Reimplantation of Anomalous Left Coronary Artery From the Pulmonary Artery to the Ascending Aorta

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This is a case of a 9-month-old male who presented to the emergency department with a two-week history of cough, intermittent fever, and poor oral intake. His respiratory panel was positive for respiratory syncytial virus (RSV), and he was found to have significant cardiomegaly on chest X-ray. Electrocardiogram (EKG) demonstrated Q-waves in leads I and aVL, along with diffuse ST segment elevation in precordial leads. 

On echocardiogram, the patient demonstrated an anomalous left coronary artery from the pulmonary artery with coronary steal and collateral formation, severe dilated cardiomyopathy, severely diminished left ventricular function, and severe mitral regurgitation. The patient’s B-type natriuretic peptide (BNP) level was greater than 4000, and the troponin level was 132.  

Recognizing the critical nature of this patient, the cardiothoracic surgical team elected to proceed with anomalous left coronary artery from the pulmonary artery (ALCAPA) repair with coronary artery lengthening to compensate for the 13.8 mm distance between the ascending aorta and coronary ostia. The extracorporeal membrane oxygenation (ECMO) team was on standby due to concomitant conditions. 

Operation 

The patient was brought into the operating room and prepped in the standard fashion. The patient was then cannulated high on the ascending aorta. Cardiopulmonary bypass commenced in the standard fashion, with subsequent snaring of the branch pulmonary arteries. The aorta was then cross-clamped, and 15 cc/Kg of antegrade del Nido cardioplegia was applied to the aortic root, with simultaneous application of 10 cc/Kg of del Nido cardioplegia instilled into the main pulmonary artery and down the anomalous left coronary system. 

The middle pulmonary artery was then divided proximal to the branch pulmonary arteries to allow for direct visualization of the anomalous left coronary artery ostia that arose from the posterior sinus of the middle pulmonary artery. A generous coronary button was excised for planned elongation, taking care to preserve as much native tissue to the pulmonary leaflet annulus. The aortic root was then dissected to evaluate the position of the coronary button to minimize stretch.  

To accommodate ideal positioning, extra button tissue was purposely harvested to augment the coronary to facilitate translocation onto the native aorta, given the length required for reimplantation. The coronary elongation was accomplished using a two-layered running continuous technique with 7-0 Prolene. Two and 3 mm dilators were then passed through, showing no evidence of obstruction or stenosis. Since the planned coronary button anastomosis was low on the ascending aorta, the aorta was transected to ensure that there was no compromise to the aortic valve leaflets. An aortic punch was then used to fully create the ostia opening, and the coronary artery button was anastomosed using 7-0 Prolene in a running continuous fashion.  

The back wall of the button anastomosis was left long to create a cobra hood anastomosis, which aimed to reduce the risk of postoperative complications. After ensuring patency of the anastomosed button graft, the transected ascending aorta was then anastomosed back together using 7-0 Prolene in a running continuous fashion. 

Attention was redirected to the main pulmonary artery. Cormatrix was brought to the surgical field, and a patch was created for the replacement of the coronary button harvest site, which was completed using 7-0 Prolene in a running fashion. The main pulmonary artery was then anastomosed back to the branch pulmonary arteries using 6-0 Prolene in a continuous fashion.  

After adequate hemostasis, the patient was rewarmed, taken off bypass, and transitioned to central veno-arterial extracorporeal membrane oxygenation (VA ECMO) as planned, given the patient’s poor preoperative cardiac function. The patient was able to be switched over without any hemodynamic compromise. 

Postoperative 

Cardiopulmonary bypass time was 137 minutes, and aortic cross-clamp time was 84 minutes. Postoperative transesophageal echocardiogram confirmed the anastomosis of the left coronary artery to the ascending aorta. Due to prior cardiomegaly diagnosis, the patient at the immediate post-op still had a dilated left ventricle with diminished left ventricular function.  

The patient remained on VA ECMO for 73 hours and was transferred out of the cardiovascular intensive care unit (CVICU) on day 28. He was discharged home and is currently being followed as an outpatient, with recent echocardiograms showing mild mitral valve insufficiency. The patient is clinically doing well and continues to grow and show improvement. 

References

1. Li D, Zhu Z, Zheng X, Wang Y, Wang Y, Xu R, Wang T, Liu K. Surgical treatment of anomalous left coronary artery from pulmonary artery in an adult. Coron Artery Dis. 2015 Dec;26(8):723-5. doi: 10.1097/MCA.0000000000000286. PMID: 26180997; PMCID: PMC4635871.
2. Micovic S, Milacic P, Milicic M, Brkovic M, Vukovic P, Zivkovic I. Surgical Reconstruction of the Anomalous Left Coronary Artery From the Pulmonary Artery. Tex Heart Inst J. 2023 Jan 1;50(1):e217817. doi: 10.14503/THIJ-21-7817. PMID: 36735611; PMCID: PMC9969781.

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