- Email: [email protected]
Improved definition of anomalous left coronary artery by magnetic resonance coronary angiography
Volume 130, Number 3, Part 1
American Heart Journal
Manning et al.
615
4. Daoud EG, Niebauer M, Bakr O, Jentzer J, Man KC, Williamson BD, Hummel JD, Strickberger A, Morady F. Placement of electrode catheters into the coronary sinus during electrophysiology procedures using a femoral approach. Am J Cardiol 1994;74:194-5. 5. Momma K, Linde LM. Abnormal rhythms associated with left superior vena cava. Pediatr Res 1969;3:210-6. 6. Winter FS. Persistent left superior vena cava: survey of world literature and report of thirty additional cases. Angiology 1954;5:90-132.
Improved definition of anomalous left coronary artery by magnetic resonance coronary angiography Warren J. Manning, MD, a, b, d Wei Li, MD, b, d Stafford I. Cohen, MD, a, d Robert G. Johnson, MD, c, d and Robert R. Edelman, MD b, d Boston, Mass.
Congenital coronary artery anomalies are rare, occurring in only 0.6% to 1.2% of adults referred for coronary angiography13 and frequently in association with other forms of congenital cardiac abnormalities. The majority of coronary anomalies are not associated with impaired myocardial perfusion. Hemodynamically significant anomalies with abnormalities of myocardial perfusion 4 include origin of the left coronary artery from the pulmonary artery, congenital coronary artery stenosis or atresia, coronary artery fistulae, and origin of the left coronary artery from the right sinus of Valsalva with subsequent passage of the vessel between the aorta and right ventricular infundibulum. The latter condition was thought to be benign until the postmortem observations of Cheitlin et al. 5 of the sudden death of 9 of 33 patients in whom the left coronary artery rose from the right sinus of Valsalva. The cause of sudden death is not known but has been hypothesized to include coronary artery compression, an abnormal, slitlike ostium, or acute take-off angles of the anomalous vessel. 5 Although the diagnosis of anomalous coronary arteries is easily made by angiography, definition of the subsequent course of these vessels (anterior or posterior to the right ventricular outflow trac0 is often difficult. 6 Such information is important because a posteriorly directed course is associated with a poor prognosis. Magnetic resonance coronary angiography (MRCA), with the ability
From the Departments of aMedicine (Cardiovascular Division), bRadiology, and CSurgery, Charles A. Dana Research Institute, and the dHarvardThorndike Laboratory of the Beth Israel Hospital and Harvard Medical School. Supported in part by a grant from the National Institute of Health (R01 HL48538), Bethesda, Md. Dr. Manning is supported in part by the Edwar d Mallinckrodt Jr. Foundation, St. Louis, Mo. Reprint requests: Warren J. Manning, MD, Cardiovascular Division , Beth Israel Hospital, 330 Brookline Ave., Boston, MA 02215. AM HEARTJ 1995;130:615-7. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4]4]65074
Fig. 1. Coronary angiogram after contrast injection into right coronary ostia (solid arrow) demonstrates anomalous left coronary artery (open arrows). Pulmonary artery catheter is also noted curved arrows).
to noninvasively acqmre data in double-oblique orientations, is uniquely suited for the evaluation of anomalous coronary arteries. In this report, we describe a case of an anomalous left anterior descending coronary artery in which MRCA aided in defining its course. A 67-year-old man underwent diagnostic catheterization at an outside hospital 2 years before admission for evaluation of atypical chest pain. Coronary angiography demonstrated anomalous origin of the left coronary artery from the right sinus of Valsalva and a 50% mid right coronary artery (RCA) stenosis. The specific course of the left coronary artery could not be determined, and he was treated medically. He did well for 18 months but then had typical symptoms of exertional angina. Exercise thallium testing was stopped after 9 minutes of the Bruce protocol because of 9/10 angina that required sublingual nitroglycerin for relief at a rate-pressure product of 8.3 K. He had nondiagnostic 0.5 mm horizontal ST depressions anterolaterally, but thallium scintigraphy revealed reversible inferior, apical, and posterobasal perfusion deficits. Coronary angiography demonstrated an occlusion of the mid RCA with distal filling via collaterals. The left coronary artery rose from the proximal RCA (Fig. 1) and appeared to course posteriorly to the pulmonary outflow tract (which was identified by a pulmonary artery catheter). Mild systolic narrowing of this segment was also noted. No left circumflex coronary artery was identified. The patient was referred for MRCA to confirm the course of the anomalous left coronary artery. MRCA was performed with a superconducting 1.5 T echo-planar-capable system (Siemen's Medical Systems, Iselin, N.J.) with a standard planar elliptical spine coil as radiofrequency receiver. An ECG-gated gradient-echo sequence with fat saturation and k-space segmentation was used in conjunction with breath holding. 7A repetition time (TR) of 13 msec and an echo time (TE) of 8 msec were used with a 3 mm slice thickness, i mm overlap, and 230 x 230 mm field of view. Transverse images demonstrated the origin of the RCA and anomalous left coronary artery (Fig. 2, A). The left
616
Manninget al.
September 1995 American Heart Journal
Fig. 2. A, Transverse magnetic resonance coronary angiographic image at level of right coronary artery ostia (solid arrow) and origin of anomalous left coronary artery (open arrow). B and C, Successive transverse images depict course (open arrows) of left coronary artery. D, Oblique MR image demonstrates course of anomalous left coronary artery (solid arrows) between aorta and pulmonary artery and around pulmonary artery. AO, Ascending aorta;.LA, left atrium; PA, pulmonary artery; RV, right ventricular outflow tract.
coronary artery was then identified as it traveled between the ascending aorta and right ventricular outflow tract, finally extending along the interventricular groove (Fig. 2, B and C). Oblique imaging of the left coronary artery demonstrated its path around the pulmonary artery (Fig. 2, D). Transverse and oblique imaging of the RCA demonstrated loss of signal distal to the mid RCA. The patient was referred for coronary artery bypass surgery, at which time the posterior course of the left coronary artery was confirmed. A reverse saphenous vein graft was placed to the posterior descending coronary artery, and the left internal mammary artery was grafted to the mid left anterior descending coronary artery. His postoperative recovery was uneventful, and 6 months later he underwent a repeat exercise thallium test during which he exercised 14 minutes of the Bruce protocol to a rate-pressure product of 26.6 K. He had no anginal symptoms, ECG demonstrated no evidence for ischemia, and thallium scintigraphy was normal. Origin of both coronary arteries from the right sinus of Valsalva is a rare congenital anomaly identified in 0.6% to 1.2% of patients referred for diagnostic angiography.l-a Although the origin of anomalous coronary arteries is readily identified by angiography, delineation of their more distal course is often difficult. Such information is important because patients with vessels passing between the aorta and
right ventricular outflow tract have an increased risk of sudden death and myocardial infarction. Positioning of a pulmonary artery catheter during conventional angiography may be helpful. Although transthoracic echocardiography has little utility for identifying the course of these vessels, transesophageal echocardiography has recently been shown to be a valuable adjunct to angiography,s Transesophageal echocardiography is minimally invasive but has limited ability to identify the distal portion of the vessel. In addition, time-consuming transducer manipulations are required to track the course of the vessel, s Conventional spin-echo or cine gradient echo magnetic resonance imaging methods, which take several minutes of data acquisition to construct an image, are poorly suited for imaging of native coronary vessels 9 but have been shown to be useful to delineate coronary artery fistula. 1° These vascular conduits, however, are much larger than the native coronary arteries. More recently magnetic resonance coronary angiographic techniques that allow visualization of native vessels have been developed, and identification of stenoses has also been described. 7 The flexibility of imaging planes and ease of identification of mediastinal structures make magnetic resonance coronary angiography an ideal imaging tool to define the course of anomalous coronary arteries. As depicted in this example,
Volume 130, Number 3, Part 1 American Heart Journal
Cohen et al. 617
extensive portions of the anomalous vessel can be visualized its passage can be clearly visualized with respect to the aorta and right v e n t r i c u l a r outflow tract. In conclusion, magnetic resonance coronary angiography is a helpful adj u n c t to conventional coronary angiography and should be considered in patients with anomalous coronary a r t e r i e s in w h o m conventional angiography does not clearly delineate the course of the anomalous vessel. REFERENCES
1. Chaitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamicfindings in patients with anomalous origin of the coronary arteries. Circulation 1976;53:122-31. 2. Engel HJ, Tones C, Page HL. Majorvariations in anatomical origin of the coronary arteries; angiographic observations in 4,250 patients without associated congenitalheart disease. Cathet CardiovascDiagn 1975;1:157-69. 3. KimbirisD, Iskandrian AS, SegalBL, BemisCE. Anomalousaortic origin of coronary arteries, Circulation 1978;58:606-15. 4. Leviu DC, FollowsKE, Abrams HL. Hemodynamicallysignificantprimary anomalies of the coronary arteries: angiographic aspects. Circulation 1978;58:25-34. 5. Cheitlin MD, Decastro CM, McAllisterHA. Sudden death as a complication of anomalous left coronary originfrom the anterior sinus ofValsalva: a not-so-minor congenital anomaly. Circulation 1974;50:780-7. 6. Ishikawa T, Brandt PWT. Anomalousorigin of the left main coronary artery from the right anterior aortic sinus: angiographic definition of anomalous course. Am J Cardiol 1985;55:770-6. 7. Manning WJ, Li W, Edelman RR. A preliminary report comparing magnetic resonance coronary angiography with conventionalcontrast angiography. N Engl J IVied1993;328:828-32. 8. Fernandes F, Alam M, Smith S, Khaja F. The role oftransesophageal echocardiography in identifyinganomalous coronaryarteries. Circulation 1993;88:2532-40. 9. Paulin S, von Schulthess GK, FosselE, Krayenbuehl HP. MR imaging of the aortic root and proximal coronary arteries. Am J Roent 1987; 148:665-70. 10. Kugelmass AD, Manning WJ, Piana RN, Weintraub RM, Balm DS, Grossman WG. Coronary arteriovenous fistula presenting as congestive heart failure. Cathet Cardiovasc Diaga 1992;26:19-25.
Obstruction of the systemic venous pathway after closure of an adjustable atrial septal defect in the modified Fontan operation Daniel M. Cohen, MD, a J o h n J. Whe!ler , MD, b J. Terrance Davis, MD, a and H u g h D. Allen, MD b
Columbus, Ohio
The "fenestrated" F o n t a n operation is now recognized as a suitable alternative for patients who are deemed to be at From the aDepartment ofThoracic Surgeryand the bDivisionofCardiology, Children's Hospital and Ohio State University CollegeOf Medicine. Reprint requests: Daniel M. Cohen, MD, Department of Thoracic Surgery, Children's Hospital, 700 Children's Dr., Columbus, OH 43205. AMHEARTJ 1995;130:617-8. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 414]65075
Fig. 1. Angiogram: injection into systemic (right) atrium, right anterior oblique view. Obstruction of lateral t u n n e l (white arrows) after closure of adjustable ASD. Dark arrows, Surgical dips; RPA, right p u l m o n a r y artery;LPA, left p u l m o n a r y artery.
too high of a risk to undergo the complete procedure. 1, 2 A total cavopulmonary anastomosis is u n d e r t a k e n in which the lateral t u n n e l technique is combined with the superior v e n a caval pulmonary connection. 3 Additionally, a fixed communication between the systemic venous and pulmon a r y venous chambers is created to allow right-to-lei~ atrial decompression to take place. An adjustable snare is fixed to the margins of the defect, enabling its ready closure at an appropriate future date. 4 Obstruction in the lateral tunnel resulting from traction on this adjustable snare has not been previously described. This case report describes a m e c h a n i s m in which such an obstruction occurred and h o w it was relieved in the cardiac catheterization laboratory. A 6-year-old girl with double outlet right ventricle and a straddling left atrioventricular valve had undergone a central shunt at 2 years of age. Cardiac catheterization at 6 years of age d e m o n s t r a t e d normal p u l m o n a r y a r t e r y pressure and resistance and normal v e n t r i c u l a r end-diastolic pressures. A modified F o n t a n procedure w i t h an adjustable atrial septal defect (ASD) was performed. At operation a long stenotic segment of the left p u l m o n a r y artery was a u g m e n t e d with a patch of bovine pericardium, the superior v e n a cava was transected and anastomosed to the right p u l m o n a r y artery, and a lateral t u n n e l technique was used to baffle the inferior v e n a caval blood to the right pulmonary artery. Because it was necessary to reconstruct the left pulmonary artery, we decided to fenestrate the F o n t a n repair. A 5 m m adjustable ASD was left in place and encircled with a r u n n i n g suture t h a t was pulled through a polyethylene tube t h a t served as a tourniquet buried b e n e a t h the rectus abdominis fascia. Three months later, r e p e a t cardiac catheterization d e m o n s t r a t e d rightto-left s h u n t i n g through the adjustable ASD. Systemic venous pressures were 16 m m H g and there was no gradient demonstrable from the inferior v e n a cava to the
American Heart Journal
Manning et al.
615
4. Daoud EG, Niebauer M, Bakr O, Jentzer J, Man KC, Williamson BD, Hummel JD, Strickberger A, Morady F. Placement of electrode catheters into the coronary sinus during electrophysiology procedures using a femoral approach. Am J Cardiol 1994;74:194-5. 5. Momma K, Linde LM. Abnormal rhythms associated with left superior vena cava. Pediatr Res 1969;3:210-6. 6. Winter FS. Persistent left superior vena cava: survey of world literature and report of thirty additional cases. Angiology 1954;5:90-132.
Improved definition of anomalous left coronary artery by magnetic resonance coronary angiography Warren J. Manning, MD, a, b, d Wei Li, MD, b, d Stafford I. Cohen, MD, a, d Robert G. Johnson, MD, c, d and Robert R. Edelman, MD b, d Boston, Mass.
Congenital coronary artery anomalies are rare, occurring in only 0.6% to 1.2% of adults referred for coronary angiography13 and frequently in association with other forms of congenital cardiac abnormalities. The majority of coronary anomalies are not associated with impaired myocardial perfusion. Hemodynamically significant anomalies with abnormalities of myocardial perfusion 4 include origin of the left coronary artery from the pulmonary artery, congenital coronary artery stenosis or atresia, coronary artery fistulae, and origin of the left coronary artery from the right sinus of Valsalva with subsequent passage of the vessel between the aorta and right ventricular infundibulum. The latter condition was thought to be benign until the postmortem observations of Cheitlin et al. 5 of the sudden death of 9 of 33 patients in whom the left coronary artery rose from the right sinus of Valsalva. The cause of sudden death is not known but has been hypothesized to include coronary artery compression, an abnormal, slitlike ostium, or acute take-off angles of the anomalous vessel. 5 Although the diagnosis of anomalous coronary arteries is easily made by angiography, definition of the subsequent course of these vessels (anterior or posterior to the right ventricular outflow trac0 is often difficult. 6 Such information is important because a posteriorly directed course is associated with a poor prognosis. Magnetic resonance coronary angiography (MRCA), with the ability
From the Departments of aMedicine (Cardiovascular Division), bRadiology, and CSurgery, Charles A. Dana Research Institute, and the dHarvardThorndike Laboratory of the Beth Israel Hospital and Harvard Medical School. Supported in part by a grant from the National Institute of Health (R01 HL48538), Bethesda, Md. Dr. Manning is supported in part by the Edwar d Mallinckrodt Jr. Foundation, St. Louis, Mo. Reprint requests: Warren J. Manning, MD, Cardiovascular Division , Beth Israel Hospital, 330 Brookline Ave., Boston, MA 02215. AM HEARTJ 1995;130:615-7. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4]4]65074
Fig. 1. Coronary angiogram after contrast injection into right coronary ostia (solid arrow) demonstrates anomalous left coronary artery (open arrows). Pulmonary artery catheter is also noted curved arrows).
to noninvasively acqmre data in double-oblique orientations, is uniquely suited for the evaluation of anomalous coronary arteries. In this report, we describe a case of an anomalous left anterior descending coronary artery in which MRCA aided in defining its course. A 67-year-old man underwent diagnostic catheterization at an outside hospital 2 years before admission for evaluation of atypical chest pain. Coronary angiography demonstrated anomalous origin of the left coronary artery from the right sinus of Valsalva and a 50% mid right coronary artery (RCA) stenosis. The specific course of the left coronary artery could not be determined, and he was treated medically. He did well for 18 months but then had typical symptoms of exertional angina. Exercise thallium testing was stopped after 9 minutes of the Bruce protocol because of 9/10 angina that required sublingual nitroglycerin for relief at a rate-pressure product of 8.3 K. He had nondiagnostic 0.5 mm horizontal ST depressions anterolaterally, but thallium scintigraphy revealed reversible inferior, apical, and posterobasal perfusion deficits. Coronary angiography demonstrated an occlusion of the mid RCA with distal filling via collaterals. The left coronary artery rose from the proximal RCA (Fig. 1) and appeared to course posteriorly to the pulmonary outflow tract (which was identified by a pulmonary artery catheter). Mild systolic narrowing of this segment was also noted. No left circumflex coronary artery was identified. The patient was referred for MRCA to confirm the course of the anomalous left coronary artery. MRCA was performed with a superconducting 1.5 T echo-planar-capable system (Siemen's Medical Systems, Iselin, N.J.) with a standard planar elliptical spine coil as radiofrequency receiver. An ECG-gated gradient-echo sequence with fat saturation and k-space segmentation was used in conjunction with breath holding. 7A repetition time (TR) of 13 msec and an echo time (TE) of 8 msec were used with a 3 mm slice thickness, i mm overlap, and 230 x 230 mm field of view. Transverse images demonstrated the origin of the RCA and anomalous left coronary artery (Fig. 2, A). The left
616
Manninget al.
September 1995 American Heart Journal
Fig. 2. A, Transverse magnetic resonance coronary angiographic image at level of right coronary artery ostia (solid arrow) and origin of anomalous left coronary artery (open arrow). B and C, Successive transverse images depict course (open arrows) of left coronary artery. D, Oblique MR image demonstrates course of anomalous left coronary artery (solid arrows) between aorta and pulmonary artery and around pulmonary artery. AO, Ascending aorta;.LA, left atrium; PA, pulmonary artery; RV, right ventricular outflow tract.
coronary artery was then identified as it traveled between the ascending aorta and right ventricular outflow tract, finally extending along the interventricular groove (Fig. 2, B and C). Oblique imaging of the left coronary artery demonstrated its path around the pulmonary artery (Fig. 2, D). Transverse and oblique imaging of the RCA demonstrated loss of signal distal to the mid RCA. The patient was referred for coronary artery bypass surgery, at which time the posterior course of the left coronary artery was confirmed. A reverse saphenous vein graft was placed to the posterior descending coronary artery, and the left internal mammary artery was grafted to the mid left anterior descending coronary artery. His postoperative recovery was uneventful, and 6 months later he underwent a repeat exercise thallium test during which he exercised 14 minutes of the Bruce protocol to a rate-pressure product of 26.6 K. He had no anginal symptoms, ECG demonstrated no evidence for ischemia, and thallium scintigraphy was normal. Origin of both coronary arteries from the right sinus of Valsalva is a rare congenital anomaly identified in 0.6% to 1.2% of patients referred for diagnostic angiography.l-a Although the origin of anomalous coronary arteries is readily identified by angiography, delineation of their more distal course is often difficult. Such information is important because patients with vessels passing between the aorta and
right ventricular outflow tract have an increased risk of sudden death and myocardial infarction. Positioning of a pulmonary artery catheter during conventional angiography may be helpful. Although transthoracic echocardiography has little utility for identifying the course of these vessels, transesophageal echocardiography has recently been shown to be a valuable adjunct to angiography,s Transesophageal echocardiography is minimally invasive but has limited ability to identify the distal portion of the vessel. In addition, time-consuming transducer manipulations are required to track the course of the vessel, s Conventional spin-echo or cine gradient echo magnetic resonance imaging methods, which take several minutes of data acquisition to construct an image, are poorly suited for imaging of native coronary vessels 9 but have been shown to be useful to delineate coronary artery fistula. 1° These vascular conduits, however, are much larger than the native coronary arteries. More recently magnetic resonance coronary angiographic techniques that allow visualization of native vessels have been developed, and identification of stenoses has also been described. 7 The flexibility of imaging planes and ease of identification of mediastinal structures make magnetic resonance coronary angiography an ideal imaging tool to define the course of anomalous coronary arteries. As depicted in this example,
Volume 130, Number 3, Part 1 American Heart Journal
Cohen et al. 617
extensive portions of the anomalous vessel can be visualized its passage can be clearly visualized with respect to the aorta and right v e n t r i c u l a r outflow tract. In conclusion, magnetic resonance coronary angiography is a helpful adj u n c t to conventional coronary angiography and should be considered in patients with anomalous coronary a r t e r i e s in w h o m conventional angiography does not clearly delineate the course of the anomalous vessel. REFERENCES
1. Chaitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamicfindings in patients with anomalous origin of the coronary arteries. Circulation 1976;53:122-31. 2. Engel HJ, Tones C, Page HL. Majorvariations in anatomical origin of the coronary arteries; angiographic observations in 4,250 patients without associated congenitalheart disease. Cathet CardiovascDiagn 1975;1:157-69. 3. KimbirisD, Iskandrian AS, SegalBL, BemisCE. Anomalousaortic origin of coronary arteries, Circulation 1978;58:606-15. 4. Leviu DC, FollowsKE, Abrams HL. Hemodynamicallysignificantprimary anomalies of the coronary arteries: angiographic aspects. Circulation 1978;58:25-34. 5. Cheitlin MD, Decastro CM, McAllisterHA. Sudden death as a complication of anomalous left coronary originfrom the anterior sinus ofValsalva: a not-so-minor congenital anomaly. Circulation 1974;50:780-7. 6. Ishikawa T, Brandt PWT. Anomalousorigin of the left main coronary artery from the right anterior aortic sinus: angiographic definition of anomalous course. Am J Cardiol 1985;55:770-6. 7. Manning WJ, Li W, Edelman RR. A preliminary report comparing magnetic resonance coronary angiography with conventionalcontrast angiography. N Engl J IVied1993;328:828-32. 8. Fernandes F, Alam M, Smith S, Khaja F. The role oftransesophageal echocardiography in identifyinganomalous coronaryarteries. Circulation 1993;88:2532-40. 9. Paulin S, von Schulthess GK, FosselE, Krayenbuehl HP. MR imaging of the aortic root and proximal coronary arteries. Am J Roent 1987; 148:665-70. 10. Kugelmass AD, Manning WJ, Piana RN, Weintraub RM, Balm DS, Grossman WG. Coronary arteriovenous fistula presenting as congestive heart failure. Cathet Cardiovasc Diaga 1992;26:19-25.
Obstruction of the systemic venous pathway after closure of an adjustable atrial septal defect in the modified Fontan operation Daniel M. Cohen, MD, a J o h n J. Whe!ler , MD, b J. Terrance Davis, MD, a and H u g h D. Allen, MD b
Columbus, Ohio
The "fenestrated" F o n t a n operation is now recognized as a suitable alternative for patients who are deemed to be at From the aDepartment ofThoracic Surgeryand the bDivisionofCardiology, Children's Hospital and Ohio State University CollegeOf Medicine. Reprint requests: Daniel M. Cohen, MD, Department of Thoracic Surgery, Children's Hospital, 700 Children's Dr., Columbus, OH 43205. AMHEARTJ 1995;130:617-8. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 414]65075
Fig. 1. Angiogram: injection into systemic (right) atrium, right anterior oblique view. Obstruction of lateral t u n n e l (white arrows) after closure of adjustable ASD. Dark arrows, Surgical dips; RPA, right p u l m o n a r y artery;LPA, left p u l m o n a r y artery.
too high of a risk to undergo the complete procedure. 1, 2 A total cavopulmonary anastomosis is u n d e r t a k e n in which the lateral t u n n e l technique is combined with the superior v e n a caval pulmonary connection. 3 Additionally, a fixed communication between the systemic venous and pulmon a r y venous chambers is created to allow right-to-lei~ atrial decompression to take place. An adjustable snare is fixed to the margins of the defect, enabling its ready closure at an appropriate future date. 4 Obstruction in the lateral tunnel resulting from traction on this adjustable snare has not been previously described. This case report describes a m e c h a n i s m in which such an obstruction occurred and h o w it was relieved in the cardiac catheterization laboratory. A 6-year-old girl with double outlet right ventricle and a straddling left atrioventricular valve had undergone a central shunt at 2 years of age. Cardiac catheterization at 6 years of age d e m o n s t r a t e d normal p u l m o n a r y a r t e r y pressure and resistance and normal v e n t r i c u l a r end-diastolic pressures. A modified F o n t a n procedure w i t h an adjustable atrial septal defect (ASD) was performed. At operation a long stenotic segment of the left p u l m o n a r y artery was a u g m e n t e d with a patch of bovine pericardium, the superior v e n a cava was transected and anastomosed to the right p u l m o n a r y artery, and a lateral t u n n e l technique was used to baffle the inferior v e n a caval blood to the right pulmonary artery. Because it was necessary to reconstruct the left pulmonary artery, we decided to fenestrate the F o n t a n repair. A 5 m m adjustable ASD was left in place and encircled with a r u n n i n g suture t h a t was pulled through a polyethylene tube t h a t served as a tourniquet buried b e n e a t h the rectus abdominis fascia. Three months later, r e p e a t cardiac catheterization d e m o n s t r a t e d rightto-left s h u n t i n g through the adjustable ASD. Systemic venous pressures were 16 m m H g and there was no gradient demonstrable from the inferior v e n a cava to the