Incidental congenital coronary artery vascular fistulas in adults: Evaluation with adenosine-13N-ammonia PET-CT

Table 1 Symptoms, clinical presentation and physical findings of adult subjects with congenital coronary artery fistula

Case	Fistula origin and termination	Symptoms and clinical presentation	Previous history and risk factors	Physical findings	BMI	Intervention
1	RCA and LAD to PA	Chest pain	Tubular adenoma of sigmoid, celiac disease	Normal	32.7	None
		NSTEMI/PMI
		(CK 390 U/L)
2	LAD to PA	Chest pain	Old IMI	Normal	25.9	CABG and surgical closure of the fistula
		NSTEMI	Asthma
		(CK 573 U/L)
3	LCx to PA	Chest pain	Blanco	Normal	26.6	Coiling of the fistula and PCI of LAD
		Dyspnea on exertion
4	RCA to CS	Dyspnea on exertion	Diaphragmatic hernia, asthma and hypertension	Systolic ejection murmur grade 2/6 2 d ICS	28.2	None
5	LAD to PA	Non-sustained VT	DM, COPD, hypertension, hypothyroidism	Systolic ejection murmur grade 2/6 2 d ICS	33.4	PCI of OM branch and FFR of LAD. Coiling of the fistula
6	LAD to PA	Dyspnea on exertion	COPD	Apical mitral regurgitation murmur grade 2/6	25.5	Mitral valve plasty
			Severe mitral valve regurgitation
7	LAD to PA	Chest pain	Celiac disease	Normal	24.2
8	LAD to PA	Angina pectoris	Hypercholesterolemia	Systolic ejection murmur grade 2/6 2 d ICS	21.1	PCI of LAD
			Positive family history for CAD
9	LCx to PA	Palpitation	Ischemic CVA	Normal	24.8	None
		PAF and non-sustained VT
		Hypertension
10	LAD to PA	Angina pectoris	NSTEMI 2010 (CK 328 U/L). PCI of RCA 2012	Normal	24.2	Coiling of the fistula
11	LAD to PA	Chest pain	Hypertension, hypercholesterolemia	Normal	29.1	None
			Positive family history for CAD
			Smoker

BMI: Body mass index; CABG: Coronary artery bypass grafting; CAD: Coronary artery disease; CK: Creatinine kinase; COPD: Chronic obstructive pulmonary disease; CS: Coronary sinus; CVA: Cerebral vascular disease; DM: Diabetes mellitus; FFR: Fractional flow reserve; ICS: Intercostal space; IMI: Inferior wall myocardial infarction; LAD: Left anterior descending coronary artery; LCx: Left circumflex coronary artery; NSTEMI: Non-ST elevation myocardial infarction; OM: Obtuse marginal branch; PA: Pulmonary artery; PAF: Paroxysmal atrial fibrillation; PCI: Percutaneous coronary intervention; PMI: Posterior myocardial infarction; RCA: Right coronary artery; VT: Ventricular tachycardia.

Table 2 Angiographic characteristics of fistula components

Case	Fistula origin and termination	Yr of detection	Angiographic characteristics of fistula components
			LCx			RCA			LAD
			O	P	T	O	P	T	O	P	T
1	RCA and LAD to PA	2014				M	MT	M	S	MT	S
2	LAD to PA	2015							M	MT	M
3	LCx to PA	2015	S	ST	S
4	RCA to CS	2015				S	ST	S
5	LAD to PA	2016							S	ST	S
6	LAD to PA	2016							M	M	M
7	LAD to PA	2016							M	MT	M
8	LAD to PA	2015							M	MT	S
9	LCx to PA	2016	M	MT	M
10	LAD to PA	2017							M	M	M
11	LAD to PA	2017							M	MT	S

CS: Coronary sinus; LAD: Left anterior descending coronary artery; LCx: Left circumflex coronary artery; RCA: Right coronary artery; PA: Pulmonary artery; M: Multiple; MT: Multiple and tortuous; S: Single; ST: Single and tortuous; T: Termination; O: Origin; P: Pathway.

Table 3 Pharmacological adenosine stress/rest ¹³N-ammonia positron emission tomography computed tomography in 5 patients with unilateral coronary vascular fistulas

Case	CAF	Stress/rest perfusion segments				LVEF (%)		Semi-quantitative findings	CAD	Prior procedure	Management of CAF
		LCx	RCA	LAD	Global	Rest	Stress
3	LCx-PA	2.74	2.49	2.56	2.59	52	57	No perfusion defects	1-VD	PCI-LAD	PTE
4	RCA-CS	2.65	2.7	2.71	2.69	65	65	Diffuse reversible reduction of perfusion in the apical, antero-septal and partially in basal anterior segment	None	None	CMM
7	LAD-PA	2.36	2.55	2.35	2.33	60	65	Perfusion of LAD area is less than inferior segment	None	None	CMM
8	LAD-PA	3.02	2.91	2.94	2.95	33	39	Normal perfusion with reduced LVEF	1-VD	PCI-LAD	CMM
11	LAD-PA	3.2	3.6	4.5	3.9	65	65	No perfusion defects	None	None	CMM

CAD: Coronary artery disease; CAF: Coronary artery fistula; CMM: Conservative medical management; CS: Coronary sinus; LAD: Left anterior descending coronary artery; LCx: Left circumflex coronary artery; LVEF: Left ventricular ejection fraction; PA: Pulmonary artery; PCI: Percutaneous coronary intervention; PTE: Percutaneous transcatheter embolization; RCA: Right coronary artery; VD: Vessel disease.