Late Presentation of Abnormal Origin of Coronary Arteries from the

Pulmonary Artery: two case report

Hassiba Smail, MD, Andrei Baciu, MD, Francois Bouchart, MD, Pierre-Yves Litzler, MD, PhD

Department of Thoracic and Cardiovascular Surgery, Rouen University Hospital Charles Nicolle

Rouen - France.

Word count:

Correspondence and reprint requests to:

Hassiba Smail, MD
Department of Thoracic and Cardiovascular Surgery
Charles Nicolle University Hospital
1, rue de Germont
76000 Rouen - FRANCE
Tel: +33 6 76 21 30 63
Fax: +33 2 32 88 84 59
E-mail: hassibasmail@yahoo.fr

Abstract: 100 mots

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Late presentation of abnormal origin of coronary arteries from the pulmonary artery (ACAPA)

is uncommon, we described two contrasting clinical presentation and outcome of late

abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) in young

woman of 16 years old year succumbing sudden death, and abnormal origin of the right

coronary artery from the pulmonary artery (ARCAPA) in 66 years old man with mild

symptoms. They had the same surgical approach but an opposing outcome, while the young

woman is life treating 3 years after the surgery, the aged man dead one year after the surgery.

Cases report: 1500 MOTS

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We report the case of a young female of 16-year-old, without any family history of cardiac

disease, who developed cardiac arrest during sport class. After a successful cardiopulmonary

resuscitation, the EKG showed signs of anterolateral myocardial infarction. She was

immediately brought up to the hospital where an acute intracranial pathology was excluded

with a normal head CT scan. An urgent coronarography was performed that showed the

absence of the common trunk from aortic sinus and a communication between the left

coronary artery and the pulmonary artery. The ejection fraction was estimated at 63 %. A CT

coronary angiogram was performed that identified the anomalous origin of the left main

coronary artery arising from the pulmonary artery with an increased diameter of the coronary

arteries and a highly developed collaterally between the right and the left coronary artery,

confirming the ALCAPA syndrome.

A subsequent MRI showed left-right shunt rapport (QP/QS) of 1.4 and sub endocardial

delayed gadolinium enhancement in the lateral territory, consistent with partial (30%)

thickness infarct in the distribution of the left main artery. The patient underwent an urgent

surgical correction with implantation of the left coronary trunk in the corresponding aortic

sinus. The left coronary ostium was found as emerging from the posterior sinus of the

pulmonary artery closely situated of the left commissure. The ostium of the left coronary

artery was dissected and excised from the pulmonary artery root, extended towards upwards

and to the right, creating an adequate sized cuff of about 6 to 8 mm. Reimplantation of the

LMCA was performed directly on the posterior sinus of Valsalva on the aorta without any

tension using a continuous suture with prolene 6-0. The main pulmonary artery was

reconstructed with an autologous pericardial patch. The patient postoperative recovery was

uneventful,

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At 2 years follow-up the patient was asymptomatic, under beta-blocker treatment, with an

ejection fraction of 64%, A CT coronary angiogram confirmed a good flow through the

reimplanted LMCA (Figure 1 C). These findings allowed her to restart physical activity.

The second case we report is of a 66-year-old patient with antecedents of hypertension,

dyslipidemia, smoking and a two years history of effort dyspnea (NYHA II). Physical exam

was normal, The EKG revealed left anterior hemi-block, echocardiography revealed a systolic

dysfunction with a FE of 40%. An exercise stress test was performed with positive result. A

cardiac catheterization was subsequently performed which revealed the diagnosis of ARCAPA

syndrome.

The cardiac MRI confirmed the diagnosis of ARCAPA. It also showed a moderated LV

hypokinesia, viability.

The patient underwent surgical correction with the implantation of the right coronary artery a

couple of days later.

In situ findings showed the right coronary artery emerging from the right anterior sinus of the

pulmonary artery. The first part of the right coronary artery was carefully dissected then the

coronary ostia was cut out and implanted on the anterior face of the aorta without any traction

or folding. The post operative course was marked by an acute episode of kidney failure, which

required dialysis and a brief arrhythmia both of them reversed and the patient was discharged

at 10 days post operative. Unfortunately, we lost track of the patient until we were informed

two years later that the patient succumbed to sudden death. No autopsy was performed.

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Discussion

We previously described 2 case reports of late presentation of anomalous coronary artery

arising from the pulmonary artery (ACAPA) that have several opponent elements; a 66-year-

old patient diagnosed with ARCAPA with mild symptoms and a young female of 16-year-old,

free of symptoms until she developed cardiac arrest, later diagnosed with ALCAPA. They

both underwent surgical correction of the respective coronary artery, but again with 2 different

outcomes. The patient diagnosed with ALCAPA had a full recovery to the point where she

restarted physical activity, and, in opposition, the patient diagnosed with ARCAPA succumbed

to sudden death 2 years after the surgical intervention.

ACAPA accounts for approximately 0.01 pour cent of the patients undergoing coronary

angiography, being the second most frequent congenitally artery anomaly (3).

ARCAPA is an extremely rare anomaly, the first case being described in 1885 by Brooks, with

an incidence of 0,002 per cent, until now having less than 100 reported cases, with ages

ranging from 1 day to 90 years old (2). ALCAPA has an incidence estimated at 1/300.000 live

births counting for 0.24 % to 0.46 % of congenital cardiac diseases and the first reported case

was described by Konstantinowitsch in 1906 of a 2-day-old infant (1). The explanation for

ALCAPA highest incidence compared to ARCAPA is the proximity of the left coronary bud to

the pulmonary artery sinus (2).

There are 2 types of ALCAPA: the infant type where 90 pour cent of infants presenting this

syndrome die within the first year of life due to myocardial infarction and congestive heart

failure, and the adult type where those who did not died often present mitral insufficiency,

myocardial ischemia and heart failure further in life (4). In contrast with ALCAPA, which is a

symptomatic life -threatening condition, most of the patient diagnosed with ARCAPA remains

asymptomatic until adulthood. When ARCAPA patients become symptomatic, the clinical

presentation varies from dyspnea, fatigue and angina to congestive heart failure and

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myocardial infarction (9 %). There are series that show a 17 % of cases presenting cardiac

arrest without any preceding symptoms (7).

The left coronary artery supplies more blood to the myocardium, which explains why

ALCAPA has a worse symptomatology and prognosis.

Our late presentation cases perfectly fit into the general description above, ALCAPA

syndrome on young female of 16-year-old a free of symptoms until developed cardiac arrest

and ARCAPA syndrome on a 66-year-old patient with mild symptomatology.

Even thought in our cases the diagnosis wasn't put with the help of echocardiography, there

are direct and indirect criteria for diagnosing the abnormal origin of one of the coronary

arteries. As a direct sign the abnormal origin of the coronary artery can be detected as well as

the retrograde coronary artery flow. As indirect signs that can be found are abundant

intercoronary septal collaterals with anterograde flow for ARCAPA and retrograde flow for

ALCAPA and dilatation of contralateral normally originated coronary artery.

RMN: viability

In the case of the young patient presenting with cardiac arrest and diagnosed with ARCAPA

the surgical correction was unquestionable the treatment of choice. Current surgical indication

is the restoration of a dual-coronary system (6).

However 3 surgical procedures have been proposed for the correction of ALCAPA. Ligation

of the ALCAPA, the use of saphenous vein graft, and the reimplantation technique through the

translocation procedure with or without different materials reconstruction or Takeuchi

technique. The surgical indication for Takeuchi technique is considered when left coronary

artery arise too distant for direct implantation on the aorta, and the pulmonary artery trunk is

sufficiently dilated so the creation of the intrapulmonary tunnel wouldnt cause pulmonary

stenosis, or when the collaterals or non-compliant vessels around the left main trunk are

developed and mobilization would be aggressive (6). Even if the ostium of the left coronary

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artery on the pulmonary artery arose from the posterior facing sinus close to the left

commissure the translocation was possible the distance to go was only of a couple of

millimeters. The left coronary artery was implanted into the posterior sinus of Valsalva with

no tension using an extension created with an auto-pulmonary flap and a symmetric aortic

flap.

With positive result rise some surgical retrospective questions knowing that two years post-op

the patient succumbed to sudden death. Even though there is an operative indication both for

ALCAPA and ARCAPA syndrome, we think that is important to compare the operative risk

with the mortality risk, especially in cases like our with and patient that has lived 66 years

with this malformation, and who presents mild symptomatology. The lack of adequate

comparative surgical and follow-up studies for late presentations of congenital malformation

and also the fact that there is no certified risk score.

Current recommendations are corrective operation even in asymptomatic patients: AGE,

RMN viability, Symptoms.

2 years follow-up the patient was asymptomatic, under beta-blocker treatment, with an

ejection fraction of 64%.

Conclusion:

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