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Creepy Creatures and the Heart

Tue, 17/01/2012

Sometimes heart disease comes from unexpected sources. Sure, the following case is something you might not see very frequently, but we promise, once you read this unusual presentation of a rare disease you will never forget this diagnostic entity.
  From the Forests of Transylvania 

A 20-year-old man from Romania was brought to our hospital with acute peripheral limb ischemia and complete occlusion of the right femoropopliteal artery. His mental status was rather peculiar. When questioned about his health he reacted rather slow and lethargic. 

We performed thrombectomy and investigated the specimen. The specimen did not look like the typical thromboembolic material but was composed of amorphous lamellar shell-like structures. 

Histology of the specimen extracted during thrombectomy

Finding the Origin

Why would a young patient without any risk factors develop embolism? Was the source of embolism in the heart? Clearly, all patients with embolism (especially if they are young) should undergo echocardiography. And this is just what we did. 

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Four chamber view showing an oval structure (45×20 mm) in the left ventricle.

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Short axis view of the left ventricle demonstrating the close proximity of the structure to the subvalvular apparatus.

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Color Doppler displaying flow in and out of the cystic structure.

Questions, Questions, Questions

The pathology was easy to see but what is this oval structure we found in the ventricle? Is it truly part of the subvalvular apparatus? A cystic tumor? Or maybe some form of congenital malformation? Is this only an incidental finding or does it have something to do with the embolic event? 

To answer these questions we need more information. Since the young man also had a conspicuous mental status we performed a cranial CT.

Cranial CT scan with a small unilateral thalamic „hemorrhagic“ lesion.

The most prominent laboratory finding was a significant relative eosinophilia of 19%.

Calling Dr. House

Can you put the puzzle together? Embolism, eosinophilia, a cystic structure in the heart, lesions in the brain...

Yes, the suspicion is high that the patient had echinococcus cysticus. Truly, we found a very high antigen titer (1:3200). Cysts were not found in any other organs (neither in the liver spleen or lung). Since the liquor was free of E. cysticus antigen or clonal immunoglobulin expansion, our diagnosis was a primary cardiac manifestation of E. cysticus.

A Little Bit of Background

The disease is caused by a tapeworm which can live in the small intestines of numerous species such dogs, sheep, cattle, goats, pigs and fox (final host) depending on the type of tapeworm. There it lays its eggs which are passed by the faeces. Ingestion of faeces by the intermediate host can lead to infection of humans. Within the small intestines, the larvae hatch from the egg and enter the bloodstream. Usually, they settle in the liver. But as this case shows, they can be distributed anywhere. In these organs the larvae develop into hydatid cysts (what we saw in the heart). Rupture of the cysts leads to spead of the infection. The final host is infected by ingesting the hydatides.

Tape worm: Tape worms are usually only 2-5mm long.

What now?

Our patient received combined antihelmintic therapy with albendazole and praziquantel. But we felt that the cyst in the heart should also be removed so cardiac surgery was performed. The proximity of the cyst to the mitral valve also required mitral valve replacement.

Hydatid cyst seen at surgery

Postoperative echo showed that the cyst had been completely removed. However, his left ventricular function had suffered.

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Four chamber view after surgery. 

The hydatid cyst was gone, the bioprosthesis functioned well, but left ventricular function was somewhat reduced.

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Short axis view of the left ventricle demonstrating regional wall motion abnormalities were the cyst had been „pealed“ out of the myocardium.

The Story Continues

But, his reduced left ventricular function was not his main problem. Unfortunately, the parasitic titer did not decline during follow up and cerebral CT 15 months later showed new lesions in the brain and later also in the liver. 

We don‘t know what happened to the young man. But his mental status had deteriorated when we saw him last. He never came back from Romania and we fear the worst. After all, the prognosis is poor especially if the cysts rupture. Judging from the echo and the brain lesions, this is what might have happened. 

This is certainly a unique presentation of a rare disease. Even in regions where echinococcus infection is endemic, one rarely finds hydatid cysts in the heart. But this case shows you that the heart can be affected by many disorders and the power of echocardiography to make the diagnosis.

Finding the pathology with echo is only one part of the game. Interpreting the findings and putting them in a clinical perspective is the other. So stay tuned for more clinical cases and don‘t forget to visit our site:

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