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Number 09-15 Peripartum Cardiomyopathy?
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Number 09-15 Peripartum Cardiomyopathy?

Case From:  D.H. Brown, B. J. Barber, N. Kalra, Y. Naughton, V. Sorrell;  Institution - Department of Cardiology and Pediatric Cardiology, University of Arizona and Sarver Heart Center, Tucson, AZ, USA

Clinical history: A 36 year old female presented with irregular heart rate and palpitations following labor and delivery of her child.

Echocardiogram:  LV EF of 36% with a LVID 60-65mm, trace-mild MR, decreased TDI velocities at the mitral annulus, and normal E/E' (septal wall)

Additional history: The patient was a known carrier of the Duchenne Muscular Dystrophy gene mutation with deletion of exons 49-52. CMR performed to distinguish peripartum cardiomyopathy from that of Duchenne Muscular Dystrophy induced cardiomyopathy.

CMR cine:  Dilated LV cavity with multiple wall motion abnormalities and inferolateral dyskinesis.

 

 

    

 

CMR Delayed contrast enhancement: Extensive, mid-myocardial and sub-epicardial late gadolinium enhancement. This is consistent with muscular dystrophy or myocarditis, quantified as below.

 


  

   

 

Clinical Course:  The patient was initially treated with carvedilol, lisinopril, and aldactone.  There was little improvement in EF on 6 month follow-up.  Frequent ectopy remained.  This, in combination with the CMR findings of extensive scarring and fibrosis led to the recommendation for ICD implantation.  Heart failure therapy will continue with an attempt to maximize ACE-I dosing as this has been reported to improve cardiac function in boys with muscular dystrophy.

Additional comments: 
The baby was a healthy girl - who therefore has a 50% carriage rate for the DMD gene mutation.
The scanning centre's recommendations for breastfeeding mothers after gadolinium exposure is to express their milk and abstain from breast feeding for two days following the exam.  However, this risk is theoretical, and they point out that the American College of Radiology guidelines was NOT to interrupt breast feeding, but has been recently softened to a policy of discussion with informed choice with the mother potentially expressing milk and discarding.
The full dataset for this case is here

References: 
1) Oldfors A et al; Br HeartJ (1994) Dilated cardiomyopathy and the dystrophin gene:  an illustrated review;72:344-348 
2) Duboc D et al; J Am Coll Cardiol (2005)  Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy; 45(6):855–857
3) Yilmaz A et al. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach. J Cardiovasc Magn Reson. 2008;10:50.
4) Pulchalski M et al; Int J Cardiovasc Imaging (2009) Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?; 25:57–63
5) Walcher T et al; International Journal of Cardiology (2008) Cardiac involvement in a female carrier of Duchenne muscular dystrophy; in press.  


COTW handling editor: James Moon

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