Name: direct re-implantation of left coronary artery into the aorta in adults with anomalous origin of left coronary artery from the pulmonary artery (alcapa)
Uploaded: 2017-04-24T15:00:00
Description: Watch this Scientific Journal Video about Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery ALCAPA at JoVE

This work was supported by a grant of the Swiss Cardiovascular Foundation to RT.

The protocol follows the institutional guidelines of the human research ethics committee of the University of Zurich.
1. Preparation for Surgery
<ol>
	<li>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.</li>
	<li>Pre-medicate the patient by the oral administration of 5 mg of midazolam, 30 - 60 min prior to the induction of anesthesi...

<ol>
	<li>Dodge-Khatami, A., Mavroudis, C., Backer, C. L. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+origin+of+the+left+coronary+artery+from+the+pulmonary+artery:+collective+review+of+surgical+therapy.">Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy.</a> Ann. Thorac. Surg. 74 (3), 946-955 (2002).</li><li>Keith, J. D. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=The+anomalous+origin+of+the+left+coronary+artery+from+the+pulmonary+artery.">The anomalous origin of the left coronary artery from the pulmonary artery.</a> Br. Heart J. 21 (2), 149-161 (1959).</li><li>Hauser, M. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Congenital+anomalies+of+the+coronary+arteries.">Congenital anomalies of the coronary arteries.</a> Heart. 91 (9), 1240-1245 (2005).</li><li>Wesselhoeft, H., Fawcett, J. S., Johnson, A. L. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+origin+of+the+left+coronary+artery+from+the+pulmonary+trunk.+Its+clinical+spectrum,+pathology,+and+pathophysiology,+based+on+a+review+of+140+cases+with+seven+further+cases.">Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.</a> Circulation. 38 (2), 403-425 (1968).</li><li>Edwards, J. E. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=The+direction+of+blood+flow+in+coronary+arteries+arising+from+the+pulmonary+trunk.">The direction of blood flow in coronary arteries arising from the pulmonary trunk.</a> Circulation. 29 (2), 163-166 (1964).</li><li>Agustsson, M. H., Gasul, B. M., Fell, H., Graettinger, J. S., Bicoff, J. P., Waterman, D. F. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+origin+of+the+left+coronary+artery+from+the+pulmonary+artery+diagnosis+and+treatment+of+infantile+and+adult+types.">Anomalous origin of the left coronary artery from the pulmonary artery diagnosis and treatment of infantile and adult types.</a> JAMA. 180 (1), 15-21 (1962).</li><li>Roberts, S. M., Banbury, T., Mehta, A. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=A+rare+case+of+anomalous+left+coronary+artery+from+the+pulmonary+artery+(Bland-White-Garland+Syndrome)+in+a+68-year-old+woman.">A rare case of anomalous left coronary artery from the pulmonary artery (Bland-White-Garland Syndrome) in a 68-year-old woman.</a> Semin. Cardiothorac. Vasc. Anesth. , (2016).</li><li>Yau, J. M., Singh, R., Halpern, E. J., Fischman, D. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+origin+of+the+left+coronary+artery+from+the+pulmonary+artery+in+adults:+a+comprehensive+review+of+151+adult+cases+and+a+new+diagnosis+in+a+53-year-old+woman.">Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman.</a> Clin. Cardiol. 34 (4), 204-210 (2011).</li><li>Michielon, G., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+coronary+artery+origin+from+the+pulmonary+artery:+correlation+between+surgical+timing+and+left+ventricular+function+recovery.">Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery.</a> Ann. Thorac. Surg. 76 (2), 581-588 (2003).</li><li>Dodge-Khatami, A., Mavroudis, C., Backer, C. L. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Congenital+Heart+Surgery+Nomenclature+and+Database+Project:+anomalies+of+the+coronary+arteries.">Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries.</a> Ann. Thorac. Surg. 69 (4 Suppl), S270-S297 (2000).</li><li>Talwar, S., Jha, A. J., Choudhary, S. K., Gupta, S. K., Airan, B. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Repair+of+anomalous+left+coronary+artery+from+pulmonary+artery+(ALCAPA)+beyond+infancy.">Repair of anomalous left coronary artery from pulmonary artery (ALCAPA) beyond infancy.</a> Heart Surg. Forum. 16 (4), E210-E215 (2013).</li><li>Toumpourleka, M., Belitsis, G., Alonso, R., Rubens, M., Moat, N., Gatzoulis, M. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Late+presentation+and+surgical+repair+of+ALCAPA.">Late presentation and surgical repair of ALCAPA.</a> Int. J. Cardiol. 186, 207-209 (2015).</li><li>Quah, J. X., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=The+management+of+the+older+adult+patient+with+anomalous+left+coronary+artery+from+the+pulmonary+artery+syndrome:+a+presentation+of+two+cases+and+review+of+the+literature.">The management of the older adult patient with anomalous left coronary artery from the pulmonary artery syndrome: a presentation of two cases and review of the literature.</a> Congenit. Heart Dis. 9 (6), E185-E194 (2014).</li><li>Kottayil, B. P., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Anomalous+origin+of+left+coronary+artery+from+pulmonary+artery+in+older+children+and+adults:+direct+aortic+implantation.">Anomalous origin of left coronary artery from pulmonary artery in older children and adults: direct aortic implantation.</a> Ann. Thorac. Surg. 91 (2), 549-553 (2011).</li><li>Rajbanshi, B. G., Burkhart, H. M., Schaff, H. V., Daly, R. C., Phillips, S. D., Dearani, J. A. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Surgical+strategies+for+anomalous+origin+of+coronary+artery+from+pulmonary+artery+in+adults.">Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults.</a> J. Thorac. Cardiovasc. Surg. 148 (1), 220-224 (2014).</li><li>Neumann, A., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Long-term+results+after+repair+of+anomalous+origin+of+left+coronary+artery+from+the+pulmonary+artery:+Takeuchi+repair+versus+coronary+transfer.">Long-term results after repair of anomalous origin of left coronary artery from the pulmonary artery: Takeuchi repair versus coronary transfer.</a> Eur. J. Cardio-Thorac. Surg. , (2016).</li><li>Pe&#241;a, E., Nguyen, E. T., Merchant, N., Dennie, C. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=ALCAPA+syndrome:+not+just+a+pediatric+disease.">ALCAPA syndrome: not just a pediatric disease.</a> Radiographics. 29 (2), 553-565 (2009).</li></ol>

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...

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 diseases2,3. 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 (RCA) with extensive intercoronary collaterals4. During the neonatal period, high pulmonary vascular resistance and the resultant pulmonary artery (PA) pressures ensure that antegrade flow is&#160;maintained from the PA into the anomalous left coronary artery. As the pulmonary vascular resistance gradually decreases, antegrade flow to the left coronary artery reduces. This eventually leads to reversal of flow, and left-to-right shunting into the PA, thus resulting in a &#34;coronary steal5.&#34; Thus, left ventricular (LV) myocardial perfusion depends on intercoronary collaterals from an RCA5,6.
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, and malignant ventricular arrhythmias precipitated by acute myocardial ischemia7. In a subset of adult patients, the mean age at presentation is 41 years, with a shift in sex distribution toward female patients (Female-to-male ratio: 2:1)8. In this patient population, 14% are asymptomatic; 66% present with symptoms of angina, dyspnea, palpitations, or fatigue; and 17% present with life-threatening symptoms, including ventricular arrhythmias, syncope, and sudden cardiac death8. The average age of life-threatening presentation is 33 years and of sudden cardiac death 31 years8. Therefore, surgical correction is highly recommended as soon as the diagnosis is made, regardless of age or the degree of intercoronary collateralization1,9.
Depending on the origin of the anomalous left coronary artery, direct re-implantation of the ALCAPA into the aorta is the more physiologically sound repair technique to reestablish the dual-coronary perfusion system. Most commonly, ALCAPA takes off from the right-hand pulmonary sinus (sinus 1 of the PA), which faces the aortic sinus where the main left coronary artery usually originates (sinus 2 of the aorta)10. This coronary anatomy is most suited to the direct re-implantation technique. The aim of this report is to describe, in detail, the technique for the direct re-implantation of the left coronary artery in ALCAPA in adult patients. The rationale behind direct re-implantation is the advantage-the physiological reestablishment of dual-coronary perfusion-it offers over the ligation of the anomalous left coronary combined with coronary artery bypass grafting11,12,13.

<table border="1" fo:keep-together.within-page="1" fo:keep-with-next.within-page="always">
	<tbody>
		<tr>
			<td>Heart surgery infrastructure:</td>
			<td></td>
			<td></td>
		</tr>
		<tr>
			<td>Heart Lung Machine</td>
			<td>Stockert</td>
			<td>SIII</td>
		</tr>
		<tr>
			<td>EOPA 24Fr. arterial cannula</td>
			<td>Medtronic</td>
			<td>77624</td>
		</tr>
		<tr>
			<td>Quickdraw 25Fr. femoral venous cannula</td>
			<td>Edwards</td>
			<td>QD25</td>
		</tr>
		<tr>
			<td>LV vent catheter 17Fr.</td>
			<td>Edwards</td>
			<td>E061</td>
		</tr>
		<tr>
			<td>Antegrade 9Fr. cardioplegia cannula</td>
			<td>Edwards</td>
			<td>AR012V</td>
		</tr>
		<tr>
			<td>Retrograde 14Fr. cardioplegia cannula&#160;</td>
			<td>Edwards</td>
			<td>NPC014&#160;</td>
		</tr>
		<tr>
			<td>Electrocautery</td>
			<td>Covidien</td>
			<td>Force FX</td>
		</tr>
		<tr>
			<td>Name</td>
			<td>Company</td>
			<td>Catalog number</td>
		</tr>
		<tr>
			<td>Sutures:</td>
			<td></td>
			<td></td>
		</tr>
		<tr>
			<td>Polypropylene 4/0</td>
			<td>Ethicon</td>
			<td>8871H</td>
		</tr>
		<tr>
			<td>Polypropylene 5/0</td>
			<td>Ethicon</td>
			<td>8870H</td>
		</tr>
		<tr>
			<td>Polypropylene 6/0</td>
			<td>Ethicon</td>
			<td>EH7400H</td>
		</tr>
		<tr>
			<td>Braided polyesther 2/0 ligature with polybutylate coating&#160;</td>
			<td>Ethicon</td>
			<td>X305H</td>
		</tr>
		<tr>
			<td>Intergard dacron graft 8 mm</td>
			<td>Maquet</td>
			<td>IGW0008-30</td>
		</tr>
		<tr>
			<td>Micro knife Sharpoint&#160;</td>
			<td>TYCO Healthcare PTY&#160;</td>
			<td>78-6900</td>
		</tr>
		<tr>
			<td>Name</td>
			<td>Company</td>
			<td>Catalog number</td>
		</tr>
		<tr>
			<td>Drugs:</td>
			<td></td>
			<td></td>
		</tr>
		<tr>
			<td>Midazolam</td>
			<td>Roche Pharma</td>
			<td>N05CD08</td>
		</tr>
		<tr>
			<td>Rocuronium</td>
			<td><a href="https://www.compendium.ch/comp/mnr/22695/de">MSD Merck Sharp &#38; Dohme&#160;</a></td>
			<td>M03AC09</td>
		</tr>
		<tr>
			<td>Propofol</td>
			<td>Fresenius Kabi</td>
			<td>N01AX10</td>
		</tr>
		<tr>
			<td>Fentanil</td>
			<td>Actavis</td>
			<td>N01AH01</td>
		</tr>
		<tr>
			<td>Name</td>
			<td>Company</td>
			<td>Catalog number</td>
		</tr>
		<tr>
			<td>Instruments:</td>
			<td></td>
			<td></td>
		</tr>
		<tr>
			<td>Cooley Derra anastomosis clamp</td>
			<td>Delacroix-Chevalier</td>
			<td>DC40810-16</td>
		</tr>
		<tr>
			<td>Cooley vascular clamp</td>
			<td>Delacroix-Chevalier</td>
			<td>DC40810-16</td>
		</tr>
		<tr>
			<td>Dissection forceps Carpentier</td>
			<td>Delacroix-Chevalier</td>
			<td>DC13110-28&#160;</td>
		</tr>
		<tr>
			<td>Scissors Metzenbaum</td>
			<td>Delacroix-Chevalier</td>
			<td>B351751</td>
		</tr>
		<tr>
			<td>Needle holder Ryder</td>
			<td>Delacroix-Chevalier</td>
			<td>DC51130-20&#160;</td>
		</tr>
		<tr>
			<td>Dissection forceps DeBakey</td>
			<td>Delacroix-Chevalier</td>
			<td>DC12000-21&#160;</td>
		</tr>
		<tr>
			<td>Micro needle holder Jacobson</td>
			<td>Delacroix-Chevalier</td>
			<td>DC50002-21&#160;</td>
		</tr>
		<tr>
			<td>Micro scisors Jacobson</td>
			<td>Delacroix-Chevalier</td>
			<td>DC20057-21&#160;</td>
		</tr>
		<tr>
			<td>Lung retractor</td>
			<td>Delacroix-Chevalier</td>
			<td>B803990</td>
		</tr>
		<tr>
			<td>Allis clamp</td>
			<td>Delacroix-Chevalier</td>
			<td>DC45907-25&#160;</td>
		</tr>
		<tr>
			<td>O&#8217;Shaugnessy Dissector</td>
			<td>Delacroix-Chevalier</td>
			<td>B60650</td>
		</tr>
		<tr>
			<td>Vessel loop</td>
			<td>Medline</td>
			<td>VLMINY</td>
		</tr>
		<tr>
			<td>18 blade knife</td>
			<td>Delacroix-Chevalier</td>
			<td>B130180</td>
		</tr>
		<tr>
			<td>Leriche haemostatic clamp</td>
			<td>Delacroix-Chevalier</td>
			<td>B86555</td>
		</tr>
	</tbody>
</table>

direct re-implantation of left coronary artery into the aorta in adults with anomalous origin of left coronary artery from the pulmonary artery (alcapa)

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.

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...

Watch this Scientific Journal Video about Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery ALCAPA at JoVE

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

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.

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

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 ...

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