationship between the V_E/VCO₂ slope and BMI. For an increase of 1 point in BMI, the V_E/VCO₂ slope fell by 0.6 in CTEPD and 0.35 in CTEPH (p < 0.001). The mean V_E/VCO₂ slope in CTEPH and CTEPD groups was 48.6 ± 10.4 in BMI < 25 and 31.3 ± 6.5 in BMI > 35 (p < 0.001). The lower V_E/VCO₂ slope in obesity relates to an increased VCO₂/work rate relationship; there was no difference in the V_E/work relationship. Conclusions: The V_E/VCO₂ slope is markedly reduced by obesity, independent of the level of pulmonary vascular obstruction in CTEPH or CTEPD. Thus, obesity masks key physiologic evidence of pulmonary vascular obstruction on the gas exchange assessment of obese individuals.

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.

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: Pelvic venous obstruction (PVO), defined as greater than 50% stenosis or occlusion of pelvic veins, is a known risk factor for deep vein thrombosis (DVT). DVT is a known risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), but the prevalence of PVO in CTEPH is unknown. Methods: This cross-sectional study at Temple University’s tertiary referral center for Pulmonary Hypertension, Right Heart Failure, and CTEPH sought to identify the presence of PVO in patients with CTEPH who underwent cardiac catheterization, pulmonary angiography, and venography. Results: A total of 193 CTEPH patients were referred for pulmonary angiography, and among these, 148 underwent venography. PVO was identified in 65 (44%) patients. Lower extremity (LE) DVT was associated with PVO (p = 0.004). The severity of pulmonary hypertension was similar with and without PVO (mean pulmonary artery pressure 43.0 ± 10.3 mm Hg vs. 43.8 ± 12.4 mm Hg, p = 0.70), as was the need for pulmonary thromboendarterectomy (69.2% vs. 61.4%, p = 0.32). Conclusions: Pelvic vein obstruction is common and a novel clinical association in patients with CTEPH, particularly in patients with a history of LE DVT. PVO and its role in CTEPH warrants further study, including the potential role of revascularization to mitigate further risk.