European Journal of Echocardiography 2006 7(4):326-329; doi:10.1016/j.euje.2005.06.006
Copyright © 2005, The European Society of Cardiology
Coronary artery–left ventricular fistulae associated with apical hypertrophic cardiomyopathy
Omer Alyan,
Ozcan Ozeke* and
Zehra Golbasi
Yuksek Ihtisas Hospital, Department of Cardiology, Ankara, Turkey
Received 25 January 2005; received in revised form 15 May 2005; accepted after revision 1 June 2005.
* Corresponding author. Turkiye Yuksek Ihtisas Hastanesi, Kardiyoloji Klini
i, Sihhiye, 06100 Ankara, Turkey. Tel.: +90 505 383 67 73. ozcanozeke{at}hotmail.com
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Abstract
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We describe an unusual case of coronary artery–left ventricular
fistulae associated with apical hypertrophic cardiomyopathy
in a 63-year-old man who had a 2-year history of angina pectoris
without significant coronary atherosclerosis. It is important
to recognize this anomaly as it may be the source of angina
in patients without angiographic evidence of major atherosclerotic
coronary artery disease.
Keywords: Coronary artery–left ventricular fistulae; Coronary steal; Apical hypertrophic cardiomyopathy
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Case report
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A 63-year-old man was admitted to our hospital for a 2-year
history of effort angina. He had none of the classical risk
factors for coronary artery disease (CAD) and was not taking
any prescribed medications. On physical examination, the patient
appeared generally well. His blood pressure was 140/80mmHg and
his pulse was 75beats/min. General physical examination showed
no abnormalities except for a 2/6 systolic murmur at apex. Electrocardiography
revealed a normal sinus rhythm, left ventricular hypertrophy
and symmetrical and deep T wave inversions in leads V1–V5.
Laboratory findings were within normal limits and chest radiography
revealed mild cardiomegaly. Given the clinical suspicion of
CAD, he underwent cardiac catheterization, which revealed no
significant CAD, but there was a stream of dye into the left
ventricle (LV) via a tiny plexus of intramural vessels, from
the dilated and tortuous left anterior descending artery (LAD)
during systole and diastole (
Fig. 1). Two-dimensional transthoracic
echocardiography and left ventriculography showed typical apical
hypertrophic cardiomyopathy (ACM) (
Fig. 2). The ratio of apical
to basal anterior and posterior wall thickness was found to
be greater than 1.5 on M-mode scanning. There was no intraventricular
gradient at rest and with Valsalva maneuver. Color-coded Doppler
echocardiography in the apical region of the LV showed color
flow signals between the epicardium and the LV chamber, demonstrating
penetrating intramyocardial coronary arteries arising from the
LAD (
Fig. 3A). The spectral pulsed Doppler echocardiography
at the apex revealed the exclusively diastolic flow velocity
spectrum (
Fig. 3B). The patient's symptoms improved with beta
blocker therapy and he was discharged with medical therapy.
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Discussion
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ACM, a variant of hypertrophic cardiomyopathy, is rare in the
west but more common in oriental people, especially in the Japanese.
1,2 The characteristic spade-like appearance on left ventriculography
has been used to confirm the diagnosis of this condition; transthoracic
echocardiography can also be useful in this regard.
1 However,
since apical artifacts may obscure the typical appearance during
echocardiography, this type of cardiomyopathy has occasionally
been misdiagnosed as isolated non-compaction cardiomyopathy,
apical LV thrombus or tumors.
2,3
Coronary artery fistulae between a coronary artery and a cardiac chamber is a rare condition and is found in approximately 0.2% of patients undergoing cardiac catheterization.4 Approximately 20% of patients with coronary artery fistulae have other cardiac anomalies, most frequently aortic and pulmonary atresia and patent ductus arteriosus. However, association of ACM and coronary artery–LV fistulae has been rarely reported.5–10 It is not clear whether the apical hypertrophy is a reactive change in chronic volume overload of LV through the coronary artery to the LV shunt or whether it results in multiple coronary microfistulae, possibly due to disarray of myocardial cells. Morphological studies suggest a partial persistence of embryonic myocardial sinusoids that arise from endothelial protrusions into the intertrabecular spaces. Fetal regression of this structure results in the formation of the Thebesian vessels of the adult heart. Thus, interference with developmental changes might produce an abnormally prominent Thebesian system with morphological appearance of multiple coronary microfistulae.11
The clinical diagnosis of coronary artery–LV fistulae is difficult because clinical presentation, laboratory and ECG manifestations are non-specific. Most patients with coronary artery fistulae, as in our patient, present with typical angina pectoris without CAD. Both coronary artery fistulae and ACM can produce myocardial ischemia and angina, and their association could aggravate the ischemia. The main mechanism of myocardial ischemia seems to be related to the coronary steal phenomenon.12–14
The treatment of coronary artery–LV fistulae is essentially medical; conservative management with continued follow-up of these patients appears to be appropriate. Surgical treatment is exceptional and must be considered in only severe forms with refractory medical treatment. In the presence of symptoms of congestive heart failure, significant left-to-right shunt and arrhythmias, other major cardiac lesions, concomitant CAD, elective closure of coronary fistulae are generally accepted.15 In our patient the symptoms were relatively controlled with metoprolol and no surgical treatment was planned.
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References
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