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Transcatheter Coil Occlusion of Coronary Artery to Right Ventricular Fistulae in Pulmonary Atresia with Intact Ventricular Septum
CASE REPORT
Case Report
Transcatheter Coil Occlusion of Coronary Artery to Right Ventricular Fistulae in Pulmonary Atresia with Intact Ventricular Septum Sarah A. Hope, MBChB MRCP, a Samuel Menahem, MD FRACPb and Ian T. Meredith, PhD FRACPa,∗ a
Cardiovascular Research Centre, Monash Medical Centre, 246 Clayton Road, Clayton, Vic. 3168, Australia b Department of Cardiology, Monash Medical Centre, 246 Clayton Road, Clayton, Vic. 3168, Australia
We describe a young adult with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology who presented with extreme dyspnoea on exertion. Coronary artery to right ventricular fistulae and reversible myocardial ischaemia secondary to a right ventricular steal phenomenon were present. The right coronary artery to right ventricular fistula was completely occluded with coils, by a transcoronary approach, with amelioration of symptoms. However, one coil embolised and lodged uneventfully in the left atrium during the procedure. This case demonstrates that a transcatheter approach may be successfully employed not only in patients with isolated coronary artery fistulae, but also in patients with coexistent complex congenital heart disease. (Heart Lung and Circulation 2004;13:195–197) © 2004 Published by Elsevier Inc on behalf of Australian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Keywords. Pulmonary atresia intact ventricular septum; Coronary artery fistula; Interventional catheterisation
Case report
A
young girl with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology presented with decreasing exercise tolerance and extreme dyspnoea on exertion. She was known to have coronary artery to right ventricular fistulae, and myocardial ischaemia was suspected. At the age of 16 years an exercise test demonstrated electrocardiographic changes consistent with ischaemia after just 3 min of the Bruce protocol. An exercise myocardial perfusion scan was subsequently performed but demonstrated only minor changes in the anterior wall, possibly due to attenuation artefact. Coronary angiography confirmed the presence of coronary artery to right ventricular fistulae involving both right and left coronary artery systems, with direction of flow from the coronary arteries to the right ventricle (Fig. 1). There were no significant coronary artery stenoses. Right ventricular pressure was estimated at less than half systemic by Doppler echocardiography of tricuspid valve incompetence. Although the right ventricular pressure might be underestimated if the peak velocity of the regurgitant jet was not interrogated, due to the volume of the tricuspid regurgitation in this patient good Doppler traces
Received 31 March 2003; received in revised form 9 September 2003; accepted 4 March 2004 ∗ Corresponding author. Tel.: +61 3 9594 4545; fax: +61 3 9594 6939. E-mail address: [email protected] (I.T. Meredith).
were achieved with relative ease and the jet widely interrogated. It is unlikely that the right ventricular pressure was substantially underestimated. A right ventricular steal phenomenon was diagnosed with benefit thought likely from occlusion of the fistulae. The fistula between the right coronary artery and right ventricle was felt to be most significant, and coil occlusion was undertaken in the cardiac catheter laboratory by a transcoronary approach. The fistula was best visualised in a left anterior oblique projection with cranial angulation. Using a six French JR 3.5 guiding catheter (Cordis Corporation, Miami Lakes, Florida, USA) and a Balance® wire (Guidant Corporation, Santa Clara, California, USA), a three French Tracker catheter (Boston Scientific Corporation, Natick, Massachusetts, USA) was positioned within the fistula. Subsequently Balance® Middleweight (Guidant Corporation) and ACS DOC® (Guidant Corporation) wires were utilised to optimise the position. Six fiber coils (2 mm × 10 mm, 2 mm × 20 mm × 4, 3 mm × 30 mm) (Boston Scientific Corporation) and three GDC® —10 coils (2 mm × 6 cm, 2 mm × 8 cm, 3 mm × 8 cm) (Boston Scientific Corporation) were deployed via the Tracker catheter once a satisfactory position had been established, achieving complete occlusion of the fistula without obstruction to flow in the distal right coronary artery (Fig. 2). Unfortunately, due to the size of the fistula and volume of blood flow, one fiber coil, which was deployed early, became dislodged into the right ventricle from where it passed retrogradely across the tricuspid
© 2004 Published by Elsevier Inc on behalf of Australian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2004.03.016
196 CASE REPORT
Hope et al. Transcatheter coil occlusion of coronary artery to right ventricular fistulae
Heart Lung and Circulation 2004;13:195–197
Figure 1. Fistula between the right coronary artery and the right ventricle.
Figure 2. Complete occlusion with coils of the fistula between the right coronary artery and right ventricle with no obstruction to flow in the distal right coronary artery.
Hope et al. Transcatheter coil occlusion of coronary artery to right ventricular fistulae
valve. Since the atrial baffle was to the right of the tricuspid valve, the coil entered the left atrium. It could not be retrieved by a transcatheter approach. Fortunately it lodged in the left atrium, where it caused no problems and did not embolise further. Following the procedure there was a marked improvement in exercise tolerance with the patient able to resume all activities of daily living and to climb a flight of stairs in comfort.
Discussion The association between pulmonary atresia with intact ventricular septum and the occurrence of fistulae between the right ventricle and the coronary arteries has long been recognised.1 The fistulae appear to be primarily associated with small hypertensive right ventricles and their presence has been documented in more than 60% of cases on pathological examination and more than 50% by angiography.2,3 Fistulae are an early feature of the cardiac malformation and may be identified using colour flow Doppler echocardiography.4 They have also been demonstrated in the fetus by fetal echocardiography.5,6 They are thought to represent the persistence of embryological connections between the ventricle and the coronary arteries, patency of which is maintained secondary to the high right ventricular pressure.7 Coronary artery blood flow may be dependent upon the right ventricle, or may be bi-directional.8 The potential dangers of surgical decompression of the right ventricle, with development of a right ventricular steal phenomenon and myocardial ischaemia are widely recognised.9,10 The presence of fistulae in this child was recognised prior to her modified Fontan procedure at the age of 4 years, at which time her right ventricular pressure was almost twice systemic under general anaesthesia. No surgical decompression of the right ventricle was undertaken. Whether the subsequent decline in right ventricular pressure to less than half systemic may be the result of increased tricuspid regurgitation brought about by geometric changes in the heart following the modified Fontan procedure, or due to incipient myocardial dysfunction caused by ischaemia either before or during the surgical procedures, or latterly as a result of the right ventricular steal phenomenon, is a matter for speculation. Indeed, there may have been contributions from all of these mechanisms. The consequence, however, has been the development of significant myocardial ischaemia on exertion in a young adult who would previously have had a right ventricular contribution to coronary artery blood flow. This case demonstrates that a right ventricular steal phenomenon may develop over time in a patient who has previously had right ventricular-dependent coronary artery blood flow, despite having undergone no procedure to decompress the right ventricle. The symptoms can be ameliorated by coil occlusion of coronary artery to right ventricular fistulae by a transcoronary approach. However, when coronary artery to right ventricular blood flow is sufficient to cause significant symptoms there is a risk of embolisation of a coil to the right ventricle as in this case,
197
from where it may enter the systemic circulation with the potential to embolise to any systemic arterial bed. There is also the possibility that coronary artery blood flow might be impeded either by inappropriate coil position or the development of thrombus either extending from the fistula or forming in an area of disruption of the coronary endothelium. These potential risks must be considered prior to undertaking such a procedure. However, this case demonstrates that the transcoronary approach and coil embolisation may be effective not only in patients with isolated coronary artery fistulae,11 but also in patients with coexistent complex congenital heart disease. This procedure may successfully treat the right ventricular steal phenomenon, with additional potential long-term benefit for the maintenance of ventricular function. Dr. Hope is supported by Medicine International and Faculty Postgraduate Research Scholarships from Monash University and a Cardiovascular Research Centre PhD Scholarship from Monash Medical Centre.
References 1. Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 1926;13:273–83. 2. Calder AL, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987;59(5):436–42. 3. Coles JG, Freedom RM, Lightfoot NE, Dasmahapatra HK, Williams WG, Trusler GA, et al. Long-term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1989;47(2):213–7. 4. Sanders SP, Parness IA, Colan SD. Recognition of abnormal connections of coronary arteries with the use of Doppler color flow mapping. J Am Coll Cardiol 1989;13(4):922–6. 5. Baschat AA, Love JC, Stewart PA, Gembruch U, Harman CR. Prenatal diagnosis of ventriculocoronary fistula. Ultrasound Obstet Gynecol 2001;18(1):39–43. 6. Chaoui R, Tennstedt C, Goldner B, Bollmann R. Prenatal diagnosis of ventriculo-coronary communications in a secondtrimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol 1997;9(3):194– 7. 7. Garson AJ, Bricker JT, McNamara D. The science and practice of pediatric cardiology. Philadelphia/London: Lea & Febiger; 1990. 8. Sissman NJ, Abrams HL. Bidirectional shunting in a coronary artery-right ventricular fistula associated with pulmonary atresia and an intact ventricular septum. Circulation 1965;32(4):582–8. 9. Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992;86(5):1516–28. 10. Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol 2000;86(11):1272–4, A9. 11. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39(6):1026–32.
CASE REPORT
Heart Lung and Circulation 2004;13:195–197
Case Report
Transcatheter Coil Occlusion of Coronary Artery to Right Ventricular Fistulae in Pulmonary Atresia with Intact Ventricular Septum Sarah A. Hope, MBChB MRCP, a Samuel Menahem, MD FRACPb and Ian T. Meredith, PhD FRACPa,∗ a
Cardiovascular Research Centre, Monash Medical Centre, 246 Clayton Road, Clayton, Vic. 3168, Australia b Department of Cardiology, Monash Medical Centre, 246 Clayton Road, Clayton, Vic. 3168, Australia
We describe a young adult with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology who presented with extreme dyspnoea on exertion. Coronary artery to right ventricular fistulae and reversible myocardial ischaemia secondary to a right ventricular steal phenomenon were present. The right coronary artery to right ventricular fistula was completely occluded with coils, by a transcoronary approach, with amelioration of symptoms. However, one coil embolised and lodged uneventfully in the left atrium during the procedure. This case demonstrates that a transcatheter approach may be successfully employed not only in patients with isolated coronary artery fistulae, but also in patients with coexistent complex congenital heart disease. (Heart Lung and Circulation 2004;13:195–197) © 2004 Published by Elsevier Inc on behalf of Australian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Keywords. Pulmonary atresia intact ventricular septum; Coronary artery fistula; Interventional catheterisation
Case report
A
young girl with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology presented with decreasing exercise tolerance and extreme dyspnoea on exertion. She was known to have coronary artery to right ventricular fistulae, and myocardial ischaemia was suspected. At the age of 16 years an exercise test demonstrated electrocardiographic changes consistent with ischaemia after just 3 min of the Bruce protocol. An exercise myocardial perfusion scan was subsequently performed but demonstrated only minor changes in the anterior wall, possibly due to attenuation artefact. Coronary angiography confirmed the presence of coronary artery to right ventricular fistulae involving both right and left coronary artery systems, with direction of flow from the coronary arteries to the right ventricle (Fig. 1). There were no significant coronary artery stenoses. Right ventricular pressure was estimated at less than half systemic by Doppler echocardiography of tricuspid valve incompetence. Although the right ventricular pressure might be underestimated if the peak velocity of the regurgitant jet was not interrogated, due to the volume of the tricuspid regurgitation in this patient good Doppler traces
Received 31 March 2003; received in revised form 9 September 2003; accepted 4 March 2004 ∗ Corresponding author. Tel.: +61 3 9594 4545; fax: +61 3 9594 6939. E-mail address: [email protected] (I.T. Meredith).
were achieved with relative ease and the jet widely interrogated. It is unlikely that the right ventricular pressure was substantially underestimated. A right ventricular steal phenomenon was diagnosed with benefit thought likely from occlusion of the fistulae. The fistula between the right coronary artery and right ventricle was felt to be most significant, and coil occlusion was undertaken in the cardiac catheter laboratory by a transcoronary approach. The fistula was best visualised in a left anterior oblique projection with cranial angulation. Using a six French JR 3.5 guiding catheter (Cordis Corporation, Miami Lakes, Florida, USA) and a Balance® wire (Guidant Corporation, Santa Clara, California, USA), a three French Tracker catheter (Boston Scientific Corporation, Natick, Massachusetts, USA) was positioned within the fistula. Subsequently Balance® Middleweight (Guidant Corporation) and ACS DOC® (Guidant Corporation) wires were utilised to optimise the position. Six fiber coils (2 mm × 10 mm, 2 mm × 20 mm × 4, 3 mm × 30 mm) (Boston Scientific Corporation) and three GDC® —10 coils (2 mm × 6 cm, 2 mm × 8 cm, 3 mm × 8 cm) (Boston Scientific Corporation) were deployed via the Tracker catheter once a satisfactory position had been established, achieving complete occlusion of the fistula without obstruction to flow in the distal right coronary artery (Fig. 2). Unfortunately, due to the size of the fistula and volume of blood flow, one fiber coil, which was deployed early, became dislodged into the right ventricle from where it passed retrogradely across the tricuspid
© 2004 Published by Elsevier Inc on behalf of Australian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2004.03.016
196 CASE REPORT
Hope et al. Transcatheter coil occlusion of coronary artery to right ventricular fistulae
Heart Lung and Circulation 2004;13:195–197
Figure 1. Fistula between the right coronary artery and the right ventricle.
Figure 2. Complete occlusion with coils of the fistula between the right coronary artery and right ventricle with no obstruction to flow in the distal right coronary artery.
Hope et al. Transcatheter coil occlusion of coronary artery to right ventricular fistulae
valve. Since the atrial baffle was to the right of the tricuspid valve, the coil entered the left atrium. It could not be retrieved by a transcatheter approach. Fortunately it lodged in the left atrium, where it caused no problems and did not embolise further. Following the procedure there was a marked improvement in exercise tolerance with the patient able to resume all activities of daily living and to climb a flight of stairs in comfort.
Discussion The association between pulmonary atresia with intact ventricular septum and the occurrence of fistulae between the right ventricle and the coronary arteries has long been recognised.1 The fistulae appear to be primarily associated with small hypertensive right ventricles and their presence has been documented in more than 60% of cases on pathological examination and more than 50% by angiography.2,3 Fistulae are an early feature of the cardiac malformation and may be identified using colour flow Doppler echocardiography.4 They have also been demonstrated in the fetus by fetal echocardiography.5,6 They are thought to represent the persistence of embryological connections between the ventricle and the coronary arteries, patency of which is maintained secondary to the high right ventricular pressure.7 Coronary artery blood flow may be dependent upon the right ventricle, or may be bi-directional.8 The potential dangers of surgical decompression of the right ventricle, with development of a right ventricular steal phenomenon and myocardial ischaemia are widely recognised.9,10 The presence of fistulae in this child was recognised prior to her modified Fontan procedure at the age of 4 years, at which time her right ventricular pressure was almost twice systemic under general anaesthesia. No surgical decompression of the right ventricle was undertaken. Whether the subsequent decline in right ventricular pressure to less than half systemic may be the result of increased tricuspid regurgitation brought about by geometric changes in the heart following the modified Fontan procedure, or due to incipient myocardial dysfunction caused by ischaemia either before or during the surgical procedures, or latterly as a result of the right ventricular steal phenomenon, is a matter for speculation. Indeed, there may have been contributions from all of these mechanisms. The consequence, however, has been the development of significant myocardial ischaemia on exertion in a young adult who would previously have had a right ventricular contribution to coronary artery blood flow. This case demonstrates that a right ventricular steal phenomenon may develop over time in a patient who has previously had right ventricular-dependent coronary artery blood flow, despite having undergone no procedure to decompress the right ventricle. The symptoms can be ameliorated by coil occlusion of coronary artery to right ventricular fistulae by a transcoronary approach. However, when coronary artery to right ventricular blood flow is sufficient to cause significant symptoms there is a risk of embolisation of a coil to the right ventricle as in this case,
197
from where it may enter the systemic circulation with the potential to embolise to any systemic arterial bed. There is also the possibility that coronary artery blood flow might be impeded either by inappropriate coil position or the development of thrombus either extending from the fistula or forming in an area of disruption of the coronary endothelium. These potential risks must be considered prior to undertaking such a procedure. However, this case demonstrates that the transcoronary approach and coil embolisation may be effective not only in patients with isolated coronary artery fistulae,11 but also in patients with coexistent complex congenital heart disease. This procedure may successfully treat the right ventricular steal phenomenon, with additional potential long-term benefit for the maintenance of ventricular function. Dr. Hope is supported by Medicine International and Faculty Postgraduate Research Scholarships from Monash University and a Cardiovascular Research Centre PhD Scholarship from Monash Medical Centre.
References 1. Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 1926;13:273–83. 2. Calder AL, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987;59(5):436–42. 3. Coles JG, Freedom RM, Lightfoot NE, Dasmahapatra HK, Williams WG, Trusler GA, et al. Long-term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1989;47(2):213–7. 4. Sanders SP, Parness IA, Colan SD. Recognition of abnormal connections of coronary arteries with the use of Doppler color flow mapping. J Am Coll Cardiol 1989;13(4):922–6. 5. Baschat AA, Love JC, Stewart PA, Gembruch U, Harman CR. Prenatal diagnosis of ventriculocoronary fistula. Ultrasound Obstet Gynecol 2001;18(1):39–43. 6. Chaoui R, Tennstedt C, Goldner B, Bollmann R. Prenatal diagnosis of ventriculo-coronary communications in a secondtrimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol 1997;9(3):194– 7. 7. Garson AJ, Bricker JT, McNamara D. The science and practice of pediatric cardiology. Philadelphia/London: Lea & Febiger; 1990. 8. Sissman NJ, Abrams HL. Bidirectional shunting in a coronary artery-right ventricular fistula associated with pulmonary atresia and an intact ventricular septum. Circulation 1965;32(4):582–8. 9. Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992;86(5):1516–28. 10. Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol 2000;86(11):1272–4, A9. 11. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39(6):1026–32.
CASE REPORT
Heart Lung and Circulation 2004;13:195–197