coronary arteries anomalies

Cardiology Grand Rounds

Ahmed Almomani MDUAMS

6/22/2016

Case 1

• 17 year-old male with no past medical history collapsed in the school basketball game after episode of chest pain. CPR-AED

• At OSH, diagnosed with NSTEMI and undergoes a coronary angiogram.

Angiogram

Cardiac CT

Congenital Anomalies of the Coronary Circulation

Congenital Anomalies of the Coronary Circulation

• Coronary artery anomalies are defined as those angiographic findings in which the number, origin, course, and termination of these arteries are rarely encountered in the general population.

• May occur in 1% to 5% of patients undergoing coronary arteriography.

Maron BJ et al. N Engl J Med 2003;349:1064-1075.

Sudden Death in Young Athletes

Morgan et al. impedance and Causes of sudden Death in U.S. College Athletes. JACC, 2014

182 Sudden Deaths in NCAA athletes from 2002-2001

Congenital Anomalies of the Coronary Challenges

• Documentation of precise ischemia risk for some of these anomalies by conventional exercise stress testing or intravascular Doppler flow studies is poorly predictive.

• Malignant features of anolamous coronaries include– slitlike ostium– acute angle of takeoff– intramural course– compression between the aorta and the pulmonary trunk

Angelini P et al. Coronary Artery Anomalies: A Comprehensive Approach 1999.

Coronary Artery Anomalies

• Overall 1,686 patients of 126,595 catheterization patients in one database.

• 196 of 126,595 cases involved coronary origin from opposite sinus

Yamanaka, O. Catheterization and Cardiovascular Diagnosis. 21:28 1990.

Coronary Artery Anomalies

• Benign:– LCx from R sinus or RCA.– separate LAD and LCx ostia.

• Potentially serious: – Anomalous left coronary artery from the pulmonic artery.

(ALCAPA)– Anomalous right coronary artery from the pulmonic artery

(ARCAPA)– Coronary Artery fistula (CAF)– Anomalous coronary artery from the opposite sinus of

Valsalva (LM or LAD from R sinus or RCA from L sinus)

Yamanaka, O. Catheterization and Cardiovascular Diagnosis. 21:28 1990.

Bashore, T.M. ACCSAP8, 2012

Anomalous Coronary Artery from the Opposite Sinus (ACAOS)

Paolo Angelini Circulation. 2007;115:1296-1305

4 subtypes of ACAOS:

1. Retrocardiac2. Retroaortic3. Preaortic, or between the

aorta and pulmonary artery4. Intraseptal (supracristal)5. Prepulmonary (precardiac).


• LM or LAD origin from the proximal RCA or the right aortic sinus or vice versa, with subsequent passage between the aorta and the right ventricular outflow tract has been associated with sudden death during exercise in young persons.

• The increased risk of sudden death may be due to:– Slitlike ostium– Bend with acute takeoff angles of the aberrant coronary arteries– Arterial compression between the pulmonary trunk and aorta– intramural course


• In rare cases of anomalous origin of the LCA from the right sinus, myocardial ischemia may occur even if the LCA passes anterior to the right ventricular outflow tract or posterior to the aorta.

• Although CABG has been the traditional revascularization approach in patients with ACAOS, coronary stenting also has been reported to yield acceptable medium-term success.

LCx from RCA

Braunwald’s Heart Disease 10th edition

Benign


Krasuski et al. Circulation. 2011;123:154-162

RCA from left coronary sinus


Anomalous Coronary from Opposite sinus of Valsalva, How Frequent?

• 301 patients out of 210,700 cardiac catheterizations.

• 79% of the 301 were anomalous RCA from L sinus.

• 21% of the 301 were anomalous LM from R sinus.

• 54 of the 301 (21%) had interarterial course.

Krasuski et al. Circulation. 2011;123:154-162

Anomalous Coronary Artery from the Opposite Sinus (ACAOS), Diagnosis?

• Diagnosis– Angiography

• ROA• “laid-back” aortogram• IVUS

– Cardiac CT with 3D.– CMR

ACC/AHA Adult Congenital Guidelines for Management of Anomalous Coronary Origin• Class I:

– Surgical coronary revascularization for:• Anomalous LM from R sinus with course between Aorta

and PA (LOE B)• Anomalous RCA from L sinus with course between the

great vessels and documented ischemia (LOE B)• Documented ischemia with anomalous coronary course

between the great arteries (LOE B)

Warnes CA, et al. Circulation, 118:714, 2008.

ACC/AHA Adult Congenital Guidelines for Management of Anomalous Coronary Origin• Class IIa:

– Surgical coronary revascularization can be beneficial in the setting of documented vascular wall hypoplasia, coronary compression, or obstruction to coronary flow, regardless of documented ischemia (LOE C).

• Class IIb: – Surgical revascularization may be reasonable in

patients with anomalous LAD artery coursing between the aorta and PA (LOE C).


Surgical repair: unroofing to create an neo-ostium

• This is usually an intramural segment in the wall of the aorta with either the RCA or the LAD from the opposite sinus of valsalva

• CABG (Problems with competitive flow)

Frommelt, P et al. JACC, 42:148, 2003

36th Bethesda Conference in 2005

• Task Forces examine data and provide recommendations on eligibility for competitive athletics in patients with various cardiac condictions.– Coronary artery from the opposite sinus with an

interarterual course should EXCLUDE participation from all competitive sport.

– Participation 3 months post-surgery allowed in the absence of ischemia, arrhythmias, or LV dysfunction during max exercise.

Extra!!

• Abnormal origin of the LAD artery from the RCA or right coronary cusp is present in 3-7% of patients with tetralogy of Fallot.

• It usually travels anteriorly to the pulmonary outflow tract and can be damaged during tetralogy of Fallot surgical RV outflow tract repair.

• D-transposition and L-transposition!!

Case 2

• 20 year-old collage student who presented to your clinic with symptoms of CHF which are getting worse for the last year. ROS was positive for exertional chest pain that he had as far as he remembers and though it was normal.

Angiogram

Anomalous Pulmonary Origin of the Coronary Arteries (APOCA)

• The most common variant of this syndrome is an anomalous origin of the LCA from the pulmonary artery (ALCAPA).– Other variants: ARCAPA or single left vessel (LCx or LAD)

• Untreated, in the absence of an adequate collateral network, most infants (95%) die within the first year. In the presence of an extensive collateral network, patients may survive into adulthood, likely with ICM.

Anomalous Origin of the LCA from the Pulmonary Artery (ALCAPA)

• Aortography findings: Large RCA Absence of a left coronary ostium. Late phase of the, LAD and LCx branches fill by

collateral circulation from RCA branches. Later in the filming sequence, retrograde flow from the

LAD and LCx arteries opacifies the LMCA and its origin from the main pulmonary artery.

• Findings can be confirmed with Cardiac CT.

The Surgical Approach to ALCAPA 1. In young children, ligation and a left subclavian artery to LAD artery approach can

be used.

2. Ligation with subsequent bypass grafting can be done, though competitive flow places a disadvantage for graft survival on either the left internal mammary artery or saphenous vein bypass.

3. The Takeuchi procedure can be performed. This amounts to creating an aortopulmonary window, then placing a tunnel from the aorta to the orifice of the anomalous coronary. The tunnel traverses the PA to supply blood directly into the coronary.

4. The coronary and a button of tissue can be removed from the PA and then reattached to the aorta. Current guidelines recommend noninvasive testing for ischemia every 3-5 years after repair of ALCAPA.

Simply Treatment is Surgical

With CBAG or other approach

Coronary Artery FistulasAbnormal Destination

• Coronary artery fistula is defined as an abnormal communication between a coronary artery and a cardiac chamber or a major vessel.

• RCA in involved in about 50% of the cases.

• May become quite large and create a measurable left-to-right shunt.

• Coronary arteriography is the best method for demonstration of the origin of these fistulas. Also echo, MRI and CT– Congenital– Iatrogenic

Coronary-Cameral fistula


Congenital fistula to the pulmonary artery


Coronary Artery Fistulas, Treat or not to treat?

• The decision as to when to close these fistulae remains controversial.

• Should be closes if:– volume overload due to the shunt.– myocardial ischemia (due to coronary steal)– arrhythmia.– unexplained LV dysfunction

• Many can be closed using percutaneous catheter methods.

ACC/AHA Adult Congenital Guidelines for Management of Anomalous Coronary Origin• Class I

– If a continuous murmur is present, its origin should be defined by echocardiography, MRI, CT angiography, or cardiac catheterization (LOE C).

– A large fistula should be closed, regardless of symptomatology, via either a transcatheter or surgical approach, if feasible (LOE C).

– A small or moderate fistula should be closed if there is evidence for myocardial ischemia, arrhythmia, or otherwise unexplained systolic or diastolic dysfunction or enlargement, or endarteritis, if feasible (LOE C).


ACC/AHA Adult Congenital Guidelines for Management of Anomalous Coronary Origin• Class IIa

– Clinical follow-up with echocardiography every 3-5 years can be useful for patients with asymptomatic, small fistula to exclude the development of chamber enlargement (LOE C).


Congenital Coronary Stenosis of Atresia (Abnormal Wall)

• Congenital stenosis or atresia of a coronary artery can occur as an isolated lesion or in association with other congenital diseases:

• Calcific coronary sclerosis• Supravalvular aortic stenosis• Homocystinuria• Friedreich ataxia

• Coronary Artery Aneurysm.

RCA Ostial Atresia and Vieussens’ Ring

Saremi f, et al J Am Coll Cardiol Img. 2011;4(12):1320-1323

Take Home...1. Coronary anomalies are common, but rarely cause

symptoms. 2. They can be divided into anomalies of origin, course, vessel

wall, and destination. 3. It is important to understand which anomalies are expected

when evaluating patients with congenital heart disease. 4. The recognition of which anomalies can result in significant

clinical consequences, including sudden death, is important, as many of the coronary anomalies are first encountered fortuitously during routine coronary angiography.

Thank you

coronary arteries anomalies

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