Paul Forfia

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.

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: 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.

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.

Right heart (RH) structure and function are major determinants of symptoms and prognosis in pulmonary arterial hypertension (PAH). RH imaging provides detailed information, but evidence and guidelines on the use of RH imaging in treatment decisions are limited. We conducted a Delphi study to gather expert opinion on the role of RH imaging in decision‐making for treatment escalation in PAH. A panel of 17 physicians with expertise in PAH and RH imaging used three surveys in a modified Delphi process to reach consensus on the role of RH imaging in PAH. Survey 1 used open‐ended questions to gather information. Survey 2 contained Likert scale and other questions intended to identify consensus on topics identified in Survey 1. Survey 3 contained Likert scale questions derived from Survey 2 and summary information on the results of Survey 2. The Delphi panel reached consensus that RH imaging is likely to improve the current risk stratification algorithms and help differentiate risk levels in patients at intermediate risk. Tricuspid annular plane systolic excursion, right ventricular fractional area change, right atrial area, tricuspid regurgitation, inferior venae cavae diameter, and pericardial effusion should be part of routine echocardiography in PAH. Cardiac magnetic resonance imaging is valuable but limited by cost and access. A pattern of abnormal RH imaging results should prompt consideration of hemodynamic evaluation and possible treatment escalation. RH imaging is an important tool for decisions about treatment escalation in PAH, but systematically collected evidence is needed to clarify its role.

Background: Given the morbidity and mortality associated with pulmonary arterial hypertension (PAH), risk stratification approaches that guide therapeutic management have been previously employed. However, most patients remain in the intermediate-risk category despite initial therapy. Herein, we sought to determine whether echocardiographic parameters could improve the risk stratification of intermediate-risk patients. Methods: Prevalent PAH patients previously enrolled in observational studies at 3 pulmonary hypertension centers were included in this study. A validated PAH risk stratification approach was used to stratify patients into low-, intermediate-, and high-risk groups. Right ventricular echocardiographic parameters were used to further stratify intermediate-risk patients into intermediate-low- and intermediate-high-risk groups based on transplant-free survival. Results: From a total of 146 patients included in our study, 38 patients died over a median follow-up of 2.5 years. Patients with intermediate-/high-risk had worse echocardiographic parameters. Tricuspid annular plane systolic excursion (TAPSE) and the degree of tricuspid regurgitation (TR) were highly associated with survival (p < 0.01, p = 0.04, respectively) and were subsequently used to further stratify intermediate-risk patients. Among intermediate-risk patients, survival was worse for patients with TAPSE < 19 mm compared to those with TAPSE ≥ 19 mm (estimated one-year survival 74% vs. 96%, p < 0.01) and for patients with moderate/severe TR compared to those with no/trace/mild TR (estimated one-year survival 70% vs. 93%, p < 0.01). Furthermore, among intermediate-risk patients, those with both TAPSE < 19 mm and moderate/severe TR had an estimated one-year survival (56%) similar to that of high-risk patients (56%), and those with both TAPSE ≥ 19 mm and no/trace/mild TR had an estimated one-year survival (97%) similar to that of low-risk patients (95%). Conclusions: Echocardiography, a routinely performed, non-invasive imaging modality, plays a pivotal role in discriminating distinct survival phenotypes among prevalent intermediate-risk PAH patients using TAPSE and degree of TR. This can potentially help guide subsequent therapy.

(1) Background: In pulmonary arterial hypertension (PAH), pregnancy is regarded a contraindication due to high maternal and fetal morbidity and mortality. We report our experience in the management of pregnancies in PAH. (2) Methods: retrospective observational study in a nationally accredited pulmonary hypertension (PH) center from 2013 to 2021. (3) Results: seven pregnancies in six women with PAH, ranging from low to high risk and 21 to 37 years old. Half had known pre-existing PAH before pregnancy. One had a multifetal gestation, and one was pregnant twice under our care. PH medical therapy and serial clinical assessment throughout pregnancy were implemented with focused attention on optimizing right heart function. Delivery was planned by a multidisciplinary team involving PH cardiology, maternal fetal medicine, and obstetric anesthesiology. Patients delivered between 31 and 40 weeks of gestation; five of the seven were via cesarean section. All received regional anesthesia and were monitored in the PH intermediate step-down unit after delivery until discharge. In all cases, delivery was without complications with excellent outcomes for the mother and child. (4) Conclusions: Multidisciplinary and tailored management of PAH in pregnancy, emphasizing optimized right heart function prior to delivery, can result in excellent clinical outcomes in a referral PH center.