Full text of DOI:10.1080/10903120190939850

Prehospital Emergency Care
ISSN: 1090-3127 (Print) 1545-0066 (Online) Journal homepage: http://www.tandfonline.com/loi/ipec20
A N EAR-FATAL C ASE OF L ONG QT S YNDROME IN
A T EENAGED M ALE
Ryan A. Jones & Robert A. Swor
To cite this article: Ryan A. Jones & Robert A. Swor (2001) A N EAR-FATAL C ASE OF L ONG
QT S YNDROME IN A T EENAGED M ALE, Prehospital Emergency Care, 5:3, 296-299, DOI:
10.1080/10903120190939850
To link to this article: http://dx.doi.org/10.1080/10903120190939850
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Date: 15 March 2016, At: 21:52

A NEAR-FATAL CASE OF LONG QT SYNDROME IN A TEENAGED MALE
Ryan A. Jones, MD, Robert A. Swor, DO
attention deficit disorder and intermittent asthma. He was
on summer “holiday” from his Ritalin and used only his
CASE REPORT
The patient was a 15-year-old male from Texas who was vis-
iting his grandparents in Michigan for one week. The young
man awoke one morning in his usual state of health, ate
breakfast, spoke with his grandparents, and reportedly went
back to bed. Approximately two hours later, the grandfather
walked past his room and overheard abnormally loud,
sonorous breathing. As he approached, he noted the patient
had obviously experienced a loss of bladder and bowel func-
tion. He then witnessed a cessation of spontaneous breath-
ing. The grandfather immediately instructed his wife to call
911. The patient’s grandfather then performed two chest
compressions followed by approximately 2 minutes of
mouth-to-mouth ventilation without subsequent chest com-
pressions.
According to the emergency medical services (EMS) run
sheet, a call was received at 9:30 AM. The on-scene arrival
time was 9:32 AM. On arrival the EMS personnel found a
15-year-old male in bed with a palpable pulse and sponta-
neous breathing. He was noted to be awake, but not appro-
priately responsive. His vital signs included a heart rate of
92 beats/min, blood pressure of 122/82 mm Hg, and respi-
ratory rate of 16 breaths/min. Blood glucose and pulse
oximetry were quickly confirmed without a need for inter-
vention. Intravenous access was established and supple-
mental oxygen was provided by nasal cannula. Within 10
minutes the patient became more alert and began to respond
appropriately to questions. Naloxone was not deemed nec-
essary. He was then taken via stretcher to the ambulance.
During transport the emergency medical technician–para-
medic (EMT-P) monitoring the patient documented the
patient to be awake, but tired. Astutely, the EMT-P also
noted “an unusual appearance on the rhythm strip.” The
remainder of the transport was uneventful. The patient and
the EMS run summary were then turned over to the emer-
gency department physician.
albuterol as needed. He denied any illicit drug use. The fam-
ily history included a grandfather who died at 42 years of
age of sudden death of unclear cause. The physical exam
demonstrated a well-developed male without any significant
abnormal physical findings. The lung sounds were clear and
equal and the cardiac auscultation revealed a regular rate
and rhythm without murmur. The neurological exam was
completely intact without focal findings. Further workup
included serum evaluation of electrolytes, renal function,
glucose, magnesium, calcium, and comprehensive serum
drug screen. Radiological evaluation included a chest x-ray,
and computed tomography (CT) scan of the head. All the
above were found to be normal. A 12-lead electrocardiogram
(ECG) (Fig. 1) demonstrated a normal sinus rhythm, a QT
interval of 528 milliseconds, and anteroseptal T-wave
inversion. A repeat ECG showed a persistent prolonged
QTc interval of 540 milliseconds. A cardiology consultation
was obtained. With high probability it was felt the primary
event was a malignant ventricular arrhythmia predisposed
by a prolonged QT interval.
DISCUSSION
Unexplained syncope is a common presentation of
EMS patients. Often these patients do not have an
identifiable cause of the event, especially if the patient
is a child, an adolescent, or a young adult. As a result,
the young often may be dismissed as having psy-
chogenic or functional syncope. However, our under-
standing of syncope is changing as there is an ever-
increasing body of literature on syncope and sudden
cardiac death in the young.1–4 This most likely is a
reflection of improved rate of diagnosis rather than
increasing incidence. Table 1 lists other etiologies of
syncope and sudden cardiac death in the young.
The case of this young man is unusual in that it
identifies a patient without a significant medical his-
tory who is ultimately treated as having a near-fatal
dysrhythmia. His presentation is disturbing in that he
was found unresponsive and incontinent, symptoms
usually not found in typical low-acuity “syncopal”
events. Additionally, the patient’s grandfather wit-
nessed a respiratory arrest. The patient received
bystander cardiopulmonary resuscitation (CPR) by
his grandfather, which may have been lifesaving or
actually may have been unnecessary. There are
reports of patients in the literature who are “CPR only
survivors,” with some debate whether these were
events that indeed necessitated the provision of CPR.5
In the emergency department the patient’s only com-
plaint was a mild temporal headache. He had no significant
recollection of the events leading to the apparent respirato-
ry arrest. The patient denied a previous history of seizures,
syncope, or cardiac arrest. His medical problems included
Received October 25, 2000, from the Department of Emergency
Medicine, William Beaumont Hospital (RAJ, RAS), Royal Oak,
Michigan. Revision received February 22, 2001; accepted for publi-
cation February 22, 2001.
Address correspondence and reprint requests to: Robert A. Swor,
DO, Department of Emergency Medicine, William Beaumont
Hospital, 3601 West 13 Mile Road, Royal Oak, MI 48073. e-mail:
<raswor@aol.com>.
296

Jones and Swor NEAR-FATAL CASE OF LONG QT SYNDROME
297
TABLE 1. Etiologies of Syncope and Sudden
Cardiac Death in the Young Patient1–4
Other than the history of the initial presentation and
the brief period of confusion immediately following
the event, there was little clinical evidence that pro-
vided a clear diagnosis.
Hypertrophic cardiomyopathy
Acute myocarditis
Anomalous coronary arteries
Single coronary artery
The patient had no previous history of seizure dis-
order. He was of normal body habitus, making the
suggestion of valvular disease or aortic dissection due
to Marfan’s syndrome or other congenital structural
abnormalities unlikely. He was white and not particu-
larly muscular so that asymmetric septal hypertrophy
(ASH) was less likely. This event occurred at rest,
which is also not consistent with ASH. A subsequent
echocardiogram performed was normal, thus virtual-
ly excluding this as an etiology. There was no histori-
cal information, physical evidence, or subsequent test-
ing to suggest this to be a toxin or drug-induced event.
Previous medical history of attention deficit disor-
der and asthma was similarly not helpful in establish-
ing a diagnosis. The patient previously took methyl-
phenidate (Ritalin), but he had been on “holiday” for
more than a month from its use at the time of the
event. Patients with asthma do have a demonstrable
incidence of sudden death, but there was no history
that the patient was symptomatic and he denied using
his inhaler at the time of the event. However, in
patients with long QT syndrome, asthma has been
identified as an independent predictor of an increased
risk of adverse cardiac events.6 The family history
obtained later in the patient’s hospitalization identi-
fied a paternal grandfather who died suddenly at the
Tunneled coronary arteries
Conduction system abnormalities
Wolff-Parkinson-White syndrome
Long QT syndrome
Arrhythmogenic right ventricular dysplasia
Congenital valvular disease
Mitral valve prolapse
Aortic stenosis
Coarctation of the aorta
Marfan’s syndrome
Idiopathic left ventricular hypertrophy
Pulmonary vascular obstruction
Commotio cordis
Toxins
Metabolic disorders
age of 42. This may or may not have relevance to this
case.
After a careful history and physical exam, the sole
initial clue to diagnosis was the paramedic’s cogent
observation of an “unusual appearance of the rhythm
strip.” The patient had an abnormal and considerably
prolonged QT interval on both the rhythm strip and
12-lead ECG. In this case the QTc (corrected QT inter-
val) of 540 milliseconds is a clear, although frequently
overlooked abnormality. A QTc of this duration is in
FIGURE 1. The updated ECG.

298
PREHOSPITAL EMERGENCY CARE JULY/SEPTEMBER 2001 VOLUME 5 / NUMBER 3
TABLE 2. Medications That Potentially
Prolong the QT Interval7,12,14
R-R interval suggests a QT interval prolongation.
Bazzet’s formula (the measured QT interval divided
by the square root of the R-R interval7,10) is used to
account for the effect that heart rate has on the QT
interval. In the symptomatic patient the QTc is typi-
cally greater than 440 milliseconds in males and 460
milliseconds in females. A normal QT interval, how-
ever, does not entirely exclude the diagnosis of long
QT syndrome. It should also be noted that the QT
interval varies with time of day, age, and sex.10,11
Other ECG findings associated with QT prolongation
include nonspecific T-wave abnormalities. T waves
are typically inverted, biphasic, wide, or notched.12
QT prolongation and nonspecific T-wave abnormali-
ties are subtle but if missed can lead to grave medical
repercussions.13
Long QT syndrome can be acquired or congenital.
The differential diagnosis of the acquired form is
broad, with the majority of cases thought to be sec-
ondary to a pharmacological source or metabolic
derangement. Various antiarrhythmics, antibiotics
(including antifungals), antihistamines, and antide-
pressants have been frequently implicated pharmaco-
logical agents associated with QT prolongation.14
Table 2 lists medications thought to prolong the QT
interval. This list is alarmingly large and ever grow-
ing. Common metabolic abnormalities leading to
arrhythmia secondary to QT prolongation include
hypokalemia, hypocalcemia, and hypomagnesemia.
Obviously, any underlying medical condition that
could precipitate a metabolic abnormality must be
carefully considered. The patient in this case had a his-
tory of taking potentially cardioactive medications
(methylphenidate and albuterol). These were not
used, however, in temporal association with this
event. The metabolic causes were proven absent with
simple serum electrolyte analysis. Additionally, this
patient did not have any underlying medical condi-
tion that would independently predispose him to a
malignant arrhythmia. An acquired form of long QT
syndrome was unlikely.
Antibiotics
Erythromycin
Trimethoprim and sulfamethoxazole
Pentamidine
Antifungals
Fluconazole
Itraconazole
Ketoconazole
Antiarrhythmics
Quinidine
Procainamide
Disopyramide
Sotalol
Antidepressants
Amitriptyline
Phenothiazine derivatives
Rispiridone
Haloperidol
Pimozide
Asthma/anesthetics
Epinephrine
Antihistamines
Terfenedine
Astemizole
Gastrointestinal
Cisapride
Lipid-lowering
Probucol
the 99th percentile for prolonged QT intervals. QT
prolongation in the setting of a near-fatal dysrhyth-
mia, syncopal episode, or seizure is now highly sug-
gestive of long QT syndrome.
Abnormal repolarization of the ventricular
myocardium, electrocardiographically represented by
QT prolongation, predisposes the patient to torsades
de pointes (polymorphic ventricular tachycardia in
the setting of QT prolongation). Although torsades de
pointes often terminates spontaneously, there is a sig-
nificant risk of degenerating into ventricular fibrilla-
tion. Initial treatment of ventricular fibrillation con-
sists of immediate defibrillation. Urgent treatment for
torsades, or its recurrence, involves correcting under-
lying electrolyte abnormalities and recognizing
reversible causes. Intravenous magnesium is consid-
ered first line in preventing recurrent episodes.
Transvenous overdrive pacing or pharmacological
intervention with isoproterenol can also be used to
prevent QT interval lengthening associated with
bradycardia.7–9
The two primary forms of inherited congenital long
QT syndrome include the rare Jervell-Lange-Nielson
syndrome and the much more common Romano-
Ward syndrome. Jervell-Lange-Nielson syndrome is
an autosomal recessive disorder characterized by neu-
rosensory hearing loss and a pathologically prolonged
QT interval.15 The incidence is only 1 to 6 per million
individuals.12 Individuals typically present very early
in life with multiple episodes of syncope, seizures, or
cardiac arrest. Normal hearing excluded this diagno-
sis in our patient.
In contrast, the Romano-Ward syndrome is an auto-
somal dominant disorder with a reported incidence as
high as 1 in 10,000 individuals. This roughly translates
into 3,000 to 4,000 deaths per year in the United
States.16 This syndrome may be an underestimated
Recognition of the abnormal QT interval is the first
step in preventing dysrhythmias. The general appear-
ance of the QT interval being greater than one-half the

Jones and Swor NEAR-FATAL CASE OF LONG QT SYNDROME
299
cause of syncope and sudden cardiac death in the
young. To date, six genotypes and multiple pheno-
types have been recognized8, 17–19 The information at
the genetic and molecular level has grown dramatical-
ly during the last decade with increased understand-
ing of the human genome and the availability of
genetic testing. Fundamentally, arrhythmias occur as
the result of a genetic abnormality of sodium and
potassium ion channel function leading to aberrant
repolarization that electrocardiographically manifests
as prolongation of the QT interval. Two-thirds of gene
carriers have some type of symptoms.16 Symptoms
include syncope, palpitations, seizures, or even sud-
den death. The most common is syncope. Patients
having seizures as the initial presenting symptom of
an aborted adverse cardiac event are also well
described.20–23 The typical age of initial presentation is
preteen to teenage years. Events are frequently associ-
ated with emotional stress, physical stress, or noxious
stimuli (such as a loud noise), although as in our case,
occurrence at rest is not unusual. Screening ECGs of
the parents, siblings, and offspring of patients with
congenital QT prolongation are highly recommended.
Given the prior family history of sudden death and
the absence of other causes for this patient’s ECG
abnormality, the evidence supported the diagnosis of
either an inherited or a sporadic form of congenital
long QT syndrome. Recognizing that there is not a cor-
rectable or reversible cause of a congenitally pro-
longed QT interval, treatment is mandatory as ten-
year mortality rates of untreated patients may
approach 50%.9 Current long-term therapeutic options
include beta-blocker therapy or cervical sympathecto-
my to avoid excessive sympathetic simulation, pace-
maker implantation to prevent bradycardia and sec-
ondary lengthening of the QT interval, and implanted
cardioverter–defibrillator (ICD) placement to abort
malignant arrhythmias.9 Individual treatment is large-
ly based on the patient’s estimated risk of future mor-
bidity and mortality. Treatment in the asymptomatic
or low-risk patient is controversial. In the high-risk
patient, treatment is essential. Suppressing symptoms
with beta-blocker therapy and directly aborting
adverse cardiac events with an ICD are the most com-
mon treatments offered. Having what appeared to be
an aborted cardiac arrest placed our patient at a high
risk of repeated episodes. The patient was started on
beta-blocker therapy and had an ICD implanted. At
one year there had been no adverse events or recur-
rence of symptoms reported.
child or adolescent should not be dismissed as trivial
without serious consideration of all potential diag-
noses. This case specifically demonstrates that a
review of all prehospital information is essential. Any
unusual circumstances, no matter the source or per-
ceived insignificance, may be the only source of mak-
ing a proper diagnosis. Final diagnosis in this case,
and in most cases of long QT syndrome, was made
based on the clinical information obtained on scene
and on initial ECG.
The authors thank Dr. J. C. Ryan for her helpful review of the man-
uscript.
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CONCLUSION
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Prolonged QT syndrome has many known and likely
a number of yet-to-be-discovered causes. This case
exemplifies the need for a thorough evaluation of all
patients with syncope. A significant event in a young