Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a result of an alternate natural history in which there is limited resolution of thromboemboli with pulmonary artery obstruction leading to pulmonary hypertension (PH). CTEPH requires a thorough clinical assessment including pulmonary hemodynamics and radiologic evaluation in addition to consultation with an expert center. Surgical intervention remains the optimal management strategy. Select patients may be candidates for catheter-based intervention with balloon pulmonary angioplasty in centers with clinical expertise. Inoperable patients or those with post-intervention PH are treated with pulmonary hypertension-targeted medical therapy.

Purpose of review: The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis. Recent findings: The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme. Summary: Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.

This study validated a novel virtual echocardiography screening tool (VEST), which utilized routinely reported echocardiography parameters to predict hemodynamic profiles in pulmonary hypertension (PH) and identify PH due to pulmonary vascular disease (PH_PVD). Direct echocardiography imaging review has been shown to predict hemodynamic profiles in PH; however, routine use often overemphasizes Doppler‐estimated pulmonary artery systolic pressure (PASP_DE), which lacks discriminatory power among hemodynamically varied PH subgroups. In patients with PH of varying subtypes at a tertiary referral center, reported echocardiographic findings needed for VEST, including left atrial size, E:e’ and systolic interventricular septal flattening, were obtained. Receiver operating characteristic analyses assessed the predictive performance of VEST vs. PASP_DE in identifying PH_PVD, which was later confirmed by right heart catheterization. VEST demonstrated far superior discriminatory power than PASP_DE in identifying PH_PVD. A positive score was 80.0% sensitive and 75.6% specific for PH_PVD with an area under the curve of 0.81. PASP_DE exhibited poorer discriminatory power with an area under the curve of 0.56. VEST’s strong discriminatory ability remained unchanged when validated in a second cohort from another tertiary center. We demonstrated that this novel VEST using three routine parameters that can be easily extracted from standard echocardiographic reports can successfully capture PH patients with a high likelihood of PH_PVD. During the Covid‐19 pandemic, when right heart catheterization and timely access to experts at accredited PH centers may have limited widespread availability, this may assist physicians to rapidly and remotely evaluate PH patients to ensure timely and appropriate care.

Pulmonary hypertension (PH) encompasses a broad range of conditions, including pulmonary artery hypertension, left-sided heart disease, and pulmonary and thromboembolic disorders. Successful diagnosis and management rely on an integrated clinical assessment of the patient's physiology and right heart function. Right ventricular (RV) heart failure is often a result of PH, but may result from varying abnormalities in preload, afterload, and intrinsic myocardial dysfunction, which require distinct management strategies. Consideration of an individual's hemodynamic phenotype and physiologic circumstances is paramount in management of PH and RV failure, particularly when there is clinical instability in the intensive care setting.

Purpose of review: Although pregnancy in pulmonary arterial hypertension (PAH) is considered high risk and contraindicated, the incidence is rising. It is paramount to understand the pathophysiology and effective management strategies to ensure optimal outcomes for maternal and fetal survival. Recent findings: In this review, we highlight the outcomes of recent case series of PAH patients in pregnancy, with a focus on proper risk assessment and target goals of PAH therapy. These findings support the notion that the pillars of PAH management, including pulmonary vascular resistance reduction resulting in right heart functional improvement, and widening of the cardiopulmonary reserve, should serve as a blueprint for PAH management in pregnancy. Summary: Multidisciplinary and tailored management of PAH in pregnancy, with emphasis on optimizing right heart function prior to delivery, can result in excellent clinical outcomes in a referral pulmonary hypertension center.