Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.

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Kounis syndrome (KS) is an acute coronary syndrome (ACS) described in relationship with drugs or stents. KS was suspected in a full-term newborn who underwent a Rashkind-atrioseptostomy and stenting that periodically developed episodes of ACS. Skin test with nickel and titanium were performed being negatives. Basophil activation (BAT) and lymphocyte transformation (LTT) tests were performed for the suspected drugs. Non-responder basophils in BAT and a positive LTT for heparin were found. A new dose of heparin was administered by error, after which a new ACS was developed. We described the first report of KS by drug in a newborn.