Ventricular tachycardia in a patient with congenital coronary arteriovenous fistula

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4. Miyakazi M, Kato M. Third coronary artery: its development and function. Acta Cardiol 1988;43:449-57. 5. Reig-Villalonga J, Loncan-Vidal MP, Domenech-Mateu JM. Coronary arterial anastomoses. Study of their distribution in adult hearts specially emphasizing the crista supraventricularis area. Anat Anz 1987;164:1-12. 6. Yamagishi M, Haze K, Tamai J, Fukami K, Beppu S, Akiyama T, Miyatake K. Visualization of the isolated conus artery as a major collateral pathway in patients with total left anterior descending artery occlusion. Cathet Cardiovasc Diagn 1988;15:95-8.

Ventricular tachycardia in a patient with congenital coronary atteriovenous fistula C. Moro-Serrano,

MD,

J. Martinez,

MD,

A. H. Madrid, MD, J. J. Rufilanchas, MD, L. Novo, MD, E. Marin Huerta, MD, A. Vazquez MD, and J. Tamargo, MD Madrid, Spain

Most patients with congenital coronary arteriovenous fistula

remain

free

of symptoms

until

the

third

decade

of life.

Symptoms that are related to myocardial ischemia or chronic cardiac volume overload may appear with advancing age.1.2We report the caseof an adult patient with a congenital coronary artery fistula that was first observed clinically with sustained monomorphic ventricular tachycardia. To our knowledge, this is the first caseof ventricFrom

the Arrhythmia

Reprint y Cajal 4/4/38206

requests: Hospital,

Unit,

Ramhn

y Cajal

Hospital,

Madrid.

Concepci6n More-Serrano, MD, Arrhythmia Unit, Ram& Ctra Colmenar Viejo Km 9,100, 28034 Madrid, Spain.

503

ular arrhythmia complicating this infrequent congenital heart diseaseto be reported in the literature. A 47-year-old man was referred to our unit for electrophysiologic evaluation becauseof sustainedventricular tachycardia. A heart murmur wasdiscoveredat the ageof 8 years. He remained free of symptomsuntil 1 month before referral. He had been admitted to another hospital for treatment of palpitations and presyncope.The ECG showed a wide QRS complex tachycardia at a rate of 230beats/min (Fig. 1). The diagnosiswasventricular tachycardia, and he wastreated with lidocaine and procainamideadministered intravenously, but this did not prevent new episodesof tachycardia. At the time of admission,physical examination revealed a soft continuousheart murmur at the left esternal border. EGG showedT-wave changes,which suggestedsubepicardial ischemia,at the inferior and lateral leads(Fig. 2). Chest roentgenogramwasnormal. Two-dimensionalechocardiography, at the apical four-chamber view, revealed a small, echo-free, round spacenext to the apex of the right ventricle. Pulsed Doppler echocardiography at that spacewas diagnostic of systolic and diastolic high-velocity flow. A catheterization study was performed. The circumflex coronary artery was dilated and tortuous in its entire course, and it emptied in the right ventricular cavity,near the apex where an aneurysm had formed (Fig. 3). Pulse oximetry showedthe presenceof a left-to-right shunt at the right ventricle, with a QP-QS ratio of 1.4:1.Programmedelectrical stimulation could not reproduce the clinical arrhythmia. Isoproterenol wasalsoineffective in reproducing sustained ventricular tachycardia. Ventricular tachycardia appearedspontaneously,later on, with a ventricular cycle length of 260 msec,showing a right bundle branch block pattern, with an axis of -150 degreesin the frontal plane. Atrioventricular dissociation was assessedby means of

Fig. 1. Twelve-lead ECG of a wide QRS complex tachycardia at a rate of 230beats/min, with a right bun-

dle branch block pattern and a frontal axis of -150 degrees,suggestinga low septal ventricular origin. All of the patient’s clinical patterns of tachycardia were similar.

504

Brief Communications

American

August 1992 HearI Journal

Fig. 3. Contrast injection per panel, Posteroanterior Fig. 2. Basal lead ECG with T-wave and inferior leads.

Fig. 4. Standi ud and unipolar during

surgery (left)

inversion

at lateral

in the left coronary artery. Upprojection; lower panel, right anterior oblique projection. The left circumflex artery is dilated, and the anterior descending artery is not well-filled because of the coronary steal syndrome.

extremity leads of pace mapping at the site of the ventricular aneurism shows a QRS pattern that is quite similar to the clinical tachycardia (right).

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esophagic bipolar atria1 recording. During surgery a dilated circumflex coronary artery was seen. Near the apex of the right ventricle a bulging aneurysm of 2 cm in diameter was found. Ventricular tachycardia could not be reproduced by electrical stimulation of the right and left ventricles. Pace mapping through a hand-held probe at the site of the ventricular aneurysm reproduced the pattern of the clinical tachycardia (Fig. 4). Ligation of the coronary arteriovenous fistula was performed at its end with resection of the intramural fistulous cavity including the round aneurysm. Cellular electrophysiology was used to examine the excised tissue, which had previously been kept in oxygenated Tyrode’s solution. The tissue was thickly fibrous, but at several cells slow action potentials could be recorded. Mean values of the observed potentials were as follows: resting potential, -41.6 * 2.0 mV; action potential amplitude, 38.5 t 2.0 mV, V,,,, 5.0 i: 0.4V/sec, action potential duration33’7.3 * 14.6msec,conductionvelocity,0.018 ? 0.01 m/set. The patient had an uneventful postoperative course and was free of symptoms 2 years after surgery. Congenital coronary arteriovenous fistula is a rare malformation, which accounts for less than 1% of all cases of congenital heart disease.3 Drainage of the coronary fistula occurs in over 90’; of in the systemic low-pressure venous system. Drainage at the right ventricle occurs in 410; of cases in a wide review of the literature.’ In most young patients, the accidental discovery of a continuous heart murmur is the clue to the clinical diagnosis.‘-” Over the age of 30 years patients became symptomatic. Coronary ischemia as a result of coronary steal syndrome may produce angina pectoris, and less frequently myocardial infarcti0n.i It might also become apparent by heart failure due to volume overload of ventricular chambers secondary to left-to-right shunt.’ Other rare manifestations are endocarditis, coronary dissection, and mechanical interference. Supraventricular arrhythmias are relatively common with shunts associated with augmented flow through the atria. To our knowledge, no case of congenital coronary arteriovenous fistula wLth ventricular tachycardia has been previously described. We suspect that the tachycardia mechanism was not reentry but triggering activity or abnormal automaticity with focus at the intramural aneurysm.5 Ischemia could also play a trigger role in the initiation of ventricular arrhythmia. Abnormal electrical properties of the excised tissue indicate that the tachycardia was due to apparent aneurysm formation at the right ventricular apical insertion site of the fistula. Resection of the fistulous line with ligation of the fistula produced an excellent clinical result, and the ventricular tachycardia disappeared.

Brief Communications

4.

5.

505

LL. Congenital coronary arteriovenous fistula. Pediatrics 1960;25:531-60. Levin DC, Fellows KE, Abrams HL. Hemodynamically significant primary anomalies of the coronary arteries. Circulation 1978;58:25-34. Rosen MR. Mechanisms for arrhythmias. Am J Cardiol 1988;61:2A-8A.

Vasospastic syndrome

angina in Crow-Fukase

Toru Kato, MD, Eiji Kaneko, MD, Fujio Numano, Toshiyuki Oniki, MD, Yuji Hashimoto, MD, Yukio Kishi, MD, Michiyoshi Yajima, MD, Masayoshi Iwakami, MD, Toshizou Sekine, MD, Hidenori Maezawa, MD, and Tetsuo Furukawa, MDa Tokyo, Japan

MD,

A 36-year-old man with Crow-Fukase syndrome presented with a typical variant form of angina in which vasospasm was demonstrated by coronary angiography. The CrowFukase syndrome is characterized by peculiar multisystem manifestations such as polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly associated with extramedullary plasmacytoma.1-3 The patient was well until 1 year before admission, when he developed diarrhea, low-grade fever, and general malaise; he began to lose weight gradually. Six months before admission to the hospital he developed numbness of the feet, which gradually progressed to the knees, accompanied by muscular weakness in the lower extremities that resulted in an inability to walk. The patient noticed hyperpigmentation of the skin and impotence. On admission, the patient was confined to a wheelchair. Radiographs demonstrated an osteoblastic lesion that involved the thoracic spine. Bone marrow examination showed plasmacytoma (Fig. 1). A lumbar puncture yielded clear, colorless cerebrospinal Auid (CSF) and increased protein. Cytologic examination showed no tumor cells. CSF and serum electrophoreses disclosed increases in lambda light chains of immunoglobulin G (IgG-X). Neurologic evaluation showed decreased reflexes in the lower extremities. Sensation was diminished in a stocking-glove distribution. A glucose tolerance test showed a diabetic pattern. Other laboratory studies, including tests of liver, thyroid, and pituitary function, were within normal limits. Crow-Fukase syndrome was highly suspected. While the patient was confined to bed, he developed typical chest pain. Electrocar-

REFERENCES

1. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of managiment. Circulation 1979;59:849-54. 2. Wilde P. Watt I. Conaenital coronarv arterv fistulae. Six new cases with a collective review. Clin Radio1 “l980;31:301-11. 3. Gasul BM, Arcilla RA, Rell EH, Lynfield J, Bicoff JP, Luan

From the Third Department ogy, Tokyo

Medical

and

Reprint requests: Toru School 414138277

of Medicine,

of Internal Medicine, aDepartment Dental University, School of Medicine.

Kate, MD, Tokyo Medical l-5-45 Yushima, Bunkyo-ku,

of Neurol-

and Dental University, Tokyo 113, Japan.