Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

Table 1 Reviewed Asian (n = 111) and Caucasian (n = 100) group of patients

	Total reviewed subjects	Asian group	Caucasian group
n	211	111 (53%)	100 (47%)
Gender	F 124 (59%)	F 63 (57%)	F 61 (61%)
	M 87 (41%)	M 48 (43%)	M 39 (39%)
Mean age (range)1, yr	49.4 (16-85)	48.9 (19-83)	49.9 (16-85)
CAF characteristics
Unilateral	118 (56%)	42 (38%)	76 (76%)
Bilateral	87 (41%)	63 (57%)	24 (24%)
Multilateral	6 (3%)	6 (5%)	-
Mode of termination
CVFs	90 (43%)	43 (39%)	47 (47%)
CCFs	121 (57%)	68 (61%)	53 (53%)
RHC	201 (95%)	107 (96%)	94 (94%)
sPAP/RVSP	10 (5%)	4 (4%)	6 (6%)
Management
CMM	38	20	18
PTE2	29	9 (8%)	20 (20%)
SL	124 (59%)	82 (74%)	42 (42%)
WW	2	-	2
Death	2	-	2
Not mentioned	16	-	16

¹Subjects (n = 41) from ref. [35] were not included in calculation of mean age (n = 170, 70 Asian and 100 Caucasian).

²In one patient, PTE failed followed by SL treatment (ref. [147]) and another treated with hybrid procedures (ref. [133]). CAF: Coronary artery fistula; CCFs: Coronary-cameral fistulas; CVFs: Coronary-vascular fistulas; CMM: Conservative medical management; F: Female; M: Male; PTE: Percutaneous therapeutic embolization; RHC: Right heart catheterization; SL: Surgical ligation; sPAP: Systolic pulmonary artery pressure; RVSP: Right ventricular systolic pressure.

Table 2 Asian and Caucasian group of patients (n = 49) with pulmonary hypertension

	Total group	Asian group	Caucasian group
n	49	15 (31%)	34 (69%)
Age1	56 (16-80)	54.4 (24-77)	56.8 (16-80)
Gender	F 34 (69%)	F 12 (80%)	F 22 (65%)
	M 15 (31%)	M 3 (20%)	M 12(35%)
CAF
Unilateral	37 (76%)	9 (60%)	28 (82%)
Bilateral	12 (24%)	6 (40%)	6 (18%)
PHT
Mild	26 (53%)	8/15 (53%)	18/34 (53%)
Moderate	11 (22%)	2/15 (13%)	9/34 (26%)
Severe	12 (25%)	5/15 (33%)	7/34 (21%)
Mean PAP (mmHg)	35.6 (range 26-60)	36.9 (range 27-49)	34.3 (range 26-60)
Mean Qp:Qs ratio	1.9 (range 1.13-2.75)	1.9 (range 1.13-2.75)	1.9 (range 1.3-2.7)
RHC	43 (88%)	13 (87%)	30 (88%)
Doppler (sPAP)	6 (12%)	2 (13%)	4 (12%)
CAF characteristics
Origin	R 8, L 30, bilateral 11	R 2, L 8, bilateral 5	R 6, L 22, bilateral 6
Termination	RH side 45 LH side 4	RH side 13 LH side 2	RH side 32 LH side 2
Mode of termination
CVFs	37 (76%)	9/15 (60%)	28/34 (82%)
CCFs	12 (24%)	6/15 (40%)	6/34 (18%)
Associated disorders	17/49 (35%)	5/15 (33%)	12/34 (35%)
Management
SL	30 (61%)	9	21
PTE	13 (27%)	4	92
CMM	6 (12%)	2	4

¹Subjects from ref. [35] were not included in calculation of mean age. Mean age was calculated from 170 (70 Asian and 100 Caucasian) subjects.

²One PTE failed (from ref. [147]) followed by SL treatment and another treated with hybrid procedures (from ref. [133]). CAF: Coronary artery fistula; CCFs: Coronary-cameral fistulas; CVFs: Coronary-vascular fistulas; CMM: Conservative medical management; F: Female; R: Right coronary artery; L: Left coronary artery; LH: Left heart side; M: Male; PAP: Pulmonary artery pressure; PHT: Pulmonary hypertension; PTE: Percutaneous therapeutic embolization; RH: Right heart side; RHC: Right heart catheterization; SL: Surgical ligation; sPAP: Systolic pulmonary artery pressure.

Table 3 Mode of termination coronary-vascular fistulas vs coronary-cameral fistulas in the pulmonary hypertension (n = 49) and all reviewed (n = 211) subjects

	CVFs	CCFs	Mean age and range (yr)
Total n = 211	90/211 (43%)	121/211(57%)	38.3 (26-67)
Asian 15/111 (14%)	9/15 (60%)	6/15 (40%)	39.7 (27-67)
Caucasian 34/100 (34%)	28/34 (82%)	6/34 (18%)	36.8 (26-60)

CCFs: Coronary-cameral fistulas; CVFs: Coronary-vascular fistulas.

Table 4 Possible complications of coronary artery fistulas

Complication	Features
Cardiovascular	Myocardial infarction, stroke, aneurysm, rupture
Infectious	Bacterial endocarditis, septic pulmonary and septic renal embolism
Valvular	Incompetence, dysfunction, perforation
Pericardial	Hemopericardium, pericardial effusion, tamponade
Myocardial	Congestive heart failure
Arrhythmic	Supraventricular arrhythmias, ventricular arrhythmias and sudden death