Non cardiac central cyanosis in the newborn: A case report of persistent pulmonary hypertension of newborn (PPHN) and review of literature

Abstract

Abstract: PPHN is a fatal neonatal emergency resulting from poor
respiratory transition to extra uterine life, in spite of several advances in the management
thereof.
It is an uncommon disorder characterized by persistence of fetal
circulation seen in about 1 - 5 per
1000 live birth. Advances in the
management includes, inhaled
nitric oxide (iNO) and extra corporeal membrane oxygenation
(ECMO) as gold standard. However a third of patients will not
respond to standard treatment,
coupled with its dearth in some
developing countries as Nigeria.
In recent studies, treatment with
oral Sildenafil; a phosphodiesterase inhibitor type 5 (PDE5)
showed reduction in pulmonary
vascular resistance. This results in
significant increase in oxygenation
and a reduction in mortality without adverse effects. We report this
uncommon case of one mo. old
boy with PPHN who was seen in
April 2019 at University of Nigeria
Teaching Hospital Enugu. His
clinical presentation, CXR and 2D
Echo finding confirmed the diagnosis of PPHN. He was successfully managed with oral Sildenafil
and Oxygen therapy. We recommend Sildenafil may be used as
first-line treatment in settings
where iNO, High Frequency Ventilation (HFV), and ECMO are
unavailable.
Key words: Pulmonary hypertension, Newborn, Sildenafil.