Case Report
Prenatal Diagnosis of Left Coronary Artery to Right Ventricle Fistula Funda Oztunc, MD,1 Selman Gokalp, MD,1 Mehmet Aytac Yuksel, MD,2 Metehan Imamoglu, MD,2 Riza Madazli, MD2 1
Division of Pediatric Cardiology, Department of Pediatrics, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey 2 Maternal Fetal Medicine Unit, Department of Obstetrics and Gynecology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey Received 23 May 2013; accepted 8 June 2014
ABSTRACT: Prenatal diagnosis of a congenital coronary artery fistula between the left coronary artery and the right ventricle was established at 28 weeks of gestation. Hydrops fetalis developed during follow-up and the baby died on the first day after delivery. It is rare for coronary artery fistulas to become symptoC 2014 Wiley Periodicals, Inc. matic during fetal life. V J Clin Ultrasound 43:129–131, 2015; Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jcu.22215 Keywords: coronary artery fistula; fetal echocardiography; hydrops fetalis
A
congenital coronary artery fistula (CAF) involves an abnormal fistulous connection between a coronary artery and a cardiac chamber. Most often, a fistula from the right coronary artery to the right ventricle (RV) occurs in association with pulmonary atresia.1–4 In this report, we describe the prenatal diagnosis of an isolated left coronary artery (LCA) to RV fistula resulting in hydrops fetalis and death.
CASE REPORT
A 29-year-old woman, gravida 2, para 1, was referred to our unit for fetal echocardiography at 28 weeks’ gestation due to a suspected conCorrespondence to: F. Oztunc Additional Supporting Information may be found in the online version of this article. C 2014 Wiley Periodicals, Inc. V
VOL. 43, NO. 2, FEBRUARY 2015
genital cardiac anomaly detected during routine obstetrical sonographic examination. The patient’s previous pregnancy had resulted in a term delivery of a male infant. The parents were not consanguineous. Detailed fetal echocardiographic examination revealed situs solitus with atrioventricular and ventriculo-arterial concordance. The right atrium and ventricle were dilated (compared with left heart chambers) and an associated severe tricuspid regurgitation was noted. There were no other signs of heart failure. In the short-axis view of the aortic root, the LCA was dilated and had turbulent flow in it; in addition, color Doppler echocardiography showed a turbulent flow at the RV apex (Figures 1 and 2). Spectral Doppler analysis confirmed bidirectional flow pattern across the CAF close to its opening in the RV (Figure 3). The patient did not return for the scheduled follow-up examinations until 32 weeks’ gestation. US evaluation at 32 weeks’ gestation revealed signs of fetal ascites and hydrops. Fetal echocardiography revealed evidence of cardiovascular impairment with right ventricular dilatation, and marked deviation of the interventricular septum into the left ventricle was noted as well. Spectral Doppler analysis showed a negative a-wave in the ductus venosus and persistent end-diastolic flow in the umbilical artery. An urgent cesarean delivery was performed with the diagnosis of fetal distress secondary to severe variable decelerations and yielded a live female neonate weighing 1,814 g. The Apgar scores were 2 and 4 at 1 and 5 129
OZTUNC ET AL
FIGURE 1. Color Doppler echocardiogram shows a coronary artery fistula tract from left coronary artery to the right ventricle. RA, right atrium; RV, right ventricle; LCA, left coronary artery.
FIGURE 2. Color Doppler echocardiogram shows the entry of the coronary artery fistula into the right ventricle. CAF, coronary artery fistula; RV, right ventricle.
minutes, respectively. Following the delivery, the newborn developed bradycardia and apnea episodes, with signs of congestive heart failure, and died at 20th hour after delivery. The parents refused the autopsy.
DISCUSSION
The incidence of isolated CAFs is reported as 1:50,000 live births. In 0.25–0.4% of cases, congenital heart defects are associated with CAFs.5 The left, right, or both coronary arteries may be involved. CAFs predominantly drain into the right side of the heart (92%): into the RV in 41%, the right atrium in 26%, the coronary sinus in 7%, the pulmonary artery in 17%, and the superior vena cava in 1% of cases. Only 3% of CAFs drain into the left ventricle and 5% drain into the left atrium. Drainage into both ventricles is uncommon.6 The communications can occur in isolation or in association with other cardiac anomalies, especially obstructive disorders associated with left or right ventricular hypoplasia. Isolated CAFs are usually asymptomatic.3 The prenatal diagnosis of isolated CAF has been reported in few cases in the literature. Three of them were right coronary artery to RV fistulas,7–9 and one was LCA to right atrium fistula.10 No LCA to RV fistula like in our case has been reported. Throughout the cardiac development in the embryonic period, some branches of the coronary arteries communicate with the intramyocardial trabecular spaces, which in turn connect with the ventricular lumen. This trabeculated pattern is slightly more abundant in the developing RV than in the left.11 Ventriculocoronary fistulas can result in a steal phenomenon. The 130
FIGURE 3. Spectral Doppler waveform shows typical bidirectional flow in the coronary artery fistula.
“coronary vascular steal effect” may result in decreased intraventricular flow, thus causing mitral and aortic stenosis or atresia and related aortic and pulmonary misalignment.12 Most patients with an isolated CAF have few, if any, symptoms. However, serious complications, such as congestive heart failure, pulmonary hypertension, bacterial endocarditis, premature arteriosclerotic changes within the fistulas, aneurysm, thromboembolic events, arrhythmia, symptoms such as angina pectoris, and myocardial infarction resulting from coronary steal, have been reported.6 Verification of a dilated tortuous coronary artery at echocardiography in the postnatal period may be the first diagnostic sign of a CAF. Color Doppler echocardiography and spectral Doppler analysis can verify the presence of high-velocity and turbulent flow and point to the drainage site.10 Even though the visualization of fetal coronary JOURNAL OF CLINICAL ULTRASOUND
CORONARY ARTERY FISTULA
arteries is difficult due to the small vessel size, fetal echocardiography enables the detection of a CAF if there is significant jet flow into the cardiac cavity or a great vessel.13 In this case, the turbulent flow into the RV was detected. Almost 90% of the infants and children with CAF have been reported to be asymptomatic.14 Very few patients develop symptoms of congestive cardiac failure from a large left-to-right shunt or myocardial ischemia due to coronary artery steal in the first few years following the birth. Sharland et al reported the prenatal diagnosis of a CAF between the right coronary artery and the RV at 20 weeks’ gestation. Cardiac status of the fetus deteriorated through the pregnancy and congestive cardiac failure developed when the infant was 5 months old.9 We believe that infants diagnosed prenatally with CAF are relatively prone to develop congestive heart failure. It seems reasonable to assume that the more significant the shunt flow of the CAF, the more easily it is detected by fetal echocardiography. In addition, the divert of blood from the aorta through the shunts into the right ventricular cavity during diastole possibly leads to a “steal” effect with partial ischemia of the myocardium that is insufficiently supplied by its coronary artery.15 This effect might be responsible for the development of congestive cardiac failure at 32 weeks’ gestation in our case. However, because the patient did not return to our clinic until 32 weeks’ gestation, we were unable to document the progression of the cardiac impairment. Screening for common infectious agents that can cause hydrops was done following the delivery and the results were negative. In conclusion, diagnosis of CAFs is possible prenatally and optimal treatment options can be considered to prevent the development of cardiac failure.
REFERENCES 1. Lajos P, Love J, Salim MA, et al. Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum. Ann Thorac Surg 2004;77:1087. 2. Maeno YV, Boutin C, Hornberger LK, et al. Prenatal diagnosis of right ventricular outflow tract
VOL. 43, NO. 2, FEBRUARY 2015
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart 1999;81:661. Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849. Khan MD, Sivasubramonian S, Simpson JM. Prenatal diagnosis of tetralogy of Fallot associated with a fistula from the left coronary artery to the left atrium. Cardiol Young 2003;13:194. Apitz J Angeborene Anomalien der Koronararterien. In: Apitz J, editor. P€ adiatrische Kardiologie. Darmstadt: Steinkopff-Verlag; 1998; p 419–429. Schumacher G, Roithmaier A, Lorenz HP, et al. Congenital coronary artery fistula in infancy and childhood: diagnostic and therapeutic aspects. Thorac Cardiovasc Surg 1997;45:287. Karagoz T, Ozkutlu S, Celiker A. Percutaneous closure of a prenatally diagnosed large coronary artery fistula with an Amplatzer vascular plug immediately after delivery. Acta Cardiol 2008;63: 405. Hung JH, Lu JH, Hung J, Chen JD. Prenatal diagnosis of a right coronary-cameral fistula. J Ultrasound Med 2006;25:1075. Sharland GK, Tynan M, Qureshi SA. Prenatal detection and progression of right coronary artery to right ventricle fistula. Heart 1996;76:79. Mielke G, Sieverding L, Borth-Bruns T, et al. Prenatal diagnosis and perinatal management of left coronary artery to right atrium fistula. Ultrasound Obstet Gynecol 2002;19:612. Chaoui R, Tennstedt C, Goldner B. Prenatal diagnosis of ventriculocoronary arterial fistula in a fetus with hypoplastic left heart syndrome and aortic atresia. Ultrasound Obstet Gynecol 2002;20:75. Paladini D, Caruso G, Sglavo G, et al. Hypoplastic left heart syndrome associated with left ventriclecoronary artery-pulmonary artery fistula: prenatal diagnosis and histological correlation. Ultrasound Obstet Gynecol 2005;26:78. Mavroudis C, Backer CL, Rocchini AP, et al. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63:1235. Hayashi T, Inuzuka R, Ono H, et al. Prenatal diagnosis of coronary artery fistula: a case report and review of literature. Pediatr Int 2012;54:299. Chaoui R, Tennstedt C, Goldner B, et al. Prenatal diagnosis of ventriculo-coronary communications in a second-trimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol 1997;9:194.
131
Prenatal Diagnosis of Left Coronary Artery to Right Ventricle Fistula Funda Oztunc, MD,1 Selman Gokalp, MD,1 Mehmet Aytac Yuksel, MD,2 Metehan Imamoglu, MD,2 Riza Madazli, MD2 1
Division of Pediatric Cardiology, Department of Pediatrics, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey 2 Maternal Fetal Medicine Unit, Department of Obstetrics and Gynecology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey Received 23 May 2013; accepted 8 June 2014
ABSTRACT: Prenatal diagnosis of a congenital coronary artery fistula between the left coronary artery and the right ventricle was established at 28 weeks of gestation. Hydrops fetalis developed during follow-up and the baby died on the first day after delivery. It is rare for coronary artery fistulas to become symptoC 2014 Wiley Periodicals, Inc. matic during fetal life. V J Clin Ultrasound 43:129–131, 2015; Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jcu.22215 Keywords: coronary artery fistula; fetal echocardiography; hydrops fetalis
A
congenital coronary artery fistula (CAF) involves an abnormal fistulous connection between a coronary artery and a cardiac chamber. Most often, a fistula from the right coronary artery to the right ventricle (RV) occurs in association with pulmonary atresia.1–4 In this report, we describe the prenatal diagnosis of an isolated left coronary artery (LCA) to RV fistula resulting in hydrops fetalis and death.
CASE REPORT
A 29-year-old woman, gravida 2, para 1, was referred to our unit for fetal echocardiography at 28 weeks’ gestation due to a suspected conCorrespondence to: F. Oztunc Additional Supporting Information may be found in the online version of this article. C 2014 Wiley Periodicals, Inc. V
VOL. 43, NO. 2, FEBRUARY 2015
genital cardiac anomaly detected during routine obstetrical sonographic examination. The patient’s previous pregnancy had resulted in a term delivery of a male infant. The parents were not consanguineous. Detailed fetal echocardiographic examination revealed situs solitus with atrioventricular and ventriculo-arterial concordance. The right atrium and ventricle were dilated (compared with left heart chambers) and an associated severe tricuspid regurgitation was noted. There were no other signs of heart failure. In the short-axis view of the aortic root, the LCA was dilated and had turbulent flow in it; in addition, color Doppler echocardiography showed a turbulent flow at the RV apex (Figures 1 and 2). Spectral Doppler analysis confirmed bidirectional flow pattern across the CAF close to its opening in the RV (Figure 3). The patient did not return for the scheduled follow-up examinations until 32 weeks’ gestation. US evaluation at 32 weeks’ gestation revealed signs of fetal ascites and hydrops. Fetal echocardiography revealed evidence of cardiovascular impairment with right ventricular dilatation, and marked deviation of the interventricular septum into the left ventricle was noted as well. Spectral Doppler analysis showed a negative a-wave in the ductus venosus and persistent end-diastolic flow in the umbilical artery. An urgent cesarean delivery was performed with the diagnosis of fetal distress secondary to severe variable decelerations and yielded a live female neonate weighing 1,814 g. The Apgar scores were 2 and 4 at 1 and 5 129
OZTUNC ET AL
FIGURE 1. Color Doppler echocardiogram shows a coronary artery fistula tract from left coronary artery to the right ventricle. RA, right atrium; RV, right ventricle; LCA, left coronary artery.
FIGURE 2. Color Doppler echocardiogram shows the entry of the coronary artery fistula into the right ventricle. CAF, coronary artery fistula; RV, right ventricle.
minutes, respectively. Following the delivery, the newborn developed bradycardia and apnea episodes, with signs of congestive heart failure, and died at 20th hour after delivery. The parents refused the autopsy.
DISCUSSION
The incidence of isolated CAFs is reported as 1:50,000 live births. In 0.25–0.4% of cases, congenital heart defects are associated with CAFs.5 The left, right, or both coronary arteries may be involved. CAFs predominantly drain into the right side of the heart (92%): into the RV in 41%, the right atrium in 26%, the coronary sinus in 7%, the pulmonary artery in 17%, and the superior vena cava in 1% of cases. Only 3% of CAFs drain into the left ventricle and 5% drain into the left atrium. Drainage into both ventricles is uncommon.6 The communications can occur in isolation or in association with other cardiac anomalies, especially obstructive disorders associated with left or right ventricular hypoplasia. Isolated CAFs are usually asymptomatic.3 The prenatal diagnosis of isolated CAF has been reported in few cases in the literature. Three of them were right coronary artery to RV fistulas,7–9 and one was LCA to right atrium fistula.10 No LCA to RV fistula like in our case has been reported. Throughout the cardiac development in the embryonic period, some branches of the coronary arteries communicate with the intramyocardial trabecular spaces, which in turn connect with the ventricular lumen. This trabeculated pattern is slightly more abundant in the developing RV than in the left.11 Ventriculocoronary fistulas can result in a steal phenomenon. The 130
FIGURE 3. Spectral Doppler waveform shows typical bidirectional flow in the coronary artery fistula.
“coronary vascular steal effect” may result in decreased intraventricular flow, thus causing mitral and aortic stenosis or atresia and related aortic and pulmonary misalignment.12 Most patients with an isolated CAF have few, if any, symptoms. However, serious complications, such as congestive heart failure, pulmonary hypertension, bacterial endocarditis, premature arteriosclerotic changes within the fistulas, aneurysm, thromboembolic events, arrhythmia, symptoms such as angina pectoris, and myocardial infarction resulting from coronary steal, have been reported.6 Verification of a dilated tortuous coronary artery at echocardiography in the postnatal period may be the first diagnostic sign of a CAF. Color Doppler echocardiography and spectral Doppler analysis can verify the presence of high-velocity and turbulent flow and point to the drainage site.10 Even though the visualization of fetal coronary JOURNAL OF CLINICAL ULTRASOUND
CORONARY ARTERY FISTULA
arteries is difficult due to the small vessel size, fetal echocardiography enables the detection of a CAF if there is significant jet flow into the cardiac cavity or a great vessel.13 In this case, the turbulent flow into the RV was detected. Almost 90% of the infants and children with CAF have been reported to be asymptomatic.14 Very few patients develop symptoms of congestive cardiac failure from a large left-to-right shunt or myocardial ischemia due to coronary artery steal in the first few years following the birth. Sharland et al reported the prenatal diagnosis of a CAF between the right coronary artery and the RV at 20 weeks’ gestation. Cardiac status of the fetus deteriorated through the pregnancy and congestive cardiac failure developed when the infant was 5 months old.9 We believe that infants diagnosed prenatally with CAF are relatively prone to develop congestive heart failure. It seems reasonable to assume that the more significant the shunt flow of the CAF, the more easily it is detected by fetal echocardiography. In addition, the divert of blood from the aorta through the shunts into the right ventricular cavity during diastole possibly leads to a “steal” effect with partial ischemia of the myocardium that is insufficiently supplied by its coronary artery.15 This effect might be responsible for the development of congestive cardiac failure at 32 weeks’ gestation in our case. However, because the patient did not return to our clinic until 32 weeks’ gestation, we were unable to document the progression of the cardiac impairment. Screening for common infectious agents that can cause hydrops was done following the delivery and the results were negative. In conclusion, diagnosis of CAFs is possible prenatally and optimal treatment options can be considered to prevent the development of cardiac failure.
REFERENCES 1. Lajos P, Love J, Salim MA, et al. Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum. Ann Thorac Surg 2004;77:1087. 2. Maeno YV, Boutin C, Hornberger LK, et al. Prenatal diagnosis of right ventricular outflow tract
VOL. 43, NO. 2, FEBRUARY 2015
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart 1999;81:661. Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849. Khan MD, Sivasubramonian S, Simpson JM. Prenatal diagnosis of tetralogy of Fallot associated with a fistula from the left coronary artery to the left atrium. Cardiol Young 2003;13:194. Apitz J Angeborene Anomalien der Koronararterien. In: Apitz J, editor. P€ adiatrische Kardiologie. Darmstadt: Steinkopff-Verlag; 1998; p 419–429. Schumacher G, Roithmaier A, Lorenz HP, et al. Congenital coronary artery fistula in infancy and childhood: diagnostic and therapeutic aspects. Thorac Cardiovasc Surg 1997;45:287. Karagoz T, Ozkutlu S, Celiker A. Percutaneous closure of a prenatally diagnosed large coronary artery fistula with an Amplatzer vascular plug immediately after delivery. Acta Cardiol 2008;63: 405. Hung JH, Lu JH, Hung J, Chen JD. Prenatal diagnosis of a right coronary-cameral fistula. J Ultrasound Med 2006;25:1075. Sharland GK, Tynan M, Qureshi SA. Prenatal detection and progression of right coronary artery to right ventricle fistula. Heart 1996;76:79. Mielke G, Sieverding L, Borth-Bruns T, et al. Prenatal diagnosis and perinatal management of left coronary artery to right atrium fistula. Ultrasound Obstet Gynecol 2002;19:612. Chaoui R, Tennstedt C, Goldner B. Prenatal diagnosis of ventriculocoronary arterial fistula in a fetus with hypoplastic left heart syndrome and aortic atresia. Ultrasound Obstet Gynecol 2002;20:75. Paladini D, Caruso G, Sglavo G, et al. Hypoplastic left heart syndrome associated with left ventriclecoronary artery-pulmonary artery fistula: prenatal diagnosis and histological correlation. Ultrasound Obstet Gynecol 2005;26:78. Mavroudis C, Backer CL, Rocchini AP, et al. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63:1235. Hayashi T, Inuzuka R, Ono H, et al. Prenatal diagnosis of coronary artery fistula: a case report and review of literature. Pediatr Int 2012;54:299. Chaoui R, Tennstedt C, Goldner B, et al. Prenatal diagnosis of ventriculo-coronary communications in a second-trimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol 1997;9:194.
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