Prenatal diagnosis of ventriculocoronary arterial communication associated with pulmonary atresia

Prenatal diagnosis of ventriculocoronary arterial communication associated with pulmonary atresia
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  Ultrasound Obstet Gynecol   2003;  21 : 413–415Published online 17 March 2003 in Wiley InterScience (  DOI:  10.1002/uog.98 Picture of the Month Prenatal diagnosis of ventriculocoronary arterial communication associated withpulmonary atresia F. TADDEI, M. SIGNORELLI, C. GROLI, S. SCALCHI and U. A. BIANCHI Department of Obstetrics and Gynecology, University of Brescia, Italy Wereportourfindingsinacaseofpulmonaryatresiawithintact ventricular septum and ventriculocoronary arteryfistula in which power Doppler was used to make thediagnosis.A 30-year-old primiparous woman with no significantmedical history presented at the fetal echocardiographyservice of our prenatal diagnosis center at 21 + 2 weeksof gestation after a routine sonographic examinationhad demonstrated a slightly enlarged fetal left cardiacventricle. Sonography was performed using a SiemensElegra ultrasound machine (Siemens, Erlangen, Germany)with a 3,5C40H transabdominal transducer.This showedfetal biometry to be consistent with gestational age andthe extracardiac fetal morphology was normal. Two-dimensional imaging of the four-chamber view confirmedthat the left ventricle was slightly enlarged. The leftoutflow tract and the aortic arch were normal. ColorDoppler examination demonstrated normal blood flow tothe ventricles in diastole and absence of tricuspid valveregurgitation.The right ventricle was slightly hypoplastic witha hypertrophic myocardium and reduced movement.The main pulmonary artery diameter was reduced, thepulmonary arteries were perfused by retrograde flowfrom the ductus arteriosus and no flow through thepulmonary valve was detected. Power Doppler identifiedthe presence of an anomalous vessel on the external wallof the heart which was confirmed by color Doppler andwhich demonstrated turbulent flow from the apex alongthe external wall of the right ventricle (Figures 1 and 2).Pulsed Doppler examination of this vessel demonstratedthe presence of bidirectional flow: antegrade flow witha peak velocity of 150 cm/s and retrograde flow witha peak velocity of 110 cm/s (Figure 3). A diagnosis of pulmonary atresia with intact ventricular septum andabnormal communication between the right ventricle andleft coronary artery was made.Fetal karyotyping following amniocentesis was normaland excluded the presence of the 22q11.2 deletion.The patient was counseled about the possibility of  Figure 1  Power Doppler ultrasound image showing the fistulasurrounding the right ventricle (RV) and connecting with the aorta(arrow). surgical repair, but opted for termination of pregnancy.Pathological examination of the fetus confirmed tricuspidatresia with hypoplastic right ventricle and, in placeof the anterior descending ramus of the left coronaryartery, there was an abnormally large, thick-walledramus communicating with the right ventricular cavity(Figure 4), representing a ventriculocoronary arteryfistula.Coronary artery fistulae are rare congenital anoma-lies which have recently been diagnosed in the antenatalperiod 1–7 . They can occur in isolation or be associatedwith other cardiac anomalies, particularly flow obstruc-tion pathologies. The fistula winds around the heartin a tortuous way and drains into a cardiac chamber Correspondence to:  Dr F. Taddei, Department of Obstetrics and Gynecology, Spedali Civili, 25100, Brescia, Italy(e-mail: © 2003 ISUOG. Published by John Wiley & Sons, Ltd. PICTURE OF THE MONTH  414  Taddei et al. Figure 2  Color Doppler ultrasound image showing turbulent flowat the outer wall of the right ventricle. Figure 3  Pulsed wave Doppler flow velocity waveforms in thecoronary artery. The characteristic bidirectional flow shows analternating antegrade and retrograde perfusion during the cardiaccycle. or a proximal vessel such as the right atrium, venacava, coronary sinus, right ventricle or main pulmonaryartery.Coronary artery fistulae are mostly associated withpulmonary atresia with intact interventricular septum.In these cases, as there is a pulmonary obstruction,right ventricular outflow is limited to the fistula orthe tricuspid valve. The predominance of one of thesecompeting passages depends on the flow capacity of the fistula. If the fistula is small, the evolution  inutero  will be similar to that of pulmonary atresiawith intact septum, with tricuspid regurgitation and Figure 4  Photograph of the fetal heart at autopsy. The fistula runsfrom the apex of the right ventricle on its external wall.Scale bar = 1  cm. development of right congestive decompensation. If thefistula is sufficiently large, there are unlikely to beany particular signs  in utero . In these cases there areconsequences of an arterial–venous shunt between rightventricle and aorta, with exclusion of the pulmonarycirculation 8 , 9 .When pulmonary valve atresia is detected, the presenceofafistulashouldalwaysbesought.Theearliestdiagnosisof this condition was reported by Chaoui  et al  . 10 in a 13-week fetus and has led to a discussion on the pathogenesisof coronary fistulae. Previously, in cases associated withpulmonary atresia, the fistula was thought to be aconsequence of hypertension in the right ventricle 11 .However,thattheconditioncanariseasearlyas13 weeksthrows doubt upon this hypothesis. As Chaoui  et al  . state,the primary etiological factor could be the fistula itself,that, by deviating the right ventricular outflow, causesthe perfusion in the pulmonary valve to be reduced, thusleading to atresia.The antenatal identification of this anomaly isimportant as it enables early neonatal angiography withtracing of the coronary vascular regions, thus optimizingmanagement prior to surgery 12 , 13 .It should be borne in mind that real-time ultrasounddoes not usually allow correct diagnosis in a low-riskpatient. Examination by color Doppler and, as we haveshown, power Doppler, allows an anatomical picture thatcompares very well with the macroscopic pathologicalanatomy (Figure 4).In conclusion, when pulmonary atresia with intactinterventricular septum is identified, power and colorDoppler should be used to examine the myocardium tocheck for anomalous vascular communications. References 1. Chaoui R, Tennstedt C, Goldner B, Bollman R. Prenatal diag-nosis of ventriculo-coronary communications in a second-trimester fetus using transvaginal and transabdominal colorCopyright © 2003 ISUOG. Published by John Wiley & Sons, Ltd.  Ultrasound Obstet Gynecol   2003;  21 : 413–415.  Picture of the Month  415 Doppler sonography.  Ultrasound Obstet Gynecol   1997;  9 :194–197.2. Chang YC, Wu MH, Wang JK, Lue HC. Doppler echocardio-graphy for detection of ventriculo-coronary communication:report of a case.  J Formos Med Assoc  1995;  94 : 182–184.3. Arabin B, Aardenburg R, Schasfoort-Van Leeuwen M,Elzenga N. Prenatal diagnosis of ventriculocoronary arterialcommunications combined with pulmonary atresia.  Ultrasound Obstet Gynecol   1996;  7 : 461–462.4. Baschat AA, Love JC, Stewart PA, Gembruch U, Barman CR.Prenatal diagnosis of ventriculocoronary fistula.  Ultrasound Obstet Gynecol   2001;  18 : 39–43.5. Yang Y,Li ZA,Wang XF,Deng YB.Leftcoronaryarteryfistulaidentified by color Doppler flow imaging.  J Tongji Med Univ 1995;  15 : 175–177.6. Patel CR, Shah DM, Dahams BB. Prenatal diagnosis of acoronary fistula in a fetus with pulmonary atresia with intactventricular septum and trisomy 18.  J Ultrasound Med   1999;  18 :429–431.7. Sharland GK, Tynan M, Qureschi SA. Prenatal detection andprogression of right coronary artery to right ventricle fistula. Heart   1996;  76 : 79–81.8. Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infantswith pulmonary atresia, intact ventricular septum, and rightventricle-dependent coronary circulation.  Am J Cardiol   2000; 86 : 1272–1274, A9.9. Satou GM, Perry SB, Gauvreau K, Geva T. Echocardiographicpredictors of coronary artery pathology in pulmonary atresiawith intact ventricular septum.  Am J Cardiol   2000;  85 :1319–1324.10. Chaoui R, Machlitt A, Tennstedt C. Prenatal diagnosis of ventriculo-coronary fistula in a late first-trimester fetuspresenting with increased nuchal translucency.  Ultrasound Obstet Gynecol   2000;  15 : 160–162.11. Manner J. Embryology of congenital ventriculo-coronarycommunications: a study on quail-chick chimeras.  Cardiol Young   2000;  10 : 233–238.12. Descalzo Senorans A, Santos de Soto J, Gonzales Garcia A,Mayol Deya A. Congenital coronary fistula to right ventricle.Treatment with transcatheter coil embolization.  Rev EspCardiol   1999;  52 : 526–528.13. Lee ML, Chaou WT. Successful transcatheter coil embolizationof coronary artery fistula in an infant.  J Thorac Imaging   2000; 15 : 153–156.Copyright © 2003 ISUOG. Published by John Wiley & Sons, Ltd.  Ultrasound Obstet Gynecol   2003;  21 : 413–415.
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