Clinical features: A 32-year-old, G5, P3, SAB-1 woman in her third trimester was transferred to UCSF for cardiac and pregnancy evaluation (2/27). She had had a lifelong heart murmur, idiopathic thrombocytopenic purpura, and two episodes of postpartum hemorrhage. The sole cardiac evaluation, other than electrocardiograms, was echocardiography (2/26), which showed an atrial septal defect, dilation of the right atrium and ventricle, moderate to severe pulmonary insufficiency and mild mitral valve regurgitation. PA pressure was 120 mm Hg. She had had new onset of progressive dyspnea on exertion and dependent edema over the past 3 months.
Blood pressure was 130/70 mm Hg, pulse 82/min, and respirations 18/minute. She was receiving 10 L of oxygen/minute by face mask. Her lungs were clear. A loud fixed P2 and diastolic and systolic murmurs were heard along the left sternal border. Finger clubbing and 2+ tibial edema were present. PaO2 was 43.8 mm Hg and oxygen saturation, 80%. Platelets were 17 k/µl, hematocrit 46.6%, WBC 19.2 k/µl, AST 77 U/L, and ALT 89 U/L. A chest film was obtained. An electrocardiogram showed RVH. After a healthy baby was delivered vaginally on 2/27, a perfusion scan showed a major R to L shunt and low probability of pulmonary embolism. Despite maximal support, she became more cyanotic and hypotensive and died on 3/2. The clinical diagnosis was atrial septal defect, severe pulmonary hypertension, and reversal of shunt.
An autopsy was limited to examination of the heart and lungs.
Radiology | Image 1 | Image 2 | Image 3 | Image 4 | Image 5 | Discussion
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