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| SUNDAY, FEBRUARY 12, 2023
Risk Factors for an aortic aneurysm include age, family history, lifestyle habits, medical conditions such as high blood pressure, which is also the biggest risk factor for aortic dissection, and several genetic conditions such as Loeys-Dietz syndrome.
It started like any other day for Joshua Choquette.
ones, about half of all patients with the condition die before arriving at a hospital. At Baystate - the only tertiary care center in western By Keith O’Connor Massachusetts with cardiac Special To The Republican surgery capabilities - he would He woke up, had breakfast, soon learn the grim news that and went to work at Amherst the odds of surviving surgery were “just 50-50.” Landscape. Later he went to “You could see it in Joshua’s the gym before ending the face. His whole world was day by having a drink with turned upside down. But I a friend, a normal day for a knew the odds were better for healthy 28-year-old young a young, fit, relatively healthy man. Joshua, however, had no young man like him,” his suridea that in less than 24 hours geon Dr. Siavash Saadat said. Also in his favor, according that he would be fighting for his life at Baystate Medical to a recent study published in Center – a battle that he had the journal JAMA Cardiology, death rates to repair an aortic to win, not just for himself, but for his two older brothers dissection were nearly three as well. times higher at low-volume “I was bench pressing and centers when not done by a felt a pop, like a tear in my dedicated aortic surgeon like Dr. Saadat at an experienced chest, but I thought I had just pulled a muscle. Later high-volume center. that night after going to bed, During the nearly 8-hour, highly complex, open-heart I woke up at about 2 a.m. surgery, Dr. Saadat and his OR in excruciating pain, which had now traveled up the left team identified the tear and side of my neck to the ear,” removed the damaged portion of the aorta, replacing it with a said Joshua, who soon found himself at the Emergency De- manmade Dacron graft which partment at Baystate Medical keeps the blood from flowCenter because he was suffer- ing into the aortic layers and directs it back to the correct ing from an aortic aneurysm “lumen” or channel within that had dissected. The aorta is the largest the vessel. Joshua beat the odds - more artery in the body. It carries than 13,000 people die from all the blood being pumped an aortic dissection each year from the heart to the entire body. An aneurysm is a weak in the United States - and survived the surgery. He returned area in the wall of the arhome to recover after spendtery that develops over time ing six days - some in the and produces a balloon-like Cardiac Intensive Care Unit bulging as the arterial wall - in the hospital. But while his progresses towards failure. Dissection represents a partial surgery was a success, Joshua would find himself back in rupture. Aortic dissection the operating room just six happens when there is a tear in the inner most layer wall of months later. “When you have a patient the aorta. As the tear extends along the length of the aorta, such as Joshua come in as an emergency, you don’t have a blood flows in between the layers of the blood vessel wall good grasp of what is going on with the remainder of the aorfurther weaking it, blockta, just that you need to repair ing off branches, or simply bursting completely through. the dissection immediately to If left untreated, the condition save your patient,” Dr. Saadat results in death for over 90% said, noting the risk of death increases by 3-4% every hour of patients. Joshua was one of the lucky if left untreated.
“For that reason, patients receive post-operative screening CT scans to check the aorta for any progression of disease. What we noticed was that Joshua’s aorta continued to get larger and was growing so rapidly and susceptible to another tear, that we decided to do a second surgery on the aorta,” he added. Sometimes there is a silver lining to even the most terrible of tragedies. In Joshua’s case there was - his aortic aneurysm would go on to save the lives of his two brothers – Andrew, 30, and Daniel, 33, all of whom live in Ware. About 20% of people with
an aortic aneurysm and dissection have a genetic predisposition to the disease. Since the illness is familial, even before genetic testing was done, Andrew and Daniel underwent CT scan screening. While ECHO cardiography can detect some thoracic aortic aneurysms, CT is the “gold standard” for detection and sizing of aneurysms. “Ironically, before my brother had his dissection, I was having chest pains and assumed it was just stress, I made an appointment with my primary care doctor, which I later cancelled when I learned about the CT scan,”
Top: From Left to Right: Joshua Choquette, Daniel Choquette, Andrew Choquette and Desiree Choquette in Front. Bottom: Baystate Health Cardiac Surgeons Dr. David Deaton and Dr. Siavash Saadat. (PHOTOS COURTESY OF BAYSTATE HEALTH)
Andrew said. Joshua was worried for his brothers. “I bet my life that it was just bad luck for me and there was no way they would have aneurysms. Unfortunately, they did,” he said. Risk factors for an aortic aneurysm include age, family history, lifestyle habits, medical conditions such as high blood pressure, which is also the biggest risk factor for aortic dissection, and several genetic conditions such as Loeys-Dietz syndrome. Loeys-Dietz is a disorder that affects the connective tissue in many parts of the body and is often characterized by an enlargement of the aorta. All three brothers after genetic testing were found to have the syndrome. Symptoms of dissection may feel like a heart attack and include sudden, severe chest pain or upper back pain that radiates to the neck, and shortness of breath. Aortic dissection occurs in about 2 out of every 10,000 people, mostly seen in men ages 40 to 70, but can happen to anyone. “Aortic aneurysms are like time bombs and people are typically asymptomatic until the dissection occurs. Andrew’s aneurysm was big enough that we didn’t waste any time getting him into the hospital for surgery, in fact, his surgery preceded Joshua’s second operation, but Daniel’s was less severe and didn’t require immediate surgery,” said Dr. David Deaton, the Baystate cardiothoracic surgeon who would go on to operate on the other two brothers. Joshua’s second surgery with Dr. Saadat occurred on February 17, 2022, after Andrew’s surgery took place on October 12, 2021. Daniel’s surgery was in 2022 on the day after Thanksgiving. All three brothers had aortic root (root refers to the location on the aorta) aneurysms, which Dr. Deaton explained is
an aneurysm of the very first part of the aorta and is “as close to the heart as you can get.” “Dr. Deaton was great throughout the entire process. He is an extremely knowledgeable surgeon who uses different visual aids to explain what he is going to do, how he is going to do it, your options and the possible outcomes,” Daniel said. Joshua’s second surgery performed by Dr. Saadat was similar to what Dr. Deaton had to do for Andrew. “For a root aneurysm, you replace the section of the aorta that is enlarged with an artificial tube called a Dacron graft. Because it is the root, surgery often involves replacing the aortic valve as well with a mechanical or biological valve. When you use a mechanical valve, patients must for the rest of their lives take an anticoagulant, which many people refer to as blood thinners, in order to prevent blood clots,” Dr. Deaton said. However, Daniel’s condition, while still an aortic root aneurysm, did not require replacing his aortic valve in what is called a valve-sparing aortic root repair. Daniel noted their mother, Desiree, was “always there for us” and all three brothers agreed it took an “emotional toll” on her. She called the ordeal “18 months of chaos.” “It hit us hard. We very well could have lost Joshua. Thankfully, Andrew and Daniel were not in an emergency situation like their brother. I’m relieved now that it is all behind us,” Desiree said. “But I’ll never ever forget what Daniel said to me about having Thanksgiving. He told me he wanted me to make it, ‘The greatest ever,” just in case he didn’t make it. After he left the house, I broke down,” she added, thankful now for her three sons’ newfound health.