Prenatal diagnosis of isolated coronary artery fistula: systematic review, analysis of perinatal prognostic factors and case report

Figures & data

Table 1. Details of the reported cases with prenatal diagnosis of isolated coronay artery fistula.

Case	Study	GA at diagnosis (wk)	Origin	Drainage	Inicial size (mm)	Prenatal follow up				GA at delivery (wk)	Delivery	Gender	Postnatal follow up
						Cardiomegaly	Diastolic steal	Fistula size	Hydrops				Neonatal outcome	Age at treatment	Treatment	Clinical outcome
1	Sharland, 1996 [12]	20	RCA	RV	3	Yes	Yes	Progression	No	38	Vaginal	Female	CHF	5 mth	Coil	Death
2	Cotton, 2000 [13]	30	LCA	RV		No	No		No	34	CS		Asymptomatic			Alive, spontaneous closure
3	Mielke, 2002 [14]	23	LCA	RA	2	No		Stable	No	38	Vaginal	Female	Asymptomatic	9 days	Coil	Alive
4	Khan, 2003 [15]	22	RCA	RA		Yes		Progression	No	>37		Male	CHF	3 wk	Amplatzer	Alive
5	Hung, 2006 [16]	20	RCA	RV		Yes		Progression	No	37	CS	Female	CHF	12 days	Coil	Alive
6	Karagöz, 2008 [17]	33	RCA	RV		Yes			No	38		Male	CHF	1 day	Amplatzer	Alive
7	Daniel, 2010 [18]	23	RCA	RV		Yes	Yes	Stable	No	39	CS	Female	CHF	4 day	Surgery	Alive
8	Hayashi, 2012 [7]	33	RCA	RV	4	Yes	Yes		No	39	Vaginal	Male	CHF	3 mth	Surgery	Alive
9	Zhao, 2012 [19]	37	RCA	RV	5	No	Yes	Stable	No	39	Vaginal	Male	Asymptomatic	6 mth	Surgery	Alive
10	Matsumoto, 2012 [20]	38	LCA	RV	12	Yes	Yes		No	40	CS		CHF	1 day	Surgery	Alive
11	Nagiub, 2014 [21]	31	RCA	RV		Yes	Yes	Progression	No	37	CS	Male	CHF	2 days	Coil	Alive, T21
12	Oztunc, 2015 [22]	28	LCA	RV		Yes	Yes		Yes	32	CS	Female	CHF. Hydrops			Neonatal death
13	Sharland, 2016 [23]	22	RCA	RA	2	Yes	Yes	Progression	No	>37		Female	CHF	5 days	Amplatzer	Alive
14	Sharland, 2016 [23]	19	RCA	DA	1.6	Yes	Yes	Progression	No	>37		Female	CHF	17 days	Amplatzer	Alive
15	Sharland, 2016 [23]	21	RCA	LV	1.5	No	No	Progression	No	>37		Female	Asymptomatic	7 years	Amplatzer	Alive
16	Sharland, 2016 [23]	19	LCA	RA	1	No	No	Stable	No	>37		Female	Asymptomatic	4 years	Amplatzer	Alive
17	Sharland, 2016 [23]	20	RCA	RA	0.9	Yes	Yes	Progression	No	>37		Male	Asymptomatic	20 mth	Amplatzer	Alive
18	Kaldararova, 2016 [24]	22	LCA	RV				Stable	No	38		Female	CHF	1 day	Surgery	Alive
19	Zeng, 2016 [5]	23	LCA	RA	2.2	Yes			No	0		Female				TOP
20	Zeng, 2016 [5]	22	RCA	LV	4	No			No	0		Female				TOP
21	Wacker-Gussmann, 2018 [25]	22	LCA	RV	2.9	Yes	Yes	Progression	No	37	CS		CHF			Neonatal death
22	TekeYesn, 2018 [26]	23	LCA	RA	1.4	No		Progression	No	39	CS	Female	Asymptomatic	3 days	Surgery	Alive
23	Chae, 2018 [27]	25	LCA	CoS		No	Yes	Progression	No	39		Female	CHF. Myocardial ischemia	3 days	Surgery	Alive
24	Zhao, 2019 [6]	24	RCA	LV	3	No	No	Stable	No	38	Vaginal	Male	Asymptomatic			Alive, asymptomatic
25	Cui, 2020 [28]	35	RCA	RA	21	Yes			No	36	CS	Female	CHF	1 day	Surgery	Death
26	Li, 2020 [29]	25	RCA	RV	2.3	No		Progression	No	39	CS	Male	Asymptomatic	3 mth	Surgery	Alive
27	Current case	21	RCA	RV	2	No	Yes	Stable	No	37	Vaginal	Female	CHF	14 days	Amplatzer	Alive

CS: cesarean section; CHF: congestive heart failure; CoS: coronary sinus; DA: ductus arteriosus; LCA: left coronary artery; LV: left ventricle; mth: months; RA: right atrium; RCA: right coronary artery; RV: right ventricle; T21: trisomy 21; TOP: termination of pregnancy; wk: week.

Figure 1. Short-axis view of the fetal heart. Color Doppler shows the presence of a fistulous tract arising from the right coronary artery, running through the posterior atrio-ventricular groove and draining into the right ventricle (RV). The filling of the fistulous tract is antegrade during ventricular diastole (A) and reversed during systole (B). Ao: aorta; CAF: coronary artery fistula.

Figure 2. Selective right coronary artery angiography showing dilation of the fistulous tract with a distal narrowing (arrow) prior to its drainage into the right ventricle (RV). Ao: aorta; CAF: coronary artery fistula.

Table 2. Clinical characteristics and perinatal outcome of prenatally diagnosed isolated coronary artery fistula.

VARIABLE	VALUE
Maternal age (mean, SD)	29.16 ± 5.5 years
GA at diagnosis (mean, SD)	25.22 ± 5.7 weeks
Initial size (mean, SD)	4.04 ± 4.91 mm
Origin (n, %)
RCA	17/27 (63)
LCA	10/27 (37)
Drainage (n, %)
RA	8/27 (29.6)
RV	15/27 (55.6)
LV	3/27 (11.1)
CoS	1/27 (3.7)
Prenatal evolution (n, %)
Cardiomegaly (n, %)	15/26 (57.7)
Diastolic steal (n, %)	13/17 (76.5)
Progressive dilation of the fistula	13/18 (72.2)
Hydrops	1/27 (3.2)
GA at birth (mean, SD)	37.84 ± 1.88 weeks
Delivery (n, %)
Vaginal	6/16 (37.5)
Cesarean section	10/16 (62.5)
Gender (n, %)
Male	8/24 (33.3)
Female	16/24 (66.7)
Postnatal symptoms (n, %)	16/25 (64)
Intervention (n, %)	21/25 (84.0)
Age at treatment (mean, SD)	0.3 ± 20.5 months
Neonatal intervention (n, %)	14/21 (66.7)
Type of intervention (n, %)
Coil	4/21 (19.0)
Amplatzer	8/21 (38.1)
Surgical ligation	9/21 (42.9)
Outcome (n, %)
Alive	21/25 (84.0)
Neonatal death	3/25 (12.0)
Infant death	1/25 (4.0)

CS: cesarean seccion; CoS: coronary sinus; GA: gestational age; LCA: left coronary artery; LA: left atrium; LV: lef ventricle; RA: right atrium; RCA: right coronary artery; RV: right ventricle; SD: standard deviation.

Table 3. Analysis of prenatal variables for the prediction of postnatal severity.

Variable	Postnatal symptoms				Neonatal intervention
	Yes (n, %)	No (n, %)	p	OR (CI 95%)	Yes (n, %)	No (n, %)	p	OR (CI 95%)
Origin from RCA	11/16 (68.8)	5/9 (55.6)	.671	2.00 (0.35–11.55)	9/15 (60.0)	5/6 (83.3)	.613	0.30 (0.03–3.25)
Drainage
Right heart	16/23 (69.6)	0/2 (0)	.120	11.00 (0.47–258.43)	14/20 (70.0)	0/1 (0)	.333	6.69 (0.24–187.29)
Atrium	4/9 (44.4)	10/13 (76.9)	.187	0.24 (0.04–1.51)	6/9 (66.7)	6/10 (60)	1.00	1.33 (0.20–8.71)
Cardiomegaly	13/14 (92.9)	2/10 (20.0)	<.001	52.00 (4.03–670.63)	9/12 (75.0)	4/8 (50.0)	.356	3.00 (0.45–20.15)
Diastolic steal	11/13 (84.6)	0/4 (0)	.006	41.40 (1.64–1042.48)	7/11 (63.6)	0/2 (0)	.192	8.33 (0.32–215.69)
Progressive dilatation	9/13 (69.2)	1/5 (20)	.118	9.00 (0.75–108.31)	8/12 (66.7)	2/4 (50.0)	.604	2.00 (0.20–19.91)
GA at diagnosis*	27.9 (7.2)	25.2 (5.8)	.848	1.02 (0.86 − 1.20)	25.3 (6.3)	24.8 (3.4)	.560	1.05 (0.88 − 1.25)

*Data presented as mean (standard deviation).

CI: confidence interval; GA: gestational age; OR: odds ratio; p: p-value; RCA: right coronary artery.

Data availability statement

The datasets used and/or analyses performed during the study are available from the corresponding author upon reasonable request.