Establishment of a Two Coronary Artery System in Anomalous Origin of Left Coronary Artery from Pulmonary Artery

Review Article

J Dis Markers. 2014;1(2): 1009.

Establishment of a Two Coronary Artery System in Anomalous Origin of Left Coronary Artery from Pulmonary Artery

Nguyen Luong Tan*, Thai Viet Tuan, Doan Duc Hoang, Nguyen Xuan Hung, Nguyen Thi Le and Bui Duc Phu

Cardiovascular and Thoracic Surgery Department, Hue Central Hospital, Vietnam

*Corresponding author: Nguyen Luong Tan, Cardiovascular and Thoracic Surgery Department, Hue Central Hospital, 6/72 Le Ngo Cat Street, Hue, Vietnam

Received: August 11, 2014; Accepted: August 30, 2014; Published: September 02, 2014


Anomalous origin of the left coronary artery arising from the pulmonary artery is a relatively rare disease. Within 2 years, from August 2011 to June 2013, Cardiovascular Centre of Hue Central Hospital had received and treated 5 cases of this disease. The patients were from 4 weeks to 17 months old (median 4 months), and had an average weight of 6 kg (range 3-10 kg). All 5 cases were accurately diagnosed by echocardiography and operated successfully with reconstruction of left main coronary artery. Troponin T was used as a marker to identify the myocardial infarction after surgery. All patients had been supported with interlope after cardiac repair and in the postoperative period, the supporting time varied from several days to several weeks, depending on the hemodynamic and cardiac function, but none of the patients required postoperative extracorporeal membrane oxygenation or died. Ejection fraction returned to normal values in 4 patients after 3 months; however, in one patient with preoperative ejection fraction decreased to 18%, this figure was almost unimproved during 12 months of follow-up (20%). Early diagnosis and early surgery is to avoid chronic heart failure, requiring the surgeon to choose the most appropriate technique for each patient.


Anomalous origin of Left Coronary Artery from Pulmonary Artery (ALCAPA) has an anomaly of coronary blood supply for the heart. The left coronary artery is connected to a low blood pressure of pulmonary artery and receives blood flow from the right coronary artery by collateral vessels. This abnormal coronary anatomy leads to blood steal syndrome of the left coronary artery to the pulmonary artery. This coronary-pulmonary artery steal syndrome may cause congenital myocardial ischemia at a very young age, consequently a left heart failure and further lesions such as left ventricular dilation, and severe mitral valve regurgitation [1,2]. Newborns are usually asymyomatic until physiologic pulmonary hypertension period ended, resulting in lower left coronary pressure. Natural evolution of this anomaly is frequently fatal with a mortality of 90% by 1 year of age [3]. The diagnostic of this anomaly is usually at the late stage of congestive heart failure, therefore the treatment becomes more difficult and complicated, the prognostic is poor in comparison with the repair at early stage when the heart is only at the onset of ventricular dysfunction without the consequences of myocardial infarction.

One of the traditional surgical repairs is ligation of the anomalous origin of the left coronary artery in order to avoid the steal phenomenon. However without enough collaterals between the two coronary systems. This technique may lead to a severe myocardial ischemia, high rate post operated morbidity and mortality [4]. Dual coronary system repair restores physiological ante grade flow to the ischemic left ventricular myocardium, which has improved surgical outcome and is currently the ideal treatment for this anomaly [5].

The origin of anomalous left coronary artery locates at different sinuses or high on the trunk of pulmonary artery; surgical techniques have to vary in every single individual anatomic form in order to reimplant the left coronary artery to the aortic root [6].

In addition, severe mitral valve regurgitation need to be repaired; although some surgeons hope this concomitant lesion will disappear after the coronary reimplantation. Because of severe conditions of the heart before surgery, after a cardiopulmonary bypass and cardioplegic period, early results are not easily predicted. Therefore, most ALCAPA repairs need an Extracorporeal Membrane Oxygenation (ECMO) reserved for an acute post operated heart failure.

Patients and Methods

Clinical history and operative technique

Between 8/2011 and 6/2013, 5 children were diagnosed with ALCAPA and referred for surgical repair. All of these patients delayed weight gain. Analysis of ECG showed myocardial ischemia with pathologic large and deep Q waves, elevated ST segment and inverted T waves in leads DI, AVL and V4-6. Preoperative diagnosis was established by Doppler echocardiography. 4 patients with left main coronary artery orifice come from the left sinus of the pulmonary artery root and 1 patient had left coronary artery orifice originating from the posterior sinus of pulmonary artery root. Right coronary artery is larger than normal and reflux blood flow from the left coronary artery into the pulmonary artery (Figure 1).