It started like any other day for Joshua Choquette.
He woke up, had breakfast, and went to work. Later he went to the gym before ending the day by having a drink with a friend, a normal day for a healthy 27-year-old young man.
Joshua, however, had no idea that in less than 24 hours that he would be fighting for his life at Baystate Medical Center – a battle that he had to win, not just for himself, but for his two older brothers as well.
WHEN IT ALL BEGAN
“I was bench pressing and felt a pop, like a tear in my chest, but I thought I had just pulled a muscle. Later that night after going to bed, I woke up at about 2 a.m. in excruciating pain, which had now traveled up the left side of my neck to the ear,” said Joshua, who soon found himself at the Emergency Department at Baystate Medical Center because he was suffering from an aortic aneurysm that had dissected.
The aorta is the largest artery in the body. It carries all the blood being pumped from the heart to the entire body. An aneurysm is a weak area in the wall of the artery that develops over time and produces a balloon-like bulging as the arterial wall progresses towards failure. Dissection represents a partial rupture. Aortic dissection happens when there is a tear in the innermost layer of the aorta wall. As the tear extends along the length of the aorta, blood flows in between the layers of the blood vessel wall further weakening it, blocking off branches, or simply bursting completely through. If left untreated, the condition results in death for over 90% of patients.
Joshua was one of the lucky ones - about half of all patients with the condition die before arriving at a hospital. At Baystate - the only tertiary care center in western Massachusetts with cardiac surgery capabilities - he would soon learn the grim news that the odds of surviving surgery were “just 50-50.”
THE DISTURBING NEWS
“You could see it in Joshua’s face. His whole world was turned upside down. But I knew the odds were better for a young, fit, relatively healthy young man like him,” his surgeon Dr. Siavash Saadat said.
Also in his favor, according to a recent study published in the journal JAMA Cardiology: death rates to repair an aortic dissection were nearly three times higher at low-volume centers than when done by a dedicated aortic surgeon like Dr. Saadat at an experienced high-volume center.
During the nearly 8-hour, highly complex, open-heart surgery, Dr. Saadat and his OR team identified the tear and removed the damaged portion of the aorta, replacing it with a manmade Dacron graft which keeps the blood from flowing into the aortic layers and directs it back to the correct lumen, or channel, within the vessel.
A SECOND SURGERY
Joshua beat the odds - more than 13,000 people die from an aortic dissection each year in the United States - and survived the surgery. He returned home to recover after spending six days - some in the Cardiac Intensive Care Unit - in the hospital. But while his surgery was a success, Joshua would find himself back in the operating room just six months later.
“When you have a patient such as Joshua come in as an emergency, you don’t have a good grasp of what is going on with the remainder of the aorta, just that you need to repair the dissection immediately to save your patient,” Dr. Saadat said, noting the risk of death increases by 3-4% every hour 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.
A SILVER LINING
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 and Daniel.
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 echocardiography 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,” 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.
A GENETIC FACTOR
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. After genetic testing, all three brothers 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.
ROOT ANEURYSMS DISCOVERED
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 I 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.
18 MONTHS OF CHAOS
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.