Beware of Long QT Syndrome in the ED: How Long Will You Be Liable?

The legal concept of proximate cause and foreseeable consequences

By Melanie Heniff, MD, FAAEM, FAAP, Assistant Professor of Clinical Emergency Medicine, Department of Emergency Medicine, Indiana University School of Medicine; and Gregory P. Moore, MD, JD, Attending Physician Kaiser Permanente Sacramento, Volunteer Clinical Faculty UC- Davis Emergency Medicine Residency.

The authors appreciate the contributions of Malia J. Moore, UC-Santa Cruz, in the preparation of this manuscript.

Legal Concept: Proximate Cause and Foreseeable Consequences

How can a misread on an EKG years prior, which led to no immediate negative outcome, be held up at a distant time in the future as malpractice? It doesn't seem right to the practicing ED physician. One needs to be aware that two of the key concepts in establishing negligence in general, and malpractice specifically, are proximate cause and foreseeability.

Proximate cause can be confusing. The governing rules, and how they work, are sometimes uncertain. The topic is controversial and the subject of numerous articles in the legal literature. Proximate cause may be thought of as a legal policy that states that even when someone has behaved negligently, they should not be responsible for all of the consequences, no matter how unlikely or far-reaching. The plaintiff must show that the mistake or negligence was the actual "cause in fact" of the injury.

In deciding what is proximate cause, there are two conflicting views: direct causation and foreseeability. In the medical arena, direct causation would impose liability for any harm that directly resulted from the provider's negligence no matter how unforeseeable or unlikely it may have been at the time the provider rendered care. As long as the injury flowed directly from the negligent act, the provider is liable. If some other intervening cause for the injury occurred, it would break the chain of direct causation. Even though there was a prior negligent act, the injury was not directly caused by it.

Foreseeability is the view that more often is utilized to determine proximate cause. This concept advocates that the provider is only liable for negligent acts that were reasonably foreseeable at the time that they acted. Two classic legal cases illustrate how foreseeability is determined in the courts. It is interesting that "non-medical" legal cases determine how malpractice cases are evaluated.

Palsgraf v. Long Island R.R. Co. This is one of the most famous cases,1 in which a man running to catch a train was about to fall as he rushed to board. A train employee pushed him from behind to help him make the train. This push dislodged a package from the man's arms which contained fireworks that exploded. The explosion caused something to fall at the other end of the platform, injuring another bystander.

The bystander then sued the train company because their employee had pushed the passenger and caused the package to fall, which then caused the explosion that caused the item to fall on them. Even though it was clearly negligent for the employee to push the passenger, the court ruled that this was not a foreseeable event and that the defendant was not liable. While it was foreseeable that a pushed passenger could be hurt by the negligence; it couldn't be foreseeable that a pushed passenger would have a package of explosives in his arms that would explode and dislodge something far away. This verdict may have been different if a "direct cause" viewpoint had been used.

Marshall v. Nugent. Another classic legal case on foreseeability comes up with a different ruling. In Marshall v. Nugent,2 a man was a passenger in a car driving up a snowy hill. An oil truck coming down the hill made a wide turn and forced the car off of the road. The passenger was not hurt. The passenger then began to walk up the hill on the side of the road to summon help. Another car, coming down the hill, swerved to avoid the oil truck in the road, and hit the passenger, injuring him. The passenger sued the oil truck driver even though he had not directly injured the passenger in the original accident. The court ruled that the liability did not end with the initial episode, it was foreseeable that having the truck in the road could lead to further accidents. Often it is the jury who must decide what is foreseeable. This leads to outcomes like the long QT syndrome cases that will be mentioned later in the article and the one noted below.

Estate of Jordan Carlson v. Community Medical Center. A 3-year-old had a splenectomy for which he received a single dose of pneumococcal vaccine, because asplenics have increased risk of fatal pneumococcal infection. On Nov. 10, 1996, the child, now a 12-year old presented with a 103.6 °F fever and secondary bacterial infections. Despite the 1996 Center's for Disease Control and Prevention (CDC) recommendation that asplenic patients be reconsidered for revaccination, the physician assistant did not verify the boy's immunization status and left that portion of his chart blank. He was not seen by a physician on this visit. The child recovered fully from the episode.

The decedent returned to the hospital on Sept. 19, 2000, with a fever and disorientation; he died of pneumococcal sepsis shortly later. The plaintiff claimed that the physician assistant's failure to identify vaccination status and administer an immunization during the boy's 1996 hospital visit played a significant part in his death. The defense contended that the immunization was irrelevant to the treatment the boy received; his symptoms were treated, and he recovered from them after that visit. The plaintiff was awarded $1,365,092 in damages by a jury.3

Long QT Syndrome: What is it and how is it identified?

Long QT syndrome (LQTS) is an electrophysiologic disorder in which ventricular repolarization is delayed. This disorder can be either hereditary or can be acquired, as occurs in the case of electrolyte abnormalities or medications that can cause the QT interval to be prolonged. LQTS, in its congenital form, has an estimated incidence of 1 per 10,000 to 1 per 15,000 and is thought to be the cause of 3000-4000 cases of sudden death annually in the United States.4 The LQTS is manifested as a prolonged QT interval of greater than 440 milliseconds in men and greater than 460 milliseconds in women. QT interval prolongation, in the absence of structural heart disease, electrolyte abnormalities, or medications known to prolong the QT interval, suggests congenital LQTS. Patients with this syndrome may present with syncopal or near-syncopal events that may be mistaken for seizures. Milder presentations may include palpitations or lightheadedness. These events most often are triggered by emotional stress, vigorous physical activity, or even a startle response to a loud noise or frightening experience;5 however, sudden death is the only event in 30-40% of patients. The clinical significance of this QT abnormality is that it can be associated with the development of polymorphic ventricular tachycardia, also known as torsades de pointes.6 During this dysrhythmia the cardiac output is impaired and although the event may be self-limited, it can degenerate into ventricular fibrillation and sudden death.

In any patient who presents with a history of syncope or near-syncope, the possibility of LQTS should be considered and an EKG should be obtained. Suspicion for this disorder particularly should be raised in any patient with a family history of syncope, seizure, SIDS (sudden infant death syndrome), or sudden death.7 While symptomatic congenital LQTS most often presents in patients ages 9-15, acquired LQTS usually occurs in the fifth or sixth decades of life.4 Acquired LQTS may be caused by a variety of medications, including antiarrhythmics, antipsychotics, antifungals, antimicrobials, organophosphate insecticides, and tricyclic antidepressants.8,9 (See Table 1.) Electrolyte abnormalities that can cause LQTS include hyperkalemia, hypokalemia, and hypocalcemia. In the setting of hypokalemia, the QT interval appears to be prolonged even though the QT interval is actually normal. This abnormally prolonged appearance is due to a fusion of the T-wave with a U-wave.10

Cases of QT Prolongation Patients not Immediately Identified

Case of "heat stroke." A 15-year-old female was playing softball in the summer. While rounding the bases during a game in 1993, she experienced a seizure-like episode. A neurologist was consulted and diagnosed "heat stroke." A year later she began complaining of headaches, nauseas, and dizziness, and a year after that she had several near fainting spells. She went to a Kaiser ED in June 1995 but had no work-up or diagnosis. On Sept. 22, 1995, she was seen at the same hospital and her mother demanded further evaluation and testing. An EKG was done that revealed QT prolongation, but this was not mentioned to the mother and no treatment was done. On June 30, 1997, as she was rounding the bases in a baseball game, she felt a "seizure" coming on so she laid down on the ground. She then lost consciousness and stopped breathing. She died. A suit was filed for failure to diagnose and treat prolonged QT syndrome. The case was settled for $225,000.11

Case with patient history of seizures and dizziness. A 22-year-old mother of two presented to the ED of Brooke Army Medical Center. She reported a history of seizures and dizziness and a frightening episode the day before in which she dropped to her knees. She was evaluated by a medical student and a resident who felt it was unlikely she had experienced a seizure since there had been no postictal period.

No EKG was generated. Her pulse was slow and she was released with a diagnosis of vasovagal syndrome. Eighteen days later, she was transported to the same ED by EMS.

During this visit, the rhythm strip clearly suggested prolonged QT syndrome. A resident again doubted a seizure and an EKG was not ordered. She was released. Over the next 24 hours she experienced a "strange" heartbeat and overwhelming fear. She said she was afraid they would "let me die" if she returned to the same facility. She was brought to Northeast Methodist Hospital, where she was placed in a quiet room. The ED physician prescribed a tranquilizer and discharged her home based on the nursing assessment of anxiety. The next day she was found unresponsive, without pulse or respiration. Her father performed CPR until EMS arrived.

An EKG was done and revealed prolonged QT syndrome. She was resuscitated but survived with severe brain damage due to anoxia. She required a long-term care facility.

Family members were then tested, and all have prolonged QT syndrome. They have subsequently been treated without complications. Her family sued the hospital. A jury returned a verdict of $16,662,154.12

Case of apnea and posturing. A 6-year-old boy suffered three episodes of apnea and posturing of his right arm at 1 a.m. He was taken by his parents to the ED where he was seen by a physician. He was alert and appeared normal upon arrival. A pediatrician was consulted, and the patient was admitted. An EKG was ordered the next day; it was normal. He was discharged with a diagnosis of questionable seizure. Two months later, the child had a similar episode but suffered cardiac arrest and suffered severe brain damage. He was hospitalized and diagnosed with long QT syndrome and died several days later of brain damage and dysrhythmias. The administrator of the estate then sued, claiming a failure to diagnose a congenital syndrome that would have led to transfer to an appropriate facility and proper and life-saving treatment.13

The defendants claimed that the child's seizure led to a cardiac arrest and that the arrest led to a resultant acquired long QT syndrome. Thus, an EKG on the initial visit would not have revealed the condition. The jury awarded $885,000.13

What is the ED treatment and disposition of a patient presenting with symptomatic LQTS?

QT interval prolongation alone does not necessarily predict risk, and risk stratification should take into consideration the context of the clinical presentation and family history.

In a patient presenting with an episode of torsades de pointes or polymorphic ventricular tachycardia, immediate intervention should consist of IV magnesium (25-50 mg/kg IV, maximum 2 g). Beta blocker therapy also may be used acutely to suppress catecholamines, thereby preventing further dysrhythmia. Use of beta blockers is effective for ongoing suppressive treatment of LQTS and has been shown to substantially decrease mortality in this population of patients. In some patients, an implantable cardioverter defibrillator may become necessary.14 Cardiology consultation should be obtained and admission should be arranged in patients with symptomatic LQTS, especially in the scenario of ongoing symptoms and cardiovascular compromise. Outpatient follow-up with cardiology is acceptable in stable, asymptomatic patients with LQTS. Potentially contributing medications should be stopped, and electrolyte abnormalities should be corrected. Individuals with this diagnosis should be restricted from competitive, but not necessarily from recreational sports. They also should be instructed regarding avoidance of precipitating events including emotional stress, potentially startling/ frightening situations, and dehydration. If a patient is diagnosed with congenital LQTS, family members should be advised to have EKGs to screen for this disorder.15

How is LQTS diagnosed?

Computerized EKG readings automatically calculate QT and QTc intervals (adjusting for heart rate since the QT interval is inversely related to the QT interval length). However, the most accurate method consists of measuring three consecutive QT and RR intervals, taking an average, then calculating the QT interval using the Bazett formula QTc = QT/ÖRR.4 Unfortunately, 6-12% of cases of individuals with congenital LQTS may present with a normal QTc.6

Caveats in LQTS

1. Consider dysrhythmias and conduction problems in patients with syncope or seizures.

2. Get an EKG on all patients with syncope and consider it in patients with "seizures." They may not be actual seizures, and pre-existing seizure patients may have a conduction abnormality as a result of medication.

3. When evaluating an EKG, be sure to focus on the QT interval.

4. Beware of the diagnosis of anxiety ... patients get anxious when they are dying. This is a common complaint in undiagnosed dysrhythmias, cardiac ischemia, and pulmonary embolus in young patients and in sepsis in the elderly.

5. Realize that you may be medicolegally responsible for an unapparent mistake, far into the future, until it becomes obvious.

References

1. Palsgraf v. Long Island R.R. Co., 62 N.E. 99 (N.Y. 1928).

2. Marshall v. Nugent, 222F.2d 604 (1st Cir. 1955).

3. Estate of Jordan Carlson v. Community Medical Center, Missoula County (MT) District Court, Case No. DV-03-621. Medical Malpractice Verdicts, Settlements & Experts 2005;21(11):12.

4. Doniger SJ, Sharieff GQ. Pediatric dysrhythmias. Pediatr Clin North Am 2006;53:85-105.

5. Meyer JS, Mehdirad A, Salem BI, et al. Sudden arrhythmia death syndrome: importance of the long QT syndrome. Am Fam Physician 2003;68:483-488.

6. Dovgalyuk J, Holstege C, Mattu A, et al. The electrocardiogram in the patient with syncope. Am J Emerg Med 2007;25:688-701.

7. Huff JS, Decker WW, Quinn JV, et al. Clinical policy: critical issues in the evaluation and management of adult patients presenting to the emergency department with syncope. Ann Emerg Med 2007;49:431-444.

8. Ott P, Marcus FI. Electrocardiographic markers of sudden death. Cardiol Clin 2006;24:453-469.

9. Prybys KM. Deadly drug interactions in emergency medicine. Emerg Med Clin North Am 2004;22:845-863.

10. Mattu A, Brady W. ECGs for the Emergency Physician. London: BMJ Books; 2003.

11. Estate of Christina Garavito, Janine Matelko and Paul Garavito v. Kaiser Foundation Health Plan, Inc. Kaiser Foundation Hospitals and Southern California Permanente Medical Group. Medical Malpractice Verdicts, Settlements & Experts 2000;16(4):12.

12. Bowman v. U.S.A., Methodist Healthcare Systems of San Antonio, Ltd. d/b/a Northeast Methodist Hospital and Rene B. Lopez MD. U.S. District Court (TX) No. SA-01-CA-307-0G.

13. Rebecca Streets, Administrator for the Estate of Daniel Mullen v. Robert Goff, D.O., Ashtabula Clinic, Ashtabula County Medical Center, Nafisa Kondru, M.D., Kade Raghupathy, M.D. and Pediatric Associates, Inc., Cuyahoga County (OH) Court of Common Pleas, Case No. CV02467983.

14. Al-Khatib SM, Sanders GD, Bigger JT, et al. Preventing tomorrow's sudden cardiac death today: part I: Current data on risk stratification for sudden cardiac death. Am Heart J 2007;153: 941-950.

15. Link MS, Estes NA. How to manage athletes with syncope. Cardiol Clin 2007;25:456-466.