On February 6, 2017 my 29 year old husband had open heart surgery in NYC to repair PAPVR and ASD – two rare congenital heart defects that if left untreated would dramatically shorten his life. I can’t believe that was our reality, but it was something we had to face and something we overcame. It took me awhile to decide if I was going to share our story, actually my husband’ story, about his journey into open heart surgery. I told him it could help people going through this very scary news and he agreed we should share. I wish there were more real stories out there we could have read when John got the news, and before his surgery, but all we found online were medical journals, language we couldn’t understand, or heart surgery for older people – nothing that could help us prepare so we knew we had to write his story.
I have to remind myself everyday that God gives his toughest battles to his strongest soldiers. I know that this news seems surreal, scary and like a nightmare, but the best thing that ever happened was the one doctor who kept searching for answers, and never quit on pushing John to get this surgery. If it wasn’t for him finding these defects who knows what could have happened years from now. We are so thankful that it was found, we had an amazing surgical team and nursing staff and now John can live a healthy, long life (with me<3).
PAPVR & ASD : Being Diagnosed and Treatment
My husband had been suffering from dizziness, shortness of breath, terrible fatigue and tightness in his chest out of the blue. He knew something was wrong and just didn’t feel right at all. He went on a mission to find out the reason for these symptoms because he refused to believe it was nothing to worry about or anxiety or “maybe you’re just a little out of shape.”
None of these answers made sense at all especially if you see the shape my husband is in – he’s super fit and muscular and very healthy. After rounds of tests, MRI’s, echocardiograms, etc. the doctors at Morristown Hospital in NJ read his charts and found out he has PAPVR and ASD; two very rare congenital heart defects. We were struck with the terrible news and were shocked that this was happening – how do you comprehend that at the young age of 29 you will need open heart surgery?!
If you’re reading this post odds are you or someone you know has been diagnosed with PAPVR, also known as Partial Anomolous Pulmonary Venous Return. This congenital heart defect is when one or two of the pulmonary veins returns blood to the right atrium instead of the left atrium. Only 1-5% of the world’s population has this structural deformity and sadly my husband John is one of them. On top of having PAPVR my husband also has an Atrial Septical Defect (ASD) which is a hole between the top two chambers of his heart; allowing the red and blue blood to go back and forth. In a normal heart the chambers are completely separate: one gets oxygenated blood and one doesn’t. With ASD both types of blood pass freely and mix making you feel very lethargic, dizzy and out of breath. These defects were both found after doing a cardiac MRI, but he had done other tests before that which didn’t show these defects at all. If it wasn’t for that MRI he would never have known about this at all. It’s his relentlessness to get an answer that helped save his life.
If PAPVR is not detected in childhood you can lead a normal adult life with no symptoms, however at some point in your later 20’s or early 30’s you can begin to feel them. Many people don’t go to the doctor though to find out why. They think it’s lack of activity or they are out of shape or a million other reasons – because no one in their right mind would ever assume they have a heart defect. No one talks about it. It’s not common knowledge that these things exist and this is why WE wanted to write this. Maybe we can help save one more persons life. ❤
My Husbands History & Story
My husband has always been an athlete. His entire life he played sports at a very competitive level. His sports of choice were basketball and baseball; both of which require great physical endurance and stamina. He was a stellar athlete and likely would have gone on to the pros in baseball had it not been for a few shoulder and hip surgeries along the way. We both love working out, being active, lifting weights – we follow a very healthy lifestyle.
Out of the blue his ability to do physical activity just started to come to halt. The task of carrying a case of water up the stairs left him winded and out of breath. Lifting at the gym made him dizzy for the rest of the day. You would have never guessed he had a heart issue at all if you saw him….but he KNEW something was wrong. Something deeper because why would he see such a decline in his ability to be active?
The point of the story is that his entire life he was extremely healthy, showing no signs of any heart issues. He could run like the wind, play basketball for hours and he was always in the gym lifting weights and strength training. It wasn’t until he went looking for answers that he found this all out. I hate to think of all the people who just blow this off and never go to get tests done. Some people don’t find out they have PAPVR and ASD until they are in their 60’s and then surgery and recovery is much more complicated. Sometimes it is found when surgeons go in for a different heart operation and find it or sadly it is found too late during autopsy.
The view of a normal heart:
In a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body. When you have PAPVR everything is routed wrong; it’s like having the pipes in your home installed incorrectly. You still have water and a flushing toilet so you don’t know anything is wrong but underneath in the basement the pipes are all wrong and some are being overworked and eventually will fail. That’s what PAPVR is like; the pulmonary veins are in the wrong place so what is meant to go into the left side of your heart, spills into the right side. Everything on the right side of his heart is now enlarging because it’s getting too much blood and the left side doesn’t get enough. Over time this can cause heart failure which is why it’s not “should” I get this fixed it’s “when” and the sooner the better
Blood Clot History & Other Risks
In the last five years my husband had multiple incidences of blood clots that formed in his legs (DVTs), one in his arm and one time they even traveled to his lungs (pulmonary emboli). At this point you’re probably saying to yourself “my God he’s been through so much” – I know it’s awful.
He was placed on coumodin (blood thinners) long term and takes them to this very day. We never could understand why he kept developing blood clots, but in order to keep him clot free and have piece of mind he has been on the medication for what seems to be off and on for the last four to five years. He has to have his blood work checked bi weekly but as you can imagine it made open heart surgery even more complicated. We don’t know if the clots are because of his heart defects but they surely could be.
The Signs & Symptoms of PAPVR & ASD
In the last six months my husband (at age 29) started to complain of tightness in his chest, having shortness of breath when working out, being more light headed than normal and dizzy. He would go to the gym and struggle to run on the treadmill for ten minutes. He felt very winded and just “not like himself”. Mind you he could run for 5 miles at a time before and this felt impossible now. It continued to get worse and finally he went to the doctor to get to the bottom of it. After some testing and blood work the doctor sent him for an echocardiogram- a test of the action of the heart using ultrasound waves to produce a visual display, used for the diagnosis or monitoring of heart disease.
This test was completed and they also did what they call “the bubble test” on his heart. This test will detect the flow of blood between the right atrium and left atrium. With this approach, a sterile salt solution is shaken until tiny bubbles form and then is injected into a vein. The test determined that my husband had ASD, an Atrial Septical Defect. In order to get blood to the left atrium and left ventricle and out to the body, some of this “mixed” blood, which has lower than normal oxygen levels, must pass through a hole from the right atrium to the left atrium. Surgery is needed, since oxygen-poor (blue) blood cannot meet the body’s demands, either at rest or with activities. If the bubbles are seen in both atriums it means you have a hole and he does.
He then sent to get a cardiac MRI at Morristown Hospital in NJ. This test required him to stay in position the MRI machine for almost 90 minutes so they could take images of his heart and lunges. A cardiac MRI is a painless imaging test that uses radio waves, magnets, and a computer to create detailed pictures of your heart. The results of the MRI alarmed the doctor and said not to hesitate and make an appointment to meet with Dr. Robert Sommers, a doctor from Columbia University who specializes in cardiovascular disease and also does pediatric cardiology. Needless to say we got scared and confused. We didn’t know why he had to see him or why he seemed so insistent but it all started to make sense after we met with Dr. Sommers.
Right away we made an appointment to find out what was on the MRI that was so important to address. The imaging showed John’s right side of his heart was enlarged and was working 2.2 times harder than his left. He had ASD and PAPVR and it can only be fixed with open heart surgery. I went with my husband to that doctor appointment and the moment this news came out of Dr. Sommers mouth my heart sank. I wanted to cry because I felt so awful that he would have to go through this but I stayed strong for him. I wanted him to know it would be okay. Even though I wanted to crumble I kept on a brave face. I trusted this doctor and he gave a sense of calm to him. He said without this surgery his symptoms will get worse and worse and at his age it would be smart to just get it done and focus on living a long and healthy life.
After meeting Dr. Sommers it was time to find a surgeon and one that knows how to tackle this surgery. Most adult heart surgeons do not perform these often, the heart surgeons who are experts in these heart surgeries are usually pediatric heart surgeons. So the challenge was to find a hospital that was top ranked, with a heart surgeon who performs these in adults and children. It was either Columbia University in NYC or Boston Children’s Hospital – the top 2 in cardiac surgery and congenital heart defects and disease.
Luckily there was a doctor at Columbia University in NYC that Dr. Sommers highly recommended – Dr. Chai. We made the appointment to meet with him in NYC and then set up the day for his open heart surgery. I can’t tell you how amazing he was throughout this entire process but I knew we were in the best hands in the world with him.
My husband’s open heart surgery was on Monday February 6. Now that the surgery is over I will have another post detailing the lead up and day to day after his operation to help those of you going through it or a loved one who is.
The good news is PAPVR and ASD surgery will correct the issue and in a few months or a year later your heart will slowly go back to normal and you will feel so much better. Your heart and body will work as it was always intended to. I made a follow up post on what you can expect and what you should do to prepare for your surgery, and your recover. It is here http://www.jerseygirltalk.com/2017/02/12/open-heart-surgery-preparation-tips-and-what-to-expect/
John’s surgery happened the day after the Super Bowl, which was a great distraction but as soon as it was time to say good night to friends and family a wave of sadness and anxiety arose. As terrible as it is to say, we did not know what would happen within the next 24 hours. When you know that there is a chance your surgery could have complications, you fear this is the last time you may see your loved one. You have to remind yourself that you have the BEST team around you and you have to have faith and trust that things will be okay. That night we went home and John had to wash with a special soap to remove any bacteria. After some tossing and turning he finally fell asleep, then a few short hours later we were up at 4 am to shower again and head into NYC. That morning was hard, because John didn’t want to go. It all hit him and me like a wave that this was happening, he was going to have open heart surgery and putting it off was not an option. My heart broke over and over knowing that he couldn’t opt not to have the surgery, it was not something that would go away on its own – he HAD to go through with it.
We arrived early and he was the first OHS of the day. On little sleep and without food or coffee on a cold February morning we sat in the waiting room, filling out forms and waiting to be called into the OR waiting area. John met the surgeon, we tried to just talk and remain calm, and then they came to bring him to the operating room. He literally walked himself into the room, laid down on the table and woke up a few hours later in the cardiac ICU. The surgery took about four to five hours. He went in around 9am and the surgeon came to find us in the waiting room around 11:30am.
I can’t tell you how the world stands still like it does when your loved one is in the surgery. I held my breath the entire time, sitting their with our family, praying, trying to remain calm, hoping to hear good news. All I wanted to know is that it was over, that he made it out okay and any bad recovery I could deal with, but I needed him to be okay. For 45 minutes his heart was stopped and running on a heart and lung machine, I just prayed they’d fix his heart and there would be no issues. When I saw the surgeon walk in, I clapped and yelled and hugged all my family members. He was out of surgery, thank the Lord.
After the surgery they bring the patient right to cardiac ICU. You won’t be able to see them for about an hour later and only for a few minutes. I will warn you, when you see them after OHS you will not be prepared for what you see. I almost fainted when I saw my husband laying there with dozens of tubes out of his body, a tube down his throat, a tube in his neck, a mask over his face, so many machines and IVs; he was lifeless and it made me ill. He was not awake yet but he could hear our voice and I made sure to say in his ear “you made it out, it’s all behind you now”. I couldn’t stop crying tears of joy. A few hours later as he came to we were allowed back in, the tubes were out of his throat and he could breath on his own but he was groggy and in and out. The nurses were kind enough to let me stay in ICU overnight. There were 3 drainage tubes in his belly that pumped out fluid and he had so many medicines and tubes all over. He slept a lot and was visibly in pain anytime he tried to breath in or move. His sternum was wired back together and any movement was awful for him. The nurse came in many times to make him cough with his heart pillow which was so painful. He also had to practice breathing in a tube and they moved him a few times in the bed that night to prevent pneumonia. The first night was really hard, but he did get to sleep and I never left his side.
He stayed in cardia ICU for one night and by the second day he was forced to get up out of the bed and sit in a chair. The physical therapist came to visit him and get him moving, because he said the worst thing you can do is stay in the bed. It was hard to move him and he was in a lot of pain but he had to do it. He was moved to a step down room by the 2nd night and thankfully we got a private room. The 2nd night he started to have a fever and was sweating a lot which made us nervous. He was monitored a lot that night and he felt pretty awful, but by the 3rd day his fever broke. After day 3 he was up and walking around the hallways to get mobile. Once he was up and moving he started to feel a lot better. There was a lot of practice with his breathing tube and his lungs got stronger everyday. there were many tests each day, Xrays, etc and he went along with everything and just took it day by day.
Going Home & After
John had surgery on Monday morning and he was discharged on Friday afternoon. Because he has suffered from blood clots in the past they had to have special protocol of how they would take him off blood thinners before surgery and then put him back on after. I think this added more complexity to his surgery, but thankfully it all worked out. Once he got home it was two weeks of rest and just minimal walking around the hallway to stay active. He and I slept in our living room; he on his motorized recliner and me on the couch. The first week he needed help to do everything, lift anything, showering etc so make sure you have someone to help you and be with you at home at least for one week. I took 3 weeks off from work for the week of surgery and 2 weeks after. It will be hard to even lift a cup of coffee at first, but each day it gets better.
John was at the gym on the treadmill in 3 weeks doing light walking. He did not drive a car for I think the first month, but he wanted to lol. After one month he felt so much better and four months later he was back in the gym lifting light weights 🙂
I hope this is helpful and feel free to ask any questions. Me and my husband would be happy to answer any questions you have about PAPVR, ASD, heart surgery or anything related. He is now 6 months post surgery and doing AMAZING!!!
Thank you to all of my friends and family for being there for all of this. You all know who you are and we thank you so much ❤
Need tips on how to prepare for PAPVR surgery? Click here
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