Solving the Mysteries of Heart Disease

by Gerald D Buckberg

  History is the ultimate determiner of when truth will win. Yet along the way, new ideas, and most importantly, the subsequent human reaction to them, will create dynamic contrasts. It always has. A perfect example can be seen in the responses to theories about how the heart fills.

  Opposition to both new — and old — medical concepts has pervaded history. We’ve already cited that Harvey contradicted Galen by saying the heart filled by pressure and was not a sucking cup. Galen had made his fundamental observation of the ventricles suctioning blood long before, in AD 180, when reporting the “violent sucking of blood” from the vena cava (two large veins) as he looked within the chest of a gladiator whose injury exposed the heart’s biology.

  Yet even though Erasistratus (the previously mentioned father of physiology) had also observed this same suction used by the heart to achieve venous return, Galen was dismissive of him. Galen freely notes that he did not like Erasistratus as he felt him dishonest in how he came to his theories. In fact, Galen disliked Lycus of Macedonia (an anatomist with additional theories) even more. He thought Lycus was pompous and not worthy to be considered alongside many other wonderful Greeks.

  But Galen also made the most wonderful and insightful comment on the concept of learning. Despite all of this criticism, he said that Lycus “…is not to be disregarded; he may, perhaps, be stating some wonderful truth, unknown to any of his predecessors.” Galen knew that no matter how much we personally like or dislike someone, or accept or reject their overall approach — we must continue to listen in order to learn.

  This affected me profoundly, and led to my own observation: Ignorance is being unknowledgeable, but able to learn… while arrogance is being knowledgeable, but unable to be taught. It characterizes the pundits that believe in the yesterdays of tradition, but fail to look toward the future.

  Symbol of Medicine… More than a Symbol

  To advance medicine, we must retain our student-like ways. As we do, we enter what I call the “spiral pathway of learning.”

  This caduceus, (Figure 14) the symbol of the medical profession, is fittingly composed of two reciprocal spirals. These are interwoven, and from my perspective, this shape reflects a path to learning and then to wisdom. Near the top where a spiral is largest, we gather and enlarge our knowledge. As we move down one spiral loop, we interact with this information to analyze and differentiate it. Simultaneously, in the other spiral, we synthesize these facts and bring them together into a concept. We develop wisdom.

  Figure 14: Caduceus, the signpost of medicine, displays the same reciprocal spirals as in the helix, but contains a staff between them, conveying the need for action.

  But that is not the end point. We must do something with this wisdom. A surgeon cannot be like a monk sitting on a hill. There must be movement of knowledge. A concept, an approach, or a technique must be created and then tested. As cardiac surgeons, we are particularly fortunate because we can learn, we can understand, and we can act on the behalf of our patients.

  Recognizing this progression, and the absolute need for us to always remain willing to learn, led me to what became the final statement I would make in my presentation. It would be another quote from Albert Einstein, who said:

  “There exists a passion for comprehension, just as there exists a passion for music. That passion is rather common in children, but gets lost in most people later on.”

  I would supplement my hero by saying: “I hope not!”

  The Presentation at AATS

  All of this thought went into the preparation for my basic science lecture at the AATS, which went extremely well. After I made my last remark and departed the stage, there was a ten-minute standing ovation from the audience of 4,000 surgeons… which I heard from my seat in the audience.

  Francis Fontan, a dear friend and legend within our cardiac surgery profession, later asked me why did I had not remained at the lectern to receive the ovation. I simply told him I left because I was finished. Looking at me knowingly, Francis said, “You gave them your mind, and took your body away.”

  While I was elated to give the lecture, it wasn’t about my receiving glory or applause. I was not looking to take credit for being a great lecturer. What was astounding was the information and correlations presented. Nature is the actual presenter and should get all the credit.

  Francis and I both knew the true worth of science is only appreciated when the mind of the listener begins to accept new knowledge, after which they test this novel information to verify it, and a fresh view of reality then follows to create a new biological truth. That was my intention and hope behind delivering the talk.

  Of course, people will respond however they respond. Andy Wechsler, who was the editor of the Journal of Thoracic and Cardiovascular Surgery for eight years, once described me as the “Intellectual Provocateur of Heart Surgery.”

  That portrayal may have been accurate, but I preferred the comment made by a physician from Russia who remarked, “You saw the invisible and taught it to us.” It was the nicest thing anyone has ever said to me.

  Yet I am not the first, nor will I be the last, to teach “the invisible.” Our basic observations of cardiac suction began with the Greeks, were altered by Harvey, and are now changing back again to authentically reflect what occurs in nature. The reason for such progress — this “revolution” — is that the truth always wins. New knowledge changes the thinking of the listener, who in turn changes their actions.

  Circling Around

  While writing this book and reflecting on my experience of creating this lecture, I came to a fascinating recognition. Looking again at Monet’s series of paintings cited earlier, I noted all his haystacks had a wide base and an apical tip like an inverted heart, while his cathedral possessed lateral support from flying buttresses. These are the two primary components of the heart — its base and helix.

  Was this Monet’s mindset, or did it reflect my imagination? What is certain was that Monet saw a new world every time he looked. It is just like the experience I shared with my daughters as we walked down the same street and saw new things. That is what Monet did. He played the game of discovery.

  And so, the tradition of learning continues.

  The Helix and Heart lecture can be viewed at:

  www.youtube.com/watch?v=ArZ8GEFUQaw

  Further, the talk was formally written up with greater detail in the Journal of Thoracic and Cardiac Surgery, which can be accessed at:

  http://www.jtcvsonline.org/article/S0022-5223(02)00169-1/fulltext

  CHAPTER 20

  The Helix and Heart Failure: The Dilated Heart, Footballs, and Basketballs

  Curious colleagues often ask me why this memoir is being written for the non-medical public, since until now, all of my publications have been directed exclusively at the medical community.

  I recall a conversation I had with another colleague and friend, Connie Athanasuleas, who related a discussion he had with his mother. While sharing their time-honored traditional Greek salad, his mom, who had congestive heart failure, asked her heart surgeon son about a new procedure she saw on the television news (three years before our RESTORE team was formed). A surgeon from Brazil had removed a piece of heart muscle in a dilated heart in order to change its size and shape. Connie’s mom, despite her lack of medical knowledge, thought this seemed like an interesting idea. Connie was unaware of the procedure, but immediately appreciated the approach, and agreed that it introduced an intriguing solution to an otherwise lethal problem.

  The surgeon was Randas Batista, who I’ve mentioned before, and whose work will be described in this chapter. When confronted with new knowledge like this, a person can respond in one of two ways. Inquisitive physicians like Connie may not be aware of novel treatment breakthroughs that might solve conditions previously thought unsolvable, but they listen and are spurred to learn more. On the other hand, there are physicians, unlike Connie, who might know of some new procedure, but are hesitant to change. Their own traditional v
iewpoints are so limiting that they dismiss innovations that could have life-changing and life-saving importance.

  My hope is that readers will mirror the curiosity of Connie’s mother and welcome new discoveries such as those described in this and other chapters. Discoveries that might lead to compelling, persuasive conversations with their doctors to potentially open their eyes to new possibilities.

  I believe patients must inform themselves. They should not just wait passively for someone to tell them what to do. Whether it is for their own life, the life of a loved one, or simply from intense curiosity, we all need to pay attention to forward-looking treatments. Groundbreaking advances are constantly being made in all areas of medicine, as pioneers search out new solutions to age-old problems. Fresh answers must be encouraged and outmoded mindsets changed. Sometimes it is the patient that instigates the reversal of medical inflexibility.

  A New Angle on Dilated Hearts

  After learning of Paco’s discoveries, I grew even more fascinated with the heart. Paco presented glorious new information when he unveiled its true structure. This deeper understanding of how the heart becomes distorted would provide answers about how to treat the world’s greatest health hazard: congestive heart failure.

  We had already recognized the structural and functional differences that occur when heart failure causes the heart’s size and shape to enlarge from the normal elliptical football shape to become spherical like a basketball (Figure 1a and b). We knew the changed heart shape was the source of the problems generated by heart failure. But the full understanding of why had eluded us… until Paco’s structural analysis furnished that answer.

  Figure 1a: Normal conical heart on left, compared to the dilated spherical heart in heart failure on right.

  Figure 1b: Shows sports analogy, with spherical “heart” on left and conical heart on right.

  Paco let us explore the mechanical reasons behind the problem of heart failure by revealing that heart musculature is formed by a helix and a basal wrap. (Figure 2 upper) We learned that the transverse (horizontal) muscle fibers of the wrap caused squeezing or compression (like a blood pressure cuff on your arm). Conversely, the diagonal fibers of the helix permitted the predominant function of twisting — as they crossed each other at 60° angles. The simplicity and elegance were astounding.

  It suddenly was clear that as heart failure alters the cardiac structure to make the elliptical heart become spherical — this shape change makes its oblique (slanted at 60°) helix fibers more horizontal — to now resemble the wrap’s flatter fiber angles. (Figure 2 lower)

  But why does this geometric change create such a functional problem?

  I discovered a breathtaking study by Edward Sallin that explained the relationship between the angle of muscle fibers and function. Sallin was a bio-mathematician, who in 1969, identified how the orientation of heart muscle fibers determined the heart movement. He showed that each individual heart muscle fiber shortens by only 15% during compression at each heartbeat — yet in a heart with an oblique (angled) fiber orientation — the heart will eject 60% of its blood to circulate into the body (the normal response) during each heartbeat. Conversely, performance is impaired when these same fibers have a horizontal orientation — as their contraction yields only a 30% ejection fraction.109

  Figure 2: Upper drawing is normal architecture, with the helical arms reciprocally crossing each other at 60° angles. Lower drawing of dilated heart as fibers become more transverse (horizontal), at approximately 30° angles.

  Sallin’s contribution was enormous because he had structurally solved the missing gap, as he used form to explain function!

  I now fully understood why heart performance worsens when the cardiac form changes from the normal elliptical shape that contains obliquely angled fibers — into a dilated spherical shape where fibers are more horizontal. These observations validate the fundamental objective of rebuilding normality: we must return the fibers to their expected 60° angles in order to permit recovery of the heart’s natural function. (Figure 2)

  Different Heart Diseases — Same Focus

  What a revelation! Yet while I now had the complete explanation behind the need to restore the heart’s natural shape, I also knew that all conventional medical efforts were still totally aimed only at solving the illness that caused the heart to dilate (such as restoring adequate blood flow and preventing leaky valves) — rather than restoration of ventricular structure. Now more than ever, the spotlight needed to shine on correcting the dilation itself.

  The medical community needed to realize that this missing knowledge leads to a critical error in judgment. The circular shape is the consistent detrimental factor in the three major causes of heart failure with dilated hearts:

  The heart attack — in which the ventricle has a scar the causes the remote muscle to stretch as it dilates

  A leaky valve — that makes the ventricle dilate

  The heart muscle itself — when infections or viruses cause dilation, despite the presence of normal blood vessels and valves

  Figure 3: The three causes of a dilated heart include: on left, a heart attack due to a closed coronary artery; in center, a leaky aortic or mitral heart valve; and on right, direct damage of the heart muscle.

  These three origins of heart failure are shown in Figure 3. They all share the same inescapable destructive spherical shape. A circle is a circle is a circle, regardless of the basic underlying disease causing this globular shape.

  Heart failure cannot be cured until you convert the failing heart’s spherical shape into its natural elliptical form. However, this is never the singular solution. Successful treatment must also remedy the disease that produced this shape distortion (such as coronary disease or leaky heart valves). Embracing these dual goals can have an immense impact — because dilated hearts exist in half of the 15 million patients with heart failure.

  Heart Attacks — New Ideas, Old Resistance

  Heart attacks are the primary cause of heart dilation in the United States. Before exploring the focus of this chapter, let me review how such dilation is now typically treated — and how the development of rebuilding natural ventricular shape has restored the heart’s function.

  The first prior approach was angioplasty by cardiologists, since this treatment restores blood flow and corrects the surface bulge (aneurysm). But it leaves a non-contracting, thickened muscle in its place. Cardiac surgeons sometimes participated in the initial treatment response by performing a bypass graft to renew flow in obstructed arteries, and also correct leaky mitral valves. Despite satisfactory early survival of patients, the long-term outcomes are problematic when the heart becomes dilated.86 Patients with such stretched spherical hearts have a worsened quality of life due to heart failure or may develop lethal arrhythmias. Yet this fundamental cause of heart failure remains untreated.

  Dor’s concepts got me thinking about the geometric reasons for heart failure. The target area of our thinking was his focus upon the scar. While this is what causes the heart to stretch, conventional medicine ignores it. The role of the heart is to contract and support the circulation, and while the scarred region stops working immediately, the patient is kept alive by the still-functioning remote muscle that compensates for the dead scar. But we must now focus upon how to improve its function, because heart failure will develop progressively, despite such compensation. The reason for such impairment is linked to understanding the heart’s anatomy, because cardiac performance is determined by how the heart’s muscle fibers are oriented within its structural form. Healthy function occurs when they are slanted (as in a normal heart shaped elliptically like a football), but performance is dramatically diminished when the fibers become horizontal (as when the heart is stretched like a basketball).

  These lessons were not only illuminated by Dor, Paco, and Sallin. Richard Gorlin stated in 1967 that the prelude to heart failure was having a scar that occupied more than 20% of the ventricular muscle.77 He also observed that this s
tructural change stretched the functioning remote muscle, which then compensated by contracting more forcibly. Returning a more normal shape was the objective of the surgical breakthroughs in 1983 by Dor79 and Jatene.83 Each observed that their patients fared much better, characterized by vastly improved cardiac function — when their ventricular approach excluded the scar by restoring the natural elliptical shape — to a previously failing spherical heart.

  The Dor and Jatene surgical solutions departed from tradition… and a revolution was in the making. Validation of their approaches was powerfully shown in the superb clinical successes reported by our RESTORE Group, and in the 5,000 other patients described in the congestive heart failure chapter.94

  But still, no one listened.

  Not the Whole Answer

  I was undaunted that traditionalists blindly held onto conventional treatment. Rather, I was exhilarated that our international confirmation of the Dor procedure in dilated hearts might help set a new standard for treating heart failure.

  However, my enthusiasm was incomplete. Another dilemma confronted us.

  As good as our outcomes had been, they were not always perfect. Only limited recovery occurred after rebuilding very dilated ventricles. I pondered the reason for this failure. Was the operation incorrect? Or was this technique simply less effective in very stretched hearts?

  This much was very clear to me: these pivotal questions needed answering!