Sudden Cardiac Death: Resuscitation or Resurrection? (Essay 23)

Bernard Lown, MD

In early January 2011 my son, Fred, drove me to the Brigham and Women’s Hospital emergency ward for a recurrent intestinal obstruction. The streets from our upscale suburban home are full of potholes. For the first time I experienced each one as a painful jab in my distended belly. Arriving at the hospital I was in no mood to socialize. Beside me at the check-in desk was a familiar face, a heavy-set, elderly, unkempt woman in a wheelchair. Scraping my distracted memory evoked no name, no identity, no hint of recognition.

The woman made an announcement for all in earshot: “This is Doctor Lown, the greatest doctor in the world.” My contorted bowel remained indifferent to this ego enhancing proclamation.  It did however awake a dormant memory.  I finally recognized her. She was Priscilla , the wife of the Reverend Keith Johnston, who had been a patient 36 years ago on my service in this very hospital. The reason for his hospitalization then  was due to a cardiac arrest, from which he had been  resuscitated with difficulty. Priscilla told me that her husband had been now admitted with a massive cerebral bleed and was not expected to survive. I later learn that he died that very week.

In 1975 my service was filled with patients who had been resuscitated from cardiac arrest. This was before the days of implantable defibrillators. Our aim was to establish which antiarrhythmic drugs would prevent a recurrence. The Reverend Johnston was unique among these patients, as will become clear.

The pastor was a tall, slim, muscular, boyish-looking man appearing a decade younger than his age of 39. He had been in blissful good health, rarely had any illness that required visiting a doctor. On the fateful day of his cardiac arrest he had returned early from his church and was “roughhousing” with two daughters, aged 13 and 15, when he collapsed, stopped breathing, convulsed several times, and turned an ashen blue. It was his good fortune that his wife was home. She was a registered nurse who two weeks earlier was certified in cardiopulmonary resuscitation. Paramedics arrived promptly and raced him to the local community hospital. The outlook was dire since it required several defibrillations to get a sustained heartbeat. He regained consciousness after 12 hours without residual neurological deficits. His underlying heart rhythm continued to be convulsed by numerous extra systoles, auguring a possible recurrence of ventricular fibrillation, the hallmark chaotic rhythm disorder of a cardiac arrest. Several weeks thereafter he was transferred to the Peter Bent Brigham Hospital, now the Brigham and Women’s Hospital.

On first meeting the Reverend Johnston I was struck by a boyish grin seemingly bent on mischief. I was puzzled by the contradiction between his near-death experience and his overt mood of levity and ready laughter. It soon became clear that this was a facade to conceal the terror of impending doom. He was desperate to understand why he was stricken. If God would smite him, who could protect him? More troubling was why the Lord had tested him in such an incomprehensible manner.

If he was puzzled about the cardiac arrest, I was dumbfounded. Over the previous 15 years I had been intensely investigating the syndrome of sudden cardiac death. It claimed an American life every 90 seconds, or more than 350,000 victims annually. My service at the Brigham Hospital had become an international center for studying malignant disorders of the heart’s rhythm. Johnston did not fit the prototype of those afflicted.

Invariably victims had had significant, if not far advanced, coronary artery disease or congenital cardiac anomalies. Usually they were middle-aged or older. Commonly they were stressed at work or afflicted with dysfunctional families. Many were frustrated high achievers. Johnston was free of any evidence of heart disease. He did not even harbor any of the risk factors that long antedate the onset of cardiovascular disease. His parents were in robust good health. There was no heart disease or diabetes in his family. He had never smoked; his blood pressure was low normal. He jogged as much as 10 miles several times a week and enjoyed riding his horse. He claimed a good marriage, a normal sex life, well-behaved daughters, and little stress in his occupation. As the pastor of a small Methodist congregation, he did not have an exceptional workload and was in control of his own schedule.

Johnston described that fateful day, before “it happened,” as extraordinary in its ordinariness,  although he had returned from church early in the afternoon, which was not his habit. When I probed why he had gone home early, he was evasive. I gave up when he grew seemingly uncomfortable. Apparently, as he was wrestling with one of his teenage daughters, she punched him in the lower  chest. A few minutes later said goodbye  and collapsed. For this deeply religious man the only explanation was that God had punished him. But for what sin? Before sleuthing for the sin, imagined or real, I had to invest a prodigious amount of time in understanding what had transpired and what the underlying cardiac pathology was, which I was certain we would identify. The reason for my cock-sureness was straightforward: we had never before failed to identify a cardiac basis.

His wife was the most intimate witness to the cardiac arrest. She was a large, obese woman who talked in an unhurried monotone. She had none of the exuberance of her husband. I felt as though I was conversing, not with a spouse, but with a protective older sister who was frequently running interference for her errant and mischievous sibling. She remembered vividly the fateful moment though it differed in some perhaps important details from her husband’s recollection. About 10 minutes after his daughter punched him in the upper belly, a neighbor rang the doorbell. At this point Johnston uttered, “Oh, I am sorry,” and collapsed with agonal breathing and clonic movements. The most striking difference in recalling his final words was that he claimed to have said goodbye, she maintained hat he uttered an apology.

Priscilla felt Keith he was highly emotional but bottled up. When pressed, she related that he faced a contentious congregation. Though the parish was small, with only 225 families, the bickering was endless. Their intense disputes strained relations among everyone. Furthermore, the church hierarchy was not supportive of his ministry. Her palpable anxiety was beyond reassurance. Her husband  was caught up in what she deemed a no-exit situation; another cardiac arrest was inevitable.

Upon examination I was struck with Johnston’s exuding vitality and seeming good health. The stubble of an unshaved beard lent his boyish face some gravitas. There were no telltale marks of heart disease. Blood values showed no evidence of diabetes. Cholesterol and other lipid moieties were normal. The electrocardiogram was normal. The heart was small, the aorta was not dilated, peripheral pulses were intact. Angiographic visualization of coronary vessels showed none to be obstructed or even narrowed. He was able to exercise on a motorized treadmill adhering to a stressful protocol up to 17 minutes without the heart muscle anoxia experienced by patients with impaired coronary artery blood flow. However, he did exhibit many ventricular skipped beats, some coming in salvos, a possible omen for malignant arrhythmia that can degenerate into ventricular fibrillation, the arrhythmia of the cardiac arrest.

We confronted a puzzle: how to explain a life-threatening perturbation of the heartbeat in someone without demonstrable heart disease. Being clueless impeded implementing a medical program to protect the pastor against a recurrence of ventricular fibrillation. The next such episode might occur without anyone present and could be his death knell. Further, we could not allay his anxiety without understanding why he had been a victim. Our ignorance  further stoked his already ingot-glowing apprehension.  Unexpectedly the pastor himself came to the rescue, pointing us in a new direction.

At 4 a.m. on the fourth day in the hospital, while fast asleep, he experienced a second cardiac arrest. This is the time of night when one dreams. He vehemently denied dreaming. Two years earlier we had investigated the effect of sleep on the stability of the heart’s rhythm.(1) We found that with sleep, even among patients with coronary heart disease, the heart rate slowed and rhythm perturbations lessened or disappeared entirely. Ventricular extra systoles, including those of advanced grade that augur a possible cardiac arrest, decreased by 50 percent. What drove the pastor’s heartbeat in an opposite direction? Perhaps what he was dreaming might provide clues about the recurrent cardiac arrests. It was the proverbial dog that didn’t bark in the genre of a Sherlock Holmes mystery.

We immediately started him on a continuous intravenous lidocaine infusion.(2) Some years earlier I had discovered that this anesthetic agent, then used almost exclusively by dentists, was effective against ventricular disordered heart rhythms such as those afflicting the Reverend Johnston. We also intensely examined whether emotional factors might have played a role. He underwent psychiatric evaluation and a battery of psychological tests.( 3) While asleep, he was monitored with a Grass Polygraph(4) that enabled us to record throughout the night brain wave activity, eye motion, heart rhythm, and muscle tone. Thereby we were able to correlate dreaming periods with changes in heart rhythm. I also spent much time probing the content of the dreams.

Johnston  was initially very reluctant to see a psychiatrist. During four such interviews, each monitored electrocardiographically, extra heartbeats, ventricular extra systoles, increased several-fold, including the fast salvos that augur potentially malignant tachycardia. In fact, an hour long session with the psychiatrist resulted in more extra systoles than in the previous 24 hours combined.

The psychiatrist found him to be tormented and raging against being bottled up in a small community, a diminutive parish without chance for advancement under an oppressive church hierarchy. Adding to this brew were the oft-recurring titillating erotic fantasies that he was battling. He both rebelled against and welcomed his controlling wife, who kept him on the straight and narrow. The psychology tests lent support to these assessments, pointing to the centrality of his struggle to control aggressive and hostile thoughts that he deemed mortally sinful against an all-loving God.

The sleep studies added further evidence, pointing to the primacy of emotional and psychological factors. Our past investigations had demonstrated that sleep ameliorated cardiac rhythm disorders.(1) But the opposite was the case with Johnston. During REM sleep, the time of the night devoted to dreaming, he had twice as many extra systoles as during the waking state. Even more striking were those ominous salvos. These accounted for 2 percent of all extra systoles while he was awake but increased by 32 percent during REM sleep.

Ultimately our lengthy conversations provided decisive insights as to what caused the pastor’s heart to disorganize into the chaotic rhythm leading to the near-death experiences. I pressed him hard to divulge what he dreamt the night of the cardiac arrest. Repeatedly he rebuffed my questions with “I didn’t dream” or “I don’t remember what I dreamt.” He was not budged by exhortations that his doctors needed to be informed if they were to protect him against a potentially fatal recurrence. At times I felt like an obdurate police detective pressuring a potential suspect to confess his guilt. He was unfazed by the line of argument that sooner or later we would have to discontinue the intravenous infusion of lidocaine. However, he became very restless when informed that the lidocaine would be stopped that night. We also intended to repeat the polygraphic monitoring of his sleep to confirm whether dreaming was associated with a cardiac arrest should it occur. His only response was a neutered one: ”Doctor, you have to do what you think is best.”

Two hours later I was paged by a cardiac care unit nurse that a very disturbed Reverend Johnston demanded to see me. He pleaded with me not to stop the lidocaine. My response was, “OK, what about tomorrow night, or the next night, or the night after that? How long can this go on?”

Appearing extraordinarily agitated, he asked me to close the door. As though in a séance of free association, gazing at a remote spot on the ceiling, without once looking at me, he acknowledged having had a “disgusting dream.” He related something of the following: In his dream, he was parked on the edge of a cliff with a woman of his congregation with whom he was collaborating on a church project. The motor is running as they begin “to make out.” Suddenly he hears a police siren and sees approaching headlights in the rearview mirror. At this point he steps on the accelerator, and the car plunges into the ravine below. The next thing Johnston was aware of was being surrounded by doctors and nurses who informed him of his second cardiac arrest. A long silence followed. I thanked him for his trust and assured him that we knew how to protect him against recurrence.

I was struck by the similarities in the precipitating events of both cardiac arrests, namely, the prominence of erotic content and the auditory signal of an unexpected intruder. I recalled his evasive circumlocution about why he had returned home at midday just before his first cardiac arrest. He told me that he had been horsing around with his teenage daughters. I was taken aback upon meeting them. They were precociously mature and resembled grown women rather than prepubescent teenagers. In each episode there was a precipitating sound, the doorbell or a police car siren. In the first the intruder was a neighbor, in the second a police officer. Just before the first cardiac arrest, he apologized, saying, “I’m sorry.” In the second he inflicted the ultimate self-punishment in an act of suicide and homicide by stepping on the car’s accelerator and toppling into an imagined void.

Treatment was largely focused on obtunding nerve traffic from brain to heart.(5) We focused on drugs that diminished sympathetic and increased parasympathetic neural outflow to the heart. We used three different class drugs, aiming to provide a safety net should one not adequately protect against a recurrence.(6) Johnston was taught how to eliminate ventricular extra systoles by means of meditation, and an active exercise program was instituted. He was encouraged to obtain psychiatric counseling. Once the program was in place, cardiac arrhythmia could no longer be provoked whatever the aversive stimulation. Visits by the psychiatrist, REM sleep, strenuous exercise, and provocative conversations failed to induce ventricular arrhythmia.

When it came to hospital discharge. I noted that Priscilla Johnston, instead of being pleased by her husband’s homecoming, requested that we keep him hospitalized for an additional week. She offered no explanation. Out of the clear blue and without comprehending my own words, I turned to the pastor and asserted, “If you want to get out of here, you will have to shave off your beard.” Without a word of protest he promptly shaved his face clean. His wife embraced me tearfully, indicating that she no longer objected to his immediate hospital discharge.

An explanation was soon forthcoming. Apparently he was directing an Easter passion play, wherein he was cast in the role of Jesus. It was to take place the following week. Priscilla was certain that once on the cross, his religious fervor and identity with Christ would provoke a fatal cardiac arrest. She was also certain that once the beard was gone he would abandon the play.

When I saw the pastor in follow-up three months later, the first question was about the passion play. Indeed there was no play, as his wife had predicted; instead he had delivered an Easter sermon. The church was packed, and his parents for the first time visited from Pennsylvania. He related that compared to the sermon, no action of his  ever was as meaningful. He was charged with emotion, his legs were trembling, though his heart was quiet while delivering the sermon.

“So what was your sermon about?” I inquired eagerly.

He responded with pride, “It was titled, ‘ Sudden Death: Resuscitation or Resurrection?’”

That title evoked a long-forgotten experience. In 1961 I was visited in my laboratory at the Harvard School of Public Health by the abbess in charge of nursing at Catholic hospitals throughout southern Europe, with headquarters in the Vatican. Accompanying her was a State Department representative and an Italian translator. I had just developed the direct current defibrillator and the cardiovertor for treating heart rhythm disorders. She was eager to learn about American medical innovations developed in the aftermath of WWII, when Europe was at a standstill. She grew seemingly uneasy as I explained the new technology. Presuming some miscommunication, I asked the translator to inform the abbess that with the new instruments we did not resurrect; we merely resuscitated. She let go with peals of laughter and invited me to be her guest in Rome.

It is worth commenting how the Reverend Johnston fared after his hospital discharge. The aftermath of a cardiac arrest is not kind on victim or family. During the first three years a deep-seated anxiety shrouded the Johnstons. Major apprehension was provoked each time he had a minor symptom. They all avoided the common room, where the cardiac arrest had occurred. Eventually they were forced to sell their home and relocate to another community. Sleep was often restless and easily disrupted. His wife continued to wake with a startle and move close to determine whether he was breathing. At times panicked, she found herself searching for his pulse. He continued to moan and groan during sleep. He told her that this was the result of a struggle “to put human beings together again.” He admitted to his doctors that he still had disturbing dreams but was quite philosophic about their occurrence, rationalizing that they purged his mind of  “evil thoughts during daytime.” After 26 years of uneventful follow-up, to spare him the long journey to Boston, I discharged the pastor to the care of his family physician.

I was well aware that he was at high risk of having a recurrence. A study emanating from Seattle reported that patients whose cardiac arrest was not provoked by a heart attack, as was the case with the pastor, were highly likely to have a recurrence. The median time lapse to the next cardiac arrest was a mere 20 weeks.(7) In fact, current guidelines mandate the implanting of a defibrillator in anyone who has experienced two or more cardiac arrests. Yet, on the antiarrhythmic drug program we had evolved, the Reverend Johnston had no cardiac arrests nor did he have any hospital admissions during the ensuing 36 years.

The New England Journal of Medicine published this case report and featured it as a lead article.(8) A number of key concepts were promulgated. These  deserve brief enumeration:

1. Transient risk factors change the excitable properties of the heart and can trigger a cardiac arrest.

2. Higher nervous activity, generated by stressful psychological states, is among the key transient risk factors.

3. Higher nervous activity can trigger ventricular fibrillation, the heart rhythm disorder of sudden death, in the absence of demonstrable heart disease.

4. Dreaming can trigger cardiac arrest.

5. An effective therapeutic program can be structured to prevent higher nervous activity triggering cardiac arrest and to protect patients over their lifetime.

Our published report did not alter the treatment of cardiac arrest. None of the principles we had enunciated were adopted. Doctors generally regard clinical data as scientific when a reproducible relationship is supported in a large population sample. The larger the sample, the more attention is paid to the study. Judgment based on a single case report is regarded as anecdotal and harks back to pre-scientific medicine. But as the philosopher of science Karl Popper maintained, a conclusion that all swans are black is not convincing, irrespective of sample size.

Observing merely a single white swan refutes such a contention about the color of swans. The Reverend Johnston was that white swan, implicating the power of psychological factors in provoking sudden death.

The fact that little attention was paid to our report in the New England Journal of Medicine. I believe was not due to the philosophic issue of what constitutes relevant scientific data but rather to the psychological orientation of the authors. Most modern physicians are ill trained to address the psychological dimension of their patients’ ailments. I found support for this conclusion when reviewing the voluminous patient’s chart for this essay.

During the Reverend Johnston’s annual visits, a postdoctoral fellow in our training program reviewed his record, interviewed and examined him, participated during my meeting with the patient, and finally wrote an extensive summary of his or her observations. These young doctors had finished their internships and residency training and were among the best and the brightest. They were a self-selected group attracted to our program because of its holistic approach to patient care. Reading their reports of these visits left me dismayed. Only two of 16 fellows reflected in their notes any interest in the emotional factors that had led to the cardiac arrest. Their reports largely focused on externalities relating to what various technologies divulged about the state of his heart. Of the two post-docs who dealt at length with emotional triggers, one had been coauthor of the published report.

 References and Notes

1. Lown B, Tykocinski M, Garfein A, Brooks P: Sleep and ventricular premature beats. Circulation 1973; 48: 691-701.

2. Lown B, Fakhro A, Hood WB, Thorn GW. The coronary Care Unit: New perspective and Directions. JAMA 1967: 199:188-198.

3. The psychological tests included the Minnesota Multiphasic Personality Index (MMPI), Wechsler Adult Intelligence Test, and Rorschach ink blot testing.

4. The Grass Polygraph provided recordings through nearly nine hours of sleep. It included an electroencephalogram (brain waves), elecro-occulogram (eye movements), electromyogram (muscle contractions), and electrocardiogram (heart rhythm). This enabled us to correlate REM sleep, which is associated with dreaming, and heart rhythm abnormalities.

5. We introduced a host of new methodologies to protect patients from recurrence who were threatened with sudden cardiac death. These included Trendscription, permitting expeditious and gestalt assessments of large amounts of data enabling us to grade trends in arrythmias. Acute drug testing enabled a prompt determination of both the efficacy and safety of an antiarrhythmic drugs. The acetylstrophanthin tolerance test provided nearly instantaneous exposure to whether digitalis glycosides would exert an anti- or pro-arrhythmic effect.

6. The three arrhythmic drugs that totally suppressed all ventricular ectopic activity against all types of provocations were a beta blocker, the digitalis glycoside Digoxin, in a large daily dose, and the anti-epilepsy drug Dilantin.

7. Schaeffer WA, Cobb LA: Recurrent ventricular fibrillation and modes of death in survivors of out-of-hospital ventricular fibrillation. New England Journal of Medicine 1975:293, 259-262.

8. Lown B, Temte JV, Reich P, Gaughan C, Regenstein Q, Hai H: Basis for recurring ventricular fibrillation in the absence of coronary heart disease and its management. New England Journal of Medicine 1976:294, 62


11 responses to “Sudden Cardiac Death: Resuscitation or Resurrection? (Essay 23)

  1. Barbara H. Roberts, MD

    A fascinating story on many levels. I would give anything to know what prompted you to suggest the patient shave off his beard. To me that is the most amazing part of this case report.

  2. Warren Green

    Dear Bernard,

    Thank you for the latest essay, a most fascinating one that raises some unanswered questions for me. First, it strikes me as an essay that any medical student interested in cardiology and psychiatry should read. But of course I have no idea about any of the medical issues. My questions have to do with Rev. Johnston’s behavior, and you probably can’t adequately answer them or you would have in your essay. For example, why did he not want to say why he came home from church early that fateful day? Did it have anything to do with the passion play he got himself involved in? And was the project he was involved in with the woman who inhabited his erotic dream on the edge of the cliff the passion play project? And the punch to the lower chest—nu? Not related? What of it? Is it left unexplored because a full ten minutes elapsed before the collapse? And there’s an implication of a sexual element to the roughhousing with the physically mature teenage girls, but it’s not explored. So I’m left frustrated, which I’m sure was your intent. Nevertheless, I much enjoyed all that I could understand. I shall likely remain mystified about Mr. Johnston’s mysterious inner life.

    All best,

    Warren

  3. Unquestionably believe that which you stated. Your favorite reason appeared to be on the net the simplest thing to be aware of. I say to you, I definitely get annoyed while people think about worries that they just do not know about. You managed to hit the nail upon the top and also defined out the whole thing without having side-effects , people can take a signal. Will probably be back to get more. Thanks

  4. Danny Williams

    Affairs of the heart are not to be trifled with.

  5. A most sobering story in many ways. Thank you for taking the great amount of time needed to assemble it.

  6. Eileen Pruette

    My son survived a cardiac arrest three years ago at age 19, due to the external defibrillator which restarted his heart and the hypothermia protocol which allowed him to be released from the hospital without apparent cardiac or neurological damage just 6 days after he had collapsed while sitting in English class. The event was diagnosed as idiopathic. That was not a very comforting diagnosis (what could I do to prevent another with that diagnosis!) so I pressed the EPs on the possible association between the arrest and my son’s anxiety issues, which had beset him from a very young age. Not much credence was given to the issue, but an ICD was implanted “just in case”. Less than a year later, it was called into action. Then again a month later. He was eventually diagnosed with Brugada Syndrome, We found he had a defect in SCN5A, specifically ARG 105 TRP, which had only been previously associated in the literature with 1 other cardiac arrest. The hunt began for others at risk in the family who might not be so lucky to suffer their cardiac arrest in a room with 30 others, at least one of whom had the presence of mind to yank an AED from the wall. Much to my surprise, we found the errant gene in my family. As the family historian, I had traced our family back to the mid 1800’s when my forefathers had survived crossing the oceans in steerage class to escape poverty in Ireland. They were a hearty bunch – no premature deaths. I myself had 8 siblings, all living, who had produced 22 grandchildren, all living, none ever having had a cardiac event of any kind. My younger son was given an ICD as his ECG showed the Brugada cove and the very same intraventricular conduction delay of his older brother. That was enough to convince me. Then my brother got an ICD, then his daughter. So far, none of them have been even charged up in response to an arrhythmia (thank heavens!). So I have gone back to wondering what had set my older son apart from what was presumably the many others in my large family who have had the gene, but never a cardiac event. In my mind, I had already settled upon the anxiety issues and have made sure he has access to a good psychiatrist. Before I read your blog, I thought I was just waiting for science to catch up with my conclusion. I see now you already figured it out – just like you figured out that machine that brought my son back to life. Thanks for both.

  7. Pingback: Sudden Cardiac Death: Resuscitation or Resurrection? (Essay 23) « calvinjgarci

  8. Pingback: Sudden Cardiac Death: Resuscitation or Resurrection? (Essay 23) | deandouglers

  9. Pingback: Sudden Cardiac Death: Resuscitation or Resurrection? (Essay 23) « maryzgarnette

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