Abstract
Persistent pulmonary hypertension of newborn (PPHN) is a very rare manifestation of congenital cytomegalovirus (CMV) infection. PPHN associated with CMV can be severe but is usually transient and responds well to antiviral therapy. We report a rare case of PPHN that occurred in the setting of fulminant congenital CMV infection and successful treatment with antiviral therapy along with review of the very few cases reported in literature. A male appropriate for gestational Age (AGA) newborn developed rapidly progressive respiratory distress starting at 11 hours of life requiring ventilatory support and 100% oxygen. He developed hypotension and wide difference between pre and postductal saturations. Echocardiography revealed findings consistent with severe PPHN. Examination also revealed multiple purpuric skin lesions and soft hepatosplenomegaly. MRI Brain showed intraventricular hemorrhage, bilateral periventricular calcification, bilateral cerebral and cerebellar intraparenchymal hemorrhage. Complete Cell Count (CBC) revealed severe thrombocytopenia and blood serum showed positive Immunoglobulin M (IgM) for CMV and Urinary CMV was positive by nucleic acid test. He was treated with ganciclovir, inhaled nitric oxide and inotropes. He recovered and was discharged on day 24 of life. Severe PPHN is a rare manifestation of congenital CMV infection and carries a high risk of morbidity and mortality. Congenital CMV should be considered in neonates with PPHN of unknown etiology. Early institution of antiviral therapy in these babies is associated with favorable outcome.