Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA)

Summary

Surgical correction of ALCAPA is highly recommended, regardless of age or the degree of intercoronary collateralization. This protocol presents a technique for the direct re-implantation of adult-type ALCAPA into the aorta to re-establish the dual-coronary perfusion. Whenever feasible, direct re-implantation is preferred to other surgical correction techniques.

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly which is one of leading causes of myocardial ischemia and infarction in children. If left untreated, it results in a 90% mortality rate in the first year of life. In patients who survive to the adulthood, the coronary steal phenomenon and retrograde left-sided coronary flow provide a substrate for chronic subendocardial ischemia, which may lead to left ventricular dysfunction, ischemic mitral regurgitation, malignant ventricular arrhythmias, and sudden cardiac death. The average age of life-threatening presentation is 33 years and of sudden cardiac death 31 years. Therefore, surgical correction is highly recommended as soon as the diagnosis is made, regardless of age. In adult-type ALCAPA originating from the right-facing sinus of the pulmonary artery, direct re-implantation of the ALCAPA into the aorta is the more physiologically sound repair technique to re-establish the dual-coronary perfusion system and is recommended. This protocol describes the technique of direct re-implantation of adult-type ALCAPA into the aorta.

Introduction

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly usually seen as an isolated lesion¹. The incidence of ALCAPA is estimated at 1 in 300,000 live births, comprising between 0.24% and 0.46% of congenital cardiac diseases^2,3. It is one of the most common causes of myocardial ischemia and infarction in children and, if left untreated, results in a 90% mortality rate in the first year of life⁴. Only 10 - 15% of infants survive to adulthood due to the rapid development of a large dominant right coronary artery ....

Protocol

The protocol follows the institutional guidelines of the human research ethics committee of the University of Zurich.

1. Preparation for Surgery

1. Clean and prepare the surgical suite in a typical manner. To facilitate communication between the surgeon and the perfusionist, place the heart-lung machine to the left of the patient, opposite to the surgeon.
2. Pre-medicate the patient by the oral administration of 5 mg of midazolam, 30 - 60 min prior to the induction of anesthesia.
3. Let the patient be monitored according to standard guidelines, with direct arterial and central venous pressure access by the anesthesiol....

Results

Presentation

The patient was a 48-year-old woman presenting with the recent onset of angina Canadian Cardiovascular Society (CCS) grad III and occasional palpitations. She reported three uneventful pregnancies. Moderate smoking was the main cardiovascular risk factor. Trans-thoracic echocardiography showed a moderately impaired (45%) left ventricular ejection fraction and no mitral regurgitation. A coronary angiography was then per.......

Discussion

This protocol describes a detailed technique for the direct re-implantation of the ALCAPA into the aorta in an adult patient with the origin of main left coronary artery from the right-facing sinus of the pulmonary artery according to the Dodge-Khatami classification¹⁰. The myocardial protection strategy and the reconstruction of the pulmonary artery are clearly demonstrated. The major critical step of this technique is represented by the generous mobilization of the left coronary artery to achiev.......

Materials

List of materials used in this article

Name	Company	Catalog Number	Comments
Heart surgery infrastructure
Heart Lung Machine	Stockert	SIII
EOPA 24Fr. arterial cannula	Medtronic	77624
Quickdraw 25Fr. femoral venous cannula	Edwards	QD25
LV vent catheter 17Fr.	Edwards	E061
Antegrade 9Fr. cardioplegia cannula	Edwards	AR012V
Retrograde 14Fr. cardioplegia cannula	Edwards	NPC014
Electrocautery	Covidien	Force FX
Sutures
Polypropylene 4/0	Ethicon	8871H
Polypropylene 5/0	Ethicon	8870H
Polypropylene 6/0	Ethicon	EH7400H
Braided polyesther 2/0 ligature with polybutylate coating	Ethicon	X305H
Intergard dacron graft 8 mm	Maquet	IGW0008-30
Micro knife Sharpoint	TYCO Healthcare PTY	78-6900
Drugs
Midazolam	Roche Pharma	N05CD08
Rocuronium	MSD Merck Sharp & Dohme	M03AC09
Propofol	Fresenius Kabi	N01AX10
Fentanil	Actavis	N01AH01
Instruments
Cooley Derra anastomosis clamp	Delacroix-Chevalier	DC40810-16
Cooley vascular clamp	Delacroix-Chevalier	DC40810-16
Dissection forceps Carpentier	Delacroix-Chevalier	DC13110-28
Scissors Metzenbaum	Delacroix-Chevalier	B351751
Needle holder Ryder	Delacroix-Chevalier	DC51130-20
Dissection forceps DeBakey	Delacroix-Chevalier	DC12000-21
Micro needle holder Jacobson	Delacroix-Chevalier	DC50002-21
Micro scisors Jacobson	Delacroix-Chevalier	DC20057-21
Lung retractor	Delacroix-Chevalier	B803990
Allis clamp	Delacroix-Chevalier	DC45907-25
O’Shaugnessy Dissector	Delacroix-Chevalier	B60650
Vessel loop	Medline	VLMINY
18 blade knife	Delacroix-Chevalier	B130180
Leriche haemostatic clamp	Delacroix-Chevalier	B86555