A review of the management of pulmonary arterial hypertension associated with congenital heart disease

Abstract

Approximately 5-10% of adolescent and adult patients with congenital heart disease (CHD) will develop pulmonary arterial hypertension (PAH). Patients with PAH associated with CHD (PAH-CHD) exhibit several similarities to those with idiopathic and other associated forms of PAH, especially with regards to their non-specific, cardinal symptoms. The development of PAH-CHD can lead to lifelong impairment although, paradoxically, survival may be better versus idiopathic PAH. Patients with PAH-CHD may experience social limitations, and emotional and psychological issues, arising from their disease burden. Nurses and other allied healthcare professionals are well placed to deliver the individually-tailored care that patients with PAH-CHD require. Activities known to be of particular benefit include patient engagement and education, patient empowerment, colleague training, and ensuring effective communication across the multidisciplinary team. Recent developments in the management of PAH-CHD have led to changes in the medical needs and optimal care of this patient population. This review aims to provide an overview of the natural course, diagnosis, symptoms and impact of PAH-CHD. We also aim to communicate the current standards in management of patients with PAH-CHD, and how their outlook can be improved in the future.

Risk factors for pulmonary arterial hypertension in children and young adults

“…CHD, a very heterogeneous condition, is a known risk factor for PH. Depending on the type of defect and previous repair approximately 5–10% of patients with CHD will develop pulmonary arterial hypertension . By adjusting for CHD, we ruled out this confounding factor in our study.…”

Information Experiences and Needs in Patients with Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension

“…Establishing clinical nurse specialists at PAH centres may be a way to support both patients and nurses and others in the patient's surrounding [10, 32]. Clinical nurse specialists can improve their own professional practice and achieve excellence in healthcare in collaboration with physicians and others in PAH teams, to support patients with special healthcare needs, their families, and even other healthcare professionals in different positions.…”

Some neonatal risk factors for adult pulmonary arterial hypertension remain unknown

“…CHD‐PH is a heterogeneous condition. Some studies suggest that approximately 5–10% of with CHD will develop pulmonary arterial hypertension, depending on the type of defect and previous repair strategies . The SPAHR registry aims to include all PH cases in Sweden, and thus, we believe that patients with PH were not included among the controls.…”