CLINICAL CASE REPORTS and
as
Letters to the Editor will be published if suitable, space permits. They should not exceed 1,000 words (double in length, and may be subject to editing or abridgment.
spaced)
Bilateral Coronary-Artery-to-Pulmonary-Artery Fistula Coexistent with Apical Hypertrophic Cardiomyopathy— A Case Report Yau-Ting Tai, M.B., M.R.C.P., F.A.C.A. Ping-Ching Fong, M.B., M.R.C.P. and
Wing-Hing Chow, M.B., M.R.C.P.,
F.I.C.A.
HONG KONG
Abstract
sixty-four-year-old man presented with repolarization abnormalities on the electrocardiogram. Echocardiography and cardiac catheterization revealed that he had the rare combination of apical hypertrophic cardiomyopathy with bilateral coronary-artery-to-pulmonary-artery fistula. An exercise thallium scan was negative, suggesting that the marked electrocardiographic changes were most likely secondary to the apical myocardial hypertrophy, instead of to coronary-stealA
induced ischemia.
Introduction
Coronary arteriovenous fistulas, while a rare congenital anomaly, have been well characterized. 1,2 The association of an anomaly of the coronary vasculature with primary diseases of the myocardium is, however, rarely described.’ This report details the rare coexistence of bilateral coronary-artery-to-pulmonary-artery fistula with apical hypertrophic cardiomyopathy in a patient who was otherwise asymptomatic. The potential effect of such a combination of vascular and myocardial abnormalities is addressed. Case Report A sixty-four-year-old Chinese man was referred for evaluation of electrocardiographic abnormalities discovered on routine checkup. He had a five-year history of mild systemic hypertension (blood pressure on presentation 150-160/100 mm Hg), well controlled with a thiazide diuretic. There was no history of exercise intolerance, palpitation, syncope, or chest pain. Physical examination produced normal findings; in particular no murmur was heard. The electrocardiogram showed sinus rhythm with deep symmetrical inverted From the Division of
Cardiology, Department of Medicine, University
of
Hong Kong, Hong Kong
72
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
73
FIG. 1. Left ventriculogram in a right anterior oblique (30 degrees) projection, showing the &dquo;ace-ofspade&dquo; appearance at end-diastole (A) and the active ventricular contraction with apical obliteration during systole (B).
precordial leads V2 to V6 (maximum 9 mm). Two-dimensional echocardiography revealed marked hypertrophy of the left ventricular apex and the distal one third of the ventricular septum and the inferior wall, below the level of the papillary muscle. Left ventricular contraction was active with systolic apical obliteration. On cardiac catheterization, the left ventricular end-diastolic pressure was 12 mm Hg, and there was no intraventricular pressure gradient. Left ventriculography showed active left ventricular contraction with apical obliteration and an &dquo;ace-of-spade&dquo; appearance at end-diastole (Fig. 1). Coronary angiography revealed a dilated and smooth proximal left anterior descending artery, giving rise to multiple tortuous and plexiform vessels that coursed superiorly and drained into the main pulmonary artery (Fig. 2A). The proximal T
waves over
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
74
FIG. 2. Left (A) and right (B) coronary angiography in a right anterior oblique projection, showing the coronary-artery-to-pulmonary-
artery fistula (arrow).
right
coronary artery gave rise to two conal branches that drained into the same plexiform malformation and then into the main pulmonary artery (Fig. 2B). The rest of the
coronary anatomy was normal. There was insignificant oxygen step-up at the main pulmonary artery, and the calculated pulmonary-to-systemic flow ratio was less than 1.1. An exercise thallium scan revealed mild increase of uptake at the apical region, consistent with apical hypertrophy. No reversible perfusion defect was identified. A twentyfour-hour ambulatory electrocardiographic recording did not show any tachyarrhythmia. The patient’s regimen was changed to a beta blocker for blood pressure control plus an-
tibiotic
prophylaxis
for dental and
surgical procedures.
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
75 Discussion
This report documents the unusual concurrence of anomaly of the ventricular myocardium together with a malformation of the coronary anatomy. The electrocardiographic, echocardiographic, and ventriculographic features of this patient were consistent with apical hypertrophic cardiomyopathy of the true &dquo;Japanese&dquo; or &dquo;Asian&dquo; type.4-6 As not infrequently found in patients of the Japanese series,’ this patient had mild systemic hypertension, the severity of which was considered inadequate to account for the myocardial hypertrophy. Coronary angiography revealed in addition abnormal vascular communications between both the left and right coronary arteries and the main pulmonary artery. The major clinical significance of coronary &dquo;arteriovenous&dquo; fistulas lies in their propensity to lead to myocardial ischemia through the &dquo;steal&dquo; phenomenon.’ In our patient, this risk was potentially greater, for the fistula drained partly from the left anterior descending artery, which supplied an area of myocardium that was hypertrophic and that was also likely to have an abnormal histologic infrastructure.’ This consideration is particularly relevant in the light of the recent report of apical myocardial infarction in a patient who had apical hypertrophic cardiomyopathy with normal epicardial coronary arteries.’ Conclusion
Our patient had never had symptoms of angina, and the exercise thallium scan did not reveal any evidence suggesting significant myocardial ischemia. Thus, the electrocardiographic changes of this patient, similar to those of other patients with this condition, probably represented genuine repolarization abnormalities associated with apical hypertrophy and did not reflect myocardial ischemia. This man was also asymtomatic from his cardiomyopathy. There were no overtly abnormal hemodynamic sequelae, nor was there electrocardiographic documentation of significant tachyarrhythmia. This is in accord with earlier reports on the generally more &dquo;benign&dquo; nature of this specific variant of hypertrophic cardiomyopathy.4’6 Yau-Ting Tai, M.B., M.R.C.P., F.A.C.A. University Department of Medicine Professorial Block
Queen Mary Hospital Pokfulam Road Hong Kong . References 1. Levin DC, Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries. Angiographic aspects. Circulation 58:25-34, 1978. 2. Gobel FL, Anderson CF, Baltaxe HA, et al: Shunts between the coronary and pulmonary arteries with normal origin of the coronary arteries. Am J Cardiol 25:655-661, 1970. 3. Chia BL, Yan PC, Choo MH: Combined apical hypertrophic cardiomyopathy and coronary arteryleft ventricular fistula (letter). Chest 94:219, 1988. 4. Yamaguchi M, Ishimura T, Nishiyama T, et al: Hypertrophic nonobstructive cardiomyopathy with giant negative T-waves (apical hypertrophy): Ven-
triculographic and echocardiographic features in 30 patients. Am J Cardiol 44:401-412, 1979. 5. Chia BL, Tan LK: Apical hypertrophic cardiomyopathy (letter). Am J Cardiol 53:1413, 1984. 6. Maron NJ: Apical hypertrophic cardiomyopathy: The continuing saga. J Am Coll Cardiol 15:91-93, 1990. 7. Sumino
8.
S, Sugimoto T, Koide T, et al: Necropsy finding in a patient with apical hypertrophic cardiomyopathy. Jpn Heart J 6:297-303, 1985. Webb JG, Sasson Z, Rakowski H, et al: Apical hypertrophic cardiomyopathy: Clinical follow up and diagnostic correlates. J Am Coll Cardiol 15:83-90, 1990.
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
as
Letters to the Editor will be published if suitable, space permits. They should not exceed 1,000 words (double in length, and may be subject to editing or abridgment.
spaced)
Bilateral Coronary-Artery-to-Pulmonary-Artery Fistula Coexistent with Apical Hypertrophic Cardiomyopathy— A Case Report Yau-Ting Tai, M.B., M.R.C.P., F.A.C.A. Ping-Ching Fong, M.B., M.R.C.P. and
Wing-Hing Chow, M.B., M.R.C.P.,
F.I.C.A.
HONG KONG
Abstract
sixty-four-year-old man presented with repolarization abnormalities on the electrocardiogram. Echocardiography and cardiac catheterization revealed that he had the rare combination of apical hypertrophic cardiomyopathy with bilateral coronary-artery-to-pulmonary-artery fistula. An exercise thallium scan was negative, suggesting that the marked electrocardiographic changes were most likely secondary to the apical myocardial hypertrophy, instead of to coronary-stealA
induced ischemia.
Introduction
Coronary arteriovenous fistulas, while a rare congenital anomaly, have been well characterized. 1,2 The association of an anomaly of the coronary vasculature with primary diseases of the myocardium is, however, rarely described.’ This report details the rare coexistence of bilateral coronary-artery-to-pulmonary-artery fistula with apical hypertrophic cardiomyopathy in a patient who was otherwise asymptomatic. The potential effect of such a combination of vascular and myocardial abnormalities is addressed. Case Report A sixty-four-year-old Chinese man was referred for evaluation of electrocardiographic abnormalities discovered on routine checkup. He had a five-year history of mild systemic hypertension (blood pressure on presentation 150-160/100 mm Hg), well controlled with a thiazide diuretic. There was no history of exercise intolerance, palpitation, syncope, or chest pain. Physical examination produced normal findings; in particular no murmur was heard. The electrocardiogram showed sinus rhythm with deep symmetrical inverted From the Division of
Cardiology, Department of Medicine, University
of
Hong Kong, Hong Kong
72
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
73
FIG. 1. Left ventriculogram in a right anterior oblique (30 degrees) projection, showing the &dquo;ace-ofspade&dquo; appearance at end-diastole (A) and the active ventricular contraction with apical obliteration during systole (B).
precordial leads V2 to V6 (maximum 9 mm). Two-dimensional echocardiography revealed marked hypertrophy of the left ventricular apex and the distal one third of the ventricular septum and the inferior wall, below the level of the papillary muscle. Left ventricular contraction was active with systolic apical obliteration. On cardiac catheterization, the left ventricular end-diastolic pressure was 12 mm Hg, and there was no intraventricular pressure gradient. Left ventriculography showed active left ventricular contraction with apical obliteration and an &dquo;ace-of-spade&dquo; appearance at end-diastole (Fig. 1). Coronary angiography revealed a dilated and smooth proximal left anterior descending artery, giving rise to multiple tortuous and plexiform vessels that coursed superiorly and drained into the main pulmonary artery (Fig. 2A). The proximal T
waves over
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
74
FIG. 2. Left (A) and right (B) coronary angiography in a right anterior oblique projection, showing the coronary-artery-to-pulmonary-
artery fistula (arrow).
right
coronary artery gave rise to two conal branches that drained into the same plexiform malformation and then into the main pulmonary artery (Fig. 2B). The rest of the
coronary anatomy was normal. There was insignificant oxygen step-up at the main pulmonary artery, and the calculated pulmonary-to-systemic flow ratio was less than 1.1. An exercise thallium scan revealed mild increase of uptake at the apical region, consistent with apical hypertrophy. No reversible perfusion defect was identified. A twentyfour-hour ambulatory electrocardiographic recording did not show any tachyarrhythmia. The patient’s regimen was changed to a beta blocker for blood pressure control plus an-
tibiotic
prophylaxis
for dental and
surgical procedures.
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
75 Discussion
This report documents the unusual concurrence of anomaly of the ventricular myocardium together with a malformation of the coronary anatomy. The electrocardiographic, echocardiographic, and ventriculographic features of this patient were consistent with apical hypertrophic cardiomyopathy of the true &dquo;Japanese&dquo; or &dquo;Asian&dquo; type.4-6 As not infrequently found in patients of the Japanese series,’ this patient had mild systemic hypertension, the severity of which was considered inadequate to account for the myocardial hypertrophy. Coronary angiography revealed in addition abnormal vascular communications between both the left and right coronary arteries and the main pulmonary artery. The major clinical significance of coronary &dquo;arteriovenous&dquo; fistulas lies in their propensity to lead to myocardial ischemia through the &dquo;steal&dquo; phenomenon.’ In our patient, this risk was potentially greater, for the fistula drained partly from the left anterior descending artery, which supplied an area of myocardium that was hypertrophic and that was also likely to have an abnormal histologic infrastructure.’ This consideration is particularly relevant in the light of the recent report of apical myocardial infarction in a patient who had apical hypertrophic cardiomyopathy with normal epicardial coronary arteries.’ Conclusion
Our patient had never had symptoms of angina, and the exercise thallium scan did not reveal any evidence suggesting significant myocardial ischemia. Thus, the electrocardiographic changes of this patient, similar to those of other patients with this condition, probably represented genuine repolarization abnormalities associated with apical hypertrophy and did not reflect myocardial ischemia. This man was also asymtomatic from his cardiomyopathy. There were no overtly abnormal hemodynamic sequelae, nor was there electrocardiographic documentation of significant tachyarrhythmia. This is in accord with earlier reports on the generally more &dquo;benign&dquo; nature of this specific variant of hypertrophic cardiomyopathy.4’6 Yau-Ting Tai, M.B., M.R.C.P., F.A.C.A. University Department of Medicine Professorial Block
Queen Mary Hospital Pokfulam Road Hong Kong . References 1. Levin DC, Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries. Angiographic aspects. Circulation 58:25-34, 1978. 2. Gobel FL, Anderson CF, Baltaxe HA, et al: Shunts between the coronary and pulmonary arteries with normal origin of the coronary arteries. Am J Cardiol 25:655-661, 1970. 3. Chia BL, Yan PC, Choo MH: Combined apical hypertrophic cardiomyopathy and coronary arteryleft ventricular fistula (letter). Chest 94:219, 1988. 4. Yamaguchi M, Ishimura T, Nishiyama T, et al: Hypertrophic nonobstructive cardiomyopathy with giant negative T-waves (apical hypertrophy): Ven-
triculographic and echocardiographic features in 30 patients. Am J Cardiol 44:401-412, 1979. 5. Chia BL, Tan LK: Apical hypertrophic cardiomyopathy (letter). Am J Cardiol 53:1413, 1984. 6. Maron NJ: Apical hypertrophic cardiomyopathy: The continuing saga. J Am Coll Cardiol 15:91-93, 1990. 7. Sumino
8.
S, Sugimoto T, Koide T, et al: Necropsy finding in a patient with apical hypertrophic cardiomyopathy. Jpn Heart J 6:297-303, 1985. Webb JG, Sasson Z, Rakowski H, et al: Apical hypertrophic cardiomyopathy: Clinical follow up and diagnostic correlates. J Am Coll Cardiol 15:83-90, 1990.
Downloaded from ang.sagepub.com at East Carolina University on July 2, 2015
