Solving the Mysteries of Heart Disease

by Gerald D Buckberg

  Once settled on this new plan, I decided to put on paper my concept of the journey we would all undertake together. The ideas flowed endlessly throughout my flight home from Argentina. While everyone else was seeking sleep during the 17-hour trip to Los Angeles, the little ceiling light above my seat stayed lit as I fervently wrote. Energy raced through me as I composed page after page after page, creating 70 consecutive handwritten pages of notes describing every ramification of this whole process of countering heart failure and all the studies that could be done and how to make best use of them. These pages would serve as the blueprint for our study, which I would take with me as I traveled the world to meet with each of these renowned surgeons. These meetings will be described in the second of these next two chapters on heart failure.

  I completed my pages and leaned back in my seat. I was alive again. My capacity to think was returning as well, no doubt thanks to the remarkable capacity of the brain to form new neural pathways to compensate for damaged tissues. My internal voyage back to myself — had worked.

  New Journey

  Riding in the car toward my house after landing in Los Angeles, I took a moment to reflect on my ordeal. Styron had been right on target, since the deep depression that had consumed my world during the past year and a half… had simply evaporated within the seven days in Argentina that now launched me onto this new creative effort. His describing the depths of depression had an astounding impact, as my course remarkably paralleled his.

  During my depression, I shared the power of his book, Darkness Visible, with my daughters, sending each a copy so they could better comprehend what was occurring with their father. My youngest daughter, Gia, happened to be getting her master’s degree in social work at the University of Minnesota when she read it and was astonished by the remarkable parity of Styron’s story and mine. With my consent, Gia wrote about my experience as part of her postgraduate studies and sent me a copy. I was moved that someone so close to me could so fully grasp my private trials.

  Wishing to acknowledge Styron for his contribution to me, I located his address and forwarded him a note, describing myself and adding Gia’s report. He promptly responded and defined our liaison by stating, “We have a brotherhood.”

  Styron’s creativity was the road to his recovery, and the remainder of this memoir will define how I followed my own internal path in resuming my life and passions. For me, creativity means filling an empty room with new ideas, testing them, while making ongoing changes during learning. My return to normality was not through drugs. It was generated by the excitement of uncovering the new uncharted realms that would consume my spirit, energize my attitudes, and rekindle a new sense of myself.

  My imaginative future was brimming, as initiation of this congestive heart failure project carried with it the chance to develop a means to help patients — the centerpiece of my universe. It would lay out the game plan for the next ten years of my life.

  I had returned home. I was me.

  CHAPTER 16

  Congestive Heart Failure: Education to Enactment

  After returning to Los Angeles from Argentina, I fully involved myself in trying to solve the immense, worldwide problem of congestive heart failure. I had found direction.

  My secretary, who 18 months earlier had been the first to notice changes in my memory, now recognized a huge shift in my demeanor. She asked, “What happened to you?”

  “Everything. I’ll tell you about it later. We have work to do.”

  Her face lit up, her long-absent smile returning.

  We set about getting my office in order. Books came out of boxes and onto shelves. Clutter was removed. I could suddenly appreciate the view out of my sixth-floor window… and envision what I was about to undertake.

  I thought about how the medical community presently viewed congestive heart failure. Even though it was the largest problem in cardiac care, almost nobody understood why it impaired heart efficiency, or had any idea how to fix it. Treatment focused on creating better drugs to offset the symptoms. These may have helped patients live longer… but the quality of their lives still suffered terribly, ultimately ending in premature death. This was the best that the medical community could offer.

  But I knew something they did not.

  The Truth

  A guiding principle is that you cannot treat what you do not understand. Too often it is assumed that what we imagine to be reality is the truth, and conclusions are drawn from that belief.

  What I knew was this: heart failure developed because impaired heart muscle function caused inefficient performance. And I knew why.

  Everything that most everyone believed about heart motion was wrong. To begin with, ask nearly anyone in medicine and they would describe heart action as compression and dilation — portrayed by making a clenched fist and then opening it. This type of function was first described by William Harvey, who famously discovered and wrote about the body’s circulation in the 1600s.76 His basic belief of how a heart functions has persisted ever since. In fact, the earliest two-dimensional imaging tools (injecting dye into a contracting heart and observing its image, or from an echocardiogram) supported that the heart acts almost like a piston — by compressing its volume to effectively drive out blood to meet our body’s needs — and then drawing backward to refill for the next beat.

  Harvey’s conclusions replaced all earlier theories and became the standard for the next 400 years. His beliefs were taught in all medical schools.

  It just happens that these beliefs aren’t true.

  How could that be? As is so often the case, people only see what they expect to see. This includes the world of medicine. For example, surgeons like myself, who watched the heart beat inside an open chest, thought it displayed the pumping action that Harvey described — because that’s what we were taught to expect. We did not perceive the twisting motion that Galen, a prominent Greek physician, surgeon, and philosopher, had reported in AD 180. He was the physician to the gladiators… and witnessed this natural twisting motion after looking at the heart through the open chest wound of a fallen warrior.

  Galen’s observations have since been verified by today’s three-dimensional measuring tools (like magnetic resonance imaging and speckle tracking echocardiograms), which now can document that the normal heart twists to eject blood, and then uncoils to fill.

  Consequently, it is impossible for a hand to accurately mirror a normal heartbeat by clenching the fist, a motion that makes all the fingers simultaneously close and then open. Instead, a sequential clenching motion must be used — moving from little to ring to middle to index finger — to create a dynamic whorl as they close and compress.

  Functionally, the biggest determinant of cardiac efficiency is the heart’s twisting spiral motion, as these whorling movements reflect the same motion that exists within a hurricane or tornado. By contrast, the commonly believed compression or piston movements play only a minor role in the normal heart’s effectiveness.

  The Battle of the Bulge

  Sitting in my office after returning from Argentina, I realized our first step toward solving the enduring riddle of heart failure would involve these functional differences between the normal spiral twisting heart — and the much less effective heart that occurs in cardiac failure, which only squeezes as its natural elliptical shape changes to a spherical dilated (stretched) form.

  I recognized that heart failure has continued to be an awful problem in cardiac care precisely because its causes had not been clearly understood. What was known is that a heart attack causes death in a cardiac region where there had been an interrupted or insufficient blood flow, so that a scar replaces the normal muscle. Please understand that this term “scar” does not mean the narrow scar most people envision when they have a cut on their arm or leg. Instead it describes the dead and functionless muscle area in the heart that has lost its blood supply.

  The result of this scar is that a bulge or aneurysm now develops, as this previously normal cont
racting region (when the heart’s shape was elliptical or conical) will now stop squeezing, as it thins and billows with each heartbeat. (Figure 1, left image) The patient is kept alive because the heart continues beating thanks to the remaining live muscle that did not suffer a heart attack (as its blood supply had not been obstructed).

  Yet all the patient knows is they feel chest pain, the tell-tale indicator of a heart attack.

  Figure 1: On left: cardiac shape when there is no blood reflow after a heart attack. Lighter gray is V-shape of normal heart, which becomes spherical (darker shading) after injured muscle thins and billows.

  On right: cardiac shape after blood reflow showing extensive damage in darkened area of scar in inner shell and deeper muscle. The outer muscle shell is undamaged, but function does not recover, despite return of its shape toward normal.

  As previously described in the chapter on heart attacks, the victim is rushed to the hospital, where a cardiologist performs an angioplasty to open the blockage in the artery (or uses drugs called thrombolytics that dissolve the clot). When the artery opens, symptoms rapidly improve: the chest pain lessens, and the blood pressure and the cardiac output come up, while the filling pressure in the lungs and the pulmonary artery pressure go down. The bulge disappears. The patient survives the heart attack and the angioplasty team takes pride in a job well done. The patient’s life has been saved by thwarting the immediate risk of dying from the heart attack.

  But the picture is not as clear as had been hoped. The formerly bulging dead muscle within the heart attack region has now shrunk to a smaller, thickened… but still non-contracting region. (Figure 1, right image) Clues to a more gloomy future may already be forming — for even if only 20% of this injured muscle is without function, the scenario of progressive heart failure will begin to rear its ugly head.77

  Remote Compensation

  However, this grim future isn’t immediately evident. That’s because the body is truly remarkable. It strives to stay alive, and the way it does this after there’s a scar, is to have its remote muscle (the still-functioning muscle area away from the scarred tissue) compensate to keep the heart pumping.

  Some of this was already known. In fact, cardiac surgeons can also become involved after an acute heart attack — to perform coronary artery bypass grafting to better restore blood flow to this remote muscle that is now so critical to the patient’s survival (and also may repair or replace a leaking mitral valve when needed).

  Unfortunately, despite being able to surgically improve the remote muscle’s viability, surgeons are also helpless to stop adverse long-term outcomes — since congestive heart failure may develop, despite the presence of successful coronary artery bypass grafts. And the disease will finally prevail.

  But why?

  Not Making a Difference

  This quandary deeply troubled me. I joined many other cardiac surgeon colleagues in realizing that many of our heart attack patients suffer a degradation of their health over time. This tragedy had played out in my own life, with my father’s experience. It pained me greatly to watch this robust man growing short of breath after only minor exertions. His walking became difficult, normal activities became impossible, and finally, he became incapacitated and was stuck in bed at home much of the time. He had to go to the emergency room every two weeks and no longer enjoyed living. It was terrible. He was a cardiac cripple.

  Yet in all the intervening years, I watched other patients suffer just as my father had, these same dire circumstances repeating themselves despite advances in medical treatment and new drugs. In fact, studies showed that these surgical procedures that restore blood flow to the remote muscle and / or remedy a leaking valve after a heart attack — offered no better long-term results than patients who had no surgery.78

  As a result, an astounding conclusion was reached. Cardiologists suggested we abandon these operations entirely, in favor of keeping these patients on drugs instead.78 To me, this seemed like throwing the baby out with the bath water… instead of finding out what the problem was with the bath water.

  I kept asking myself, “How can this be? I did everything correctly in these procedures. We improved flow though the coronary vessels, made leaky valves function properly, and prevented cardiac damage during the operation by using our heart protection methods. Yet, despite these advantages, our surgical operation did not fulfill our expectation of having the patient experience a long and healthy life.”

  To me, this failure to have a long-term improvement meant a fundamental cardiac problem had not yet been corrected. The answer had to be that we did not get to the disease component that caused this heart failure. We all were frustrated by this dilemma. Worse, no one knew what to do to change it.

  …Except someone had found the missing piece.

  Monaco: Gaming Tables… or Operating Tables?

  My work on controlled reperfusion had generated international interest, and years before my trip to Argentina, I was invited to Monaco, France to share my theories and knowledge.

  Being a kid from the Bronx, my image of Monaco centered upon the grand gaming rooms and financial intrigue glamorized in Hollywood movies. But from a personal perspective, gambling had never been important, because those pursuits involve only educated guesses… and guesses were not my passion. Thus, the allure of the casino tables had no effect on me. Instead, my goal was to expose medical audiences to a sure-fire winner by demonstrating the benefits of controlled reperfusion in treatment of an acute heart attack.

  Little did I know then that my visit to this fabled city would be the catalyst to my eventual search for the answer to heart failure.

  Teacher Becomes Pupil

  This quest was initiated when I visited my host, Vincent Dor, in Monaco. Dr. Dor had been Chief of Cardiothoracic Surgery at the University of Nice. As with so many of my trips, I went to teach — in this case, about using controlled reperfusion to prevent a scar from forming after a heart attack. Yet I came upon someone from whom I could learn.

  It was at this time that Dr. Dor declared he had discovered something startling: he knew why heart failure occurred… and had found a way to fix it.

  He had my full attention!

  Dor confidently stated that it was “this scar — this dead tissue inside the heart after a heart attack — that was the cause of heart failure.” It was the first time I had heard of this analysis.

  Further, he claimed the secret behind solving heart failure — was to rebuild the ventricle into its natural elliptical shape.

  I still remember my immediate reaction. The ventricle? Nobody had ever tried to deal with the ventricle to treat heart failure. Why would they? Once a patient received angioplasty or thrombosis drugs, the ventricle would again have a normal surface. The heart’s bulge would have disappeared.

  It was then that Dor looked at me and reiterated something I already knew: when the thin bulge disappears after angioplasty, that area of the heart becomes thickened — but it remains akinetic (does not move). He pointed out that confusion arises in the minds of the surgeons when they see that heart surface has no visible scar (bulge). They believe no incision should be made into this normal-looking region (even though a scar still exists within its inner shell). But he then emphatically pointed out: “If it doesn’t squeeze, what difference is there if it doesn’t squeeze because it is bulging, or if it doesn’t squeeze because it contains a scar beneath its normal surface?”

  That’s when he reemphasized, “The essential problem is the scar that remains.”

  His observation astounded me. I knew it was a straightforward procedure for surgeons to treat the thin-walled aneurysm to eliminate this bulge. They simply remove the billowing portion to return the heart back to its normal shape. But performing this procedure had become a rare event, since most patients underwent angioplasty instead, so that the heart attack region became a smaller, thicker — though still functionless — muscle.

  Yet nobody except Vincent Dor worried about how to surgically tre
at the scar remaining in the non-contracting muscle after angioplasty. Why would we? We knew the remote muscle (that region of live heart muscle away from the scar) would compensate to pump blood and keep the heart going.

  So while a scar remained after a heart attack, there was not a broad understanding that it was related to heart failure.

  Until now.

  Dor again stressed that the irreversible scar caused by the heart attack must be surgically excluded from the working (or functioning) ventricular chamber, as its presence causes the abnormal changes in ventricle size — and that altered shape is the structural basis behind the progression toward heart failure.79, 80

  The Shape of Things to Come

  Dor’s work showed that the world was wrong. The answers to heart failure were not a mystery! It had to do with geometry — and the twisting heart.

  Though described previously in different ways, I will summarize this to further clarify its role in congestive heart failure:

  A starting point for understanding this process is that the heart is naturally elliptical in shape, with a helical (spiral) structure that provides a powerful and efficient twisting motion to pump blood.

  But when there is a heart attack, a scar (dead tissue) will form in the entire section of the damaged muscle. This involves 100% of the heart attack muscle’s ventricle wall, leading to billowing or bulging of this non-contracting muscle mass.