Optimal management of severe pulmonary arterial hypertension

Abstract

Over the past decade, awareness among the medical profession of pulmonary arterial hypertension (PAH) being a treatable disease has increased. Despite this, approximately one-fifth of newly diagnosed patients are classified as being in the most severely compromised functional class (i.e. New York Health Association/World Health Organization functional class (NYHA/WHO FC) IV). The prognosis for patients in NYHA/WHO FC IV is poor, with 3-yr survival being around 40%, even with treatment. Poor prognosis coupled with severe functional impairment means it is vital that these patients receive optimal treatment. There are also subgroups of patients, who, although classified as NYHA/WHO FC III, may actually be severely haemodynamically compromised and at risk of rapid deterioration. Such subgroups include patients with PAH associated with systemic sclerosis or certain heritable mutations. These patients should be considered as being at the more severe end of the disease spectrum. In this article we will discuss the optimal management of patients with severe PAH. This includes newly emerging evidence from small-scale, open-label studies that use upfront combination therapy with intravenous epoprostenol plus oral PAH-specific drugs. We also review treatment strategies that may offer clinical benefits to patients with more severe PAH.

Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension

“…Three major mechanistic pathways, the endothelin, prostacyclin and nitric oxide pathways, are known to be involved in the pathogenesis of PAH and therapies that target these pathways are available [1][2][3]. Combining agents that target more than one pathway is an attractive option for the treatment of PAH and may offer additional long-term benefits compared with monotherapy [4,5].…”

Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights

“…This highlights the need for further improvements in treatment, particularly since these longer-living but morbidity-prone patients would undoubtedly suffer additional complications that require hospitalisation, such as heart failure, haemoptysis, angina and arrhythmias. Indeed, for some patients, such as those with severe haemodynamic impairment, a more timely combination of therapies may be warranted [3].…”

“…While the pathogenesis of PAH is complex, three key mechanistic pathways: 1) the endothelin, 2) the nitric oxide, and 3) prostacyclin pathways, are known to be involved in the disease [1]. Therapies that target these pathways have contributed to improved survival in patients with PAH [2][3][4]; nevertheless, the disease remains a fatal condition.…”

Perioperative Management with Upfront Combination Therapy in a Patient Exhibiting Idiopathic Pulmonary Hypertension with Central Pulmonary Thrombosis

“…In the present case, the patient was successfully treated with gynecological surgery under general anesthesia. Recently, upfront combination therapy for severe PAH associated with a low CI and high PVR has achieved dramatic improvements in pulmonary hemodynamics, although no randomized control trials have been performed (12). Since our institution specializes in pulmonary hypertension, the management of PAH, including the use of triple combination therapy, can be implemented early for well-controlled conditions to reduce the risk of complications associated with elective surgery.…”