Case Presentation:

A 19–year–old woman with a history of migraines, depression and a bony cyst in her jaw presented with abdominal pain. Three days prior, she acutely developed sharp, non–radiating epigastric pain which was worse with inspiration. She denied nausea, vomiting, diarrhea, appetite changes, or fevers. She was diagnosed with gastritis and was given ranitidine. Her pain worsened and shifted to her left upper quadrant with radiation to her left shoulder. On examination, oxygen saturation was 84% on room air with otherwise normal vital signs. She had tenderness to palpation of the left upper quadrant, a grade 2/6 systolic murmur at the left mid–sternal border, a continuous 2/6 murmur heard over her back, and mild clubbing with some nail bed cyanosis. Upon further questioning, she denied shortness of breath, cough or chest pain. Laboratories were unremarkable. CT of the chest showed no evidence of deep venous thrombosis or pulmonary embolism; left atrial enlargement and a splenic infarct were noted. There was suggestion of a possible connection between the right pulmonary artery and the left atrium. She denied any recent travel, oral contraceptive use, or family history of clotting disorders. On further history, she was incidentally noted to be hypoxic during routine excision of a bony cyst of her jaw at age fourteen. An echocardiogram was performed which was reportedly normal. No further work up or pulse oximetry checks were performed until her current presentation. Retrospectively, she has had lifelong dyspnea. She has no family history of congenital heart disease. Transthoracic echocardiogram confirmed a moderate to large fistulous communication between the right pulmonary artery and the left atrium which was thought to be the route that led to her embolic event. She was treated with enoxaparin and subsequently underwent surgical ligation of the fistula.

Discussion:

Abdominal pain is a common presenting complaint in hospitalized patients. While a rare cause of abdominal pain, splenic infarction needs to be considered in the differential, especially with severe, acute pain in the left upper quadrant. Embolic events are one cause of splenic infarction. Although emboli are more likely to originate from the left side of the circulation, they may originate from the right side if a direct communication exists. Congenital heart disease, which is typically diagnosed in childhood, can cause a pathway for this direct communication while also being a cause of persistent hypoxia. As demonstrated in this patient who had unexplained hypoxia with a normal initial echocardiogram, this diagnosis can be difficult to make if there is not a high clinical index of suspicion.

Conclusions:

Cyanotic congenital heart disease rarely presents in adulthood. If left undiagnosed, it may provide a pathway for embolic events causing splenic infarcts.