Pulmonary Origin of Left Anterior Descending Coronary Artery in Tetralogy of Fallot Masahiro Yamaguchi, MD, Takuro Tsukube, MD, Yuhei Hosokawa, MD, Hidetaka Ohashi, MD, and Yoshihiro Oshima, MD Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Japan
We report the case of a 4-year-old girl with an anomalous origin of the left anterior descending coronary artery from the pulmonary artery in association with tetralogy of Fallot. Creation of an intrapulmonary tunnel from a newly created aortopulmonary window to the coronary
ostium in the pulmonary trunk and total repair of tetralogy of Fallot were successfully carried out. The advantage of intrapulmonary tunnel technique (Takeuchi method) for this very rare combination is stressed. (Ann Tkorac Surg 1991;52:310-2)
dilated LAD with retrograde filling through collaterals from the right coronary artery and left circumflex branch and draining into the pulmonary artery. Thallium 201 myocardial scintigraphy showed reduced accumulation in the apex area and markedly increased accumulation in the right ventricle. Operation was performed on April 21, 1988, with the diagnosis of anomalous origin of the LAD from the pulmonary trunk in association with tetralogy of Fallot. Exposure of the heart with a median sternotomy revealed a tortuous and dilated LAD originating from the left posterior aspect of the pulmonary trunk. With the aid of hypothermic (22°C) cardiopulmonary bypass, an atrial septal defect of the central type (9 mm) was closed directly and the main pulmonary artery just above the valve was opened. The orifice of the LAD was found on the left posterior wall of the pulmonary trunk. The bicuspid pulmonary valve with an orifice of 5 mm was enlarged to accept a No. 14 Hegar dilator by commissurotomy along two commissures. The rest of the procedures were effected by aortic cross-clamping, blood cardioplegia, and topical cardiac cooling. Hypertrophic infundibular muscle bundles were resected, and a large ventricular septal defect of perimembranous trabecular type, 20 mm in size, was closed with a Dacron patch through a transverse ventriculotomy in the outflow tract. After creation of a window between the ascending aorta and the pulmonary trunk, a flap of pulmonary arterial wall, 10 mm wide and 20 mm long, was made. An intrapulmonary tunnel between the aortopulmonary window and the orifice of LAD was then created using this flap in the same way as described by Takeuchi and co-workers . The anterior wall of the pulmonary artery was reconstructed using a horse pericardial (Xenograft; Xenomedica AG, Switzerland) patch. The patient had an uneventful postoperative course, and cardiac catheterization 1 month after the operation confirmed satisfactory postoperative status with decrease of right ventricular pressure and pressure gradient across the pulmonary valve. The aortogram showed normal
nomalous origin of the coronary artery from the pulmonary artery occurs rarely in association with other congenital cardiac defects. In the repair of complex congenital cardiac defects, it is important to recognize a concurrence of this anomaly preoperatively, as it will substantially add to the risks of operation [l].In this report, we review our clinical experience with anomalous origin of the left anterior descending coronary artery (LAD) from the pulmonary trunk in association with tetralogy of Fallot. A 4-year-old girl, weighing 16 kg and 104 cm in height, was admitted for cardiac evaluation on August 10, 1987. A heart murmur was noticed at the age of 1 month, and at 6 months of age she was given a diagnosis of tetralogy of Fallot by echocardiography. Physical examination on admission revealed mild cyanosis, clubbing of finger and toe nails, and squatting on hard exercise. A grade 416 systolic murmur was heard in the second to third intercostal spaces along the left sternal border. An electrocardiogram demonstrated a QRS axis of +225 degrees with right ventricular hypertrophy. Chest roentgenography showed mild cardiomegaly (cardiothoracic ratio, 0.59) and normal pulmonary vascularity. Echocardiography disclosed an overriding of the aorta, large ventricular septal defect, and infundibulovalvular pulmonary stenosis. Cardiac catheterization revealed a step-up of oxygen saturation in the right atrium and the main pulmonary artery and a pressure gradient of 80 mm Hg across the pulmonary valve (Table 1). Right ventriculography showed a large ventricular septal defect, infundibular and valvular pulmonary stenosis, and a opacification of the dilated LAD in later frames (Fig 1). On left ventriculography, left ventricular end-diastolic volume was normal with no evidence of mitral insufficiency. Aortography and selective right coronary arteriography showed a markedly Accepted for publication Jan 15, 1991. Address reprint requests to Dr Yamaguchi, Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Takakura-dai 1-1-1, %ma-ku, Kobe 654, Japan.
0 1991 by The Society of Thoracic Surgeons
CASE REPORT YAMACUCHI ET AL ANOMALOUS CORONARY ARTERY WITH TETRALOGY
Ann Thorac Surg 1991;52:310-2
Table 1 . Cardiac Catheterization Data Pressure (mm Hg)
Superior vena cava Inferior vena cava
0.70 0.69 0.74
1218 1218 1317
0.70 0.74 0.78 0.98 0.98 0.96 0.90
10412 9713 24117 1819 1714 10411 99162
Right atrium Right ventricle
Apex outflow Pulmonary artery Pulmonary vein Left atrium Left ventricle
End-diastolic pressure, 13 mm Hg. Hg. SaO, = oxygen saturation.
after total correction of tetralogy of Fallot  and the postoperative left ventricular function is reported to be better with a two-coronary arterial system than with simple ligation . This is clearly demonstrated in the report by Wilcox and associates [l]. They repaired the pulmonary origin of LAD with the use of a saphenous vein graft from aorta to anomalous vessel at a separate operation 4 months after total repair of tetralogy of Fallot
51" 48b 20 14 13 51b
End-diastolic pressure, 12 m m
filling of the LAD through the newly created tunnel and disappearance of tortuous dilatation seen preoperatively (Fig 2). The postoperative thallium 201 myocardial scintigram revealed increased accumulation in the apex area and marked decrease of accumulation in the right ventricle.
Comment Anomalous origin of the coronary artery from the pulmonary artery in association with tetralogy of Fallot is extremely rare. Only 6 cases of this combination have been reported in the literature [I, 3-71. Three of them were found at autopsy [3, 5, 71, and surgical intervention was undertaken in 2 cases [l, 51. In these 6 patients, the left coronary artery arose from right pulmonary artery in 1 , right coronary artery from pulmonary trunk in 2 [6, 71, left coronary artery from pulmonary trunk in 2 [4, 51, and LAD from pulmonary trunk in 1 [ 11. Our patient is similar to that reported by Wilcox and associates [l],in whom LAD arose from pulmonary trunk. Clinical manifestation of anomalous origin of the coronary artery from pulmonary artery in association with tetralogy of Fallot depends on the amount of left to right shunting through the coronary arterial system acting as systemic to pulmonary anastomosis and to the severity of pulmonary stenosis [ 11. There are two options in the surgical management of anomalous origin of the LCA in infants and small children . One is simple ligation, which may be recommended in critically ill infants. But this can be successful only when adequate collateral intercoronary circulation exists. The other is reestablishment of a two-coronary arterial system, which is a preferable procedure if it can be done with an acceptable low operative risk and patency for the long term. The Of a two-coronary system is Of particular importance in the surgical treatment of this anomaly in association with tetralogy of Fallot as the left ventricular work is reported to increase
Fig 1 . Preoperative right ventriculograms in anteroposterior (A) and lateral fB) views. Large ventricular septal defect, infundibular and valvular pulmonary stenosis, and retrograde filling of dilated left anterior descending coronary artery (arrow) are demonstrated.
Ann Thorac Surg 1991;52:310-2
CASE REPORT YAMAGUCHI ET AL ANOMALOUS CORONARY ARTERY WITH TETRALOGY
with resultant gradual improvement of left ventricular contractility. Akasaka and associates  were the first to report a successful simultaneous repair of pulmonary origin of the LCA and tetralogy of Fallot. They directly reimplanted the anomalous vessel into the aorta, but the postoperative angiogram 1 month after the operation showed only vague opacification of the implanted LCA in antegrade fashion. Among various options for the establishment of a two-coronary arterial system, the intrapulmonary tunnel repair with a pulmonary arterial flap is appealing because it can be applied regardless of the patient’s age and the distance from aorta to coronary orifice in the pulmonary artery; it also can avoid mobilization of the anomalous coronary artery, which still remains a technical difficulty . It is emphasized that this technique is particularly advantageous, in terms of sparing operative time, in patients in whom concomitant reconstruction of the right ventricular outflow tract and the pulmonary artery are required as in our patient.
B Fig 2. Postoperative aortograms in anteroposterior (A) and lateral (B) views demonstrating normal filling of left anterior descending coron a y a r t e y from a newly created intrapulmonay tunnel (thick arrow) and disappearance of tortuous dilatation seen preoperatiuely. (Ao = aorta; LAD = left anterior descending coronary artery; RCA = right coronary artery.)
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