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endothelial dysfunction (including vasoconstriction); (3) vascular remodelling (which may occur in both venules and/or arterioles); (4) RV dilatation/dysfunction and functional TR;653–656 and (5) altered RV–PA coupling.655–657 The haemodynamic profile of CpcPH vs. IpcPH and elevated PVR reflects pulmonary vascular abnormalities, which contribute to an increased RV afterload. Resulting dysfunction of the RV is frequent and associated with a worse prognosis in patients with PH-LHD. In HFpEF, where RV dysfunction may occur via distinct mechanisms (Figure S1), deterioration of RV, but not LV systolic function, has been observed over time, and both prevalent and incident RV dysfunction are predictors of mortality.658 The occurrence of PH in patients with LHD may also be due to other causes, including undetected CTEPH or PAH. Further, respiratory comorbidities such as COPD and sleep apnoea are also common in patients with LHD and may contribute to PH and impact prognosis. Patients with HFpEF and PH associated with HFpEF75,76 may also present with a low DLCO, which is an independent predictor of outcome.75

8.2 Diagnosis

In patients with LHD, symptoms (e.g. exertional dyspnoea) and physical signs of PH (e.g. peripheral oedema) frequently overlap with those of the underlying left heart condition and are mostly nonspecific. However, while pulmonary congestion or pleural effusion indicate LHD as the underlying cause of PH, other features may suggest the presence of relevant PH (see Section 5.1.1). Routine diagnostic tests including BNP/NT-proBNP, ECG, and echocardiography may show signs of underlying LHD, but may also indicate PH. While BNP/NT-proBNP cannot discriminate between left- or right-sided HF, ECG findings such as right axis deviation or RV strain may suggest the presence of PH in patients with LHD. Echocardiography can diagnose HFrEF and HFpEF; identify specific cardiac conditions, including those with restrictive filling pattern; and diagnose additional valvular heart disease; it may also detect elevated sPAP and other features of PH (RA area, PA enlargement, RV/ LV ratio, LV eccentricity index, RV forming the apex), leading to an echocardiographic probability of PH (see Section 5.1.5). A stepwise, composite echocardiographic score may discriminate pre- vs. postcapillary PH and predict PVD in patients with LHD.659,660 Additional information may be gathered from further testing, including biomarkers, imaging-derived markers of RV dysfunction, and CPET-derived variables.142 Given the complexity and variability of cardiopulmonary haemodynamics in patients with LHD, the distinction between post- and pre-capillary PH and the diagnosis of PH-LHD vs. other forms of PH can be challenging. Diagnostic clues in the evaluation of suspected PH in LHD include: (1) diagnosis and control of the underlying LHD; (2) evaluation for PH and patient phenotyping; and (3) invasive haemodynamic evaluation, when indicated.

8.2.1 Diagnosis and control of the underlying left

heart disease Patients with suspected PH-LHD will have an established diagnosis of LHD, such as HFrEF/HFmrEF, HFpEF, valvular heart disease, and/or CHD. The distinction between PH associated with HFpEF and other forms of PH (e.g. PAH, CTEPH) may be challenging, particularly given the increased burden of cardiovascular comorbidities in real-world PAH populations.142,450,661 In this context, validated scores for diagnosing HFpEF (HFA-PEFF, H2FPEF)16,662,663 may be helpful for detecting it as an underlying condition in PH, and the presence or absence of risk factors for PAH or CTEPH should be determined. Patients with signs of predominant RV strain and/or PH should be further evaluated. Patients should be assessed or reassessed when they are fully recompensated and in a clinically stable condition.

8.2.2 Evaluation of pulmonary hypertension and

patient phenotyping Patients with LHD and suspected PH should be evaluated following the diagnostic strategy for PH (see Section 5). This requires identifying clinical features and a multimodal approach using non-invasive diagnostic tests such as echocardiography, ECG, and BNP/ NT-proBNP levels. In the presence of mild PH and predominant LHD, no further testing may be necessary. Otherwise, CTEPH and significant lung disease should be ruled out by V/Q scan and PFTs, and additional cardiac imaging including cMRI may be considered in selected cases. For phenotyping, a combination of variables may help to determine the likelihood of LHD, and HFpEF in particular, vs. other causes of PH (Table 23). Pulmonary hypertension associated with left heart disease is likely in the presence of known cardiac disease, multiple cardiovascular comorbidities/risk factors, atrial fibrillation at diagnosis, and specific imaging findings (LV hypertrophy, increased LA size, and reduced LA strain). Although exercise echocardiography has been proposed to uncover HFpEF, it is unable to diagnose or classify PH in this context. A combination of clinical findings and phenotyping is required to decide about the need for further invasive assessment.

8.2.3 Invasive assessment of haemodynamics

The decision to perform cardiac catheterization and to invasively assess cardiopulmonary haemodynamics should depend on the presence of an intermediate to high echocardiographic probability of PH, and should be determined by the need to obtain relevant information for prognostication or management. In patients with a high likelihood of LHD as the main cause of PH, or with established underlying LHD and mild PH (Table 23), invasive assessment for PH is usually not indicated. Indications for RHC in LHD include: (1) suspected PAH or CTEPH; (2) suspected CpcPH with a severe pre-capillary component, where further information will aid phenotyping and treatment decisions (Figure S2); and (3) advanced HF and evaluation for heart transplantation. While several haemodynamic measures (mPAP, PVR, pulmonary arterial compliance [PAC], transpulmonary pressure gradient, and DPG) are associated with outcomes in PH-LHD,142,632,635 the most robust and consistent data are available for PVR. Invasive assessment should be conducted in experienced centres, when management of the underlying LHD has been optimized and patients are in a clinically stable condition. With respect to respiratory variations of intrathoracic pressures, all pressure readings should be taken at end-expiration. Additional testing during RHC may be useful for distinguishing between PAH and HFpEF,18,23,664–669 and to uncover LHD in patients with a high likelihood of PH-LHD and normal resting PAWP;670–673 both exercise testing and fluid challenge may be considered in special situations (see Section 5.1.12). Conditions associated with reduced LV diastolic compliance or valvular heart disease may be associated Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026