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Virtual Pediatric Hospital: Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease: Case 2 Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease

Case 2

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Chief Complaint:
Twenty seven year old female with complex congenital heart disease, fever, fatigue, and left upper quadrant pain.

Clinical History:
The patient was a 27 year old female with complex congenital heart disease which included a single left ventricle, dextrocardia, pulmonary atresia, and left pulmonary hypertension. She was status post Waterston (aortic to pulmonary artery) shunt, Glenn (superior vena cava to right pulmonary artery) shunt, and a patent ductus arteriosus closure with a patent ductus arteriosus closure device. One year ago she had a superior vena cava to right atrium leak at the site of the Glenn and this was occluded with coils, but one of the coils migrated into her left pulmonary artery. She presented on this admission with a 4-5 week history of low grade fever, fatigue, cyanosis, and 3 days of left upper quadrant pain. A chest x-ray on admission showed left lower lobe infiltrate. Bronchoscopy demonstrated a narrowed bronchus to the lingula, presumably by an extrinsic mass, and there was pus coming from the bronchus to the left lower lobe. A chest CT exam was performed to check for an extrinsic mass and to rule out post obstructive pneumonia.

Clinical Physical Exam:
The patient had a low grade fever, and was found to have tenderness in the left upper quadrant.

Clinical Labs:
A culture from the bronchoalveolar lavage grew out Pseudomonas.

Clinical Differential Diagnosis:
Post obstructive pneumonia

Imaging Findings:
A chest film from the day of admission showed dextrocardia and an infiltrate in the left lower lobe. A body CT exam done on the second hospital day (not provided) showed peripheral patchy airspace disease in the left lower lobe. A V/Q scan from the second hospital day demonstrated a huge V/Q mismatch. A cardiac angiogram from the fourth hospital day (not provided) showed slow flow in a dilated left pulmonary artery and attenuated pulmonary perfusion to the left lung. A transesophageal echocardiogram done on the 12th hospital day (not provided) showed right atrial thrombus. A chest CT exam from the 17th hospital day showed massive thrombus in the left pulmonary artery.

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Imaging Differential Diagnosis:
Pulmonary Embolus

Operative Findings:

Pathological Findings:

Final Diagnosis:
Pulmonary Embolus

Follow-up and Prognosis:
The diagnosis of pulmonary embolus was unsuspected by the clinicians, despite it being suggested to them on every imaging study before the spiral CT exam. After the spiral CT exam she was placed on heparin and then switched to Coumadin. Several weeks later, the patient was rehospitalized and found to have developed leukemia. She died several weeks later. The patient had undergone a large number of cardiac catheterizations during her lifetime, many of which were very lengthy. There was some concern that the leukemia may have been radiation induced.

Similar Cases:
Case 21


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