Prenatal Diagnosis of a Large Left Coronary Artery to Right Ventricle Fistula With 3-Dimensional/4-Dimensional Spatiotemporal Image Correlation Rendering Followed by Successful Neonatal Transcatheter Closure.

HomeCirculation: Cardiovascular ImagingVol. 15, No. 11Prenatal Diagnosis of a Large Left Coronary Artery to Right Ventricle Fistula With 3-Dimensional/4-Dimensional Spatiotemporal Image Correlation Rendering Followed by Successful Neonatal Transcatheter Closure Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessCase ReportPDF/EPUBPrenatal Diagnosis of a Large Left Coronary Artery to Right Ventricle Fistula With 3-Dimensional/4-Dimensional Spatiotemporal Image Correlation Rendering Followed by Successful Neonatal Transcatheter Closure Balu Vaidyanathan, Roma Verma, Mahesh Kappanayil and Raman Krishna Kumar Balu VaidyanathanBalu Vaidyanathan Correspondence to: Balu Vaidyanathan, DM, Department of Pediatric Cardiology, Head, Fetal Cardiology division, Amrita Institute of Medical Sciences, Kochi, Kerala, India. Email E-mail Address: [email protected] https://orcid.org/0000-0001-6636-8308 The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. Search for more papers by this author , Roma VermaRoma Verma The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. Search for more papers by this author , Mahesh KappanayilMahesh Kappanayil https://orcid.org/0000-0002-1331-6720 The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. Search for more papers by this author and Raman Krishna KumarRaman Krishna Kumar The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. Search for more papers by this author Originally published26 Aug 2022https://doi.org/10.1161/CIRCIMAGING.122.014247Circulation: Cardiovascular Imaging. 2022;15Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: August 26, 2022: Ahead of Print Isolated congenital coronary artery fistulas are rare congenital anomalies in which there is an abnormal communication between a coronary artery and one of the cardiac chambers or great vessels.1 These occur with a prevalence of 1:50 000 live births and accounts for 0.2% to 0.4% of all congenital heart defects. Prenatal diagnosis of isolated congenital coronary artery fistulae is relatively rare and is restricted to short series or case reports.2,3 We report a case of isolated congenital coronary artery fistulas from the left coronary artery to the right ventricle in which prenatal imaging using advanced 3-dimensional (3D)/4-dimensional (4D) spatiotemporal image correlation (STIC) rendering was used to delineate the precise anatomy, thereby aiding planned peripartum care and expedited catheter closure of the fistula immediately after birth with excellent outcomes.A 30-year-old second gravida was referred for fetal echocardiography to our center in view of suspicion of a channel with continuous flow from aorta to the left ventricle. Fetal echocardiography evaluation (Voluson E10 systems, GE healthcare, Zipf) at 24 weeks showed a to and fro flow in a channel arising from the aorta in the direction of the left ventricle without evidence of cardiomegaly. On reassessment at 27 weeks and again at 29 weeks (Figure 1; Video S1), the left coronary artery was found to be significantly dilated (Figure 1A) with a turbulent high velocity color jet coursing toward the right ventricular apex (Figure 1B). There was cardiomegaly at 29 weeks and the 3-vessel tracheal view showed evidence of significant flow reversal into the aortic arch (Figure 1C) suggesting a hemodynamically significant shunt. Spectral Doppler showed a characteristic to and fro pattern with forward flow into the aorta in systole and retrograde flow into the fistula in diastole (Figure 1D). We did a 3D/4D STIC rendering of the anatomy using volume datasets acquired and this clearly delineated the anatomy of the fistula as arising from the left coronary artery and coursing downward toward its entry point in the right ventricular (RV) apex (Figure 2A; Video S2). The fetus was carefully followed up during the rest of the prenatal period for evidence of hydrops or heart failure. All fetal Dopplers remained within normal range till term and there were no associated anomalies.Download figureDownload PowerPointFigure 1. Prenatal imaging of the coronary fistula from left coronary artery to right ventricle. A, Short-axis view of the outflow tracts showing the aorta (Ao) in the center with the right ventricle (RV) and its outflow tract. The left coronary artery (LCA) appeared dilated. The fistula (arrows) seemed to track toward the right ventricular outflow tract. B, Short-axis view of the outflows profiling the entire course of the fistula. From the dilated LCA, the fistula coursed toward the right ventricle and joined the RV near its apex (arrow). C, The 3-vessel tracheal view showing a reverse flow (red color with arrows) toward the aorta suggesting significant run-off through the fistula, indicating its hemodynamic severity. D, Spectral Doppler across the fistula showing the typical to and fro flow pattern with systolic flows toward aorta and diastolic flow toward the RV. PA indicates pulmonary artery.Download figureDownload PowerPointFigure 2. Comparison of anatomy of the coronary fistula rendered using prenatal 3-dimensional (3D)/4-dimensional (4D) spatiotemporal image correlation (STIC) versus postnatal 256 slice computed tomography. A, 3D/4D STIC rendering of the coronary fistula anatomy using prenatal volume datasets. The dilated left coronary artery (LCA) can be seen arising from the aortic sinus (Ao). The fistula arises from the LCA and courses down as a very dilated channel toward the right ventricle (RV) where it can be seen entering (arrow). The proximal portion of the fistula was especially large with a constriction in the terminal portion. B, The fistula anatomy reconstructed using a 256 slice post-natal computed tomography study. The RV is superimposed using blue color. The origin, course and the distal end of the fistula joining the RV apex is seen and is very similar to the prenatal anatomy demonstrated using 3D/4D STIC rendering. RA indicates right atrium.At 38 weeks’ gestation, a baby boy was delivered by normal vaginal delivery with birth weight of 3.32 kg. The baby had normal APGAR scores (Appearance, Pulse, Grimace, Activity, and Respiration) at birth. Cardiac evaluation showed oxygen saturation of 99% with evidence of a continuous murmur. Neonatal echocardiography confirmed the diagnosis of a large coronary fistula from left coronary artery to the RV apex with significant dilatation of the right heart structures with flow reversal in aortic arch. A computed tomography pulmonary angiography with 3D reconstruction was done on day 2 of life and confirmed the diagnosis of a large coronary artery fistula from left coronary artery to the RV apex. There was excellent correlation between the computed tomography anatomy (Figure 2B) and the prenatal 3D/4D STIC rendering (Figure 2).In view of the hemodynamically significant fistula, the baby was taken up for a fistula occlusion by cardiac catheterization on day 4 of life under general anesthesia. Angiogram confirmed a large fistula from left coronary artery into RV with an area of constriction in the vertical limb of the fistula before entering the RV (Figure 3A; Video S3). The fistula was occluded using a 5F right coronary guiding catheter from the venous side using a 6-8 Konar-MF muscular VSD occluder (Lifetech Scientific, Shenzen, China; Figure 3B). There was some persistent residual shunt immediately post-procedure which disappeared over the next 1 month. The baby was weaned off anti-failure medications on follow-up. At 3-months post-procedure, the baby was clinically well with a weight of 6 kg and no heart failure.Download figureDownload PowerPointFigure 3. Cardiac catheterization images of the coronary fistula before and after device closure. A, Angiogram taken in the left anterior-oblique (LAO) view. The catheter coursed through the femoral venous access into the right heart and the fistula was entered through its right ventricle (RV) entry point (bold arrow) and tracked through the fistula toward the left coronary artery (LCA). The aorta (Ao) also filled retrograde along with the native coronary branches (small arrows). B, The angiogram taken from the aortic root (Ao) after deployment of the Konar-MF device (arrow) in the vertical limb of the fistula. The native LCA is seen well.This case report illustrates the significant benefit of prenatal diagnosis in planning the neonatal management of a neonate with a hemodynamically significant coronary artery fistulas. Isolated congenital coronary artery fistulae are rarely reported in the prenatal literature.1–3 More commonly, these fistulas arise from the right coronary system than the left with the vast majority draining into the right sided cardiac chambers.1 Prenatal findings suggesting high volume shunting through the fistula include cardiomegaly, flow reversal in the aortic arch, presence of turbulent flow throughout the cardiac cycle and presence of hydrops fetalis.1,2 Prenatal identification of these high-risk features should prompt careful surveillance and planned peri-partum care in a pediatric cardiac facility with an intent for expedited intervention of the fistula in the early neonatal period, as illustrated by our case (Figure 1; Video S1). We used advanced 3D/4D fetal echocardiography with STIC rendering to delineate the precise anatomic details of the fistula in the prenatal period (Figure 2A; Video S2). We had previously reported the incremental benefits of 3D/4D STIC technique in predicting the postnatal surgical pathway in fetuses with double outlet right ventricle.4 There was excellent correlation between the anatomy of the coronary artery fistulas as delineated by prenatal 3D/4D STIC compared with postnatal 256 slice computed tomography based reconstruction (Figure 2). The precise delineation of the anatomy in the prenatal period along with the identification of features of high-volume shunting enabled us to precisely plan the neonatal cardiac care for this baby culminating in a successful trans-catheter closure of the fistula (Figure 3; Video S3).In conclusion, prenatal diagnosis and precise delineation of the anatomy is feasible for isolated congenital coronary artery fistulae using fetal echocardiography assisted by newer techniques like 3D/4D STIC rendering. This helps in planning the neonatal care for these critically sick infants ensuring excellent clinical outcomes.Article InformationSources of FundingNone.FootnotesSupplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.122.014247.For Disclosures, see page 855.Correspondence to: Balu Vaidyanathan, DM, Department of Pediatric Cardiology, Head, Fetal Cardiology division, Amrita Institute of Medical Sciences, Kochi, Kerala, India. Email baluvaidyanathan@gmail.comReferences1. Walter A, Calite E, Herberg U, Breuer J, Berg C, Geipel A, Gembruch U. Prenatal detected Isolated congenital coronary artery fistula (ICCAF) characteristics and impact on the fetal hemodyanamic situation: systematic literature review.Interv Cardiol. 2021; 13:316–322. doi: 10.1002/ccr3.3779CrossrefGoogle Scholar2. Sharland GK, Konta L, Qureshi SA. Prenatal diagnosis of isolated coronary artery fistulas: progression and outcome in five cases.Cardiol Young. 2016; 26:915–920. doi: 10.1017/S1047951115001535CrossrefMedlineGoogle Scholar3. Nagiub M, Mahadin D, Gowda S, Aggarwal S. Prenatal diagnosis of coronary artery fistula: a case report and review of literature.AJP Rep. 2014; 4:e83–e86. doi: 10.1055/s-0034-1386636CrossrefMedlineGoogle Scholar4. Karmegaraj B, Kumar S, Srimurugan B, Sudhakar A, Simpson JM, Vaidyanathan B. 3D/4D spatiotemporal image correlation (STIC) fetal echocardiography provides incremental benefit over 2D fetal echocardiography in predicting postnatal surgical approach in double-outlet right ventricle.Ultrasound Obstet Gynecol. 2021; 57:423–430. doi: 10.1002/uog.21988CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Karapurkar S, Kappanayil M, Kumar R and Vaidyanathan B (2023) Incremental value of fetal spatiotemporal image correlation echocardiography in the diagnosis of tetralogy of Fallot with disconnected pulmonary arteries with ductus arteriosus supplying the left pulmonary artery, Annals of Pediatric Cardiology, 10.4103/apc.apc_163_22, 16:2, (150-153), . November 2022Vol 15, Issue 11 Advertisement Article InformationMetrics © 2022 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.122.014247PMID: 36017699 Originally publishedAugust 26, 2022 Keywordscatheter closurecoronary fistulaprenatal diagnosisPDF download Advertisement SubjectsUltrasound