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

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.

Keywords

• pulmonary hypertension
• newborn
• sildenafil