CVIA A Case of Right Coronary Artery CASE REPORT pISSN 2508-707X / eISSN 2508-7088 Fistula to Right Ventricle with https://doi.org/10.22468/cvia.2021.00101 Cardiovasc Imaging Asia 2021;5(3):107-109 Associated Aneurysm and Thrombus in Pregnant Lady

Norain Talib, Kama Azira Awang Ramli, Yusri Mohammed Department of Radiology, Hospital Serdang, Jalan Puchong, , ,

A 27-year-old pregnant female presented to our clinic for a cardiac murmur that was de- Received: March 3, 2021 tected during an antenatal check-up. She had no cyanosis, and she was asymptomatic for Revised: March 31, 2021 heart failure. The echocardiogram revealed an enlarged right coronary sinus with right coro- Accepted: April 4, 2021 nary artery (RCA) dilatation. The electrocardiographic-gated coronary computed tomogra- Corresponding author phy angiogram showed aneurysmal RCA dilatation with fistula to the inferobasal region of Norain Talib, MD, MMed (Rad) the right ventricle. A few small thrombi were visualized in the right ventricle and within the Department of Radiology, fistula. She delivered a healthy baby via caesarean section at 31 weeks of gestation, and fis- Hospital Serdang, Jalan Puchong, tula correction was planned within one year. 43000 Kajang, Selangor, Malaysia Tel: 60-389475555 Key words Coronary vessel anomalies · Multidetector computed tomography · Fax: 60-3894732 Echocardiogram. E-mail: [email protected]

INTRODUCTION course of antibiotics. An echocardiogram had detected minimal tricuspid valve vegetation (image not shown), and no murmur Cameral fistula is defined as an abnormal congenital connec- had been documented. Her firstborn baby delivered uneventful- tion of a coronary artery to a cardiac chamber. This rare condi- ly, but the patient defaulted her cardiac follow-up. tion is usually asymptomatic, but a continuous murmur is usu- On current presentation, an echocardiogram showed right cor- ally detected in adulthood. The complications of cameral fistula onary sinus and right coronary artery (RCA) dilatation (Fig. 1A). include coronary thrombosis or myocardial infarction that pres- An echogenic right ventricular lesion measuring 2.6 cm was not- ents as angina and results in symptomatic cardiomyopathy. A high ed near the visualized septal tricuspid valve annulus (Fig. 1B and index of suspicion from clinical findings is required to lead to the C). Streptococcus mitis was diagnosed based on a blood culture, appropriate imaging modalities. and the patient received treatment. In view of the cardiac lesion detected on echocardiogram and CASE REPORT for its characterization, a cardiac magnetic resonance (CMR) was performed using Magnetom Symphony 1.5 T (Siemens Health- A 27-year-old, gravida 2 female at 28 weeks of pregnancy was ineers, Elargen, Germany). Cine images showed similar findings, referred to our clinic for asymptomatic anemia associated with aneurysmal dilatation of the RCA from the dilated coronary si- cough for three months. She denied fever, failure symptoms or nus origin. During the scan, however, the patient became unco- reduced effort tolerance. Clinically, examination revealed stable operative, and CMR was abandoned. vital signs, warm peripheries, a systolic murmur best heard at Therefore, the imaging investigation was continued with com- the apex and normal lung auscultation. puted tomography. Electrocardiogram-gated coronary comput- A few years ago, she had been diagnosed with infective endo- ed tomography angiogram using 128-Slice Dual Source multi- carditis during a previous pregnancy, and she completed a month’s slice computed tomography (MSCT Somatom Definition Flash,

cc This is an Open Access article distributed under the terms of the Creative Commons Siemens Healthineers) revealed RCA aneurysm with fistulous Attribution Non-Commercial License (https://creativecommons.org/licenses/by- communication to the inferobasal right ventricle (RV) (Fig. 2C). nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduc- tion in any medium, provided the original work is properly cited. Small thrombi were seen within the fistula and the RV (Fig. 2C

Copyright © 2021 Asian Society of Cardiovascular Imaging 107 CVIA Right Coronary Artery Fistula to Right Ventricle in Pregnant Lady and D). No mass was evident within the heart chambers. In ad- consistent with cameral fistula of RCA aneurysm to RV compli- dition, chronic thromboembolism of the subsegmental pulmo- cated by thrombus within the fistula and RV. Furthermore, pul- nary arteries was identified by a filling defect within these ves- monary infarcts and septic emboli were due to migrated throm- sels. A few pulmonary infarcts and septic emboli were seen in bus into the subsegmental pulmonary arteries. the lung parenchyma (Fig. 2E and F). Overall, these findings were A multidisciplinary conference consisting of the cardiothorac-

A B C Fig. 1. Echocardiogram. (A) Dilated right coronary sinus seen in the aortic valve window (red arrow). (B and C) An echogenic lesion (blue arrow) without intravascular color flow showed in the normal B-mode and color Doppler. This lesion proved to be an avascular thrombus on computed tomography.

A B C

D E F Fig. 2. Electrocardiogram-gated coronary computed tomography angiogram image (A) to (B). Trans-axial images of aneurysmal dilatation of the proximal-to-mid right coronary artery segment, following dilatation of the right coronary sinus (red arrow). The maximal luminal diam- eter of the RCA was 1.4 cm. (C) Trans-axial images of the inferobasal segment defect (black arrow) of the right ventricle wall close to the interventricular septum, consistent with the fistula’s entry point. (C and D) Note the thrombus (blue arrows) within the right ventricle and fis- tula. (E) Arrow shows the filling defect at the distal subsegmental right posterobasal pulmonary artery complicated with a pulmonary infarct. (F) Cavity within the peripheral pulmonary infarct (green arrow), suggestive of septic emboli.

108 Cardiovasc Imaging Asia 2021;5(3):107-109 Norain Talib, et al CVIA ic team, radiology, obstetrics and pediatrics was assembled for be treated using a multidisciplinary approach during the prena- case management, and the patient finally underwent elective cae- tal period. sarean section at 31 weeks of gestation and delivered a healthy Our patient has pre-existing coronary/cardiac thrombosis and 1.6 kg baby. The fistula correction was planned within one year. pulmonary infarcts. Although her presentation was considered late, the patient’s unborn child was healthy, and she had no symp- DISCUSSION toms of heart failure. Fistula correction was planned after her re- covery from childbirth. A coronary artery cameral fistula involves a sizable commu- Supplementary Video Legends nication between a coronary artery that bypasses the myocar- Video 1. 3D volume rendering image from electrocardiogram-gated coro- dial capillary bed and enters either chamber of the heart. The nary computed tomography angiogram of a 27-year-old pregnant female RCA is the most commonly involved artery while the RV is the with right coronary artery fistula to the right ventricle with aneurysm. commonly involved cardiac chamber. This is simply due to a low- Supplementary Materials er resistance flow, shunting blood directly from the RCA into The online-only Data Supplement is available with this article at https:// the RV [1]. More than 50% of patients are usually asymptomatic, doi.org/10.22468/cvia.2021.00101. other than a continuous murmur that may appear when they reached adulthood [2]. The most common complication is cor- Conflicts of Interest onary thrombosis causing myocardial infarction and angina lead- The authors have no potential conflicts of interest to disclose. ing to symptomatic cardiomyopathy [3]. Acknowledgments Echocardiogram is an easily available and relatively fast diag- Special thanks to consultant cardiac radiologists, namely YM and KAA, nostic tool for every cardiac assessment, but it is known to be for giving guidance, moral support and encouragement during this case re- highly operator dependent. Once a fistula is identified, the ability port preparation. Many thanks to our dedicated radiographers for their com- to quantify shunt volume and flow using color Doppler is essen- mitment and participation. tial for first and preliminary imaging modality. An additional ORCID iDs complimentary maneuver using contrast micro-bubbles signals Norain Talib https://orcid.org/0000-0002-8707-2167 can be used to better define the fistula [4]. Kama Azira Awang Ramli https://orcid.org/0000-0001-9853-1552 Nevertheless, MSCT coronary angiography or coronary com- Yusri Mohammed https://orcid.org/0000-0002-8611-5946 puted tomography angiogram (CCTA) is the preferred investiga- Author Contributions tion of choice for comprehensive imaging of the coronary anat- Supervision: Kama Azira Awang Ramli, Yusri Mohammed. Validation: omy [4]. CCTA has the advantage of showing the course of the Kama Azira Awang Ramli. Writing—original draft: Norain Talib. Writing— review & editing: Norain Talib. fistula separate from other adjacent structures. It can also detect any thrombosis, abnormal dilatation, or stenosis along the fistula REFERENCES [5]. CCTA can provide an accurate assessment of the size and lo- 1. Yun G, Nam TH, Chun EJ. Coronary artery fistulas: pathophysiology, cation of an aneurysm [6]. Advantages of CCTA over conven- imaging findings, and management. 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The suitable time for intervention 46:368-371. in pregnant women is unclear. An asymptomatic patient should

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