Broken Heart Syndrome: The Octopus Trap

“Doctoring her seemed to her as absurd as putting together the pieces of a broken vase. Her heart was broken. Why would they try to cure her with pills and powders?”  Leo Tolstoy, writing about Kitty’s heartbreak over Vronsky in Anna Karenina

 

Sometimes people say that a spouse who dies unexpectedly within hours to weeks after the partner’s death has “died of a broken heart,” though a variety of different medical conditions are responsible for the increased death rate among grieving partners, who are often elderly. In 1990 a paper appeared in the Japanese medical literature that described a peculiar heart problem, documented by modern technology, that the popular press seized upon as a possible explanation for the correlation between grief or fright or other emotional stress and sudden, unexpected death. The cardiomyopathy the authors described was an abnormality in the heart muscle of the left ventricle, the chamber of the heart that pumps blood out to the body. That part of the heart acted as if it had been “stunned” into inactivity and caused pain and other symptoms commonly associated with heart attacks, but the patients did not have any coronary artery disease.  These facts seemed fit neatly into the concept of a “broken heart.”

Why an octopus trap?

The ventriculograms, or dye studies, of the hearts of the Japanese patients described in the 1990 paper showed peculiarly dilated left ventricles, ballooned at their tips so that they resembled octopus traps – narrow-necked, flask-shaped contraptions that are easy for the tentacled animals to enter but hard to escape. In the Japanese language an octopus trap is a takot-subo and by the mid-2000s the name Takotsubo cardiomyopathy, or TCM, was widespread and many more cases had been described. Risk factors for the stress-induced cardiomyopathy were both physical and mental and included stays in ICUs, near drownings, major physical injuries, bad medical or financial news, legal problems and natural disasters, and, of course, unexpected death of a loved one. Cases have also been attributed to cocaine and methamphetamine use, as well as to exercise stress testing. These patients who acted as if they had had a heart attack were most often women and they had no history of heart problems prior to the events that hospitalized them.

Who is at risk? What are the symptoms?

Takotsubo syndrome is not common, but also not rare. It accounts for 1-2% people who have symptoms initially thought to be caused by regular coronary artery disease. In women, some people estimate that as many as 5% of heart attacks are actually TCM.  Most TCM patients are Asians or Caucasian, over 90% are post-menopausal women and most cases come to attention because of heart attack-like symptoms such as acute chest pain and shortness of breath.  But unusual presentations also occur as a result of the effects of the poor heart muscle function. When it’s pump action fails, the heart sends hormonal signals that affect water and salt balance in the body.  Fluid retention occurs in some people. Low sodium levels cause symptoms of profound fatigue in others. Clots may form in the poorly contracting ventricle, break loose and cause strokes. Lethal complications such as ventricular fibrillation and actual rupture of the impaired ventricle are very rare, but have occurred.

What’s the cause?

Diagnosis of Takotsubo syndrome requires new abnormalities in the electrocardiogram, absence of coronary artery disease and no evidence of heart inflammation from an infection or autoimmune disease. While the enzyme markers for a heart attack may rise, they do so earlier and fall back to normal more quickly than they do in a routine heart attack. In addition, the muscle abnormalities in the left ventricle can’t be mapped to the territory supplied by one coronary artery as they can when a blockage is responsible for the damage. Doctors who make a TCM diagnosis must also make certain the patient does not have a tumor called a pheochromocytoma, which produces stress hormones.

Most patients recover completely

By now TCM is known to be transient, with supportive care leading to complete recovery within 1-2 months in over 95%of patients. Recurrence is extremely rare. However, the actual cause, or mechanism by which the transient heart damage occurs, remains unknown. A number of theories have been proposed and all of them have something to do with a temporary derangement in function of the cells of the inside layer of cells of the left ventricular chamber of the heart. In these cells normal energy production from fatty acids is halted. The area of the heart involved happens to have a high concentration of receptors for catecholamines (adrenaline like hormones), perhaps making it susceptible to overstimulation and damage by severe stress. The high preponderance of postmenopausal women in case reports suggests that perhaps sex hormones are somehow protective factors.

Do people really die from broken hearts?

But is the Takotsubo syndrome responsible for deaths that seem to come from emotionally broken hearts? The mortality rate in cases of Takotsubo syndrome that come to medical attention is low. Recovery rates are high. Broken heart deaths most often occur in older people who have multiple health problems which might play a role. For example, when singer/actress Debbie Fisher died as she was planning her daughter Carrie Fisher’s funeral this year, a NYT reporter speculated about the cause of death being the Takotsubo syndrome. But Debbie Fisher had suffered several strokes in recent years and had high blood pressure. Later stories attributed her death to a fatal stroke related to high blood pressure.

Grief and stress do raise the risks of dying for the bereaved, but the causes of death are many and varied and mostly related to longstanding health problems.  The pills and powders Kitty scorned for her broken heart in Anna Karenina have a place in the treatment of the many other problems that occur in the setting of grief, especially depression. While it is tempting to attribute sudden, unexpected deaths in emotionally stressed people to an odd and mysterious heart problem named after an octopus trap, science requires objectivity and evidence.  So far the evidence about sick hearts that resemble octopus traps suggests that, at least in the people in whom the diagnosis is made, death is a very rare outcome and complete recovery is the rule.

Torn Aortas: Saving Life Depends on Recognizing Symptoms

“There is no disease more conducive to clinical humility than aneurysm of the aorta.”   William Osler, 1849-1919

    John Ritter, star of television’s Three’s Company, died unexpectedly at age fifty-four from aortic dissection, a catastrophic event which starts as a small tear in the lining of the aorta, the largest blood vessel in the body. His tear, like most aortic dissections, occurred in the part of the aorta that exits the heart and ascends toward the head, but dissections can anywhere along the course of the aorta as it turns and then descends along the back wall of the chest and abdomen. Actor Alan Thicke’s recent death has also been attributed to an aortic tear. In neither case was the cause of death confirmed by autopsy, presumably because diagnosis was made clinically and by imaging studies once the men reached medical care. Diagnosis in both cases came too late for their lives to be saved. The first step in saving the life of someone with an aortic dissection is recognition of the symptoms. As Osler accurately stated, this is not necessarily easy. 

Upper Aortic Symptoms 
Symptoms of aortic tears vary according to the part of the aorta involved. In the upper aorta, as in Ritter’s case, the tear appears without warning. The first symptoms, such as severe chest pain, confusion, dizziness, nausea and vomiting, come from blood tunneling its way into the tear and under the aortic lining, separating it from the thick outer wall of the blood vessel. Heartbeat by heartbeat, the tunnel enlarges and a growing clot of blood extends around the inner circumference of the aorta and along its length, stretching from the ascending part of the thoracic aorta into the curved aortic arch branches where large arteries branch off and carry blood to the head and arms. 

The differences between dissection and heart attack symptoms

    Sadly, like John Ritter, almost 40 percent of people with upper aortic dissection who get to medical attention are not diagnosed in time for doctors to attempt surgical repair. Within forty-eight hours, half of them are dead. Diagnosis depends on recognition of subtle and qualitative characteristics of symptoms that differ from similar heart attack symptoms. Chest pain is severe from the beginning and sometimes described as ripping or tearing. Its most distinctive quality is sudden onset of maximally severe pain . Often people report a sense of impending doom. The pain may radiate up the neck or into the back, as it can in a heart attack. As the dissection progresses, clotting blood can block the openings to the aortic arch branches and even work its way backwards to damage the coronary arteries that nourish the heart. The aortic valve may be damaged and begin to leak. 

Symptoms From the Descending Aorta

     While the involvement of so many other structures in an upper aortic dissection can produce a host of symptoms that manifest themselves in the heart, the brain, the neck, the face, and the extremities, sometimes confusing physicians, descending aortic symptoms are more straightforward. Pain from dissection in the descending thoracic aorta bores through to the back. In the abdominal aortic segment dissection pain may be felt in the flank, lower back, or groin. Because the descending aorta is more tightly bound to surrounding structures, tears may be more confined and symptoms less severe. 

Dissections and Aneurysms

   Sometimes a dissection begins in an aortic wall already weakened enough to have ballooned out into an aneurysm, which is a distended spot in an artery wall. Ninety-five percent of aortic aneurysms are located in the abdominal aorta, and aortic dissections in the abdomen are often triggered by the prior development of an aneurysm. A tear in the wall of an aneurysm can cause the aorta to rupture completely causing internal bleeding, with mortality rates between 75 percent and 90 percent of aortic aneurysms Fortunately, aneurysms are often found incidentally on imaging tests for other problems, or as part of an investigation of vague abdominal or back pain or of a pulsating sensation in the lower abdomen allowing time for surgical repair of the damaged artery before rupture occurs. 

Who’s at Risk
    Most upper aortic dissections occur in people between the ages of forty and seventy, with men affected three times as often as women. In otherwise healthy and relatively young people like John Ritter, the tear begins because the aortic wall is weakened by genetic processes that are often poorly understood. Pregnancy and cocaine use are also risk factors below age 40. In older people and in smokers high blood pressure, atherosclerosis are responsible for the breakdown in the aortic lining, but even in these patients, dissections tend to run in families. 

     While smoking, hypertension, and atherosclerosis are risk factors in all types and locations of aortic disease, the abdominal aorta is particualry susceptible to their degenerative effects. Abdominal aortic disease is far more common than the forms of aortic disease that affect younger people and most aneurysms of the abdominal aorta reflect age-related (over age sixty) vascular degeneration. An estimated 5 percent of men over age sixty-five have some degree of degenerative abdominal aortic dilatation. 

The importance of family history
Assessment of a patient’s family history is very important in diagnosing aortic diseases like aortic dissection because genes control the proteins that make up the connective tissue of the thick aortic wall, and there can be a hereditary predisposition to dissection and aneurysm formation, particularly in younger people like John Ritter. One relatively common condition (1 in 5000 people), which can affect the connective tissue and lead to aortic dissection, is Marfan’s syndrome. The Olympic volleyball star Flo Hyman had Marfan’s syndrome, which accounted for her six feet five stature and long arms and fingers. She died at age thirty-two of aortic dissection. Dissections can also occur in people with congenital heart abnormalities, particularly those that affect the aortic valve.  

Treatment
Surgical repair of aortic aneurysms and dissections is a serious and complicated undertaking. Incidentally discovered abdominal aneurysms and aneurysms found by screening programs should be followed carefully with ultrasound or computerized tomography scans because the risk of rupture is correlated with the size of an aneurysm. Surgical repair is far less dangerous when done before dissection or rupture but carries risk enough to warrant waiting if the aneurysm is less than five centimeters in diameter. 

    Once a dissection has begun, the outcome of surgical repair rests heavily on the condition of the patient going into surgery, on the experience of the surgical team and hospital involved, and on the complexity of the procedure required. In recent years, radiologists and cardiologists have developed procedures to repair the inside of the aorta by deploying stents and grafts via catheters inserted though the arms or legs and guided by x-ray. Initially, these procedures were only used on patients who were too frail or sick to undergo the rigors of open surgery. Increasingly, though, these less invasive procedures are gaining favor and are even being used to repair dissections of the descending thoracic aorta, which have traditionally been treated by careful control of blood pressure. More research will have to be done to assess the long-term outcome of stent and graft treatments. 

Not even recent technological advances would have helped John Ritter, however, because the proper diagnosis was not made prior to his death. His family has since set up an educational foundation called the John Ritter Foundation for Aortic Health (http://johnritterfoundation.org/your-aortic-health/) with the goal of increasing knowledge and awareness about a disease which is still a very humbling clinical problem for the medical professio

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