Return to Case Studies Index Case Study - Spongiform Cardiomyopathy

     
 

Patient’s History: The patient is a 37-year-old male with no known co-morbidity, who presented to the emergency room (ER) of the Aga Khan University Hospital (AKUH) with severe shortness of breath. He narrated a two-week history of high grade fever of sudden onset, not associated with sweating. He proceeded to develop dry cough and was cognizant of yellowish saliva, which he was informed was apparently due to hepatitis. His other complaint was that of epigastric pain.

Chest examination revealed bilateral coarse crepitations, while abdominal examination unearthed the problem of hepatomegaly. An echocardiogram was done, which brought to the fore the diagnosis of Spongiform Cardiomyopathy.

Discussion: Spongiform Cardiomyopathy is an idiopathic cardiomyopathy is caused by the intrauterine arrest of compaction of the left ventricular endocardial myocytes in the absence of other cardiac abnormalities. The recesses and clefts formed by noncompaction of the endocrinal tissue become filled with blood that is derived from the left ventricular cavity. The authors believe this entity is different from the condition known as persistent sinusoids, which may be associated with congenital right and left ventricular outflow tract obstruction.

Patients with noncompaction of the left ventricle can develop heart failure, embolic events from left ventricular thrombi, and ventricular arrhythmias. The mortality rate for the condition is high, as it is with other types of cardiomyopathy. Management includes treatment of heart failure, arrhythmia, and emboli. Some patients may need an implanted internal defibrillator, and others may need cardiac transplantation.

 
     
  Submitted by:
The Aga Khan University Hospital, Cardiopulmonary Department

Dr Nasir Rahman, Dr Sajid Dhakam, Dr Aamir Hameed
and Dr Sohail Abrar
 


 



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