Current characteristics of congenital coronary artery fistulas in adults: A decade of global experience

Table 1 Origin, termination, pathway and management of unilateral fistulas in adult patients with congenital coronary artery fistulas (n = 243)

Origin	LMT (n = 17) (7%)	LAD (n = 102) (42%)	Cx (n = 48) (20%)	RCA (n = 76) (31%)
Termination
PA	47%	75%	23%	16%
RA/CS	29%	7%	52%	37%
RV	6%	9%	4%	25%
SVC	12%	none	6%	5%
LV	none	3%	4%	12%
Aneurysm	18%	7%	17%	17%
Management
SL	47%	63%	52%	54%
CMM	41%	13%	31%	17%
PTE	12%	24%	17%	29%

LMT: Left main trunk; LAD: Left anterior descending coronary artery; Cx: Circumflex coronary artery; RCA: Right coronary artery; PA: Pulmonary artery; CS/RA: Coronary sinus/right atrium; RV: Right ventricle; SCV: Superior vena cava; LV: Left ventricle; SL: Surgical ligation; CMM: Conservative medical management; PTE: Percutaneous therapeutic embolization.

Table 2 Comparison between unilateral, bilateral and multilateral fistulas related to site of origin, aneurysmal formation, mode of termination and management

	Unilateral (n = 243) (80%)	Bilateral (n = 55) (18%)	Multilateral (n = 6) (2%)
Termination
PA1	44%	73%	75%
CS/RA	28%	18%	10%
LA	2%	5%	--
LV	6%	4%	15%
RV	13%	4%	--
Aneurysm	13%	16%	33%
Management
SL	57%	51%	66%
CMM	20%	32%	17%
PTE	23%	17%	17%

¹Statistical significance was reached between termination into pulmonary artery (PA) (29%) vs exit to all other sites (10.6%) (P = 0.000). CS/RA: Coronary sinus/right atrium; LA: Left atrium; LV: Left ventricle; RV: Right ventricle; SL: Surgical ligation; CMM: Conservative medical management; PTE: Percutaneous therapeutic embolization.

Table 3 Distribution of origin of bilateral fistulas in adult patients n (%)

Bilateral fistulas	n = 55
LMT-LAD	3 (5)
LMT-RCA	2 (4)
LAD-RSV	3 (5)
LAD-Cx	1 (2)
LCA-RCA	2 (4)
RCA-LAD	31 (56)
RCA-Cx	8 (15)
RSV-Cx	1 (2)
RCA-D	3 (5)
RCA-im	1 (2)

LMT: Left main trunk; LAD: Left anterior descending coronary artery; RCA: Right coronary artery; RSV: Right sinus of Valsalva; Cx: Circumflex coronary artery; LCA: Left coronary artery; D: Diagonal branch; im: Intermediate branch.

Table 4 Comparison of demographic data, major fistula characteristics and fistula-related complications between coronary-cameral fistulas and coronary-vascular fistulas n (%)

	CCFs (n = 135) (44%)	CVFs (n = 169) (56%)	P value
Mean age (range, yr)	46.2 (18-85)	55.6 (18-86)	0.000
Female gender	68 (50.4)	93 (55)	NS
Aneurysm	24 (18.2)	19 (11.2)	NS
MI	None	5 (3)	0.042
IE	11 (8)	None	0.000
PE	4 (2.9)	4 (1.8)	NS
Abnormal chest X-ray	3 (2)	8 (5)	NS
Origin from LMT and LAD	33 (24.1)	94 (55.6)	0.000
Fistulas having multiple origins (bilateral and multilateral)	17 (13)	44 (26)	0.003

CCFs: Coronary-cameral fistulas; CVFs: Coronary-vascular fistulas; MI: Myocardial infarction; IE: Infective endocarditis; PE: Pericardial effusion; LMT: Left main trunk; LAD: Left anterior descending coronary artery.

Table 5 Fistula characteristics and fistula-related major complication in relation to demographic data

	Age (yr), mean (range)	Female gender (%)
Origin of fistulas
Unilateral
LMT	50.4 (21-74)	47
LAD	55.6 (18-86)	51
Cx	51.4 (21-82)	52
RCA	44.9 (18-78)	53
Bilateral	57.4 (24-83)	60
Multilateral	58.0 (38-79)	67
Clinical presentation
CHF	60.6 (27-86)	70
IE	41.8 (22-65)	36
MI	58.0 (44-78)	33

LMT: Left main trunk; LAD: Left anterior descending coronary artery; Cx: Circumflex coronary artery; RCA: Right coronary artery; CHF: Congestive heart failure; IE: Infective endocarditis; MI: Myocardial infarction.