Ricerc@Sapienza

Pulmonary arterial hypertension (PAH) is a rare disease characterized by a narrowing of the small arteries and arterioles, which cause an elevation of pulmonary vascular resistance and lead to the development of right ventricular failure and death, if untreated. Despite advances in drug development, life expectancy remains poor and similar to that of many advanced cancers.
Currently, little is known about modality of death in these patients. In a recent prospective, monocentric study by Tonelli et al, PAH acted as the substratum, but not as the direct cause of death in a significant amount of deceased patients. In fact, progressive right ventricular failure or sudden death were the sole cause of death in half of patients. Several precipitants (mainly respiratory, infective and renal diseases) were therefore often crucial in determining clinical worsening and then triggering death. Furthermore, the main risk scores that have been found predictive of one-year mortality in patients with PAH have never systematically investigated the modality of death of patients with PAH nor it is clear whether specific risk factors may relate to specific modalities of death, in particular those triggered by extra-cardiac causes.
In left heart failure, different cofactors, such as pneumonia, ischemia, arrhythmia, nonadherence to diet or medications, hypertension, worsening of renal function, have a well-established role in triggering recrudescence of the pathology and increasing mortality. This knowledge allowed to adopt specific management strategies to prevent clinical worsening and death.
Similarly, understanding precipitants that contribute to exacerbations of Pulmonary Hypertension and lead to hospitalization and death, particularly those that are avoidable as triggered by preventable causes, is of great importance to clinicians and could favorably influence Pulmonary Hypertension disease management.