Transplanted Anomaly: Acquired Congenital Heart Disease

Article Citation:

John R. Lesser, B. Kelly Han, Mosi K. Bennett, Dan L. Lips, Robert S. Schwartz, and Benjamin Sun (2017) Transplanted Anomaly: Acquired Congenital Heart Disease. Journal of the Minneapolis Heart Institute Foundation: Fall/Winter 2017, Vol. 1, No. 2, pp. 142-144.

Case Report

John R. Lesser, MD

B. Kelly Han, MD

Mosi K. Bennett, MD

Dan L. Lips, MD

Robert S. Schwartz, MD

Benjamin Sun, MD

Minneapolis Heart Institute® and Foundation, Minneapolis, MN; Children’s Hospital and Clinics of Minnesota, Minneapolis, MN

Address for correspondence:
John R. Lesser, MD
Minneapolis Heart Institute Foundation
920 E 28th Street
Minneapolis, MN

E-mail: John.Lesser@allina.com

ABSTRACT

A 53-year-old man with an ischemic cardiomyopathy had a heart transplant from a previously healthy 18-year-old following a motor vehicle accident. A routine posttransplant invasive coronary angiogram demonstrated a narrowing in the left main artery. This was shown to be from an extrinsic compression by an intracoronary ultrasound. A subsequent coronary computed tomography angiogram revealed that the transplanted heart had a potentially malignant left main origin from the right coronary sinus with an interarterial course. Coronary computed tomography angiography is the gold standard for assessment of coronary anomalies and in our case led to prophylactic surgery with “unroofing” of the left main artery.

Keywords: coronary artery anomaly, left main origin from the opposite sinus, cardiac transplant, coronary CT angiography

HISTORY

A 53-year-old man developed an ischemic cardiomyopathy and severe congestive heart failure. He subsequently had an orthotropic cardiac transplant from an 18-year-old man who died in a motor vehicle accident. Our patient did well postoperatively and returned for his first yearly routine invasive coronary angiogram. Although difficult, coronary catheters finally engaged his “abnormally rotated” coronary ostia. The left main artery appeared to have a moderate eccentric stenosis (Figure 1a,b). Given the unusual nature of this finding, an intracoronary ultrasound probe was placed and the minimal left main area was 4.7 mm2 with 10 mm2 at its largest. Importantly, no intimal hyperplasia or atherosclerosis was identified on ultrasound. Because of the prior difficulty cannulating his coronary arteries, a coronary computed tomography (CT) angiogram was ordered the following year.

FIGURE 1
a. The catheter is not directly cannulating the left main artery during an angiogram in a cranial view. This arrow shows a moderate left main narrowing. b. A left anterior oblique view of the left main shows a moderate left main stenosis (arrow).

The coronary CT angiogram demonstrated that the left main origin was from the right coronary sinus (Figure 2a,b). The left main origin was interarterial traveling between the aorta and the pulmonary artery (Figure 3). No coronary plaque or intimal thickening was present in the coronary tree. Despite his asymptomatic state, this anatomic finding led to subsequent successful surgical “unroofing” of the left main artery.

FIGURE 2
a. A volume-rendered technique of the coronary computed tomography (CT) angiogram shows the anastomosis of the native with the donor aorta (small arrow). The left main artery has its origin from the right coronary sinus (long arrow). b. A 2-dimensional multipurpose rendering of a coronary CT angiogram shows the right coronary ostium (short arrow) off of the right coronary cusp (RCC). The left main (long arrow) has its origin off of the RCC and an interarterial course between the aorta and pulmonary artery.

FIGURE 3
A volume-rendered short axis view of the heart shows the relationship of the right coronary cusp (RCC); the left main ostium; interarterial left main course (medium-sized arrow); and the anterior course off the right coronary artery off of the RCC (short arrow).

DISCUSSION

An anomalous left coronary artery from the right coronary sinus with an interarterial course has most often been discovered postmortem.1 It is the second most common cause of sudden death in athletes or young patients2,3 and was the only coronary anomaly associated with sudden death in a large retrospective cohort assessing nontraumatic sudden deaths in military recruits.3 Invasive coronary angiography is a 2-dimensional technique that projects images with excellent spatial and temporal resolution. It does not directly visualize the surrounding anatomic structures and this limits its diagnostic accuracy for the determination of the cusp of origin and the course of an unusually positioned coronary artery. Cardiac CT and magnetic resonance angiography are current gold standard techniques for imaging the presence and assessing the possible risk of a coronary anomaly.4 These techniques show whether an interarterial course between the aorta and pulmonary artery may subject the coronary artery to external compression. In addition, it is possible to visualize the coronary artery in an intramural aortic location or identify the presence of a “slit-like” orifice.4

Cardiac transplantation has excellent short- and intermediate-term outcomes, but the scarcity of suitable organs is a major limitation. Our young apparently healthy donor had normal ventricular function. More intense assessment of a healthy young donor’s heart is generally not required. The intracoronary ultrasound demonstrated external coronary compression from an interarterial or intramural course, but such an unlikely possibility was not entertained until the coronary and great vessels were literally visualized on the coronary CT angiogram.

Prophylactic surgical correction is the therapy of choice because a patient may present with sudden death as the initial symptom.5 An operation that “unroofs” the left main artery incorporates the proximal portion of the vessel into the coronary sinus and eliminates the problem posed by the intramural and interarterial course. Our patient is doing well 2 years after the “unroofing” procedure.

REFERENCES

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2475-0190
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