Pulmonary Hypertension

Use right-heart catheterization (RHC) to establish the diagnosis and obtain mean pulmonary artery pressure (mPAP), pulmonary arterial wedge pressure (PAWP), cardiac output (CO), and pulmonary vascular resistance (PVR). Resting PH is defined by mPAP greater than 20 mm Hg; contemporary classification then depends on PAWP and PVR rather than mPAP alone. AHA Journals+1AHA JournalsEvaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association | CirculationjaccCardiopulmonary Hemodynamics in Pulmonary Hypertension and Heart Failure: JACC Review Topic of the Week

Obtain CO during the diagnostic RHC and calculate PVR. Incorporating PVR distinguishes pulmonary vascular disease from high-flow states with normal resistance, while PAWP or left-ventricular end-diastolic pressure measurement reduces inappropriate labeling of left-heart disease as PAH. jaccjaccDefinitions and Diagnosis of Pulmonary Hypertension | JACC

In patients with established PH, assess right-ventricular adaptation rather than relying only on pulmonary pressure. Progressive right-ventricular dilation signals loss of ventricular-arterial coupling and is a key follow-up parameter. jaccjaccThe Relationship Between the Right Ventricle and its Load in Pulmonary Hypertension | JACC

When PAWP exceeds 15 mm Hg, manage the case as PH associated with left-heart disease until proven otherwise. In HFpEF cohorts, PH due to left-heart disease has been defined by mPAP at least 25 mm Hg with PAWP greater than 15 mm Hg, and PVR, transpulmonary gradient, and diastolic pressure gradient have been used to identify a combined pulmonary vascular component. jacc+1jaccPulmonary Arterial Capacitance Is an Important Predictor of Mortality in Heart Failure With a Preserved Ejection Fraction | JACC: Heart FailurejaccCardiopulmonary Hemodynamics in Pulmonary Hypertension and Heart Failure: JACC Review Topic of the Week

When PAWP is 15 mm Hg or less and PVR is elevated, pursue pre-capillary etiologies. The current classification framework separates PAH, chronic thromboembolic disease, PH associated with chronic lung disease, and other PH groups; chronic lung disease-associated PH is classified as World Symposium group 3. AHA Journals+2AHA JournalsEvaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association | CirculationWileyAbstracts from the 17th International Neonatal and ...WileyExercise pulmonary hypertension in chronic thromboembolic pulmonary disease: A right heart catheterization study - Dhayyat - 2024 - Pulmonary Circulation - Wiley Online Library

Actively identify chronic thromboembolic disease because it changes treatment from medical management alone to potential mechanical intervention. Detection of peripheral chronic thromboembolic lesions helps distinguish CTEPH from other PH causes and determines whether lesions are surgically accessible or better suited to balloon pulmonary angioplasty. Wolters KluwerWolters KluwerChronic thromboembolic pulmonary hypertension anno 2021 : Current Opinion in Cardiology

At diagnostic RHC, measure mPAP, PAWP, and CO under standardized conditions and derive PVR. Contemporary hemodynamic assessment emphasizes meticulous RHC because the same mPAP elevation has different diagnostic, prognostic, and therapeutic implications when PAWP or PVR differs. jacc+1jaccDefinitions and Diagnosis of Pulmonary Hypertension | JACCjaccCardiopulmonary Hemodynamics in Pulmonary Hypertension and Heart Failure: JACC Review Topic of the Week

Use acute vasoreactivity testing selectively in PAH phenotyping rather than as a general test for all PH. In PAH associated with congenital heart disease, inhaled iloprost during RHC improved hemodynamic parameters and was well tolerated in the reported pediatric cohort; acute changes in PVR are also used when considering reversibility before defect correction in selected congenital-shunt patients. Wolters KluwerWolters KluwerInvasive Hemodynamic and Vasoreactivity Testing with... : Cardiology Discovery

Do not extrapolate a single provocative maneuver across all phenotypes. Exercise, passive leg raise, and fluid challenge correlate only moderately to strongly in practice, and their variable reclassification rates require integration with the patient’s left-heart phenotype and the entire invasive dataset. ATS JournalsATS JournalsHemodynamic Responses to Provocative Maneuvers during Right Heart Catheterization | Annals of the American Thoracic Society

Pulmonary endarterectomy (PEA) is first-line treatment for operable CTEPH. Surgical candidacy depends on whether chronic thromboembolic lesions are technically accessible, making anatomic evaluation at an experienced CTEPH center essential before concluding that disease is inoperable. ScienceDirect+1ScienceDirectChronic thromboembolic pulmonary hypertension and balloon pulmonary angioplasty – Where are we in 2024? - ScienceDirectWolters KluwerChronic thromboembolic pulmonary hypertension anno 2021 : Current Opinion in Cardiology

Use balloon pulmonary angioplasty (BPA) for selected patients with technically inoperable distal disease, prohibitive surgical risk, or recurrent PH after PEA. BPA has emerged as a therapeutic option for these groups, whereas PEA remains preferred when operable disease is present. ScienceDirect+1ScienceDirectChronic thromboembolic pulmonary hypertension and balloon pulmonary angioplasty – Where are we in 2024? - ScienceDirectWolters KluwerChronic thromboembolic pulmonary hypertension anno 2021 : Current Opinion in Cardiology

PH-directed medical therapy is a third established CTEPH treatment modality and may be combined with mechanical therapy. In a systematic review of five studies, riociguat followed by combination therapy with BPA was associated with larger gains in WHO functional class, PVR, CO, and 6-minute walk distance than either therapy alone; the pooled evidence base was limited and should not replace individualized multidisciplinary selection. ScienceDirect+1ScienceDirectBalloon pulmonary angioplasty and riociguat in the management of chronic thromboembolic pulmonary hypertension: a systematic review - ScienceDirectScienceDirectManagement of chronic thromboembolic pulmonary hypertension - ScienceDirect

Discuss treatment at an expert center because modern procedural risk is center-dependent. Reported peri-interventional mortality is below 3% for PEA and below 1% for BPA in expert centers. ScienceDirectScienceDirectManagement of chronic thromboembolic pulmonary hypertension - ScienceDirect

Track right-ventricular size and function longitudinally. Right-ventricular hypertrophy can maintain coupling early in PH, whereas progressive dilation indicates late failure of adaptation and should prompt reassessment of hemodynamics, disease mechanism, and treatment strategy. jaccjaccThe Relationship Between the Right Ventricle and its Load in Pulmonary Hypertension | JACC

In PH due to left-heart disease, an elevated PVR identifies a greater pulmonary vascular burden than isolated post-capillary PH. Reconsider occult or undertreated elevation in left-sided filling pressure when symptoms, PAWP, and PVR are discordant, and use protocolized provocative RHC selectively rather than assuming a fixed PAH phenotype. jacc+2jaccPulmonary Arterial Capacitance Is an Important Predictor of Mortality in Heart Failure With a Preserved Ejection Fraction | JACC: Heart FailurejaccCardiopulmonary Hemodynamics in Pulmonary Hypertension and Heart Failure: JACC Review Topic of the WeekATS JournalsHemodynamic Responses to Provocative Maneuvers during Right Heart Catheterization | Annals of the American Thoracic Society

After CTEPH intervention, evaluate persistent or recurrent PH for residual proximal or distal obstruction and reassess whether BPA, medical therapy, or an additional multimodal approach is appropriate. Both PEA and BPA may be considered in chronic thromboembolic pulmonary disease even without resting PH in selected patients, underscoring the importance of symptom and anatomic reassessment rather than mPAP alone. ScienceDirectScienceDirectManagement of chronic thromboembolic pulmonary hypertension - ScienceDirect