Presentation
Increasing shortness of breath
Patient Data
Dextrocardia. There is the impression of a figure 3 appearance to the descending aorta though no evidence of rib notching.
Dextrocardia. Two bilobed lungs with hyperarterial bronchi suggest a degree of left isomerism, although there is normal abdominal situs.
Appearance of the aorta may represent pseudocoarctation (rather than true coarctation) due to the absence of significant collateral vessels (though this is not well evaluated due to the phase of the study).
Case Discussion
The patient was seen in cardiac outpatient clinic for dilated cardiomyopathy and investigated with echo and a right heart catheter. She was found to have an ejection fraction of 48%, GLS -12.5%, severe left atrial dilatation (LA volume 63) with moderate-to-severe mitral regurgitation and normal pulmonary artery pressures. She also had a bicuspid aortic valve.
Pseudocoarctation represents kinking of the descending aorta at the level of the ligamentum arteriosum without a pressure gradient like in true coarctation. It is often associated with other congenital cardiac anomalies as in this case where the patient had dextrocardia as well as a bicuspid aortic valve.
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