scimitar syndrome presenting in adults

16
J CARD SURG 2008;23:63-78 REDDY, ET AL. REGIONAL RECONSTITUTION OF IMA 63 CASE REPORTS Regional Variation in the Reconstitution of Flow in the Internal Mammary Artery Graft Proddutur R. Reddy, M.D., Mamdouh Bakhos, M.D.,and Ferdinand Leya, M.D. Department of Medicine, Division of Cardiology; and Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, Maywood, Illinois ABSTRACT We report an 81-year-old man with coronary artery disease and bypass surgery with a sequential internal mammary artery (IMA) to the diagonal and then the anterior descending, who developed regional variations in the flow through his arterial conduit. Four years after his initial surgery, he developed atresia of the proximal seg- ment of the arterial conduit due to competitive flow. After reoperation, the patient reconstituted flow in his proximal segment, but developed atre- sia of the distal segment. We describe for the first time, regional variation in arterial conduit pa- tency and discuss factors controlling patency in the sequential arterial conduit. doi: 10.1111/j.1540- 8191.2007.00492.x (J Card Surg 2008;23:63-65) The internal mammary artery (IMA) has properties unique from saphenous vein grafts (SVG) that distin- guish it as the ideal bypass conduit. Atresia of arterial grafts after thrombosis or low flow states has been previously described, including its reversal to patent flow. 1 We describe for the first time a case demon- strating regional patency and regional reconstitution in a sequential IMA conduit. CASE REPORT An 81-year-old man was evaluated and treated at an outside hospital in 2000 for dyspnea on exertion and fatigue. Cardiac catheterization revealed a normal left ventricle but severe mitral regurgitation (MR) and triple vessel coronary disease. He underwent mitral annu- loplasty and bypass surgery including a sequential in- ternal mammary artery (IMA) to the first diagonal (D1) Address for correspondence: Proddutur R. Reddy, M.D., Department of Medicine, Division of Cardiology, Loyola University Medical Center, Bld. 110, Room 6231, 2160 S. First Ave., Maywood, IL 60153. Fax: 708-327-2771; e-mail: Dr [email protected] No funding was involved in this work. There is no conflict of interest with any of the authors. branch then to the left anterior descending (LAD) artery. Four years later, he presented to our hospital with recur- rent symptoms. Cardiac catheterization revealed mildly dilated left ventricle with preserved ejection fraction, severe MR, severe tricuspid regurgitation, and moder- ate coronary artery disease. The IMA graft to D1 was patent, but the sequential segment of the IMA from D1 to LAD had become atretic (Fig. 1). The patient un- derwent a second operation with mitral and tricuspid valve repair and single vessel bypass with saphenous vein grafts (SVG) to the mid-LAD (distal to the previous IMA graft). Most recently, the patient presented again with symptoms of dyspnea on exertion and fatigue. Cardiac catheterization revealed mild MR, mild aortic stenosis, and moderate coronary artery disease. The diagonal was occluded proximally, the mid-LAD steno- sis worsened, and the SVG’s were patent. The proximal IMA segment grafted to D1 had now become atretic, but the distal IMA segment connecting D1 to LAD was now widely patent (Fig. 2) with reverse flow from the well-perfused LAD toward D1. The atretic distal internal thoracic artery (IMA) seg- ment was likely due to competitive flow from the na- tive LAD. The larger SVG to LAD had significantly more blood flow to supply not only the LAD territory, but also the D1. As a result, the proximal IMA now became atretic while the distal IMA was reconstituted. DISCUSSION The IMA has vascular properties unlike the SVG that give them long-term patency advantages. The patency rates of the IMA approaches 99% at one year and from 85% to 95% in 10 years. 2 Unlike arterial conduits, SVG’s are more susceptible to intimal hyperplasia, ar- teriosclerosis, progressive stenosis, thrombus forma- tion, and occlusion. The one-year patency rates are from 80% to 90%. However, this significantly decrease thereafter: 1% to 2% per year occlusion rate for the first five years up to 4% to 5% occlusion rate from years 5 through 10. Overall the 10-year patency rates of SVG’s are only 60% and only 30% of those are free of any stenosis. 3 These advantages in patency have led to better survival and freedom from ischemic events in patients with multiple grafts including the IMA. 4 The use of the IMA, particularly to the LAD, is the standard of care. Because of improved patency rates, the IMA is se- quentially grafted to one or more diagonal branches and occasionally to two sites on the LAD. This prac- tice has been proven effective and is practiced by many surgeons; 5 however, the greatest risk is the potential to jeopardize the distal IMA, which is usually the most important anastamosis. IMA failures are infre- quent and most often occur due to intimal compromise and thrombosis secondary to faulty technique, severe

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J CARD SURG2008;23:63-78

REDDY, ET AL.REGIONAL RECONSTITUTION OF IMA

63

CASE REPORTS

Regional Variation in theReconstitution of Flow in theInternal MammaryArtery Graft

Proddutur R. Reddy, M.D.,∗ MamdouhBakhos, M.D.,† and Ferdinand Leya,M.D.∗

∗Department of Medicine, Division ofCardiology; and †Department of Thoracicand Cardiovascular Surgery, LoyolaUniversity Medical Center, Maywood, Illinois

ABSTRACT We report an 81-year-old man withcoronary artery disease and bypass surgery witha sequential internal mammary artery (IMA) to thediagonal and then the anterior descending, whodeveloped regional variations in the flow throughhis arterial conduit. Four years after his initialsurgery, he developed atresia of the proximal seg-ment of the arterial conduit due to competitiveflow. After reoperation, the patient reconstitutedflow in his proximal segment, but developed atre-sia of the distal segment. We describe for thefirst time, regional variation in arterial conduit pa-tency and discuss factors controlling patency in thesequential arterial conduit. doi: 10.1111/j.1540-8191.2007.00492.x (J Card Surg 2008;23:63-65)

The internal mammary artery (IMA) has properties

unique from saphenous vein grafts (SVG) that distin-

guish it as the ideal bypass conduit. Atresia of arterial

grafts after thrombosis or low flow states has been

previously described, including its reversal to patent

flow.1 We describe for the first time a case demon-

strating regional patency and regional reconstitution in

a sequential IMA conduit.

CASE REPORT

An 81-year-old man was evaluated and treated at an

outside hospital in 2000 for dyspnea on exertion and

fatigue. Cardiac catheterization revealed a normal left

ventricle but severe mitral regurgitation (MR) and triple

vessel coronary disease. He underwent mitral annu-

loplasty and bypass surgery including a sequential in-

ternal mammary artery (IMA) to the first diagonal (D1)

Address for correspondence: Proddutur R. Reddy, M.D., Department

of Medicine, Division of Cardiology, Loyola University Medical Center,

Bld. 110, Room 6231, 2160 S. First Ave., Maywood, IL 60153. Fax:

708-327-2771; e-mail: Dr [email protected]

No funding was involved in this work.

There is no conflict of interest with any of the authors.

branch then to the left anterior descending (LAD) artery.

Four years later, he presented to our hospital with recur-

rent symptoms. Cardiac catheterization revealed mildly

dilated left ventricle with preserved ejection fraction,

severe MR, severe tricuspid regurgitation, and moder-

ate coronary artery disease. The IMA graft to D1 was

patent, but the sequential segment of the IMA from

D1 to LAD had become atretic (Fig. 1). The patient un-

derwent a second operation with mitral and tricuspid

valve repair and single vessel bypass with saphenous

vein grafts (SVG) to the mid-LAD (distal to the previous

IMA graft). Most recently, the patient presented again

with symptoms of dyspnea on exertion and fatigue.

Cardiac catheterization revealed mild MR, mild aortic

stenosis, and moderate coronary artery disease. The

diagonal was occluded proximally, the mid-LAD steno-

sis worsened, and the SVG’s were patent. The proximal

IMA segment grafted to D1 had now become atretic,

but the distal IMA segment connecting D1 to LAD was

now widely patent (Fig. 2) with reverse flow from the

well-perfused LAD toward D1.

The atretic distal internal thoracic artery (IMA) seg-

ment was likely due to competitive flow from the na-

tive LAD. The larger SVG to LAD had significantly more

blood flow to supply not only the LAD territory, but also

the D1. As a result, the proximal IMA now became

atretic while the distal IMA was reconstituted.

DISCUSSION

The IMA has vascular properties unlike the SVG that

give them long-term patency advantages. The patency

rates of the IMA approaches 99% at one year and

from 85% to 95% in 10 years.2 Unlike arterial conduits,

SVG’s are more susceptible to intimal hyperplasia, ar-

teriosclerosis, progressive stenosis, thrombus forma-

tion, and occlusion. The one-year patency rates are

from 80% to 90%. However, this significantly decrease

thereafter: 1% to 2% per year occlusion rate for the

first five years up to 4% to 5% occlusion rate from

years 5 through 10. Overall the 10-year patency rates

of SVG’s are only 60% and only 30% of those are free

of any stenosis.3 These advantages in patency have led

to better survival and freedom from ischemic events in

patients with multiple grafts including the IMA.4 The

use of the IMA, particularly to the LAD, is the standard

of care.

Because of improved patency rates, the IMA is se-

quentially grafted to one or more diagonal branches

and occasionally to two sites on the LAD. This prac-

tice has been proven effective and is practiced by

many surgeons;5 however, the greatest risk is the

potential to jeopardize the distal IMA, which is usually

the most important anastamosis. IMA failures are infre-

quent and most often occur due to intimal compromise

and thrombosis secondary to faulty technique, severe

64 REDDY, ET AL.REGIONAL RECONSTITUTION OF IMA

J CARD SURG2008;23:63-78

Figure 1. Proximal segment of the sequential IMA to D1 ispatent and the distal segment of the IMA graft from D1 to LADis atretic. A = Proximal segment of sequential IMA to D1;B = Native first diagonal; C = Distal segment of sequentialIMA from D1 to LAD; D = Native LAD.

coronary artery disease, or competitive flow causing

thrombosis.

Arterial conduits are able to autoregulate their flow

based on demand. Competitive flow from patent na-

tive arteries is important in determining patency and

flow-induced thrombosis of the arterial graft. Vein

grafts are not bothered by competitive flow because

of their larger size. The IMA is also disadvantaged be-

Figure 2. Proximal segment of the sequential IMA to D1 isclosed and the distal segment of the IMA graft from D1 to LADnow patent. B = Native first diagonal; C = Distal segment ofsequential IMA from D1 to LAD; D = Native LAD; E = SVGgraft to LAD.

cause the relative resistance to flow is proportional

to the greater length of travel from the subclavian

artery.

Initial studies examining the importance of native

artery stenosis prior to IMA anastamosis were conflict-

ing. Canine experiments with arterial conduits to non-

stenotic native arteries were shown to remain patent;

however, the follow-up was only two months.6 The ini-

tial f/u studies with arterial conduits in humans with

patent native arteries were also relatively short term.7

More recently, long-term follow-up has suggested that

degree of stenosis of native vessel is a major predic-

tor of arterial graft patency. Occlusion of the IMA was

found to be as high as 79% in arteries without signifi-

cant native artery stenosis.8 As a result, the SVG may

be better conduits than arteries when bypassing mod-

erately stenotic native vessels because of their resis-

tance to closure with competitive flow. In fact, because

of its large size, the right coronary artery (RCA) may

demonstrate greater competitive flow than the LAD

system. As a result, the IMA is rarely used for RCA

lesions.9

Baseline patency in arterial conduits is dependent

on vasorelaxant factors that allow control of blood flow

based on oxygen demand. This process is invaluable

because oxygen extraction within the cardiac myocytes

is already maximal and further increases in oxygen de-

livery can only be maintained by increased blood flow.

Nitric oxide (NO) and prostacyclin are potent vasodila-

tor produced by vascular endothelial cells. In addition

to basal vascular patency, NO also significantly reduces

catecholamine-induced vasoconstriction. Hypoxia is a

potent stimulant for the release of endothelium-derived

NO and prostacyclin.10 Veins produce less prostacyclin

than arterial grafts11 and have less NO-mediated re-

laxation.12 Baseline NO is 50% higher in human IMA

than in SVG.13 Other vasodilators known to provoke

the release of or increase vascular sensitivity to NO in-

clude endothelium-derived relaxing factor,12 vascular

endothelial growth,13 and platelet-derived adenosine

nucleotides.14 These protective mechanisms are either

absent or decreased in the SVG.

Our case is unique because it reveals that the IMA

endothelium is not only “physiologically active” but its

dynamic adaptive capacity is complex and highly local-

ized. Mechanisms for arterial patency have more re-

gional variation than previously reported. Dincer and

Barner described the restoration of patency in an ap-

parently occluded IMA. However, the patient did not

have a sequential graft or demonstrate this degree of

regional variation.1 More commonly, the entire IMA will

become atretic, or occasionally, just the distal portion

will close. It is known that the distal part of the IMA

is more muscular and less elastic, and therefore, more

prone to vasomotor response.15 The change in luminal

patency in our patient occurred over at least a two-

year period, suggesting that the conduit maintains its

ability to vasodilate or constrict years after the initial

surgery.

This case demonstrates that arterial grafts have

highly regionalized mechanisms to control luminal pa-

J CARD SURG2008;23:63-78

MACKIE AND CLEMENTSLEFT VENTRICULAR TO CORONARY SINUS FISTULA

65

tency that remain viable for years. Further investi-

gation into the mechanism and extent of regional-

ity may clinically improve grafting patterns and our

understanding of hemodynamic effects on vascular

physiology.

REFERENCES

1. Dincer B, Barner HB: The “occluded” internal mammary

artery graft: Restoration of patency after apparent occlu-

sion associated with progression of coronary disease. J

Thorac Cardiovasc Surg 1983;85(2):318-320.

2. Tatoulis J, Buxton BF, Fuller JA: Patencies of 2127 arte-

rial to coronary conduits over 15 years. Ann Thorac Surg

2004;77:93-101.

3. Fitzgibbon GM, Kafka HP, Leach AJ, et al: Coronary by-

pass graft fate and patient outcome: Angiographic follow-

up of 5,065 grafts related to survival and reoperation

in 1,388 patients during 25 years. J Am Coll Cardiol

1996;28:616-626.

4. Cameron A, Davis KB, Green G, et al: Coronary bypass

surgery with internal-artery-grafts on 10-year survival and

other cardiac events. N Eng J Med 1986;314:1-6.

5. Dion R, Glineur D, Derouck D, et al: Long-term clinical

and angiographic follow-up of sequential internal thoracic

artery grafting. Europ J Cardiothorac Surg 2000;17:407-

414.

6. Lust RM, Zeri RS, Spence PA, et al: Effect of chronic

native flow competition on internal thoracic artery grafts.

Ann Thorac Surg 1994;57:45-50.

7. Sabik JF, Lytle BW, Blackstone EH, et al: Does compet-

itive flow reduce internal thoracic artery graft patency?

Ann Thorac Surg 2003;76:1490-1497.

8. Berger A, MacCarthy PA, Siebert U, et al: Long-term pa-

tency of internal mammary artery bypass grafts. Circula-

tion 2004;110(2 Suppl):II-36-II-40.

9. Sabik JF, Lytle BW, Blackstone EH, et al: Comparison

of saphenous vein and internal thoracic artery graft pa-

tency in coronary system. Ann Thorac Surg 2005;79:544-

551.

10. Pearson PJ, Evora PR, Discigil B, et al: Hypoxia in-

creases vasodilator release from internal mammary artery

and saphenous vein grafts. Ann Thorac Surg 1998

May;65(5):1220-1225.

11. Chaikhouni A, Crawford FA, Kochel PJ, et al: Human in-

ternal mammary artery produces more prostacyclin than

saphenous vein. J Thorac Cardiovasc Surg 1986;92:88-

91.

12. Luscher TF, Diederich D, Siebenmann R, et al: Differ-

ences between endothelium-dependent relaxation in ar-

terial and in venous coronary bypass grafts. N Eng J Med

1988;319(8):462-467.

13. Broeders MA, Doevendans PA, Maessen JG, et al: The

human internal thoracic artery releases more nitric oxide

in response to vascular endothelial growth factor than

the human saphenous vein. J Thorac Cardiovasc Surg

2001;122:305-309.

14. Yang Z, Luscher TF: Basic cellular mechanisms of

coronary bypass graft disease. Eur Heart J 1993;14(1

Suppl):193-197.

15. Angelini GD, Bryan AJ, Dion R: Surgical anatomy and

histological characteristics. In Van Son JA, Smedts FM

(eds): Arterial Conduits in Myocardial Revascularization.

1st ed. New York: Oxford University Press; 1996, pp.

13-22.

Left Ventricular to CoronarySinus Fistula Following MultipleMitral Valve ReplacementSurgeries

Benjamin D. Mackie, M.D.,∗ and StephenJ. Clements, M.D.†∗Department of Medicine, J. Willis HurstInternal Medicine Residency Program,Emory University School of Medicine,Atlanta, Georgia; and †Division ofCardiology, Department of Medicine, EmoryUniversity School of Medicine, Atlanta,Georgia

ABSTRACT Development of left ventricular to coro-nary sinus fistula is a rare complication of mitralvalve surgery. Three of the seven previously re-ported cases occurred following multiple valve re-placement surgeries, all of which were thought tobe secondary to a complication of surgery and allwere treated with surgical closure of the fistula.We report a case of left ventricular to coronary si-nus fistula occurring after two mitral valve replace-ment surgeries that was treated medically withfavorable long-term results. doi: 10.1111/j.1540-8191.2007.00491.x (J Card Surg 2008;23:65-67)

Frank Gerbode first described congenital left ventric-

ular to right atrial shunts in 1958. However, Chambers

and Rogers were the first to describe a left ventricular

to coronary sinus fistula following mitral valve replace-

ment in 1972.1,2 Since that time, additional cases have

been reported; taken together they suggest a greater

risk of fistula formation following multiple mitral valve

replacement surgeries and/or after excessive debride-

ment of the mitral valve annulus.3-7 Our case describes

a patient with late development, five years out from the

second mitral valve replacement, of a left ventricular to

coronary sinus fistula who presented with a two-month

history of increased dyspnea on exertion and new car-

diac murmur.

CASE REPORT

A 77-year-old female, with no previous cardiac his-

tory, originally presented with ruptured chordae ten-

dinae and underwent mitral valve replacement with

a 29 mm Hancock tissue valve at that time. She did

well postoperatively until four years later, when she

developed severe mitral regurgitation requiring a sec-

ond valve replacement, again with a 29 mm Hancock

valve. The postoperative course was uneventful except

Address for correspondence: Benjamin D. Mackie, 69 Jesse Hill Drive,

Atlanta, GA 30303. Fax: 404-778-3508; e-mail: [email protected]

66 MACKIE AND CLEMENTSLEFT VENTRICULAR TO CORONARY SINUS FISTULA

J CARD SURG2008;23:63-78

Figure 1. Cardiac angiography showing origin of LV to coro-nary sinus fistula depicted by the white arrow. Other struc-tures include the right atrium = RA; coronary sinus = CS;right ventricle = RV; metallic ring of Hancock valve in the mi-tral position = MV; and ascending aorta = Ao.

for bradycardia for which a permanent pacemaker was

placed.

A year later, she presented with increasing dys-

pnea on exertion, shortness of breath at rest, and

fatigue. Physical exam showed a blood pressure of

152/78 mmHg, pulse of 80 beats/min, respiratory rate

of 20 breaths/min, and a temperature of 96.5 ◦F. She

was in no acute distress. Cardiac auscultation revealed

a regular rate and rhythm, normal S1 and S2 with a III/VI

holosystolic murmur loudest at the left lower sternal

border and apex, radiating to the neck and axilla. Vis-

ible jugular venous distention to the angle of her jaw

at 45 degrees was observed and hepatojugular reflux

was present. Lung fields were clear to auscultation.

Abdominal exam showed right upper quadrant tender-

ness and a liver spanning 8 cm. Trace pretibial edema

was present.

Electrocardiogram showed a paced rhythm. Chest

X-ray showed mild cardiomegaly and left atrial enlarge-

ment. Routine labs were within normal limits.

Further evaluation with cardiac catheterization re-

vealed a fistula between the posterior left ventricular

wall and the coronary sinus leading to opacification of

the coronary sinus, the right atrium, and both cavae

(Fig. 1). The shunt ratio was 1.75/1 and pulmonary

artery pressures were 45/18 mmHg with a mean pul-

monary capillary wedge pressure of 16 mmHg without

a large v-wave. Left ventricular ejection fraction was

65%.

Following cardiac catheterization, a transthoracic

echocardiogram showed a high-velocity jet inferior to

the mitral valve and high-velocity systolic flow into the

coronary sinus and right atrium, both consistent with a

left ventricular to coronary sinus fistula. The patient im-

proved symptomatically with optimization of her blood

pressure, diuresis, and the addition of verapamil to her

medical regimen. She deferred any additional surgeries

and continued to do well for 16 years when she died

of another cause.

DISCUSSION

This case illustrates a very rare, yet significant, com-

plication following mitral valve replacement surgery.

The increased pulmonary pressures, illustrating mild

pulmonary hypertension, provide the physiological

explanation behind the patients presenting clinical

symptoms. Even though all previously reported cases

were treated with surgical closure; this patient was ad-

equately managed with medical therapy alone since her

frail health caused her to decline surgery.

The decision to treat medically versus surgically

should be individualized taking into account the pa-

tients age, degree of pulmonary hypertension, and

other medical comorbidities. However, surgical inter-

vention should remain the definitive therapy.

Two etiologies have been postulated to account for

the formation of left ventricular to coronary sinus fistula

formation following mitral valve replacement surgery.

The first is direct injury during surgery leading to forma-

tion of a fistulous tract. Second is injury to the posterior

ventricular wall, inferior to the mitral valve leaflets, sec-

ondary to excessive debridement of a usually calcified

mitral valve annulus. This predisposes to spontaneous

dissection and eventually results in fistula formation.6

In our case, fistula formation was likely the result of the

second etiology described above based upon the dis-

tant chronological relationship between valve replace-

ment surgery and fistula recognition.

Obviously, the exact time of fistula development can-

not be determined, but can be estimated based upon

time of clinical presentation.

Even though uncommon, left ventricular to coronary

sinus fistula should be a diagnosis of consideration in

patients who have undergone multiple mitral valve re-

placement surgeries and present with symptoms con-

sistent with pulmonary congestion. One needs a high

index of suspicion to make this diagnosis clinically. The

murmur created from the fistula closely mimics that

of mitral regurgitation, and the presenting symptoms

are those of heart failure. In all reported cases, angio-

graphic visualization, echocardiographic evaluation, or

direct surgical visualization/palpation has been required

to confirm the diagnosis. All patients responded well

to repeat surgery. Based upon the small number of

reported cases in the literature, we feel that postop-

erative left ventricular to coronary sinus fistula carries

a favorable prognosis if diagnosed in a timely fashion,

but does require repeat surgery in most cases.

REFERENCES

1. Gerbode F, Hultgren H, Melrose D, et al: Syndrome of left

ventricular-right atrial shunt. Ann Surg 1958;148:433.

2. Chambers RL, Rodgers MA: Left ventricular-to-coronary-

vein fistula following mitral valve replacement. Ann Thorac

Surg 1972;14:305-308.

3. Miller DC, Schapira JN, Stinson EB, et al: Left ventricular-

coronary sinus fistula following repeated mitral valve re-

placements. J Thorac Cardiovasc Surg 1978;76:43-45.

4. Morritt GN, Jamieson MPG, Irwing JB, Marquis RM, Wal-

baum PR: Development of left ventricular-coronary sinus

fistula following replacement of mitral valve prosthesis. J

Thorac Cardiovasc Surg 1978;76:381-384.

J CARD SURG2008;23:63-78

TAGHIPOUR, ET AL.RARE CASE OF SINGLE CORONARY ARTERY ANOMALY

67

5. Rogers AG, Rossi NP: Left ventricular-coronary sinus fis-

tula after mitral valve replacement. J Cardiovasc Surg

1987;94:637-638.

6. Paolini G, Gallorini C, Triggiani M, et al: Mitral valve

prosthetic endocarditis: Development of left ventricular-

coronary sinus fistula following replacement. Eur J Cardio-

thorac Surg 1993;7:663-664.

7. Almodovar LFL, Rufilanchas JJ, Enriquez F, et al: Left

ventricular-coronary sinus/right ventricular fistula late after

mitral valve replacement. Ann Thorac Surg 2004;77:1441-

1443.

A Very Rare Case of SingleCoronary Artery Anomaly, withLeft Anterior Descending andLeft Circumflex ArteriesOriginating Separately fromProximal Right Coronary Artery

Hamid R. Taghipour, M.D.,∗ YahyaDadjou, M.D.,† Ahmad R. Mafi, M.D.,M.Sc.,‡and Yashar Moharamzad, M.D.§∗Department of Cardiothoracic Surgery,Baqiatallah University Hospital, Tehran, Iran;†Department of Cardiology, BaqiatallahUniversity Hospital, Tehran, Iran; ‡ResearchCenter of Baqiatallah University Hospital,Tehran, Iran; and §Research Center ofBaqiatallah University Hospital, Tehran, Iran

ABSTRACT Coronary artery anomalies are rare,with the reported prevalence of 0.2% to 1% inroutine angiographic studies. Among them, pres-ence of a single coronary artery is one of therarest anomalies, comprising less than 3% of allcoronary anomalies. In this article we report acase of single coronary artery anomaly with leftanterior descending and left circumflex arteriesarising separately from proximal right coronaryartery, and left anterior descending artery reach-ing atrioventricular sulcus by passing between aor-tic and pulmonary artery trunci. The patient under-went off-pump coronary artery bypass grafting andis currently symptom-free. doi: 10.1111/j.1540-8191.2007.00496.x (J Card Surg 2008;23:67-69)

CASE REPORT

A 45-year-old female attended the outpatient clinic

complaining of chest discomfort for more than four

Address for correspondence: Ahmad R. Mafi, Research Center of Baqi-

atallah Hospital, Mollasadra Avenue, Tehran, Iran PO: 19945/581. Fax:

00-98-21-8805-37-66; e-mail: [email protected]

weeks. The pain was mainly in the left sternal border ra-

diating to back, aggravating by exercise and alleviating

by rest. Coronary risk factors included positive family

history and hypercholesterolemia.

On physical examination, blood pressure was 110/70

mmHg, lungs were clear, and the rest of physical exam-

ination was unremarkable. ECG showed a sinus rhythm

at the rate of 80 per minute, with ST-T wave changes

from V1 to V5.

Chest X-ray was normal. Transthoracic echocardiog-

raphy showed no abnormality. Exercise Tolerance Test

was positive with ST changes in anterior leads.

Myocardial perfusion scan demonstrated ischemia

in anterior wall. During coronary angiography, which

was performed from the standard right femoral Jud-

kins technique, attempts to cannulate the left coronary

artery were unsuccessful. Cannulation of the right coro-

nary artery (RCA) showed left circumflex (LCx) and left

anterior descending (LAD) originating separately from

the proximal RCA without left main trunk (Figs. 1 and 2).

LAD had 85% stenosis in its proximal portion, and was

observed to proceed between the aorta and pulmonary

trunci to reach the atrioventricular sulcus. LCx and RCA

were normal without any narrowing. Left ventricular

ejection fraction was also normal. Off-pump coronary

artery bypass graft was performed by anastomosing

left internal thoracic artery to LAD. The aberrant vessel

was not ligated. Postoperative period was uneventful.

She remained symptom free two months after the op-

eration.

DISCUSSION

Coronary artery abnormalities are a common cause

of sudden death from cardiac causes in young people.

In one study of sudden death from cardiac causes in pa-

tients between the ages of 18 years and 35 years, 23%

of the deaths were due to coronary artery disease.1

None of the patients, who were nearly all men, had

any history of angina pectoris or myocardial infarction.

The majority had single-vessel disease, mainly affect-

ing proximal LAD. Acute thrombosis happened in only

25% of them. The most frequent congenital coronary

artery anomalies causing sudden death in the young

people is believed to be anomalous origin of either the

right or left coronary artery in the left or the right aortic

sinus of Valsalva, respectively. If the aberrant artery is

a dominant artery, which perfuses a large region of my-

ocardium, the patient will be at highest risk for sudden

death and the risk seems to be greatest during the first

three decades of life.2

Coronary artery anomalies are found in about 0.2%

to 1% of angiographies and about 0.3% of autopsies.3

The most common coronary abnormality is that LAD

and LCx arteries originate separately from left sinus of

valsalva (52%). Second most common anomaly is that

LCx artery originates from RCA or right sinus of valsalva

(27%). Coronary artery fistulae comprise 5% of cases.4

Coronary artery atherosclerosis has been reported to

happen in about 11% of anomalous arteries.3 Among

all types of coronary artery anomalies, presence of a

single coronary artery is a very rare entity, especially

68 TAGHIPOUR, ET AL.RARE CASE OF SINGLE CORONARY ARTERY ANOMALY

J CARD SURG2008;23:63-78

Figure 1. Left anterior descending artery (LAD) originatesfrom the proximal part of right coronary artery (RCA).

in the absence of any structural heart disease.5, 6 Its

prevalence has been reported to be less than 3% of all

coronary anomalies.4 Although a majority of patients

are asymptomatic, this anomaly may result in myocar-

dial ischemia presenting with angina pectoris, myocar-

dial infarction, syncope, congestive heart failure, and

even life-threatening ventricular arrhythmias and sud-

den death.7

Our case presented with no artery originating from

left sinus of valsalva, with LAD and LCX originating sep-

arately from proximal right coronary artery. In one re-

cent angiographic study of more than 70,000 patients,

the prevalence of single coronary artery with LAD orig-

inating from proximal RCA was reported to be about

0.006%.3 In the case of LAD originating from right si-

Figure 2. Left circumflex artery (LCx) originates from proximalpart of right coronary artery (RCA) which is not filled in thisview .

nus of valsalva or RCA, there are three variations of the

initial course of LAD:3

1. anterior to the right ventricular infundibulum (an-

terior type, which is the most common)

2. between the aorta and the pulmonary trunk (inter-

arterial type)

3. in the ventricular septum beneath the right ven-

tricular infundibulum (septal type)

As in our case, the single coronary artery was orig-

inated from right sinus of valsalva and LAD and LCx

were originated from proximal RCA with LAD having

a course between aorta and pulmonary arteries, it can

be classified as type R-III B coronary anomaly accord-

ing to Lipton’s classification.8 Interarterial type is the

most dangerous type, and it is reported to be the sec-

ond most common cause of sudden death in young

athletes, after hypertrophic cardiomyopathy.7

In this type, even in the absence of significant

atherosclerosis and narrowing of the vessel, ischemic

complications are frequent, whose exact mechanisms

are not fully understood.

Some possible mechanisms include:

1. Compression of the LAD by aorta and the pul-

monary trunk.3

2. Acute angle of origin of the anomalous artery with

respect to the ostium may form a “slit-like” ori-

fice that could become further compressed during

systole.3

3. Anomalous artery can be kinked as it courses be-

tween the great vessels.9

4. Myocardial squeezing.3

5. Vasospasm.3

6. Small artery.3

7. Endothelial dysfunction.10

None of the above mechanisms are proven. For

example, some authors argue that the compression

mechanism is unlikely, as the intravascular pressure

inside the anomalous artery and inside the aorta is al-

most equal during all cardiac cycles, and the pulmonary

trunk usually has a lower pressure.7

Although rare, coronary artery anomalies should be

always kept in mind while encountering a young pa-

tient with ischemic heart disease symptoms, where

accurate diagnosis and early intervention can be cura-

tive and life-saving.

REFERENCES

1. Corrado D, Basso C, Poletti A, et al: Sudden death in the

young: Is acute coronary thrombosis the major precipitat-

ing factor? Circulation 1994;90:2315-2323.

2. Liberthson RR: Sudden death from cardiac causes in chil-

dren and young adults. N Engl J Med 1996;334(16):1039-

1044.

3. Tuncer C, Batyraliev T, Yilmaz R, et al: Origin and distri-

bution anomalies of the left anterior descending artery

in 70,850 adult patients: Multicenter Data Collection.

Catheter Cardiovasc Interv 2006;68(4):574-585.

J CARD SURG2008;23:63-78

CHRISTIANSEN, ET AL.SURGERY FOR CORONARY ANEURYSM

69

4. Kosar F, Ermis N, Erdil N, et al: Anomalous LAD and CX

artery arising separately from the proximal right coronary

artery:-A case report of single coronary artery with coro-

nary artery disease. J Card Surg 2006;21(3):309-312.

5. Surucu H, Okudan S, Tatli E: Rare coronary artery

anomaly: A single coronary artery arising from the

right sinus of Valsalva. Anadolu Kardiyol Derg 2007;7(1):

113-114.

6. Namboodiri N, Harikrishnan S, Tharakan JA: Case reports:

Single coronary artery from right aortic sinus with sep-

tal course of left anterior descending artery and left cir-

cumflex artery as continuation of right coronary artery: A

hitherto unreported coronary anomaly. J Invasive Cardiol

2007;19(4):E102-E103.

7. Vianna CB, Gonzalez MM, Dallan LA, et al: Anomalous

coronary artery causing transmural ischaemia and ven-

tricular tachycardia in a high school athlete. Resuscitation

2007, doi:10.1016/j.resuscitation.2006.11.012.

8. Lipton MJ, Barry WH, Obrez I, et al: Isolated single coro-

nary artery: Diagnosis, angiographic classification, and

clinical significance. Radiology 1979;130(1):39-47.

9. Davis JA, Cecchin F, Jones TK, et al: Major coronary

artery anomalies in a pediatric population: Incidence and

clinical importance. J Am Coll Cardiol 2001;37(2):593-

597.

10. Tuncer C, Gokce M, Sokmen G: An anomalous left

main coronary artery with coronary torsion originat-

ing from the right sinus Valsalva. Int J Cardiol 2007,

doi:10.1016/j.ijcard.2006.11.198.

Surgical Management of a LeftMain Stem Coronary ArteryAneurysm

S. Christiansen, M.D., A. Klocke, M.D., A.Hoffman, M.D., and R. Autschbach, M.D.

Department of Cardiothoracic Surgery,University of Aachen, Aachen, Germany

ABSTRACT Left main stem aneurysms are rarely de-scribed and the optimal treatment is controver-sially discussed. A majority of these patients un-dergo medical treatment with antiplatelet or anti-coagulation drugs. Surgery is just recommendedin symptomatic patients or when there is the riskof thromboembolic events or rupture. We reporton a 51-year-old patient suffering from intermit-tent angina pectoris in whom an aneurysm of theleft main stem was diagnosed by coronary an-giography. The patient underwent successful sur-gical management with aneurysm closure and re-construction of the left main stem by a segmentof the great saphenous vein. This report summa-rizes the main treatment options for left main

Address for correspondence: PD Dr. med. S. Christiansen, Depart-

ment of Cardiothoracic Surgery, University of Aachen, Pauwelsstr.

30, 52074 Aachen, Germany. Fax: +49-241-80-82454; e-mail:

[email protected]

stem aneurysms and discusses the role of cardiacsurgery for this rare disease. doi: 10.1111/j.1540-8191.2007.00498.x (J Card Surg 2008;23:69-70)

Coronary artery aneurysms are defined as coronary

dilatation exceeding the diameter of the normal coro-

nary artery or the patient’s largest coronary vessel by

1.5 times.1 The incidence varies from 1.5% to 5% with

left main stem aneurysms often associated with two-

or three-vessel disease being extremely rare [0.1%].1-3

Coronary aneurysms may be caused by atheroscle-

rosis, Kawasaki’s disease, coronary angioplasty (bal-

loon, laser, atherectomy), arteritis, dissection, trauma,

connective tissue disorders, and metastatic tumors

or may be a congenital disorder.1,4 Here, we report

on a patient with a probably atherosclerotic left main

stem aneurysm undergoing surgical treatment and

discuss the various therapeutic options for this rare

disease.

CASE REPORT

A 51-year-old male patient was admitted to our de-

partment due to instable angina pectoris with typical

chest pain radiating into the left arm. The symptoms

were independent from physical activity and occurred

irregular and unpredictable. The coronary angiography

did not reveal any high-grade stenoses of the right or

left coronary artery except a proximal 50% stenosis of

the first marginal branch. Surprisingly, we also found

an aneurysm of the left main stem (Fig.1).

The left ventricular function was not impaired un-

til now as demonstrated by the ventriculography and

a transthoracic echocardiography. The patient was

treated with acetylsalicylic acid and phenprocoumon

but the symptoms persisted. It was hypothesized that

the symptoms were caused by repetitive minor em-

bolizations from the aneurysm despite the anticoagu-

lation therapy. Therefore, we decided to operate the

patient to exclude the aneurysm as source of the em-

boli to avoid further myocardial infarctions.

After median sternotomy, the heart-lung-machine

was instituted via the ascending aorta and the right

atrium and cardioplegia was administered. The main

pulmonary artery was divided and the aorta incised into

the left main stem till the bifurcation into the left an-

terior descending coronary artery and the circumflex

artery. The aneurysm was situated in the middle of the

left main stem at the inferior site and was closed by a

suture. The incision of the left main stem was closed

with a vein patch from the great saphenous vein, which

was also used for closure of the aortotomy. Further-

more, a vein graft to the first marginal branch was con-

structed. After extensive deairing and weaning from

cardiopulmonary bypass the procedure was finished.

The intraoperative angiography demonstrated a com-

plete exclusion of the aneurysm and a patent vein graft.

The patient was extubated on the same day. The post-

operative recovery was uneventful and the patient was

discharged home six days later.

70 CHRISTIANSEN, ET AL.SURGERY FOR CORONARY ANEURYSM

J CARD SURG2008;23:63-78

Figure 1. Depicts the aneurysm of the left main stem (whitearrow) at its inferior site.

DISCUSSION

Complications of coronary aneurysms are throm-

bosis and distal embolization, rupture, and spasm.

Whereas in an older publication some authors de-

scribed an incidence of rupture of an aneurysm of

12%, in recent reports, no cases of rupture were doc-

umented.1 The risk of aneurysm rupture cannot be ex-

cluded but it appears to be very difficult to estimate

the real frequency of this event because it can only

be diagnosed by an autopsy.1 In a postmortem study,

a thrombus within the aneurysm was found in 70% of

these patients.1 Thrombosis may lead to embolizations

and myocardial infarctions but its prevalence is not ex-

actly defined until now.1 Regarding aneurysm spasms

there are only few reports on single patients, which do

not allow to draw any meaningful conclusions.1 But in

principle, aneurysms may lead to vasoconstriction play-

ing a role in the etiology of downstream ischemia.1

There is a controverse discussion about the appropri-

ate treatment of coronary aneurysms. Numerous dif-

ferent strategies were described: implantation of en-

dovascular stents, occlusion by coils, ligation, closure

of the aneurysm neck, resection of the affected part of

the coronary artery and direct reanastomosis or inter-

position of a vein or graft, resection and patch repair,

and/or coronary bypass.5, 6 A majority of patients pri-

marily undergo a medical treatment with antiplatelet

and/or anticoagulation agents.1 In the presence of

an atherosclerotic aneurysm a surgical procedure is

preferred because of the often concomitant coronary

artery disease requiring a revascularization procedure

anyway. Furthermore, surgery is recommended in pa-

tients with aneurysm complications and especially for

saccular aneurysms due to the higher risk of thrombo-

sis and rupture.1

In our patient we decided to perform a surgical treat-

ment in order to prevent further myocardial infarctions

because of repetitive thromboembolic events, which

could not be managed with the anticoagulation ther-

apy. A coil embilization was not indicated because of

the form of the aneurysm: It had a wide neck and prob-

ably the coil would be flushed out leading to an oc-

clusion of the left main stem. Different surgical proce-

dures are described: Usually it is recommended to ex-

cise or ligate the aneurysm and perform a simultaneous

bypass procedure when necessary.2,5 Other authors

also described coronary artery bypass grafting without

ligation or isolation of the aneurysm but this strategy

does not exclude the risk of rupture or embolization.7

In patients with left main stem aneurysms reconstruc-

tive procedures were described:8 After resection of the

aneurysm the left main stem was reconstructed by a

femoral circumflex artery or internal iliac artery graft. Si-

multaneously, bypass grafts were performed to avoid

a myocardial ischemia due to an early spasm of the

interposed graft.8 We are of the opinion that it is not

necessary to resect another arterial vessel for left main

stem reconstruction. Direct suture of the aneurysm ori-

fice and closure of the left main stem with a vein patch

appears to be an adequate therapy.

CONCLUSION

To our opinion, symptomatic patients with left main

stem aneurysms should be treated surgically. Closure

of the aneurysm with reconstruction of the left main

stem and performance of a revascularization procedure

seems to be the therapy of choice.

REFERENCES

1. Syed M, Lesch M: Coronary artery aneurysm: A review.

Prog Cardiovasc Dis 1997;40(1):77-84.

2. Barettella MB, Bott-Silverman C: Coronary artery

aneurysms: An unusual case report and a review of

the literature. Cathet Cardiovasc Diagn 1993;29:57-61.

3. Topaz O, DiSciascio G, Cowley MJ, et al: Angiographic fea-

tures of left main coronary artery aneurysms. Am J Cardiol

1991;67:1139-1142.

4. Takeda K, Matsumiya G, Nishimura M, et al: Giant cir-

cumflex coronary aneurysm associated with cystic me-

dial necrosis in a non-Marfan patient. Ann Thorac Surg

2007;83:668-670.

5. Jonsson A, Nilsson T, Pernow J, et al: Resection of a gi-

ant left main stem coronary artery aneurysm. J Cardiovasc

Surg 2001;42:489-491.

6. Kim H, Park H: Percuaneous treatment of trisaccu-

lar coronary aneurysm with coil-embolisation. Heart

2006;92:1826.

7. Izumi Y, Yoshida H, Shimizu N, et al: Emergent operation

for aneurysm of the left main coronary artery presenting

acute myocardial infarction. Ann Thorac Cardiovasc Surg

2004;10:195-197.

8. Anderson CA, Filsoufi F, Kadner A, et al: Repair of the

left main coronary artery aneurysm using the circumflex

femoral artery as a Y-interposition graft. Ann Thorac Surg

2004;78:314-316.

J CARD SURG2008;23:63-78

TJANG, ET AL.SCIMITAR SYNDROME IN ADULTS

71

Scimitar Syndrome Presentingin Adults

Yanto Sandy Tjang, M.D., M.B.A., M.P.H.,M.Sc., D.Sc., F.I.C.S.,∗ Ute Blanz, M.D.,∗

Stanley Kirana, M.D.,† and Reiner Korfer,M.D., Ph.D.∗

∗Department of Thoracic and CardiovascularSurgery, Heart and Diabetes CenterNRW/Ruhr-University Hospital of Bochum,Bad Oeynhausen, Germany; and †DiabetesCenter, Heart and Diabetes CenterNRW/Ruhr-University Hospital of Bochum,Bad Oeynhausen, Germany

ABSTRACT Scimitar syndrome is a rare and com-plex congenital anomaly, which is characterizedby the image of a Turkish sword on the chestX-ray. Very few cases in adults are reported inthe literature. The long-term results of scimitarsyndrome after surgical correction remain disap-pointing. We report an adult patient with scimitarsyndrome undergoing surgery at our center. doi:10.1111/j.1540-8191.2007.00497.x (J Card Surg2008;23:71-72)

Scimitar syndrome is a rare and complex congenital

anomaly, mostly associated with a variety of thoracic

abnormalities. The anomalous pulmonary venous re-

turn of all or most of the right lung to the inferior vena

cava just below or above the right hemidiaphragm cre-

ates the image of a Turkish sword on the chest X-ray.

Very few cases presenting in adults are reported. The

long-term results of scimitar syndrome after surgical

correction remain disappointing. We report an adult pa-

tient with scimitar syndrome undergoing surgery at our

center.

CASE REPORT

A 19-year-old female was admitted to our center for

systolic murmur. Medical history revealed syncope at

11 years old. No recurrent pneumonia or cyanosis ex-

isted. Physical examination revealed no other abnor-

malities. The ECG showed right axis deviation with right

ventricular hypertrophy. The chest X-ray demonstrated

dextroposition of the heart with an irregular shadow

in the right lower lung field and increased vascularity

in the right lung. Cardiac catheterization revealed mod-

erate elevation of right heart pressure. Ejection frac-

tion and pulmonary artery pressures were normal. The

scimitar vein resulted in an estimated left/right shunt

volume of 43% (Qp/Qs = 1.8:1). There was no evi-

dence of atrial septal defect. Magnetic resonance imag-

ing demonstrated an anomalous venous drainage of

Address for correspondence: Yanto Sandy Tjang, M.D., M.B.A., M.P.H.,

M.Sc., D.Sc., F.I.C.S., Wielandstrasse 11, 32545 Bad Oeynhausen,

Germany. Fax: +49-5731-971820; e-mail: [email protected]

the right lung to the subphrenic inferior vena cava, con-

firming the diagnosis of scimitar syndrome (Fig. 1). The

patient underwent surgery via median sternotomy. The

scimitar vein was identified in the right hemithorax and

followed distally below the diaphragm. Due to close

proximity of the hepatic vein confluence and the infe-

rior vena cava, the latter could not be snared in order to

isolate the venous return to the right atrium. The entire

surgical procedure was performed under hypothermic

circulatory arrest at 18◦C. The right atrium was incised

to create an atrial septal defect. Surgical repair was

performed by baffling the orifice of the scimitar vein

from its entry into the inferior vena cava to the cre-

ated atrial septal defect using a large diameter of au-

tologous pericardium. The postoperative course was

uneventful. Four years after surgery, the patient was

completely free of symptoms. Echocardiography re-

vealed no stenosis of the intraatrial tunnel.

DISCUSSION

Scimitar syndrome occurs in 1–3/1,00,000 live

births.1 Described by Cooper in 1836, the term “scimi-

tar” was firstly used by Halasz in 1956.2 The age at pre-

sentation varies. The mean age of diagnosis is seven

months.3 This malformation is classically divided into

infant and adult forms.1,4 Infant patients typically have

features of congestive heart failure due to significant

left/right shunt from the anomalous pulmonary venous

drainage or more commonly from an additional cardiac

defect such as atrial septal defect. Older children and

adults may have symptoms and the diagnosis may be

made by chest X-ray demonstrating the scimitar sign

in as many as 70% of patients.5 Cardiac catheteriza-

tion should always be performed to confirm the diag-

nosis, identify the specific course of the anomalous

pulmonary venous drainage, determine the presence

of scimitar vein stenosis and pulmonary hypertension,

measure the degree of left/right shunt, and detect any

associated cardiac abnormalities.5 Computed tomogra-

phy, angiography, and magnetic resonance imaging are

helpful if the scimitar vein is obscured by the overlying

cardiac shadow, particularly when cardiac dextroposi-

tion is pronounced.

Surgical intervention is indicated for: (1) large left/

right shunt exceeding 50%, resulting in pulmonary hy-

pertension and heart failure; and (2) lung sequestration

and/or recurrent right-sided chest infection.6 Some re-

pair options include the creation of a long baffle from

the orifice of the scimitar vein within the inferior vena

cava to the atrial septal defect that directs the anoma-

lous pulmonary venous flow to the left atrium,7 divi-

sion with reimplantation of the scimitar vein into the

right atrium with an intraatrial baffle that directs blood

flow through an atrial septal defect to the left atrium,8

partitioning of the inferior vena cava into anterior and

posterior compartments with a pericardial baffle that

channels pulmonary venous blood flow directly into

the left atrium,9 or direct anastomosis of the divided

scimitar vein to the left atrium.5 In general, the long-

term results of scimitar syndrome after surgical correc-

tion remain disappointing. Stenosis of the intracardiac

72 MARISCALCO, ET AL.ECHOCARDIOGRAPHY IN CORONARY ANEURYSM

J CARD SURG2008;23:63-78

Figure 1. Anomalous venous drainage of the right lung to thesubphrenic inferior vena cava.

baffle often develops, and thrombosis of the anasto-

mosis can lead to immediate infarction of the right

lung, pulmonary hypertension, and cataclysmic hemop-

tysis.10 In our case, we attempted to create a large

diameter of intraatrial pericardial baffle to enable im-

provement in hemodynamic conditions. We believed it

could prevent early restenosis and thrombosis of the

anastomosis. Our follow-up at four years after surgery

revealed an encouraging result.

REFERENCES

1. Dupuis C, Charaf LA, Breviere GM, et al: The “adult” form

of the scimitar syndrome. Am J Cardiol 1992;70(4):502-

507.

2. Holt PD, Berdon WE, Marans Z, et al: Scimitar vein drain-

ing to the left atrium and a historical review of the scimitar

syndrome. Pediatr Radiol 2004;34(5):409-413.

3. Najm HK, Williams WG, Coles JG, et al: Scimitar syn-

drome: Twenty years’ experience and results of repair. J

Thorac Cardiovasc Surg 1996;112(5):1161-1168.

4. Dupuis C, Charaf LA, Breviere GM, et al: “Infantile” form

of the scimitar syndrome with pulmonary hypertension.

Am J Cardiol 1993;71(15):1326-1330.

5. Brown JW, Ruzmetov M, Minnich DJ, et al: Surgical man-

agement of scimitar syndromeAn alternative approach. J

Thorac Cardiovasc Surg 2003;125(2):238-245.

6. Schramel FM, Westermann CJ, Knaepen PJ, et al: The

scimitar syndrome: Clinical spectrum and surgical treat-

ment. Eur Respir J 1995;8(2):196-201.

7. Zubiate P, Kay JH: Surgical correction of anomalous pul-

monary venous connection. Ann Surg 1962;156:234-250.

8. Shumaker HB Jr, Judd D: Partial anomalous pulmonary

venous return with reference to drainage into the inferior

vena cava and to an intact atrial septum. J Cardiovasc

Surg 1964;5:271-278.

9. Calhoun RF, Mee RB: A novel operative approach to scim-

itar syndrome. Ann Thorac Surg 2003;76(1):301-303.

10. Reddy R, Shah R, Thorpe JA, et al: Scimitar syndrome:

A rare cause of haemoptysis. Eur J Cardiothorac Surg

2002;22(5):821.

Transthoracic Echocardiographyis Adequate for the Diagnosisof Right Coronary ArteryAneurysms

Giovanni Mariscalco, M.D.,∗ FaustoSessa, M.D.,† Davide Vanoli, M.D.,‡Vittorio Mantovani, M.D., Ph.D,∗

Ferrarese Sandro, M.D.,∗ and AndreaSala, M.D.∗

∗Department of Surgical Sciences, CardiacSurgery Division, Varese University Hospital,Italy; †Department of Pathology, ClinicalInstitute Multimedica Holding Santa Mana,Castellanza, University of Insubria, Varese,Italy; and ‡Department of ClinicalPhysiology, Heart Center, University ofNorthern Sweden, Umea, Sweden

ABSTRACT Coronary artery aneurysms (CAA) arerare but potentially fatal pathologies. This case wasreferred to our Unit after occasional echocardio-graphic finding of an intracardiac mass. A new de-tailed transthoracic echocardiogram was decisivefor a diagnosis of a large CAA of the right coro-nary artery, compressing and dislocating the rightatrium. Transesophageal echocardiography wasnot performed because of the data obtained. Thediagnosis was confirmed by cardiac catheteriza-tion. The patient was managed with a surgical pro-cedure. doi: 10.1111/j.1540-8191.2007.00500.x (JCard Surg 2008;23:72-74)

BACKGROUND

Coronary artery aneurysms (CAA) are rare patholo-

gies, appearing on only 0.3% to 4.9% of coronary

angiograms.1 Spontaneous rupture, myocardial infarc-

tion, and thrombotic or embolic events can complicate

CAA.2-4 The diagnosis is most often an unexpected

finding at cardiac catheterization.5

This report shows that transthoracic echocardiogra-

phy provides sufficient information for a reliable diag-

nosis of a right CAA.

Address for correspondence: Dr. Giovanni Mariscalco, Department

of Surgical Sciences, Cardiac Division, Varese University Hospital,

21100 Varese, Italy. Fax: +39-0332-264394; e-mail: giovanimariscalco@

yahoo.it

J CARD SURG2008;23:63-78

MARISCALCO, ET AL.ECHOCARDIOGRAPHY IN CORONARY ANEURYSM

73

Figure 1. Panel A: Parasternal short axis view showing the great vessels end their relationship with the cardiac chambers. Theaorta (Ao) depicted in the center of the photograph is seen few millimeters above the cusps. Just above, the right ventricle outflowtract (RVOT) and the pulmonary valve (Pv); on the right, the main of the pulmonary artery (PA); on the left, the right ventricle(RV), the tricuspid valve (Tv), and the right atrium (RA). The coronary artery aneurysm (CAA) is also detectable, compressingand dislocating the right atrium (RA); the tricuspid valve is barely recognizable. Partial calcification of the aneurysmatic walland intraluminal thrombus (arrows) are observed. Panel B: Subcostal view providing a perpendicular view of the interatrial andinterventricular septum (IVS). The subcostal view can be made to appear as if it were a tilted four-chamber apical view. Abovethe septum, the right ventricle (RV) and the right atrium (RA); under, the left ventricle (LV) and the left atrium (LA). Tilting theprobe about 60◦ clockwise, the junction between the inferior vena cava (IVC) and the right atrium is exposed. The right atriumis compressed and dislocated by the aneurysm (CAA), the right ventricle (RV) is also compressed. The inferior vena cava (IVC) isinterestingly wide, probably because of the high diastolic pressure due to the compressed atrium.

Figure 2. Coronary angiogram showing the giant aneurysmof the right coronary artery.

Figure 3. Surgical view of the coronary artery aneurysm: (A) Initial opening of the coronary artery aneurysm with evidence of bloodclots. (B) Obliterated aneurysmatic sac (arrows). (CAA = coronary artery aneurysm; RV = right ventricle; Ao = ascending aorta)

Figure 4. Histologic features of aneurysmal wall com-pletely destroyed and substituted by fibrous tissue andabundant inflammatory cells, mainly lymphocytes andplasma cells (Hematoxylin and eosin stain; magnification×40).

74 MARISCALCO, ET AL.ECHOCARDIOGRAPHY IN CORONARY ANEURYSM

J CARD SURG2008;23:63-78

CASE SUMMARY

A 61-year-old man with an occasional echocardio-

graphic finding of a mass compressing the right atrium

was referred to our department. The patient was

asymptomatic for angina and without signs of conges-

tive heart failure. Electrocardiogram and blood analy-

sis values were normal, chest-X-ray demonstrated car-

diomegaly.

A new transthoracic echocardiogram was per-

formed, revealing decisive data for the presence of

a CAA involving the right coronary artery (RCA) and

confirming the compression and dislocation of the

right atrium. Partial calcifications of the aneurysmatic

wall and an intraluminal thrombus were also detected

(Fig. 1). Mild left ventricular dysfunction, with an es-

timated ejection fraction of 45%, and a moderate re-

gurgitation of the tricuspid valve were observed. The

specificity of the transthoracic pictures made trans-

esophageal echocardiography unnecessary. The diag-

nosis was confirmed by cardiac catheterization. A very

large and partially thrombosed CAA involving the right

coronary artery (RCA) was discovered (8 × 6 × 8 cm),

with occlusion of the middle RCA tract (Fig. 2). Critical

stenosis of the proximal left anterior descending artery

(LAD) and left circumflex (LCx) occlusion were also re-

vealed.

Surgical treatment was undertaken. Median ster-

notomy and moderately hypothermic (32 ◦C) cardiopul-

monary bypass were employed; the procedure was

done in a standardized fashion with ascending aortic

cannulation and two-stage venous cannulation of the

right atrium. The CAA was identified and longitudinally

incised in order to remove blood clots and verify the

absence of anomalous communications between the

aneurysm and the cardiac chambers (Fig. 3). The prox-

imal and distal openings were identified and occluded.

The sac was obliterated with running sutures (Fig. 3).

Finally, a saphenous bypass graft was performed dis-

tally to the excluded aneurysm. Myocardial revascular-

ization was completed by grafting the left internal tho-

racic artery to the LAD and a saphenous vein graft to

the LCx artery.

Histopathologic analysis was also obtained, revealing

atherosclerotic alteration of the vessel (Fig. 4).

DISCUSSION

CAA is a dilatation that exceeds 1.5 times the di-

ameter of a normal adjacent coronary artery.2 This

disease is a rare entity with a poor prognosis if un-

treated.1-4 Patients with aneurysmal disease have a

higher incidence of acute myocardial infarction (AMI)

and reduced survival as compared with unaffected

patients.2

The proximal and middle segments of the right coro-

nary artery are frequently involved locations.2 The most

common etiology is atherosclerotic, and destruction

of the vessel media is the usual histologic feature.2,5

Histologic and clinical studies suggest that these pro-

cesses are similar to those occurring in abdominal aor-

tic aneurysm (AAA).5,6

No clinical picture is specifically suggestive of a

CAA diagnosis.5 Angina and dyspnea are usually en-

countered in this disease.7 In cases of giant CAAs

(diameter >50 mm), chest discomfort, pain, and pal-

pitations can also be reported.8 CAAs are basically di-

agnosed as an incidental finding in coronary angiogra-

phy, which provides useful information regarding size,

shape, and location of the aneurysms.7 Angiography,

transesophageal echocardiography, and magnetic

resonance imaging can be used for the diagnosis and

follow-up of such aneurysms.9 To our knowledge, this

report is the first documentation of CAA in which

transthoracic echocardiography provided decisive infor-

mation regarding the aneurysmatic involvement of a

RCA.

According to Biglioli et al.,10 surgery is mandatory

when the aneurysm is three to four times larger

than the original vessel diameter. This is a critical

size for uncontrolled progression.10 Hence, because

of the aneurysm size and the above-mentioned com-

plications, surgical correction as mandatory in our

case.

REFERENCES

1. Lenihan DJ, Zeman HS, Collins GJ: Left main coronary

artery aneurysm in association with severe atheroscle-

rosis: A case report and review of the literature. Cathet

Cardiovasc Diagn 1991;23:28-31.

2. Swaye PS, Fisher LD, Litwin P, et al: Aneurysmal coronary

artery disease. Circulation 1983;67:134-138.

3. Virmani R, Robinowitz M, Atkinson JB, et al: Acquired

coronary arterial aneurysms: An autopsy study of 52 pa-

tients. Hum Pathol 1986;17:575-583.

4. Anabtawi IN, de Leon JA: Arteriosclerotic aneurysms

of the coronary arteries. J Thorac Cardiovasc Surg

1974;68:226-228.

5. Mariscalco G, Mantovani V, Ferrarese S, et al: Coronary

artery aneurysm: Management and association with ab-

dominal aortic aneurysm. Cardiovasc Pathol 2006;15:100-

104.

6. Kishi K, Ito S, Hiasa Y: Risk factors and incidence of

coronary artery lesions in patients with abdominal aortic

aneurysms. Intern Med 1997;36:384-388.

7. LaMendola CL, Culliford AT, Harris LJ, et al: Multi-

ple aneurysms of the coronary arteries in a patient

with systemic aneurysmal disease. Ann Thorac Surg

1990;49:1009-1010.

8. Tunick PA, Slater J, Kronzon I, et al: Discrete atheroscle-

rotic coronary artery aneurysms: A study of 20 patients:

J Am Coll Cardiol 1990;15:279-282.

9. Selke KG, Vemulapalli P, Brodarick SA, et al: Giant coro-

nary artery aneurysm: Detection with echocardiography,

computed tomography, and magnetic resonance imag-

ing. Am Heart J 1991;121:1544-1547.

10. Biglioli P, Alamanni F, Antona C, et al: Aneurysms of the

coronary arteries: One case report. Thorac Cardiovasc

Surg 1988;36:239-240.

J CARD SURG2008;23:63-78

RAFFA, ET AL.PFO CLOSURE DEVICE COMPLICATION

75

Minimally InvasiveVideo-Assisted Surgery forIatrogenic Aortic Root-to-RightAtrium Fistula After IncompletePercutaneous Occlusionof Patent Foramen Ovale: CaseReport and Review of theLiterature

Giuseppe M. Raffa, M.D.,∗ CarloPellegrini, M.D., Ph.D.,∗ Salvatore Lentini,M.D.,†Sossio Perrotta, M.D.,† Fabrizio Tancredi,M.D.,† Roberto Gaeta, M.D.,†and Mario Vigano, M.D.∗,‡

∗Department of Cardiac Surgery, Universityof Pavia, Pavia, Italy; †Department ofCardiac Surgery, University of Messina,Messina, Italy; and ‡Department of CardiacSurgery, Foundation IRCCS Policlinico “SanMatteo” Hospital, Pavia, Italy

ABSTRACT Background: The foramen ovale re-mains patent in about 25% of the population. Para-doxical embolism through a patent foramen ovale(PFO) may produce ischemic events. The closure ofa PFO may prevent recurrence of cerebrovascularevents. Percutaneous closure of a PFO is now-a-days a standard procedure and it appears to carrya low rate of complications. A surgical approach, insome cases, may be needed. Methods: A patientunderwent percutaneous closure of PFO. Therewas a residual shunt after the procedure and a fis-tula between the aortic root-to-right atrium wassubsequently discovered. Surgery was carried outusing a “Port-Access technique” through a rightanterior minithoracotomy. Results: Postoperativecourse was uneventful. Complete obliteration ofthe fistula was achieved. Conclusion: Minimally in-vasive surgery may be effective to treat PFO oreven complications after previous percutaneous at-tempts of closure. An aesthetically acceptable con-clusion, especially in young female patients, and avery low rate of morbidity may be accomplished.doi: 10.1111/j.1540-8191.2007.00483.x (J CardSurg 2008;23:75-78)

Address for correspondence: Giuseppe Raffa, Department of Cardio-

thoracic Surgery, University of Pavia, Pavia, Italy, Piazzale Golgi 2, 27100

Pavia, Italy. Fax: +39-090-2217086; e-mail: [email protected]

Foramen ovale remains patent throughout adulthood

in approximately 25% of the general population, repre-

senting the most commonly persistent abnormality of

fetal origin. Patent foramen ovale along with other atrial

septal defects is responsible for cerebrovascular mor-

bidity and reduction in life expectancy.1 Paradoxical em-

bolism through a PFO may produce ischemic events in

patients with cryptogenic stroke. Patients with a PFO

after cerebral, coronary, or systemic embolic events

of presumed paradoxical origin are at risk for recur-

rent thromboembolism. Closure of PFO is considered,

by some authors, as the most effective procedure in

stroke prevention.2-4

Therapeutic options include either percutaneous ap-

proach with specifically designed devices or surgical

treatment. Despite that percutaneous interventions are

well accepted by patients, complications rate is not

negligible5 and in some cases surgery may be needed

to manage any drawbacks.

MATERIALS AND METHODS: CASE REPORT

A 35-year-old lady was presented at our institution

both for surgical correction of an iatrogenic aortic root-

to-right atrium fistula and definitive closure of a residual

PFO.

History: the patient was affected with two acute neu-

rologic events, likely to be on ischemic grounds, re-

spectively eight and nine years before admission to

our institution. She had residual right eye motility im-

pairment and right-side hypostenia as neurologic se-

quelae. The patient was first started on oral anticoagu-

lant therapy. Later on, seven years after the last stroke,

she experienced recurrent headache episodes; there-

fore, she underwent further medical investigations. A

transesophageal echocardiography (TEE) at that time

revealed a PFO associated with aneurysm of the inter-

atrial septum. Bidirectional shunting was detected by

doppler echocardiography. Because of that the patient

underwent, in other institution, percutaneous closure

of the PFO by a 30 mm Cardia ® Starr device occluder

(Cardia Inc. Burnsville, MN, USA). Six months later, a

transthoracic echocardiogram and a TEE demonstrated

an incomplete PFO obliteration with residual bidirec-

tional shunting, (Fig. 1) and a fistula between the aortic

root and the right atrium. At first, medical treatment of

this complication was attempted by holding the anti-

coagulant therapy as described by other authors6,7 but

this did not succeed. The patient was then admitted

to our institution two months later. She was mostly

complaining of both dyspnea and palpitations. Holter

monitoring showed the presence of frequent atrial pre-

mature beats and runs of atrial tachycardia. By TEE the

fistula was located in the noncoronary sinus of the aor-

tic root, close to the commissure to the right sinus. No

interference on aortic valve function was noted. Preop-

erative angiogram of ascending aorta confirmed TEE

findings (Fig. 2).

Surgical procedure: Minimally invasive surgery was

performed through a minithoracotomy in the fourth

right intercostal space using a Port-Access technique

by a skin incision of 4.5 cm. Venous drainage was per-

76 RAFFA, ET AL.PFO CLOSURE DEVICE COMPLICATION

J CARD SURG2008;23:63-78

Figure 1. Residual interatrial shunting at Doppler TEE.

cutaneously obtained by both a cannula placed into the

right femoral vein, having the tip positioned at the junc-

tion between superior vena cava and right atrium and

a second cannula placed into the right internal jugular

vein. A thoracic port was done in the first right inter-

costal space to allow cannulation of ascending aorta us-

ing an “endodirect clamp” (Ethicon Inc, Somerville, NJ,

USA). TEE guidance was used to assess the proper po-

sition of the femoral venous cannula and the endoaortic

balloon clamp. Cold cristalloid cardioplegia was admin-

istered through the central lumen of the endoclamp. A

second thoracic port was done at the fourth right in-

tercostal space on the middle axillary line in order to

provide thoracoscopic axial vision (3D 5 mm OLIMPUS

camera) of the interatrial septum, that was eventually,

approached through a longitudinal right atriotomy run-

ning between the two venae cavae. The PFO appeared

Figure 2. Evidence of contrast medium jet between aorta andright atrium (black arrow) at preoperative ascending aorta an-giogram.

partially patent and the device, placed through the fossa

ovalis (Fig. 3), was removed leaving a septal defect

that was directly closed by five interrupted stitches.

An aortic root-to-right atrium fistula was detected by

cardioplegia flushing; the fistula was closed by two in-

terrupted stitches. Extra corporeal circulation time was

70 minutes, aortic cross clamp time was 55 minutes.

After weaning the patient off from cardiopulmonary by-

pass, TEE showed no detrimental effect of surgery on

aortic valve function and complete PFO closure.

RESULTS

Postoperative course was uneventful and patient

was discharged on the fifth postoperative day. Surgical

results were confirmed six months later by transtho-

racic echocardiography. During the 18 months postop-

erative follow up, the patient did not complain of other

episodes of headache or palpitations. Complete occlu-

sion of the PFO and the fistula, and normally function-

ing aortic valve, were seen. No aortic root pseudoa-

neurysm or dissection was reported.

CONCLUSION

PFO is a recognized cause of paradoxical embolism.

In fact, patients with a PFO and previous paradoxical

embolism have a combined cerebrovascular accidents

and TIA rate of 3.4% to 3.8% per year.1,8 Therefore,

the aim of the closure of a PFO is to prevent recur-

rent thromboembolic events. However, even after clo-

sure of PFO, thromboembolic events may still occur.

The one-year rate of recurrent neurologic thromboem-

bolism after transcatheter intervention, has been re-

ported to be between 0% and 4.9%.9 And in particular

with the Cardia PFO occluder, the yearly incidence of

recurrent thromboembolic events has been estimated

to be 2.0%.10

Although percutaneous closure of PFO gained large

popularity among patients and cardiologists, complica-

tions may occur using this technique. Supraventricular

arrhythmias, malposition, displacement and emboliza-

Figure 3. Thoracoscopic view through right atriotomy of theclosure device in situ.

J CARD SURG2008;23:63-78

RAFFA, ET AL.PFO CLOSURE DEVICE COMPLICATION

77

tion of the device, pericardial tamponade, local vascu-

lar complications, thrombus apposition on the device,

early and late residual shunting, fractures of device

arms, transient ST elevation, ventricular fibrillation, and

perforation of left atrium may be seen after percuta-

neous treatment.11

Early and late cardiac perforations are considered the

most severe complications.12 Trepels et al. described

the first case of cardiac perforation using the Amplatzer

device in 2003.13 This rare complication occurs pre-

dominantly in the anterosuperior atrial wall and adjacent

to the aorta.12 Moreover, with the same device risk of

erosion of the atrial wall is estimated to be 0.1%.14

Baykut et al. the treated on an emergency basis a late

perforation of the aortic root by an atrial septal defect

occlusion device 4 years after the percutaneous proce-

dure.15

The first case of aortic root-to-right atrium fistula with

Amplatzer septal occluder requiring surgical treatment

was reported by Chun et al. in 2003.16-18 There are

also reports of surgical repair of aortic root-to-left atrium

fistula following percutaneous closure in an adult19,20

and in a child.21 Till date, to the best of our knowledge,

there are ten reports of an aortic root to atrium fistula

(Table 1). On this ground, we believe that a strict long-

term follow-up of those patients is mandatory.

Two fistulas had a spontaneous closure and the

other eight were repaired surgically. Surgical treat-

ment was performed either by a right anterolateral tho-

racothomy22 or a standard longitudinal median ster-

notomy.23 In some cases, as for perforations, the clin-

ical status of a patient affected with a fistula required

an urgent operation.17,19,20,23

As different from previously reported cases,6,7 in our

patient, the withdrawal of oral medical therapy did not

provide spontaneous closure of the communication.

From the anatomic point of view, we believe that this

type of complication is probably the consequence of a

particular anatomic pattern, that is, when the area of

the “torus aorticus” is quite bulging within the right

atrium, and when the plane of PFO is not well aligned

TABLE 1Review of the Literature

Diagnosis AfterAuthor Device Fistula Percoutaneous Closure Treatment

Aggoun et al. 200219 Amplatzer Ao-La 21 days Surgery

Chun et al. 200315 Amplatzer Ao-Ra 3 months Surgery

Stauffer et al. 20046 PFO Star Ao-Ra 1 month Spontaneous closurea

Knirsch et at. 200520 Amplatzer Ao-La 28 days Surgery

Mello et al. 200518 Amplatzer Ao-La 6 months Surgery

Meier et al. 20057 PFO Star Ao-Ra 1 year Spontaneous closureb

Jang et al. 200516 Amplatzer Ao-Ra 2 months Surgery

Grayburn et al 200517 Amplatzer Ao-Ra 2 years Surgery

Lange et al. 200622 Cardia Star Ao-Ra 28 days Surgery

Sauer et al. 200623 Amplatzer Ao-Ra 1 day Surgery

Ao-La = aorta to left atrium fistula; Ao-Ra: aorta to right atrium fistula.aAfter one month of suspension of anticoagulant treatment.bAfter two years of suspension of antiaggregant treatment.

with it. Moreover, a diminutive aortic rim may play an

important role.24

In order to minimize the rate of complications of per-

cutaneous closure of PFO a careful selection of patients

on the basis of morphology and location of the intera-

trial defect (dimensions of border and distance from

intra-atrial structures) is required.

Even considering the continuing refinement of per-

cutaneous procedures, surgical closure of PFO should

be considered in all cases that not adhere to the selec-

tion criteria.

The surgical closure of a PFO is a low-risk and highly

successful procedure with a low probability of residual

shunt, short hospital stay, and excellent long-term re-

sults.25 Some authors even describe less hospital cost

associated with surgical treatment.26

However, many patients are not keen on having a

long residual scar in the sternal region because of

cosmetic dissatisfaction, and this is true especially in

young asymptomatic female patients.

To minimize surgical trauma and improve cos-

metic results, different minimally invasive surgical ap-

proaches have been described, both, for cosmetic rea-

son and further reduction of the already fair morbidity

of traditional surgery.27,28

In particular, minimally invasive cardiac surgery by

means of Port-Access technique is probably associated

with reduced postoperative hospital stay and costs,

better postoperative chest function, improved cosmet-

ics results, and early return to full activities.29

We have been routinely using this technique in our

Institution since 1997, and till February 2006, have

treated by this means, 875 patients (mostly affected by

mitral valve disease), and, among those, we operated

on 108 patients for abnormalities of the atrial septum.

In front of a wide-accepted procedure such as percu-

taneous closure of a PFO there is a not negligible risk

of related complications.5,9

Among the surgical options used to repair any ad-

verse event, particular attention should be paid to min-

imally invasive video-assisted surgery.

78 RAFFA, ET AL.PFO CLOSURE DEVICE COMPLICATION

J CARD SURG2008;23:63-78

The 35-year-old women in this report, even after a

complication such as aortic root to right atrium fistula

following an incomplete percutaneous closure of the

PFO, had the benefit of a definitive correction of these

defects, along with a cosmetically satisfactory result.

Therefore, we suggest, after any failed percutaneous

procedure, to consider this surgical approach in order

to offer the patient a chance of treatment characterized

by trivial risks, fast recovery, and satisfactory aesthetic

results.

Therefore, we suggest to consider this type of ap-

proach when we want to offer the patient benefits in

terms of aesthetic results and faster postoperative re-

covery after any failed percutaneous treatment.

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11. Chan KC, Godman MJ, Walsh K, et al: Transcatheter clo-

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12. Divekar A, Gaamangwe T, Shaikh N, et al: Cardiac per-

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13. Trepels T, Zeplin H, Sievert H, et al: Cardiac perforation

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14. Amin Z, Hijazi ZM, Bass JL, et al: Erosion of Amplatzer

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15. Baykut D, Doerge SE, Grapow M, et al: Late perforation of

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16. Chun DS, Turrentine MW, Moustapha A, et al: Develop-

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