IDIOPATHIC HYPERTROPHIC
SUBAORTIC STENOSIS AND
ACUTE MYOCARDIAL INFARCTION:
AN UNCOMMON ASSOCIATION
Michael Bachik, MD, S.K. Agarwal, MD, FACA, FICA, and Jacob 1. Haft, MD, FACC
Newark, New Jersey.
Acute myocardial infarction is rarely observed
in patients with idiopathic hypertrophic sub-
aortic stenosis. If seen it is usually associated
with normal coronary arteries in young sub-
jects less than 45 years of age and significant
coronary artery disease in those patients
greater than 45 years. The case reported here
briefly reviews this uncommon association.
Idiopathic hypertrophic subaortic stenosis
(IHSS) is usually a cardiomyopathy of young
adults' characterized by asymmetric septal hyper-
trophy and systolic left ventricular outflow tract
obstruction.2 Clinical presentation often suggests
coronary artery or aortic valve disease, especially
in the elderly.3 Although ischemia may occur in
the older individual due to associated atheroscle-
rotic coronary disease,4 myocardial infarction in
the young patient is rare, with the majority having
normal coronary arteries.5 This report describes
a 20-year-old man with typical features of IHSS,
who developed an acute transmural myocardial
infarction.
Requests for reprints should be addressed to Dr. S.K.
Agarwal, St. Michael's Medical Center, 268 High Street,
Newark, NJ 07102.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 75, NO. 3, 1983
CASE REPORT
The patient was a 20-year-old black man admit-
ted to St. Michael's Medical Center with chest
pain of six hours duration. The pain was described
as "crushing" and associated with diaphoresis,
shortness of breath, palpitations, and nausea.
There was no previous history of angina. Five
years prior to admission, a diagnosis of IHSS had
been made by echocardiography. One year prior
to the admission, the patient had been admitted to
another hospital with a sudden onset of left-sided
hemiplegia and dysarthria following an episode of
chest pain. This had resolved completely in less
than 24 hours. Carotid arteriography had been
consistent with a right middle cerebral artery em-
bolism. A cardiac catheterization was also per-
formed revealing an intraventricular gradient of 55
mmHg consistent with IHSS. There was no gradi-
ent recorded across the aortic valve. The proximal
portions of the main coronary arteries were well
visualized and were free of atherosclerotic narrow-
ing. After an initial course of anticoagulation, the
patient was discharged with a prescription for
60 mg of propranolol hydrochloride per day. Two
weeks prior to this admission, the patient had
stopped taking the a-blocker; past medical history
was otherwise unremarkable. Social history in-
cluded smoking two packs of cigarettes and drink-
ing four to six beers per day. Family history was
305
IHSS AND ACUTE Ml
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Figure 1. Electrocardiogram. Note the elevated ST segments in leads 11, Ill and AVF. Frequent preventricular
contractions are also seen
remarkable-the patient's father died at age 31 of
possible heart attack.
Physical Examination
Physical examination upon arrival in the emer-
gency room of St. Michael's Medical Center re-
vealed a well-developed, well-nourished, athletic
black man complaining of chest tightness. Blood
pressure was 140/80, the pulse was 90, respiratory
rate was 16 per min. There was no jugular venous
distention at 90 degrees and no carotid bruit.
Carotid upstroke was brisk. Cardiac examination
revealed the heart to be regular with occasional
ectopic beats. S I was equal to S2. An S4 was pres-
ent and there was a double apical impulse on pal-
pation at the point of maximal impulse (PMI).
306 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 75, NO. 3, 1983
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There was a grade III/VI systolic ejection murmur
heard best at the lower sternal border and apex
which increased with the Valsalva maneuver. The
PMI was I cm lateral to the midclavicular line in
the fifth intercostal space. The abdomen revealed
no organomegaly and mild epigastric tenderness
was present, with no rebound or rigidity. The
extremities showed no cyanosis or clubbing and
the pulses were normal in the upper and lower
extremities.
The ECG (Figure 1) showed regular sinus
rhythm with frequent preventricular contractions
and ST elevation in leads 2, 3, and a Vf. On admis-
sion the laboratory data showed the following:
sodium was 141 mEq/L; potassium, 4.7 mEq/L;
chloride, 106 mEq/L; CO2, 26.5 mEq/L; blood
sugar was 114 mg/dL; creatinine, I mg/dL; BUN,
 IHSS AND ACUTE Ml

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Figure 2. Carotid pulse tracing (CPT) and apex cardiogram (ACG). Both re-
veal a midsystolic retraction (arrowheads). M-mode echocardiogram reveals
midsystolic aortic valve closure (arrowhead) as well as systolic anterior mo-
tion of the mitral valve (arrow). AOaorta; LA-left atrium; IVS-interven-
tricular septum; MV-mitral valve

12 mg/dL; hemoglobin, 14 g/dL; hematocrit, 39
percent; WBC, 7,500 ,uL with a normal differ-
ential. Prothrombin and partial thromboplastin
times were within normal limits. Chest roentgeno-
gram revealed minimal cardiomegaly, while a flat
plate of the abdomen was within normal limits. On
admission the creatine phosphokinase was 3,326 U
with MB (10 percent) markedly positive and the
SGOT and lactic dehydrogenase were elevated to
160 and 1500 U respectively. The enzymes gradually

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 75, NO. 3, 1983 307
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IHSS AND ACUTE Ml

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Figure 3. Cross sectional echocardiogram. Note thickened interventricular
septum (arrowheads). LA long axis view; SA-short axis view; Aaorta;
LA left atrium; LV-left ventricle

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Figure 4. Resting thallium cardiac scan. Note the inferoapical perfusion de-
fect (anterior projection)

decreased to normal levels over a period of one date showed marked apical inferior hypokinesis
week. Phonocardiogram, M-mode echo (Figure 2) with anterodyskinesis. A resting thallium 201 car-
and 2-D echocardiograms are illustrated (Figure diac scan was performed on the second hospital
3). day (Figure 4) and showed a large apical inferior
A 2-D echocardiogram performed at a later defect with septal hypertrophy. A technetium

308 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 75, NO. 3, 1983

pyrophosphate scan was performed on the fourth
hospital day and revealed areas of uptake corre-
sponding to the apical inferior and lateral wall. A
gated blood pool scan was performed a week later
and showed apical-inferior akinesis with an ante-
rior aneurysm and ejection fraction of 60 percent.
The left ventricle was enlarged with concentric
hypertrophy.
DISCUSSION
Myocardial infarction occasionally results from
nonatherosclerotic disease of the coronary arter-
ies. These pathologic processes include arteritis,
trauma, metabolic or proliferative diseases, em-
boli, congenital anomalies, and spasm.7 Rarely, it
may occur in the presence of normal extramural
coronary arteries.8
Although coronary artery disease is common in
patients with IHSS over the age of 459 develop-
ment of acute transmural infarction is uncommon.
Kossowsky in 1973 reported a 61-year-old man
with IHSS with an occluded left anterior descend-
ing coronary artery.'0 Maron described three pa-
tients over the age of 45 who were documented to
have acute myocardial infarction in the presence
of normal coronary arteries.1'
The occurrence of myocardial infarction in
young patients with IHSS is extremely rare. Of
seven patients noted by Maron, four cases were
below the age of 45, one was only 12 years old and
had minimal or no atherosclerotic involvement of
the extramural coronary arteries."I Nair and col-
leagues recently described a 19-year-old man with
IHSS and acute myocardial infarction who had
patent coronaries on arteriography.'2
The pathophysiology of myocardial infarction
in most patients with IHSS and normal coronary
arteries remains unclear. Several mechanisms
have been postulated: (1) demand-supply mis-
match of coronary blood," (2) thromboembo-
lism,'3 (3) constriction of intramural coronary ar-
teries by septal hypertrophy or obstruction of
extramural coronary arteries by muscular bridg-
ing, '4 and (4) coronary artery spasm.'5 No single
mechanism was implicated as being responsible in
this patient, although with a prior history of tran-
sient hemiplegia, thromboembolism is likely.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 75, NO. 3, 1983
IHSS AND ACUTE MI
Maron's experience suggests that patients with
IHSS and acute myocardial infarction usually
have progressive clinical deterioration, often re-
sulting in early death. Our patient has been symp-
tom free over one year of follow-up.
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309