Abstract
Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. The pathophysiologic model is mainly characterized by an afterload mismatch in which an increased right ventricle afterload, driven by increased pulmonary vascular resistance (PVR), leads to right heart failure. International guidelines recommend optimization of treatment based on regular risk assessments to achieve or maintain a low-risk status. Current risk scores are based on a multi-modality approach, including demographic, clinical, functional, exercise, laboratory, and hemodynamic parameters, which lack significant echocardiographic parameters. The originality of echocardiography relies on the opportunity to assess in a non-invasive way a physiologically meaningful combination of easy to measure variables tightly related to right ventricle adaptation/maladaptation to increased afterload, the main determinant of a patient’s prognosis. Echo-derived morphological and functional parameters have been investigated in PAH, proving to have prognostic relevance. Different therapeutic strategies proved to have different effects in reducing PVR. An upfront combination of drugs, including a parenteral prostacyclin, has shown to be associated with right heart reverse remodeling in a greater proportion of patients than other treatment strategies as a function of PVR reduction. Adding echocardiographic data to current risk scores would allow better identification of right ventricle (RV) adaptation in PAH patients’ follow-up. This additional information would allow better stratification of the patient, leading to optimized and personalized therapeutic management.
Highlights
Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. It is characterized by the remodeling of small pulmonary arterial vessels with a subsequent increase in pulmonary vascular resistance (PVR)
International guidelines recommend optimization of treatment based on regular risk assessments to achieve or maintain a low-risk status [2] as defined by risk scores derived from different registries: Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) [3], Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) [4] Swedish Pulmonary Arterial Hypertension Register (SPAHR) [5], and the French
Standardization of data collection was not guaranteed in all published registries, with a significant loss of data. All these scores are based on survival and do not consider clinical worsening and disease progression, which do not match with clinical goals to be achieved according to current guidelines [7]
Summary
Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. A different approach characterized the FPHR, which considered the number of low-risk variables achieved among 6MWD, WHO FC, RAP, and cardiac index, BNP or NT-proBNP. In FPHR, risk assessment was performed in incident IPAH patients All these scores have the advantage of being multiparametric and based on simple parameters that are easy to obtain, allowing a patient’s risk stratification into three classes (low-intermediate-high). Standardization of data collection was not guaranteed in all published registries, with a significant loss of data All these scores are based on survival and do not consider clinical worsening and disease progression, which do not match with clinical goals to be achieved according to current guidelines [7]. Most patients end up in the intermediate-risk category, characterized by a heterogeneous population with different outcomes, representing a missed opportunity for treatment optimization and personalized medicine [8]
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