December 4th, was the two-year anniversary of the current state of my wife's journey. No she didn't have a dissection, we just had another heart valve emergency that sent her to the hospital with congestive heart failure. A CT scan revealed a 5.9 cm aortic aneurysm in the proximal descending aorta extending into the arch. Our local heart hospital spent a few days deciding how best to handle the valve repair whether a valve-in-valve transcatheter repair would work. We had the capable cardiologist interventionist, but the concern was would the valve fit and allow the heart to function. The thoracic surgeon would not open her again and only later did we find out why.
Her heart valve journey began in 2017 with mitral valve failure when congestive heart failure sent her to the ER. After meeting with the thoracic surgeon who would repair it if possible and if not replace using a porcine valve. She had a dilated ascending aorta which at the time we were not made aware of. Day of surgery the surgeon informed us the plan was to repair but if the valve needed replaced, he would also replace the aortic valve as well. Not what we were expecting but were told as they were wheeling her to the OR. In hindsight it should have been a red flag for things to come.
After surgery consultation and the news was delivered that two valves had indeed been replaced, we could only surmise it possibly might have saved her a surgery down the road. However, that was not the case, two years later we were made aware that the dilated aorta had grown significantly to a 5.5 cm aneurysm. The surgeon wanted to repair so in for her second sternotomy in two years. We don’t know the reason and can only theorize the increased force on the ascending aorta hastened its growth little did we know that it was possibly weakened by a vasculitis. Pathology didn’t find anything, so we assumed all was repaired and she recovered fully.
January 2020 came along and another trip to the ER with congestive heart failure. Her porcine replacement valve failed after only two and a half years. That is when we met a cardiologist interventionist doing TAVR procedure in our hometown. After 10 days waiting to get scheduled, the procedure was successful and we were home the next day. On follow up echo her entire aorta had dilations and we began yearly MRA scans to monitor. Not a lot of curiosity was displayed by her doctors of why her aorta apparently was prone to aneurysms.
December 2022 another trip to the ER for congestive heart failure only to find out her porcine mitral valve had failed and subsequent CT scan to gauge the size and possibility of a transcatheter valve in valve procedure could be successfully done and an incidental finding of a 5.9 cm aneurysm in the proximal descending aorta involving the arch. The mitral valve was repaired, and we were sent home without any doctor recommending we consider another higher volume facility that specialized in high risk procedures to address the aneurysm. We were simply told her aorta was a mess and our surgeon would not open her again because he was no longer her doctor. A thoughtful valve repair nurse did tell us there were other places we could go. Her cardiologist didn’t even know he hadn’t even looked at the scan. I sent her records to Cleveland Clinic.
March 2023 we headed to Cleveland where she was scheduled for an open arch repair and replacing her valve in valve mitral and aortic repairs with mechanical valves since her body clearly didn’t like the tissue valves. She endured a 12-hour open procedure, a Frozen Elephant Trunk and two valves. After a hard recovery 11 days in CICU where we had trouble getting her off the vent and another 10 days on the stepdown where she had had to be removed from her feeding tube and cleared to swallow. I really don’t know how we made it through that procedure but the surgeon and the team at Cleveland are so experienced that they got us through it. We scheduled a follow up in June 23’.
Her breathing never really recovered and at the follow-up visit I insisted we have a consultation with pulmonology where she had pulmonary function testing to make sure she could endure another procedure. TEVAR was needed to join the FET stent to a thoracoabdominal stent extending to just above the Celiac artery. Her cardiologist at Cleveland Clinic suspected unilateral diaphragmatic paralysis as a complication of her open arch repair. The phrenic nerve was either damaged or irritated and only time would tell if it would recover but that explained her symptoms of respiratory distress both post op and when we got home. We were scheduled to come back in July for the TEVAR procedure. We met the Vascular Surgeon and another 30 days of anxious waiting to have the 2nd procedure to as the Thoracic Surgeon told us “To buy her time”.
The TEVAR procedure both preparation for and recovery after were extended by the bridging process from Coumadin to Heparin and a spinal drain placement From admission to discharge it was 21 days in Cleveland. Followed by over two months of flu-like symptoms upon returning home. Some suffer from implantation syndrome post TEVAR her 450 mm, 17” stent traps blood inside the false lumen that your body reacts to. She finally felt better, and we tried to return to normal. Physically anyway the trauma of all she had gone through still left scars.
We were left with dealing with the diagnosis of Giant Cell Aortitis diagnosed from tissue sent to pathology after her arch repair. After tapering down prednisone and trying to introduce cortisone sparring anti-inflammatory drugs starting with Methotrexate which caused gastrointestinal problems without any improvement with her inflammatory markers, we moved on to Actemra. Which blocked her CRP numbers but seemed to cause hypertension after the monthly infusions which worsened till she was hospitalized with a hypertensive crisis in August 2024. She was scanned and a type 1 endoleak in the TEVAR graft was discovered. We traveled back to Cleveland to see what to do about the leak.
Upon arriving in Cleveland and receiving another scan and discovering the leak had gotten worse and her aneurysm sac was enlarging the consensus from the vascular team was she needed an open thoracoabdominal procedure. One of the biggest open procedures they do there. Her Thoracic Surgeon didn’t feel she would survive it and honestly neither did we. Her vascular surgeon was tasked with extending the TEVAR a few inches to achieve a seal just above the SMA artery. Her Celiac artery was occluded so no fear of stomach and bowel ischemia by covering it. The landing zone was a small area of good aorta 3mm in size. The surgeon was worried it may not work. I asked what plan B was to which he replied there is no plan B. This has to work.
The procedure was a success except for a complication of a dissected iliac artery where the device was inserted. A week following the extension procedure the iliac was repaired with a graft and stent. A week after that we came home. We were concerned about what was next. We had no hope for a minimally invasive procedure at Cleveland Clinic and her blood pressure was still hard to control so it was only a matter of time for this repair to fail and then what.
Her current vascular surgeon doesn’t feel she is a candidate for a fenestrated branched stent endovascular repair, so we sought a second opinion. We found a vascular surgeon who has been doing the procedure for over a decade and had a recent consultation. He thinks she is a candidate for the procedure and will take her case on. We will schedule a scan in March to monitor the existing stent. Meanwhile we are monitoring her inflammation and her blood pressure. Her new vascular surgeon saw something on her last CT that may be playing a part in her blood pressure. One of her kidney arteries has a deformity. Also, the rigid stent graft materials play a part. I’m not sure what to do about that. It seems that the length of the stent grafts and the overall repair from her ascending, arch, descending thoracic endovascular stent that was recently extended to just above her SMA artery plays a role in isolated systolic hypertension.
This last couple of years has been a constant emotional and physical assault but we have made it. We’re not ready to give up but have learned to enjoy the calm before the storms. We know what the forecast is and as we prepare to ride another storm out, we are assembling the team needed to help her get through another complicated surgery that should give her some peace of mind. Although we should be enjoying every day as a gift, it is just hard to with that dark cloud of uncertainty constantly following us around.
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