The burden of comorbidities in pulmonary arterial hypertension

Abstract

Patients with comorbidities are often excluded from clinical trials, limiting the evidence base for pulmonary arterial hypertension (PAH)-specific therapies. This review aims to discuss the effect of comorbidities on the diagnosis and management of PAH. The comorbidities discussed in this review (systemic hypertension, obesity, sleep apnoea, clinical depression, obstructive airway disease, thyroid disease, diabetes, and ischaemic cardiovascular event) were chosen based on their prevalence in patients with idiopathic PAH in the REVEAL registry (Registry to EValuate Early and Long-term PAH disease management). Comorbidities can mask the symptoms of PAH, leading to delays in diagnosis and also difficulty evaluating disease progression and treatment effects. Due to the multifactorial pathophysiology of pulmonary hypertension (PH), the presence of comorbidities can lead to difficulties in distinguishing between Group 1 PH (PAH) and the other group classifications of PH. Many comorbidities contribute to the progression of PAH through increased pulmonary artery pressures and cardiac output, therefore treatment of the comorbidity may also reduce the severity of PAH. Similarly, the development of one comorbidity can be a risk factor for the development of other comorbidities. The management of comorbidities requires consideration of drug interactions, polypharmacy, adherence and evidence-based strategies. A multidisciplinary team should be involved in the management of patients with PAH and comorbidities, with appropriate referral to supportive services when necessary. The treatment goals and expectations of patients must be managed in the context of comorbidities.

Comparative effectiveness of endothelin receptor antagonists on mortality in patients with pulmonary arterial hypertension in a US Medicare population: a retrospective database analysis

“…3,4 Elderly patients with PAH have a higher rate of comorbid conditions compared with younger patients, introducing more complications into their treatment due to considerations such as polypharmacy and drug-drug interactions. 5 Although research has indicated that comorbidities negatively affect outcomes in patients with PAH, there is limited information regarding the effect of comorbidities on survival in elderly patients. 6,7 Evidence is also sparse on the mortality benefit of PAH therapy in elderly patients not only because the low prevalence of PAH makes it unfeasible to recruit sufficient patients of any age to detect survival differences in a randomized controlled trial (RCT) but also because elderly patients with multiple comorbidities are typically excluded from PAH RCTs.…”

Multifactorial Pulmonary Hypertension in Systemic Sclerosis

“…The patient was started on oral therapy with calcium channel blockers for vasoreactive PAH, along with disease modifying drugs for SSc with mycophenolate, steroids, prostacyclin receptor agonist, endothelin receptor antagonist, diuretics, steroids, reinforcing compliance with CPAP for OSA, optimization of medications for heart failure, and continuing treatment for anemia and MDS. Such multifactorial cases with PAH, should also focus on management of co-morbidities with a multidisciplinary team approach to optimize management from every aspect of contributing factors [ 5 - 8 ].…”

Management of pulmonary arterial hypertension in patients aged over 65 years

“…Systemic hypertension, ischaemic heart disease, dyslipidaemia, diabetes, and arrhythmia are among the most common comorbidities reported in older patients, 21 , 27 , 28 and they are more prevalent in this population compared with younger adults, as evidenced across two studies that defined older as >50 years of age, 21 and ≥75 years of age 27 . Comorbid cardiovascular and metabolic diseases are also commonly observed in the general PAH population 24 and management of PAH in these cases is fully discussed in a separate review in this supplement 29 …”