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Table of Contents   
Year : 2017  |  Volume : 10  |  Issue : 3  |  Page : 306-309
Hypoplastic left heart syndrome with coronary-cameral fistulas: Echocardiographic demonstration of coronary artery steal and successful interventional treatment

1 Department of Pediatrics, Division of Cardiology, University of Oklahoma Health Sciences Center, Oklahoma, USA
2 Department of Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

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Date of Web Publication21-Aug-2017


We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.

Keywords: Coronary artery fistulas, coronary steal, hypoplastic left heart syndrome

How to cite this article:
Mir A, Niu M, Yamada M, Overholt E. Hypoplastic left heart syndrome with coronary-cameral fistulas: Echocardiographic demonstration of coronary artery steal and successful interventional treatment. Ann Pediatr Card 2017;10:306-9

How to cite this URL:
Mir A, Niu M, Yamada M, Overholt E. Hypoplastic left heart syndrome with coronary-cameral fistulas: Echocardiographic demonstration of coronary artery steal and successful interventional treatment. Ann Pediatr Card [serial online] 2017 [cited 2022 Aug 9];10:306-9. Available from:

   Clinical Summary Top

A 9-year-old male patient with hypoplastic left heart syndrome (HLHS), (mitral stenosis and aortic atresia) who had successfully completed three staged palliation presented 6 years following Fontan palliation with new-onset exertional symptoms characterized by chest pain and syncope during exercise. An exercise stress test demonstrated ST-segment depression in the inferolateral leads [Figure 1]. These findings corresponded clinically with arterial desaturation related to low cardiac output, hypotension, chest pain, and dizziness.
Figure 1: 12-lead electrocardiogram during exercise stress test showing evidence of horizontal ST-segment depression in inferior leads and lateral leads during peak exercise

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To evaluate his coronary arterial circulation, cardiac catheterization with coronary angiography and transesophageal echocardiogram (TEE) was performed. By angiography, there were multiple and prominent fistulous communications between his left coronary artery system and the hypoplastic left ventricle (LV) [Figure 2]. His right coronary artery (RCA) was normal with no evidence of fistulous communications to the LV or left coronary system. His LV systolic pressure was 258 mmHg with an end-diastolic pressure (EDP) of 15 mmHg, with mild mitral regurgitation. The simultaneous right ventricular pressure was 74 (systolic)/10 (EDP) mmHg. His systemic blood pressure was 80/51 mmHg. His baseline TEE demonstrated antegrade diastolic flow across a dilated RCA during diastole into his systemic right ventricle [Figure 3]. Isoprenaline was then infused to simulate exercise. With drug-induced chronotropic and inotropic response, there was retrograde flow from the RCA by Doppler interrogation during systole [Figure 4]. These findings suggested that diastolic runoff into the fistulas created a steal phenomenon from the RCA and was responsible for both his clinical symptoms as well as the electrocardiographic ST segment changes in the distribution of the RCA.
Figure 2: An angiogram in the native ascending aorta shows dilated let coronary system with several small fistulous communications (arrowheads) from the circumflex coronary artery into the hypoplastic left ventricle which fills because of the fistulas communications

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Figure 3: (a and b) Transesophageal echocardiogram still frame shows dilated right coronary artery system with antegrade blood flow during diastole (blue), spectral Doppler imaging to confirm antegrade blood flow in diastole

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Figure 4: (a and b) Transesophageal echocardiogram still frame at elevated heart rate of around 150 bpm now shows that there is evidence of retrograde flow in systole in proximal right coronary artery by color flow mapping and with spectral Doppler imaging. Also note that there is evidence of decreased antegrade blood flow across right coronary artery during diastole using pulse wave Doppler, electrocardiogram tracing showed increased ST depression at heart rate of 154

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Given the hemodynamic significance of these fistulas, transcatheter coil embolization of the LV was performed using several coils to completely obliterate the LV cavity. Access to the LV was obtained through the Fontan baffle. A renal double curve catheter was advanced into the LV across the mitral valve to perform a left ventricular angiogram. A 2.7-Fr Progreat catheter (Terumo Interventional systems, New Jersey, USA) was used to perform coil embolization of the LV using 7 AZUR coils (Terumo Interventional systems, New Jersey, USA). A repeat angiogram after coil embolization demonstrated minimal flow into the LV through these fistulous communications [Figure 5]. Three months later, repeat exercise stress testing showed marked improvement in exercise tolerance with no chest pain and minimal ST-segment depression in the inferior and lateral leads [Figure 6]. The patient is currently doing well with no exertional symptoms under follow-up at 6 monthly intervals.
Figure 5: A repeat angiogram in the neoaorta shows minimal flow into the hypoplastic left ventricle after coil occlusion of these fistulas

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Figure 6: A repeat electrocardiogram exercise stress test shows no significant ST-segment depression in inferior and lateral leads at peak exertion

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   Discussion Top

Coronary artery fistulous communications with the ventricular cavity, also called coronary-cameral communications, have been described in HLHS with mitral stenosis and aortic atresia. O'Connor et al. initially described these communications in an autopsy study of nine patients.[1] These fistulas are congenital and have been known to enlarge with age. The pathophysiology is that there is increased flow across these fistulas due to lower resistance than normal arterioles and capillaries, which traverse the myocardium. With larger fistulas, runoff occurs which draws blood way from the normal coronary pathway leading to coronary steal. Our patient had prominent fistulas from his left coronary system and during simulated exercise with isoprenaline, had retrograde flow in his RCA system with ST-segment depression proving that these fistulas were significant and explaining his symptoms. During elevated heart rate, the flow in the RCA is both during systole and diastole.[2] We hypothesize that during exercise, there is elevated systolic and diastolic right ventricular pressure causing an increased pressure gradient across the normal right coronary vascular bed. This leads to reversal of flow in systole and decreased diastolic flow in his normal right coronary arterial system with preferential shunting of flow through the lower resistance fistulas into the LV during diastole.

HLHS with coronary artery fistulas has been noted to have variable morbidity and mortality outcomes, with several reports identifying their presence as a risk factor for increased mortality.[3],[4] A recent paper reported an operative mortality of approximately 29% in these patients with autopsies suggesting myocardial ischemia as the cause of death.[3] At present, there is little data to guide assessment and risk stratification of these fistulas to determine which fistulas will become clinically and hemodynamically significant [4],[5] and interventions to interrupt coronary cameral fistulas are not routinely performed at the time of initial surgery in most centers.

In conclusion, we present a case of HLHS s/p Fontan palliation with significant coronary artery fistulas into the hypoplastic LV and clinical symptoms of syncope and exertional intolerance. By coil embolization, the LV was occluded and the drainage site of these fistulas was eliminated, resulting in resolution of clinical symptoms in the short term.

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Conflicts of interest

There are no conflicts of interest.

   References Top

O'Connor WN, Cash JB, Cottrill CM, Johnson GL, Noonan JA. Ventriculocoronary connections in hypoplastic left hearts: An autopsy microscopic study. Circulation 1982;66:1078-86.  Back to cited text no. 1
Watanabe N, Awa S, Akagi M, Ando Y, Oki N, Waragai T, et al. Effects of heart rate and right ventricular pressure on right coronary arterial flow and its systolic versus diastolic distribution in a variety of congenital heart diseases in children. Pediatr Int 2000;42:476-82.  Back to cited text no. 2
Siehr SL, Maeda K, Connolly AA, Tacy TA, Reddy VM, Hanley FL, et al. Mitral stenosis and aortic atresia – A risk factor for mortality after the modified Norwood operation in hypoplastic left heart syndrome. Ann Thorac Surg 2016;101:162-7.  Back to cited text no. 3
Vida VL, Bacha EA, Larrazabal A, Gauvreau K, Dorfman AL, Marx G, et al. Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2008;135:339-46.  Back to cited text no. 4
Sathanandam S, Cui W, Nguyen NV, Husayni TS, Van Bergen AH, Sajan I, et al. Ventriculocoronary artery connections with the hypoplastic left heart: A 4-year prospective study: Incidence, echocardiographic and clinical features. Pediatr Cardiol 2010;31:1176-85.  Back to cited text no. 5

Correspondence Address:
Arshid Mir
Department of Pediatrics, Division of Cardiology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_22_17

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]