The Broken Heart
The Broken Heart - Open heart surgery at 32 to repair an Atrial Septal Defect and Partial Anomalous Pulmonary Venous Return
“Mr. Forras, we need to talk.”
That’s the gut wrenching greeting my cardiologist gave me upon our second meeting. Incredible bedside manner, I know.
I nervously laughed and said “holy crap, how bad is it?” She replied that she has identified a 12mm hole in my heart and and right side ventricular enlargement and that I could expect to experience heart failure in as little as 15-20 years… (unkindly long pause) … if left untreated!
She then informed me that there are both invasive and non-invasive surgical procedures that can repair the hole and completely reverse any onset’s of heart failure, essentially resetting my clock to a normal lifespan.
Never in my life have I experienced such a swing of emotions.
Imagine being in your early 30s and being told that you can expect to die in 15-20 years… Your Adrenalin surges, your emotions begin to build up in the background, your mind begins to move a million miles per hour and just as the news is beginning to set in your doctor says, “good thing we have a way to prevent this!”
I asked the doc “what the hell is the matter with you?” “Why would you chose to deliver news in such a way?”
Never in my life had I come so close to knocking someone out that hadn’t first threatened me physically.
I left that appointment not knowing what to feel. I began my drive home up Hawaii’s beautiful H3 highway and began to digest what had just happened. Despite the obvious awesome news I received that this issue can be completely fixed and that I can expect a normal lifespan, the seed of emotion had already been planted. All I could think of was the words that “I should expect to experience heart failure in 15-20 years.”
As I began to think of my wife and my two boys, I felt a feeling that was quite foreign to me; I believe you call it emotion! I tried to fight it as much as I could but at some point I just lost it. I cried like I had never cried before. This moment will forever be burned in my memory as this was the first time I can recall expressing such emotion in almost 20 years.
Background Story
This introduction has likely left you asking “what were you diagnosed with, and how did you discover that you had this?” Great questions!
I have never experienced, or at least I had never perceived any cardiac related issues my entire life. Growing up everything was normal. I was athletic, just as any child of the 80s and 90s was and my profession as a Marine ensured that this lifestyle would be maintained. Throughout my career I had always been in the top 75% of physical performance as determined by our fitness tests.
I ran a marathon, 196 mile relay races, and maintained a fairly active lifestyle. Looking back, I did notice occasional periods of what I would describe as shortness of breath but these were always after periods of physical exertion so I believed this to be normal.
Throughout my career I had received numerous extensive physical exams and never had anything of note been recorded regarding my cardiac function.
At the end of 2016 I experienced what was likely a severe case of food poisoning. The first night of symptoms I experienced what I thought was a heart-attack and even went to the hospital to get checked out. EKGs, blood work, and imaging all showed that there was no sign of heart-attack.
Over the course of 90 days I experienced the most awful stomach issues imaginable. Most visibly, I had lost approximately 20 pounds over those 90 days; that really freaked me out.
I also began to experience what I would explain as a heart palpitation, a feeling of an occasional “skipped” beat, as well as occasional elevated heart rate. My normal resting heart rate was between 75-85 BPM yet I would occasionally experience resting rates as high as 115 BPM.
This was never fully explained however the most likely reason for the elevated HR was due to dehydration due to the “food poisoning” as each time I received IV fluids my HR would return to normal.
I underwent exploratory endoscopy and colonoscopy procedures, each coming back, luckily, with no significant findings.
My advice to all of you, do not do what I did and peruse the internet searching for your symptoms as it will only stress you out. I was dying of cancer, AIDS, Hepatitis, Lupus .... according to the internet of course... All that did was surely contribute to my stressing out.
The reason I shared with you the story of the "food poisoning" is that it really led me to be hyper focused on everything my body was doing, for better or for worse. While to this day we still do not know if it was food poisoning or not, what I do know is that whatever it was, it is what led me to continually seek medical attention, which ultimately led to the discovery of my congenital heart defect...
The Doc who saved my life with a stethoscope!
In winter of 2016 we had relocated to Hawaii and during a routine physical I would be introduced to one of the men I credit with saving my life; Flight Surgeon, Lieutenant Kramer, U.S. Navy.
During a routine physical Doc Kramer told me that he thought he had noticed a potential heart murmur and that it was likely nothing, but he was going to refer me to a cardiologist. Despite this being a bit nerve racking, I was a bit relieved that I was finally being seen by an expert. Here was this young Flight Surgeon and with only a stethoscope he finds what no other Doc had found through extensive testing... purely amazing!
The First Referral
The initial consult with the cardiologist was lackluster. The basic questions about symptoms and family history were asked and a basic exam was conducted. I was then scheduled for a few procedures to gather more additional information, a cardiac MRI and a bubble test and a TEE (Trans-Esophageal Echocardiogram).
The bubble test was rather interesting. They insert an IV catheter into the arm, in traditional fashion, and attach a special valve that allows two syringes to be attached at the same time. One tech performs an ultrasound of the heart and the other tech squeezes the saline syringes back and fourth, from syringe to syringe, agitating the saline to create tinny bubbles within the solution. When the ultrasound tech is ready the other tech opens the valve and forces the agitated saline into the bloodstream. The ultrasound then picks up the path the bubbles take within the heart.
My test showed the bubbles taking a “shortcut” through the atrium walls, suggesting that I had a hole between the two atria, known as an Aerial Septum Defect (ASD).
Arterial Septal Defect
To confirm the findings of the bubble test, I was scheduled for a cardiac TEE, which is an echocardiogram however instead of the probe being pressed onto your skin externally, it is inserted down your esophagus so that it can get an unobstructed image of the heart.This TEE confirmed the findings of the bubble test and led the cardiologist to believe that I had a 1.4-2mm hole between my right and left atriums. This was actually a bit of a relief as the MRI showed that the hole was approximately 12mm!My cardiologist informed me that this type of repair can luckily be performed non-invasively through a procedure called trans-catheter repair, where a surgeon inserts a catheter through an artery and guides a special set of tools into the heart where they install a customized closure deceive into the heart that closes the hole.
Trans-Esophageal Echocardiogram (TEE)
The second man who saved my life!
I was then referred to the the best surgeon on the island who specializes in this procedure, Dr. Christian Spies at Queen's Medical Center.
From the first appointment with Dr. Spies he earned my complete confidence. He was clean cut, well spoken and every time he spoke you knew that he was speaking from experience. His approach to my treatment was about as scientific method as you could get. With the information he had he formed his hypothesis and scheduled me for some tests to confirm.
First, he had me redo the TEE because he was not pleased with the one previously conducted at Tripler Army Medical Center. His TEE procedure showed that the ASD was in a different location than my cardiologist at Tripler originally believed, higher up, as well as it being significantly larger than initially believed, approximately 15mm verses the 1.4mm originally believed. 15mm! That meant that I had been living the majority of my life with a hole in my heart greater than 1/2 of an inch!
Based upon these findings he inferred that because of the size and location of this ASD that I likely also have another condition called Partial Anomalous Pulmonary Venous Return (PAPVR). I will oversimplify this explanation... We all know our heart is a pump. In a normal heart, the deoxygenated blood enters the right side of the heart where it is then pumped to the lungs. Once it is oxygenated by the lungs, the blood then enters the left side of the heart where it is pumped to the remainder of the body.
With PAPVR, some of the veins that should carry the already oxygenated blood from the lungs to the left side of the heart are actually incorrectly connected to the right side, which leads to already oxygenated blood entering the right side of the heart, only to be re-pumped to the lungs for oxygenation vice being sent to the body. This causes an inefficient cycle and leads to the right side of the heart to become overworked, which eventually leads to enlargement and ultimately heart failure.
Partial Anomalous Pulmonary Venous Return (PAPVR)
RUPV - right upper pulmonary vein
UPV - right upper pulmonary vein
RLPV - right lower pulmonary vein
LUPV - left upper pulmonary vein
LLPV - left lower pulmonary vein
SVC - superior vena cava
In the diagram to the left, you can see the red vein (RUPV) attached to the blue side of the heart on the left side of the diagram, right side of the heart, yea that shouldn't be there! I didn't mix up my left and right, when discussing the heart, you describe left and right as if the heart were in your chest, so when looking at a diagram of the heart, the left and right are swapped.
While PAPVR is less known and can go unnoticed, potentially for a lifetime, TAPVR, or Total Anomalous Pulmonary Venous Return is the more severe defect that is better known as it would be identified immediately after birth as the child would be born with a bluish tint. This would be because all of the veins returning oxygenated blood from the lungs would be incorrectly connected to the right side, only to be re-pumped to the lungs vice the rest of the body. This congenital defect requires immediate emergency surgery for proper repair.
Dr. Spies then scheduled me for a cardiac CT scan, which is a specialized CT scan where you wear a EKG device during the procedure that allows the CT scan machine to only take its images during the period of rest in between each heartbeat, allowing the device to capture the clearest pictures possible. To aid in the capture of the clearest image possible they also give you a Beta blocker pill which slows down the heart to approximately 60 BMP and they inject you with an iodine contrast solution.
A CT scan, while completely painless, can expose you in one scan, to the same amount of radiation as 500+ X-Rays. That is a lot of radiation! The Beta Blocker, the specialized EKG device, and the contrast dye are all utilized to ensure that the radiologist can capture all of the images needed with one scan!
The results of the procedure confirmed Dr. Spies hypothesis 100% that there was also a PAPVR and instantly solidified my confidence in his abilities!
I am not sure if my intent is to speak poorly of the quality of service at Tripler Army Medical Center however I can state that each of my civilian cardiologists from Queen's stated that they have no idea how the cardiologists at Tripler came to their conclusions based upon the tests they conducted. The cardiologists at Tripler both misinterpreted the location of my ASD, the size of the ASD, as well as completely missing the most serious of the defects, the PAPVR. Needless to say I am immensely grateful that I was referred to Queen's Medical Center for my treatment; they saved my life!
The first thing Dr. Spies said after reviewing the results of his tests was that he could not believe that I had been able to serve successfully as a Marine for the past 14 years having these two heart defects. He mentioned that I have likely have had these my entire life and that I should be considerably out of breath during physical activities and should have experienced noticeably diminished physical performance.
I suppose I can attest my ability to overcome this physical inability to my training as a Marine, were we are always taught to adapt and overcome, and that failure is never an option. Whenever a physical event seemed difficult I mentally conditioned myself to power through the difficulty.
To clarify, I never knew anything was wrong with me, I just thought I wasn’t as good a performer as others…
Dr. Spies then gave me some information I was not quite ready for, these defects would need to be repaired through open heart surgery and that he would be excited to see how my physical abilities and performance levels would increase due to the surgery enabling my body to receive 100% of the oxygenated blood that it was intended to receive.
"Um excuse me Doc, you sort of casually coasted right over the open-heart surgery part of that statement..."
He ensured me that this procedure was as basic and as straightforward as heart surgery goes and that there is nothing to worry about.
I had asked him if the benefits of this procedure outweighed the risk and he replied “100%.” He stated that this would be considered a definitive repair, meaning that once the repair is completed I can expect to live a normal life free from any related cardiac issues.
He informed me that he was going to refer me to Dr. Carlos Moreno and Dr. John Lamberti, the two surgeons who would be teaming up to perform this procedure. When I asked if these were the best surgeons available Dr. Spies informed me that these two gentlemen were world renowned and if he were to need to undergo this procedure these are the two gentlemen he would want to perform his surgery; I was sold.
The surgeons who saved my life!
I had one consultation with Dr. Moreno in mid January and it was certainly memorable. After pleasantries were exchanged Dr. Moreno spent the next 10 minutes explaining to me the complexities of my condition. How the presentation of my condition was not typical and how it was not what he expected. For 10 minutes I was waiting for this man to tell me "Mr. Forras, I'm sorry but I cannot operate on you.."
After he was done telling me how technical this repair is he wraps it up by saying "so it should be a pretty straight forward repair, and I expect that you will make a full recovery!"
I just laughed with relief and asked "why would you not just open with "it should be a pretty straight forward repair" and then explain to me the complexities!" Which I have to say I assumed this was complex, it's heart surgery!
After our brief office visit I had some definitive information, on 7 February, 2018, I would be undergoing open heart surgery at the age of 32!
Dr. Moreno informed me that he would be teaming up with Dr. Lamberti, who would be flying in from California, and together they would perform the 3+ hour open heart surgery that would save my life!
I was nervous, I was excited, I was relieved that we had a positive outlook to what began as a terrifying onset of news!
