Coronary artery disease in congenital single coronary artery in adults: A Dutch case series

Table 1 Patients' characteristics

Case/gender/age	Clinical presentation	Rest ECG	Risk factors	ETT	MIBI scan	CAD	Management	CAG 1classification	MSCT
1/F/45	AP, DOE	SR	-	Inconclusive	NA	None	CMM	R-IIP	NA
2/M/56	DOE	SR	+	Positive	NA	Intermediate lesion	CMM	R-I	Overestimation of the Cx-lesion
		RD		Ischemia IL		FFR 0.93
3/F/60	AP	SR	-	NA	NA	Mild	CMM	R-III	NA
		LBBB
4/M/86	ACS	SR	+	Negative	NA	Significant	PCI	L-I	NA
5/M/63	Effort AP	SR	+	Positive	NA	Significant	PCI	L-IIA	NA
		Negative T
		Inferior leads
6/F/43	ACP, fainting and pre- syncope	SR	+	Inconclusive	Positive 13N-adenosine PET-CT: normal	None	CABG	L-IIB	Course: between aorta and pulmonary artery
7/M/48	AP, syncope	SR	+	Negative	Negative	None	CMM	L-I	NA
		NSVT (5 beats)				Ergonovine test:
						No spasm
8/F/53	DOE, palpitation	SR	+	NA	Positive	Intermediate lesion	CMM	R-IIA	NA
		RD
		NSVT (20 beats)
9/M/46	AP, palpitation	SR	-	NA	NA	None	CMM	R-IIA	NA
10/M/63	AP	SR	+	Positive	NA	Significant	CABG	L-IIB	Course: between aorta and pulmonary artery
11/F/83	NSTEMI	SR	+	NA	NA	Significant	PCI	R-III	NA
12/F/47	ACP	SR	+	Negative	NA	None	CMM	L-IIA	NA
13/F/53	CP syncope	SR	-	Negative	Negative	None	CABG	L-IIB	Course: between aorta and pulmonary artery
14/M/72	DOE	SR	+	NA	NA	Intermediate lesion	CABG	L-IIB	Course: between aorta and pulmonary artery
15/M/41	ACP	SR	+	Negative	Negative	None	CMM	L-IIA	Benign course
		LBBB

¹Classification according to Lipton et al[1]. A: Anterior; AP: Angina pectoris; ACP: Atypical chest pain; ACS: Acute coronary syndrome; B: Between aorta and pulmonary artery; CAD: Coronary artery disease; CMM: Conservative medical management; ETT: Exercise tolerance test; F: Female; FFR: Fractional flow reserve; M: Male; CABG: Coronary artery bypass grafting; DOE: Dyspnoea on exertion; N: Normal; NA: Not available; P: Posterior to the aorta; PCI: Percutaneous coronary intervention; R: Right; L: Left; LBBB: Left bundle branch block; MSCT: Multi-slice computed tomography; SR: Sinus rhythm; -: Absent; +: Present (hypertension, smoking, obesity, hypercholesterolemia); PET-CT: Positron emission tomography-computed tomography; NSVT: Non-sustained ventricular tachycardia; RD: Repolarization disturbances; Cx: Circumflex; CAG: Coronary angiography; NSTEMI: Non-ST elevation myocardial infarction; MIBI: Methoxy-isobutyl-isonitrile; CP: Chest pain.

Table 2 Clinical-significance-based classification of coronary artery anomalies by Rigatelli et al[19,20]

Class	Subtypes	Clinical significance	Current series
A	E.g., ectopic origin of Cx from RSV1	Benign natural history, asymptomatic careful follow-up with conservative medical management or percutaneous intervention	Patients: none1
B	Ectopic origin of Cx from the RCA	Relevant, related to myocardial ischemia	Patients: 1, 2, 3, 7, 8, 9, 12, 15
	R-I, R-II, R-III anterior/posterior course2	Careful follow-up with conservative medical management or percutaneous intervention
C	L-I, L-II, L-III anterior/posterior course2	Severe, potentially related to sudden cardiac death
	R-I, R-II, R-III between/interseptal course2	Requires surgical treatment	Patients: 6, 10, 13, 14
D	L-I, L-II, L-III between/interseptal course2	Critical, class B or C with superimposed coronary artery atherosclerotic disease	Patients: 4, 5, 11
	B or C subgroups with concomitant coronary atherosclerosis	Requires urgent percutaneous management or surgical treatment

¹Not included in the current paper;

²Classification according to Lipton et al[1]. Cx: Circumflex coronary artery; L: Left; R: Right; RCA: Right coronary artery; RSV: Right sinus of Valsalva.