A middle-aged man whose heart had expanded to fill his entire chest cavity has been diagnosed with an extremely rare condition known as Wall to Wall Heart.
In a case report published in the British Medical Journal (BMJ), doctors explain how the condition, often caused by Ebstein's anomaly—a condition usually found in infants— was discovered when the man presented with breathlessness that had lasted for around six months.
Doctors performed a chest X-ray which revealed "gross cardiomegaly," which in layman's terms is an enlarged heart. This is measured using the ratio to which the heart fills the chest or thoracic cavity—the Cardiothoracic ratio (CTR).
This unnamed middle-aged man had a CTR of 1, a value that means his heart had swelled to fill his entire chest cavity, with a 100 percent cardiothoracic ratio.
University College London Institute of Child Health Cardiac Unit Professor, Robert Anderson, who was not involved in this case study, told Newsweek: "This is, indeed, a very rare occurrence in adults."
What is Wall to Wall Heart?
Anderson said that with Wall to Wall Heart, the name says it all. "The heart occupies the entirety of the thoracic cavity, and stretches when seen from the front in a chest XR, from 'wall to wall,'" he said.
"Although very rare in adults, the 'Wall-to-Wall' heart is better recognized in neonates—an infant less than four weeks old—a particular subset of those with the condition known as 'pulmonary atresia with intact ventricular septum' who have the heart filling the thoracic cavity."
Anderson said that the condition is much worse in infants because the enlarged heart "squeezes out" the lungs meaning they don't work properly.
The researcher, who has previously written papers concerning Wall to Wall Heart, said that in neonates, the condition can be spotted by echocardiography. He said it is "a very bad prognostic feature."
What is Ebstein's anomaly?
A congenital heart defect present at birth, Ebstein's anomaly is when the valve between the chambers on the right side of the heart—called the tricuspid valve—does not close correctly.
This means that rather than opening so that blood can flow from the top chamber of the heart—the right atrium—down into the bottom chamber of the heart—the right ventricle—the three small flaps of the tricuspid valve cannot move. This means blood can leak backwards into the atrium.
The misplaced valve flaps in the ventricle also cause a section of the ventricle to become part of the right atrium making it oversized and causing the right ventricle to expand as it pumps harder and harder to push the blood to the lungs. Over time, the enlarged right side of the heart weakens leading to heart failure.
The Cleveland Clinic says that around one in 20,000 infants is born with Ebstein's anomaly, which accounts for less than 1 percent of all congenital heart defects.
When discovered in adults, the Cleveland Clinic says that Ebstein's malformation tends to be mild. But, in cases when it causes the heart to enlarge it leads to decreased heart function and requires surgery.
The authors of the BMJ case study write that the unnamed man was initially treated with anticoagulation, diuretics, beta-blocker, and vasodilators—medications that open or dilate blood vessels.
Following this, he underwent a surgical resection to correct an aneurysm in the left atrium which he had possessed since birth. The patient also had a heart valve replaced with a 23-millimeter St. Jude mechanical valve.
They added that the surgery was uneventful and that the patient is stable and being treated with aspirin, diuretics, and warfarin.
After looking at the case study, Anderson concluded: "I have not seen the feature previously described in an adult. It is, indeed, very rare."
Uncommon Knowledge
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