Coronary-cameral fistulas in adults (first of two parts)

Table 1 Data of adult patients with congenital coronary artery-ventricular multiple micro-fistulas and solitary macro fistulas

Case Age/gender	Clinical presentation	Previous history	Concomitant disorders	MMFs fistula	ECG	Echocardiography	Myocardial perfusion test	Management
1, 44M	CP	TIA/Lyme disease	-	D-LV Unilateral 0-VD	SR	N	-	CMM
2, 73M	CP	SSS	COPD/RR/GN	D-LV dRCA-LV Bilateral 0-VD (Figure 1 A and B)	SR	N	Apical ischemic changes	CMM, DDDR
3, 62F	NSTEMI	-	-	D-LV dRCA-LV Bilateral 0-VD	SR	N	-	CMM
4, 45F	CP	-	RR	D-LV dRCA-LV Bilateral 0-VD	SR	N	-	CMM
5, 65F	AP	Old IMI/ breast carcinoma	COPD/RR/hypothyroidism	Cx-LV dRCA-LV Bilateral 1-VD	SR old IMI	Hypokinesia inferior	Mid baso-inferior EF 60%	CMM, PCI RCA
6, 62M	AP	-	RR	D-LV Unilateral 3-VD	SR	Anterolateral hypokinesia	-	CMM, CABG
						and apical akinesia
7, 70F	CP	TIA	-	AL-LV Unilateral 0-VD	SR	N	Negative	CMM
8, 65M	AP	Old IMI	DM/OSAS	RCA-RV Unilateral 1-VD	SR icRBBB	N	-	CMM
9, 79F	AP	-	RR	LAD-LV RCA-LV Bilateral 0-VD	SR LVH	LVH	-	CMM
10, 64F	DOE	Old ILMI	AF/AR/epilepsy	Cx-LV RCA-LV Bilateral 0-VD	AF LBBB	Moderate LV	-	CMM, AVR
						systolic function
11, 52M	DOE	MR/MVP/PAF	RR	Solitary macro CCF RCA-RA	SR RBBB	Severe MR	-	MVR/PVI/SL

AR: Aortic regurgitation; AL: Anterolateral branch; AP: Angina pectoris; AVR: Aortic valve replacement; CABG: Coronary artery bypass grafting; CP: Chest pain; CMM: Conservative medical management; COPD: Chronic obstructive pulmonary disease; Cx: Circumflex coronary artery; d: Distal; D: Diagonal branch; DM: diabetes mellitus; DOE: Dyspnoea on exertion; EF: Ejection fraction; F: Female; GN: Glomerulonephritis; ic: Incomplete; ILMI: Infero-lateral myocardial infarction; IMI: Inferior myocardial infarction; LAD: Left anterior descending coronary artery; LBBB: Left bundle branch block; LV: Left ventricle; LVH: Left ventricular hypertrophy; M: Male; MMFs: Coronary artery-ventricular multiple micro-fistulas; MR: Mitral regurgitation; MVP: Mitral valve plasty; MVR: Mitral valve replacement; N: Normal; NSTEMI: Non-ST elevation myocardial infarction; OSAS: Obstructive sleep apnoea syndrome; PCI: Percutaneous coronary intervention; PAF: Paroxysmal atrial fibrillation; PVI: Pulmonary vein isolation; RA: Right atrium; RBBB: Right bundle branch block; RCA: Right coronary artery; RR: Hypertension; SL: Surgical ligation; SR: Sinus rhythm; SSS: Sick sinus syndrome; TIA: Transient ischemic attack; VD: Vessel disease.

Table 2 Results of literature review of 243 subjects with coronary-cameral fistulas (65% congenital and 35% acquired)

Condition	n (%)	Mean age /range yr	Etiology	Management
	female %
MMFs	24 (15)	62.7 (39-85)	Congenital	CMM 100%
	Female 63%		part I
CCFs	135 (85)	46.2 (18-85)	Congenital	CMM 22%, SL 56%, PTE 22%
	Female 50%		part I
CCFs	7 (3)	24.1 (17-38)	Accidental	Emergent surgical intervention 100%
	Female 0%		part II
CCFs	8 (3.3)	55.8 (46-73)	Iatrogenic (pacing)	CMM
	Female 38%		part II	Spontaneous resolution
CCFs	7 (3)	66.5 (58-75)	Iatrogenic (PCI)	CMM
	Female 29%		part II
CCFs	25 (10.3)	50.8 (43-64)	Iatrogenic (EMB)	CMM
	Female 22%		part II	Spontaneous resolution 27%
CCFs	5 (2.1)	61 (40-78)	Iatrogenic (surgery)	CMM
	Female 20%		part II
CCFs	20 (8.2)	45 (32-74)	Iatrogenic (SM)	CMM 11%, PTE 11%
	Female unknown		part II	Spontaneous resolution 78%
CCFs	12 (5)	61 (29-75)	Spontaneous (post-MI) part II	CMM 60%, SL 30%
	Female 0%			Spontaneous resolution 10%

CCFs: Coronary cameral fistulas; CMM: Conservative medical management; EMB: Endomyocardial biopsy; MI: Myocardial infarction; MMFs: Coronary artery-ventricular multiple micro-fistulas; PCI: Percutaneous coronary intervention; PTE: Percutaneous therapeutic embolization; SL: Surgical ligation; SM: Septal myectomy.

Table 3 Fistula characteristics in congenital and acquired coronary-cameral fistulas in adults

	Congenital CCFs (0.07%)[1]			Acquired CCFs
				Latrogenic CCFs			Accidental CCFs
	Solitary Macro CCFs (large≥1.5 mm)	Solitary Macro CCFs (small ≤1.5 mm)	Multiple Micro MMFs	Post-SM	Post-EMB	Post-pacing	Blunt or sharp chest trauma
Prevalence/incidence	0.03%[1]	0.04%[1]	0.09%[1]	19%-23%[5,56]	2.8%-23.2%[57-60]	Unknown	Unknown
Fistula characteristics
Origin	Proximal segment of coronary arteries		Distal segment of coronary arteries	Septal perforator	RCA>LAD> Cx	LCA	RCA or LAD
Termination	Any cardiac chamber		LV > RV	LV	RV	Any cardiac chamber	RV or LV
Management	CMM/SL/PTE		CMM (100%)	SC (78%)/CMM 11%	SC (27%)	CMM/SC	Surgical repair (100%)
			Incidentally ICD	PTE 11%

CMM: Conservative medical management; EMB: Endomyocardial biopsy; LA: Left atrium; LAD: Left anterior descending artery; LCA: Left coronary artery; LV: Left ventricle; PTE: Percutaneous therapeutic embolization; RA: Right atrium; RCA: Right coronary artery; RV: Right ventricle; SC: Spontaneous closure; SL: Surgical ligation; SM: Septal myectomy; CCFs: Coronary-cameral fistulas.