Updated Treatment Algorithm of Pulmonary Arterial Hypertension

Abstract

The demands on a pulmonary arterial hypertension (PAH) treatment algorithm are multiple and in some ways conflicting. The treatment algorithm usually includes different types of recommendations with varying degrees of scientific evidence. In addition, the algorithm is required to be comprehensive but not too complex, informative yet simple and straightforward. The type of information in the treatment algorithm are heterogeneous including clinical, hemodynamic, medical, interventional, pharmacological and regulatory recommendations. Stakeholders (or users) including physicians from various specialties and with variable expertise in PAH, nurses, patients and patients' associations, healthcare providers, regulatory agencies and industry are often interested in the PAH treatment algorithm for different reasons. These are the considerable challenges faced when proposing appropriate updates to the current evidence-based treatment algorithm.The current treatment algorithm may be divided into 3 main areas: 1) general measures, supportive therapy, referral strategy, acute vasoreactivity testing and chronic treatment with calcium channel blockers; 2) initial therapy with approved PAH drugs; and 3) clinical response to the initial therapy, combination therapy, balloon atrial septostomy, and lung transplantation. All three sections will be revisited highlighting information newly available in the past 5 years and proposing updates where appropriate. The European Society of Cardiology grades of recommendation and levels of evidence will be adopted to rank the proposed treatments.

Comparative Effectiveness of Pharmacologic Interventions for Pulmonary Arterial Hypertension

“…[4][5][6][7] Hence, evidence regarding the best treatment, either alone or in combination, is limited, leaving such decisions to individual clinical judgment. 8,9 A network meta-analysis approach can bridge this gap and guide both clinical decision-making and future research. 10,11 Therefore, we performed a network meta-analysis combining direct and indirect evidence to evaluate comparative efficacy and safety of all US Food and Drug Administration (FDA)-approved pharmacologic interventions, alone or in combination, in patients with PAH.…”

Sildenafil in a cigarette smoke-induced model of COPD in the guinea-pig

“…Studies in experimental models of PH induced by hypoxia [10] or monocrotaline [11,12] have shown that sildenafil reduces pulmonary artery pressure (PAP), prevents right ventricular (RV) hypertrophy and exerts an anti-remodelling effect in pulmonary vessels. Sildenafil is currently used for the treatment of pulmonary arterial hypertension [13].…”

Cerebellar Syndrome as a Presentation of Pulmonary Hypertension

“…2 Primary or secondary PH classification no longer exists and a new one including 5 groups has been proposed by the World Health Organization (WHO). 3 PH in our case caused right ventricular failure (represented in the TTE and ECG findings) and presented as an acute cerebellar syndrome due to a paradoxical cerebral stroke. Both PFO and aortic regurgitation (AR) were the causes of PH here.…”