Significant stenoses of twin circumflex arteries accompanied by heart failure: a rare coronary artery anomaly.

Although coronary artery anomalies may cause some clinical symptoms, most are incidentally discovered as benign findings on coronary angiograms. A circumflex coronary artery anomalously originating from the right sinus of Valsalva is the most common coronary anomaly. However, a double circumflex coronary artery, both stenotic in their mid portions, resulting in symptomatic heart failure is a rare clinical and angiographic condition. In this case, we present a 71-year-old male patient admitted to our clinic with the diagnosis of acute heart failure. Angiography revealed stenotic double circumflex arteries, arising from the left and right sinus of Valsalva, and the patient was treated by percutaneous coronary intervention.

Introduction

Coronary artery anomalies are uncommon, being found in 0.6–1.5% of patients undergoing coronary angiography (CAG).[1] There are four common courses for the anomalously arising left coronary artery (LCA) from the right sinus of Valsalva (RSV), one common course for the right coronary artery (RCA) anomalously arising from left sinus of Valsalva (LSV), and one common course for the circumflex coronary artery (Cx), arising anomalously from RSV.[2] Although anomalously originating Cx from the RSV is the most common coronary anomaly, this anomaly is thought to be of little clinical significance unless the vessel is severely narrowed.[3] However, double Cx arteries constitute a very rare congenital coronary anomaly with just three cases reported in the literature. This report describes a Cx with stenosis in its mid portion and anomalously originating from the RSV, in addition to a left Cx with stenosis in its mid portion, originating from the left main coronary artery (LMCA).

Case Report

A 71-year-old Caucasian male patient with a history of diabetes mellitus (DM), hypertension, and chronic obstructive pulmonary disease (COPD) was brought to our emergency department with the complaints of severe dyspnoea, sweating and confusion. On physical examination, the blood pressure was 140 mmHg systolic, 80 mmHg diastolic, and his pulse rate was 110 beats/min. A pansystolic murmur was audible at the apex, and inspiratory crackles were heard throughout both lung fields. He had New York Heart Associating class II dyspnoea for a long time. There was a progressive increase in breathlessness and orthopnoea over the previous 3 weeks, and it became worse for last few days. He was diagnosed with acute pulmonary oedema and admitted into the intensive care unit (ICU). The electrocardiogram (ECG) confirmed sinus tachycardia (122 beats/min.) with nonspecific intraventricular conduction delay, and previous anterior infarction (Figure 1), and the chest X-ray confirmed cardiomegaly, interstitial oedema, and fibrotic scar from previous lung infection (Figure 2). The transthoracic echocardiography (TTE) demonstrated mild pericardial effusion, global hypokinesis of the left ventricle (LV), LV dilation, and moderate mitral regurgitation with ejection fraction estimated at 35%. His blood work-up showed moderately high cardiac enzymes levels. The patient responded very well the medical therapy instituted for his heart failure. Three days later his condition was good enough for a CAG to be carried out. The CAG revealed a double Cx; one arising from left (Figure 3) and the other originating from right coronary sinus (Figure 4). Both were 70% stenotic in their mid portions; the RCA was non-dominant, and the left anterior descending (LAD) had non-obstructive coronary artery disease. Primary percutaneous coronary intervention (PCI), including balloon angioplasty and stenting, was successfully performed for both stenoses (Figure 5, Figure 6). The patient remained uneventful and discharged on the seventh day following the procedure with a medical therapy which includes an ACE inhibitor, a β- blocker, antidiabetics, a diuretic, aspirin, and clopidogrel.

Figure 1

The electrocardiogram shows sinus tachycardia with intraventricular delay and previous anterior myocardial infarction.

Figure 2

Chest X-Ray indicates cardiomegaly, interstitial oedema, and fibrotic scar from previous lung infection.

Figure 3

The Left Cx with a 70% stenosis in its mid portion.

Figure 4

The Right Cx with a 70% stenosis in its mid portion.

Figure 5

The Left Cx after PCI.

Figure 6

The Right Cx after PCI.

Discussion

Coronary artery anomalies are found in 0.6–1.5% of coronary angiograms. Although most of them have no clinical significance, they may cause acute myocardial damage and/or chronic injuries in the area supplied by the anomalous coronary artery arising from the incorrect coronary sinus of Valsalva.[1,4,5] Some coronary artery anomalies may cause chest pain, arrhythmia, heart failure, and sudden death.[6] Myocardial ischemia can occur because of earlier and more aggressive atherosclerosis compared to a normal coronary artery[7] that was found exclusively in anomalous vessels arising from the right side with a retroaortic course.[8] The anomalously originating Cx artery generally arises posterior to the RCA, and courses inferiorly and posteriorly to the aorta to enter the left atrioventricular groove.[2] A study confirmed that the incidence of stenosis was greater in the Cx arteries originating from the right coronary sinus compared to normal Cx originating from the LMCA.[9] Although the anomaly is common, to our best of knowledge double Cx anomalies are rare with only few cases reported in the literature. Karabay et al. presented a case of twin Cx arteries arising from left and right coronary systems with acute inferior myocardial infarction treated by PCI,[6] as in our case. Including that case,[6] dual Cx arteries arising from the left and right coronary systems have been reported in only three cases.[6,10,11] Double Cx artery cases have been reported with both of them originating from the left coronary systems,[12] and from the left system and aorta, respectively.[13]

The case we presented was suffering from severe heart failure due to ischemic injury as proved by the elevated cardiac enzymes and the ECG finding. The significant stenoses of both the anomalously and normally originating Cxs were successfully treated by balloon angioplasty and stenting.

Conclusions

In patients with diminished left ventricular systolic function and/or progressive dyspnoea, coronary artery anomalies should be suspected and coronary angiography should be performed in order to exclude or show additional atherosclerotic disease, and PCI may be used as a treatment of choice in comparison with medical and/or surgical intervention.