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JACC Case Rep
JACC Case Rep
JACC Case Reports
2666-0849
Elsevier

S2666-0849(24)00261-4
10.1016/j.jaccas.2024.102468
102468
Mini-Focus Issue on Congenital Heart Disease
Imaging Vignette: Clinical Vignette: ACC.24
Right Ventricle–Pulmonary Artery Conduit Replacement Resolves Anomalous Single Coronary Stenosis in Repaired Tetralogy of Fallot
Lundin Karl Kristian MD Karl.Lundin@bcm.edu
a∗
Hickey Edward MD ab
Masand Prakash MD ac
Dimas Vivian MD d
Salciccioli Katherine Bohard MD ae
a Baylor College of Medicine, Houston, Texas, USA
b Texas Children's Hospital Division of Congenital Heart Surgery, Houston, Texas, USA
c Texas Children’s Hospital Department of Radiology, Houston Texas, USA
d Medical City Children’s Hospital, Dallas, Texas, USA
e Texas Children's Adult Congenital Cardiology, Houston, Texas, USA
∗ Address for correspondence: Dr Karl Lundin, c/o Dr Katherine Salciccioli, 6651 Main Street East 1920, Houston, Texas 77030, USA. Karl.Lundin@bcm.edu
21 8 2024
21 8 2024
21 8 2024
29 16 1024681 5 2024
27 6 2024
2 7 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
A 41-year-old man with repaired tetralogy of Fallot and a single coronary artery (CA) arising anteriorly presented with dyspnea in the setting of moderate right ventricle-pulmonary artery conduit (RV-PAC) stenosis and moderate-to-severe extrinsic left main CA compression between the aorta and RV-PAC. His CA stenosis resolved after successful RV-PAC replacement.

Graphical Abstract

Key Words

computed tomography
congenital heart defect
coronary vessel anomaly
stenosis
tetralogy of Fallot
valve replacement
Abbreviations and Acronyms

CA coronary artery

CT computed tomography

EF ejection fraction

RV-PAC right ventricle–pulmonary artery conduit
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pmcA 41-year-old man with congenital heart disease presented with worsening dyspnea on exertion after years lost to follow-up. His physical exam was notable for a III/VI systolic ejection murmur at the left mid-sternal border and a II/VI diastolic murmur at the left lower sternal border. His cardiac history included tetralogy of Fallot with pulmonary atresia repaired via placement of a 21-mm right ventricle–pulmonary artery conduit (RV-PAC) at age 5 years and a single coronary artery (CA) originating from the right coronary cusp. Additional medical history included previous nonsustained ventricular tachycardia and obesity.

Electrocardiography showed first degree atrioventricular block and right bundle branch block. Echocardiography demonstrated mildly depressed biventricular function (left ventricular ejection fraction [EF] 48%), an RV-PAC with moderate stenosis (peak velocity 3.5 m/s) and moderate regurgitation, and mild aortic regurgitation. Cardiac computed tomography (CT) showed moderate-to-severe RV-PAC calcification and a single CA arising anteriorly from the right coronary cusp with moderate-to-severe stenosis where the left main CA branch passed between the patient’s aorta and RV-PAC (Figures 1A to 1C). Magnetic resonance imaging showed mild right ventricular dilatation with moderately depressed function (EF 39%), normal left ventricular size with mildly depressed function (EF 50%), no regional wall motion abnormalities, and a moderate-to-severely dilated aortic root and ascending aorta (50 mm). An exercise perfusion scan from 5 years before and an exercise echocardiogram from 2 years before were both unremarkable.Figure 1 Preoperative and Postoperative Cardiac Computed Tomography Angiography Demonstrating Improved Coronary Artery Stenosis

(A) Preoperative cardiac computed tomography 3-dimensional reconstruction showing a single coronary artery (CA) arising from the right coronary cusp with the left main branch (yellow arrow) passing between the aorta (Ao) and right ventricle–pulmonary artery conduit (RV-PAC). (B and C) Single CA arising from the right coronary cusp with severe focal left main CA stenosis (red arrow), anterior (B) and cranial (C) views. (D and E) Single CA arising from the right coronary cusp with resolution of focal left main CA stenosis (green arrow), anterior (D) and cranial (E) views.

Per American College of Cardiology/American Heart Association (ACC/AHA) guidelines, the patient’s symptoms and significant RV-PAC dysfunction provided a Class I indication for RV-PAC replacement.1 A multidisciplinary cardiovascular team discussed management of the CA stenosis: CA angiography and/or fractional flow reserve assessment would be high risk for dissection with decreased accuracy because the left CA divided into its major branches immediately distal to the stenosis. Repeat noninvasive stress testing would have indeterminate sensitivity and not differentiate between extrinsic vs intraluminal narrowing. Pre-emptive CA intervention risked postsurgical stenosis if using arterioplasty and competitive flow and eventual graft failure if using bypass graft. Suspecting CA compression by the RV-PAC, the team recommended forgoing additional ischemic evaluation and surgically replacing the RV-PAC farther from the aorta without direct CA intervention. After successful RV-PAC replacement, intraoperative echocardiography showed stable cardiac function and low-velocity diastolic blood flow throughout the left CA. Postoperative cardiac CT showed significant improvement in the patient’s left main CA stenosis (Figures 1D and 1E). His postoperative course was unremarkable. At 6-month follow-up, his biventricular function had recovered (left ventricular EF 55%) with excellent RV-PAC function. The patient’s exertional symptoms were significantly improved.

CA stenosis via external compression occurs in patients with various pulmonary and/or aortic pathologies and procedural histories;2 anomalous CAs have an increased risk of compression.1 AHA/ACC guidelines for patients with tetralogy of Fallot recommend CA compression assessment before RV-PAC stenting or transcatheter pulmonary valve replacement, but do not discuss surgical considerations.1 Some cases of CA compression resolve with RV-PAC replacement, whereas others require direct intervention on the CA.3 Extrinsic CA compression is rare, and the heterogeneity of cases necessitates evaluation by a multidisciplinary team to determine optimal management.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
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References

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