Case Report The patient is a female with Turner’s Syndrome (45, XO) born at 32 weeks gestation by emergency caesarean section with a birth weight of 1.6KG. The neonatal course was complicated by respiratory distress, hypoglycemia, coagulopathy, gram negative sepsis and small bowel perforation. Ongoing clinical issues included transaminitis, conjugated hyperbilirubinemia and bilateral grade 4 intra-ventricular haemorrhages.
The patient was transferred from a neonatal intensive care unit to our institutions paediatric intensive care unit where her cardiorespiratory profile continued to deteriorate. She was started on noradrenaline, milrinone and intravenous diuresis to support cardiac output and ameliorate left sided pulmonary oedema. 2D echocardiography demonstrated PAPVC of the left upper pulmonary vein to the innominate vein as well as a small secundum ASD, mild right heart dilatation, moderate RVH and well preserved biventricular systolic function. There was moderate PR and mild TR with an estimated right ventricular systolic pressure approaching systemic levels.
CT angiogram confirmed a left PAPVC to innominate vein via a vertical vein and suggested only a small segment of the left lower lobe was draining to the left atrium. The CT also raised the possibility of a vertical vein stenosis.
At eight weeks corrected the patient presented for cardiac cathederisation under GA. Pulmonary artery pressure was systemic on manometry. The mean pressure in the vertical vein measured 24mmHg with a 9mmHg gradient across the stenosis.
A stent was placed across the stenosis and inflated to 22 ATM. Relief of the stenosis was achieved and the procedure was tolerated without complication.
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