Advances in paediatric pulmonary vascular disease associated with bronchopulmonary dysplasia

Abstract

Pulmonary hypertension (PH) is a common finding in infants with bronchopulmonary dysplasia (BPD). The aim of this review is to describe recent advances in the diagnosis and treatment of PH and discuss whether they will benefit infants and children with BPD related PH. Echocardiography remains the mainstay of diagnosis but has limitations, further developments in diagnostic techniques and identification of biomarkers are required. There are many potential therapies for PH associated with BPD. Inhaled nitric oxide has been shown to improve short term outcomes only. Sidenafil in resource limited settings was shown in three randomized trials to significantly reduce mortality. The efficacy of other therapies including prostacyclin, PDE3 inhibitors and endothelin receptor blockers has only been reported in case reports or case series. Randomized controlled trials with long term follow up are required to appropriately assess the efficacy of therapies aimed at improving the outcome of children with PH.

Keywords:

• bronchopulmonary dysplasia
• pulmonary hypertension
• pulmonary vascular disease

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Bronchopulmonary dysplasia (BPD) associated pulmonary hypertension (PH) has a high mortality.

• The true incidence of BPD associated PH needs to be established.

• Further work is required to identify reliable, non-invasive diagnostic techniques and, in particular, those being used in adults merit investigation in BPD patients.

• Accurate biomarkers need to be identified.

• Current treatment of BPD associated PH is based on limited evidence.

• A large randomized trial with long term outcomes comparing sildenafil and iNO should be undertaken.

• Other vasodilators need to be appropriately investigated in this population.

• The outcome of BPD related PH in childhood needs to be better understood.

• Identification of efficacious preventative therapies is key; whether in this respect sidenafil or stem cell therapy will improve outcome merit investigation.