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Background Pompe disease (PD) is a rare, progressive autosomal recessive lysosomal storage disorder that directly impacts mitochondrial function, leading to structural abnormalities and potentially culminating in heart failure or cardiogenicshock. Further genetic testing identified a homozygous mutation c.2662G>T
After discharge, she was scheduled for a 2-week postpartum visit including echocardiogram, EKG, and NT-proBNP.Discussion:Given the patient's acute decompensation and fluid overload, medical optimization was essential prior to delivery. However, stabilization was expected to be temporary due to ongoing physiologic changes of pregnancy.
A transthoracic echocardiogram showed an LV EF of less than 15%, critically severe aortic stenosis , severe LVH , and a small LV cavity. The patient was transported to the CCU for further medical optimization where a pulmonary artery catheter was placed. Authors' commentary: Cardiogenicshock in the setting of severe aortic stenosis.
I have always said that tachycardia should argue against acute MI unless there is cardiogenicshock or 2 simultaneous pathologies. The patient underwent an emergent formal echocardiogram to look for wall motion abnormality: The estimated left ventricular ejection fraction is 63 %. PR depression, which suggests pericarditis 4.
Case Continued 2 days later the patient became increasingly tachycardic, hypotensive, ashen, clammy (in cardiogenicshock) and had a new murmur. An echocardiogram showed no hemopericardium, but D oppler showed a new small ventricular septal defect with left to right shunting. The initial troponin I was 23.7 3) Oliva et al. (4)
Whenever there is tachycardia, I am skeptical of OMI unless it has led to severely compromised ejection fracction with cardiogenicshock. I suspect pulmonary edema, but we are not given information on presence of B-lines on bedside ultrasound, or CXR findings. Acute coronary occlusion and acute pulmonary edema can coexist.
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