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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 10  |  Issue : 2  |  Page : 142-144
Management of anomalous left coronary artery from pulmonary artery in an adult patient - a case report


Departments of Anaesthesia and Intensive Care, and Cardiothoracic Surgery, B. M. Birla Heart Research Centre, Kolkata., India

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How to cite this article:
Maitra G, Roychowdhury S, Ganguli S, Bhattacharya S, Kaul A. Management of anomalous left coronary artery from pulmonary artery in an adult patient - a case report. Ann Card Anaesth 2007;10:142-4

How to cite this URL:
Maitra G, Roychowdhury S, Ganguli S, Bhattacharya S, Kaul A. Management of anomalous left coronary artery from pulmonary artery in an adult patient - a case report. Ann Card Anaesth [serial online] 2007 [cited 2020 Nov 27];10:142-4. Available from: https://www.annals.in/text.asp?2007/10/2/142/37942


Anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) is a rare congenital anomaly first described in 1908 in a 60 year old woman [1] , also called Bland - White - Garland Syndrome [2] , and accounts for 0.26% of all congenital heart defects. [3]

The pathophysiological effects of this condition are related to the adequacy of perfusion of left ventricular myocardium which depends on the inflow to left coronary artery from collaterals and steal from left coronary artery to pulmonary artery. Death ensues if collaterals are poorly developed, unless immediate surgery is performed. When collaterals develop and enlarge, survival into adulthood occurs - so called adult type ALCAPA. [4] Survival beyond infancy is usually uncommon because of myocardial necrosis and left heart failure. [5] Patients with ALCAPA surviving past childhood have varying symptoms of myocardial ischaemia and heart failure, 90% dying by 35 years of age with very few older than 50 years surviving without surgical correction. [6] We report management of an adult male with ALCAPA, who underwent surgical correction.


   Case report Top


A 53-year-old male patient was admitted in our hospital with the history of chest pain and breathlessness on mild exertion, hypertension and episodes of heart failure for the past 15 years. On examination, blood pressure was normal with an irregularly irregular pulse. A holosystolic murmur was heard in the mitral and tricuspid areas. The patient was on medical treatment with enalapril, digoxin, frusemide and carvedilol. No signs of failure were noted.

Haematological and biochemical parameters were within normal limits. Chest X-ray showed hilar prominence with increased bronchovascular markings. Electrocardiogram (ECG) showed left bundle branch block with atrial fibrillation. Transthoracic echocardiogram revealed a dilated left ventricular cavity with generalised wall hypokinesia, biatrial enlargement, moderate mitral and tricuspid regurgitation and mild pulmonary arterial hypertension with an ejection fraction of 25%. Coronary angiogram revealed a left coronary artery originating from main pulmonary artery and filling retrogradely from the right coronary artery. The right coronary artery was aneurysmal and dominant.

The patient was referred by the cardiologist for surgical correction. A written, informed consent was taken and the patient was premedicated with tablet lorazepam on the morning of surgery. Induction of general anaesthesia was accomplished with intravenous (IV) midazolam (0.05 mg/kg), fentanyl (2 µg/kg) and sleep dose of thiopentone sodium. After achieving muscle paralysis with IV pancuronium (0.1 mg/kg), endotracheal intubation and mechanical ventilation were instituted. Anaesthesia was maintained with isoflurane, oxygen-air mixture and intermittent doses of fentanyl and pancuronium.

Monitoring consisted of invasive arterial pressure (via left femoral artery), central venous pressure (via right internal juglar vein), ECG, end-tidal carbondioxide, pulse oximetry and temperature. Intraoperatively left atrial pressure monitoring was performed via transthoracic left atrial line as per the institutional protocal. In addition, transoesophageal echocardio­graphy was performed, which revealed similar findings to that of transthoracic echocardiogram. Infusions of dopamine (2 µg/kg/min) and glyceryl trinitrate ( 0.5 µg/kg/min) were started via the central line before anaesthesia was induced and dobutamine and adrenaline infusions were kept ready.

After midline sternotomy and pericardial dissection, anastomosis of one saphenous venous graft from aorta to left coronary artery was performed on a beating heart. The arterial pressure remained between 80-110 mm Hg (systolic) with heart rate of around 60/min during surgery. Blood samples drawn from right atrium, right ventricle and pulmonary artery revealed oxygen tension of 40.2 mm Hg, 43.8 mm Hg and 53.3 mm Hg respectively. The surgeon then attempted to ligate the communication between the left coronary artery and the pulmonary artery. However, since the communication was large, it was decided to institute cardiopulmonary bypass for safe and complete ligation of the communication. Cardiopulmonary bypass was instituted in a standard fashion and the communication between the left coronary artery and the main pulmonary artery was ligated under fibrillatory arrest and moderate hypothermia. Thereafter patient was rewarmed and weaned from cardiopulmonary bypass with arterial pressure of 100/60 mm Hg, central venous pressure 8 mm Hg and left atrial pressure 12 mm Hg. This was achieved with dopamine 4 µg/kg/min, dobutamine 10 µg/kg/min and adrenaline 0.05 µg/kg/ min. Blood samples from right atrium, right ventricle and pulmonary artery revealed oxygen tension of 39.5 mm Hg, 41 mm Hg and 38.4 mm Hg respectively. This confirmed proper ligation of the ALCAPA preventing shunt from left coronary artery to pulmonary artery.

In the postoperative period, after 6 hours of ventilation, the patient was weaned and extubated uneventfully and postoperative echocardiogram showed no further deterioration of left ventricular function or valvular regurgitation with no residual shunt.


   Discussion Top


The clinical presentation of older ALCAPA patients usually consists of exertional dyspnoea or angina and cardiac arrythmias. [1] Clinical examination reveals a systolic murmur in 70% cases. [1],[7]

Diagnosis is usually established by coronary angiogram showing dilated and tortuous right coronary artery with collateral filling of left coronary system. Right heart catheterisation with oximetry shows increase in oxygen saturation at the point where blood from left coronary artery merges with blood from pulmonary artery. [1]

From the review of literature it is agreed that surgical correction is the method of choice as soon as possible with medical management as only supportive and temporary. One report describes a 72 years old patient with ALCAPA who was managed conservatively, as the risk of cardiac surgery seemed to outweight the natural prognosis. [8]

The two accepted corrective precedures for ALCAPA are ligation of the anomalous left coronary artery and reconstruction of a double coronary artery system. The two coronary artery system can be established in many ways - direct implantation of anomalous left coronary artery to aorta, aorta - left coronary artery saphenous vein graft, left coronary artery conduits using left common carotid artery or subclavian artery or left internal thoracic artery or by Takeuchi procedure (creation of an aorto pulmonary window and an intra-pulmonary tunnel extending from the anomalous ostium to the window). [9]

Alixi-Meskishvili et al studied four adult patients with ALCAPA aged 27, 35, 54 and 60 years in whom two coronary artery system was established and found clinical improvement in all patients and concluded that the adult patients with ALCAPA benefit from establishment of a two coronary system. [6]

Other authors have concluded that two coronary artery system appeared more physiological and should be the procedure of choice, to be performed early. [3],[7] Wilson et al after review of liturature of teenagers and adults with ALCAPA, concluded that saphenous vein grafting should be the definitive means of correction by restoring a dual coronary artery system. [1]

Schwartz et al in a study found that the degree of preoperative mitral regurgitation was predictive of outcome in ALCAPA after dual coronary repair and that mild to moderate mitral ragurgitation tended to improve without mitral valvuloplasty after establishment of dual coronary system with complete recovery of myocardial dysfunction. [10]

In the present patient, a dual coronary system was established with saphenous vein graft to anomalous left coronary artery with ligation of its communication with pulmonary artery. Mitral valve replacement or valvuloplasty was not performed as the mitral regurgitation was moderate.

We conclude that dual coronary system should be established early in patients with adult type ALCAPA and that the anaesthetic management should revolve around preventing further decrease in myocardial contractility, maintaining adaquate diastolic blood pressure and prevention of tachycardia as the myocardium is already ischaemic with poor contractivity.

 
   References Top

1.Wilson CL, Dlabal PW, Holyfield RW, et al. Anomalous origin of the left coronary artery from the pulmonary artery : a case report and review of literature concerning teenagers and adults. J Thorac Cardiovasc Surg 1977; 73: 887- 893.  Back to cited text no. 1    
2.Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J 1933; 8: 787-801.  Back to cited text no. 2    
3.Cherian KM, Bharati S, Rao SG. Surgical correction of anomalous origin of the left coronary artery from the pulmonary artery. J Card Surg 1994; 9: 386-391.  Back to cited text no. 3  [PUBMED]  
4.Bunton R, Jonas RA, Lang P, et al. Anomalous origin of left coronary artery from pulmonary artery - ligation versus establishment of a two coronary artery system. J Thorac Cardiovasc Surg 1987; 93: 103-108.  Back to cited text no. 4  [PUBMED]  
5.Shivalkar B, Borges M, Dacnen W, et al. ALCAPA syndrome : an example of chronic myocardial hypoperfusion. J Am Coll Cardiol 1994; 23: 772-778.  Back to cited text no. 5    
6.Alexi - Meskishivili V, Berger F, Weng Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery in adults. J Card Surg 1995; 10: 309-315.  Back to cited text no. 6    
7.Wollenek G, Damanig E, Salzer-Mufar V, et al. Anomalous origin of the left coronary artery : a review of surgical management in 13 patients. J Cardiovasc Surg 1993; 34: 399­-405.  Back to cited text no. 7    
8.Fierens C, Budts W, Denef B, et al. A 72 year old woman with ALCAPA. Heart 2000; 83: e2-e2 (electronic pages).  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation 1980; 62: I 180-I 189.  Back to cited text no. 9    
10.Schwartz ML, Jonas RA, Colan SD. Anomalous origin of the left coronary artery from the pulmonary artery: recovery of the left ventricular function after dual coronary repair. J Am Coll Cardiol 1997; 30: 547-553.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Gaurab Maitra
63-B, Chakraberia Road, (North) Kolkata - 700 020.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.37942

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