Case of the Month: Young man and Chest pain
A 38-year old man experienced sudden onset chest pain which radiated to his upper back along with shortness of breath. He immediately went to the emergency room. The patient appear anxious in the emergency room and complaining of chest pain. The emergency room physician was concerned for a heart attack and blood clot to the lungs. The EKG was suggestive of a possible heart attack. However, given the patient's young age, a heart attack was unlikely. A cardiologist was consulted since the patient was having EKG changes. A blood clot to the lung was also unlikely because he did not have risk factors for the development of a blood clot. The patient had a loud murmur on physical examination. The patient's chest x-ray showed signs of heart failure (i.e. fluid in lungs).
Because of the patient's presentation of sudden chest pain and radiation of pain to the back, loud murmur (the noise blood makes as it passes through an abnormal heart valve), and chest x-ray and EKG findings a CT scan with IV contrast was ordered.
The CT scan showed a Stanford Type A aortic dissection with a large aortic root aneurysm. The patient had an aberrant right subclavian artery, which originated from the descending thoracic aorta, which was also dissected.
The patient was taken to the operating room for an emergent repair of his acute aortic dissection. On route to the operating room a scar was seen his chest. A breathing tube was placed to assist the patient in the emergency room because of difficulty breathing therefore the patient could not talk. The patient's wife informed the cardiac surgical team that the patient had surgery at the age of 7 years old to correct an abnormal chest deformity called pectus excavatum. At this point, the surgical team was concerned that patient may have some form of connective tissue disorder such as Marfan Syndrome.
Transesophageal echocardiogram performed in the operating room showing the dissection flap (looks like a sail on a boat) in the ascending aorta (click on image above)
In the operating room, the echocardiogram showed that the dissection flap was protruding through the aortic valve causing severe leakage of the aortic valve. The aortic valve had three leaflets. The aortic root diameter was larger than 6 cm. On inspection, the aortic root anuerysm was also very characteristic of an aneurysm associated with a connective tissue disorder such as Marfan Syndrome (see image below).
The patient underwent an emergency replacement of the aortic root and ascending aorta with a Dacron graft and preservation of the aortic valve. The aortic reconstruction is known as a David Procedure.
The patient's post-operative course was unremarkeable and was discharged on the 7th day. He is doing well 24 months after his procedure. He and his children have been referred to the Genetics Department for counseling and genetic testing.
The post-operative CT scan showed a normal appearing aortic root with no residual dissected aorta.