The congenital porto-caval fistula: a unique presentation and novel intervention.
Congenital porto-caval fistulas are uncommon vascular malformations with a varied clinical presentation beginning in infancy and spanning late adulthood. We report a 14-year-old male who presented with dyspnea and cough. His past medical history was significant for a chronic non-immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was found to have severe pulmonary hypertension and hyperammonemia associated with a large congenital porto-caval fistula. The abnormal vessel was occluded via endovascular covered stent placement in the vena cava. His pulmonary hypertension has improved remarkably while his chronic anemia, thrombocytopenia and ammonia have normalized to allow improved cognitive performance.
Hoover
Wynton
W
Division of Pediatric Pulmonology, Riley Hospital for Children, Indiana University, Indianapolis, Indiana, USA. whoover@Peds.UAB.edu
Ackerman
Veda
V
Schamberger
Marcus
M
Kumar
Manjusha
M
Marshalleck
Francis
F
Hoyer
Mark
M
D000293:Adolescent; D000740:Anemia / Q000150:complications; D002908:Chronic Disease; D003371:Cough / Q000209:etiology; D004417:Dyspnea / Q000209:etiology; D006801:Humans; D022124:Hyperammonemia / Q000150:complications / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007859:Learning Disabilities / Q000209:etiology; D008297:Male; D011169:Portal Vein / Q000000981:diagnostic imaging; D011651:Pulmonary Artery / Q000473:pathology; D015607:Stents; D013921:Thrombocytopenia / Q000150:complications; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D016157:Vascular Fistula / Q000150:complications / Q000151:congenital* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging