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Successful repair of congenital left ventricle-to-coronary sinus fistulas
Ann Thorac Surg 1994;57757-8
9. Wakabayashi A. Expanded applications of diagnostic and therapeutic thoracoscopy. J Thorac Cardiovasc Surg 1991;102: 721-3.
Successful Repair of Congenital Left Ventricle-to-Coronary Sinus Fistulas John E. Fetter, MD, Carl L. Backer, MD, Alexander J. Muster, MD, Thomas J. Weigel, MD, and Constantine Mavroudis, MD Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children’s Memorial Hospital, and Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, Illinois A case of successful repair of congenital fistulas between the left ventricle and coronary sinus is discussed, including the pertinent diagnostic and surgical techniques. (Ann Thorac Surg 1994;57:757-8)
C
ongenital fistula between the left ventricle and the coronary sinus has been previously reported [l]. This is a report of a successful repair of such a congenital fistula between the left ventricle and the coronary sinus. A 22-month-old boy was born prematurely after a 32week gestation and was noted to have a heart murmur as a neonate. He was acyanotic and asymptomatic. Cardiac examination revealed a prominent right ventricular impulse with a 3/6 systolic ejection murmur followed by a loud diastolic rumble. The liver was palpable 2 cm below the costal margin. The chest radiograph showed marked cardiomegaly with increased pulmonary vascular markings. The electrocardiogram showed superior axis, normal sinus rhythm, incomplete right bundle-branch block, and right ventricular hypertrophy. The echocardiogram initially made the diagnosis, revealing a markedly dilated coronary sinus with Doppler color jets from the left ventricle into the coronary sinus. There was no left superior vena cava. A large secundum atrial septal defect with left-to-right shunting was demonstrated. Cardiac catheterization was performed. Right atrial pressures were normal; pulmonary artery pressure was 2318 mm Hg, pulmonary to systemic flow ratio was 1.8:l. Oxygen saturation was 83%in the coronary sinus and 80% in the right atrium. Cineangiography revealed a large secundum type atrial septal defect and a massively dilated coronary sinus, which distorted right atrial, left atrial, and right ventricular anatomy (Fig 1).
CASE REPORT FETTER ET AL LEFT VENTRICLE-TO-CORONARYSINUS FISTULAS
757
There were several fistulas communicating directly from the left ventricle to the coronary sinus (Fig 2). Operative repair was performed through a median sternotomy with routine aortobicaval cannulation, cardiopulmonary bypass, and antegrade aortic cardioplegia. Through a right atriotomy, a 2.0 x 1.5-cm ostium secundum atrial septal defect was identified as well as the large dilated coronary sinus; there were three fistulous openings deep within the sinus, each 2 mm in diameter and 4 to 5 mm apart, connecting to the left ventricle. The fistulous openings were closed with a single 1.0 x 1.5-cm Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) patch anchored with running 5-0 Prolene (Ethicon, Somerville, NJ) suture. The atrial septal defect was then closed with a pericardial patch. After atriorrhaphy, the patient was easily weaned from cardiopulmonary bypass. The postoperative course was complicated by supraventricular tachycardia, atrial fibrillation, and atrial flutter. The child was treated with digoxin and procainamide with eventual control of arrhythmias. He was discharged home in normal sinus rhythm. Follow-up Doppler echocardiographic study demonstrated no residual atrial septal defect or left ventricle to coronary sinus shunting. The arrhythmias have not recurred, and the child is doing well now 1 year postoperatively.
Comment Congenital fistula between the left ventricle and the coronary sinus is a very rare congenital anomaly. One patient was reported in 1981, a child also having transposition of the great arteries with ventricular septal defect
Accepted for publication June 24, 1993. Address reprint requests to Dr Backer, Division of Cardiovascular-Thoracic Surgery, The Children’s Memorial Hospital, 2300 Children’s Plaza, M/C 22, Chicago, IL 60614.
Fig 1. Selective angiography reveals a dilated coronary sinus (CS) communicating with the right atrium (RA) and through multiple fistulas (F) with the left ventricle.
758
CASE REPORT FETTER ET AL LEFT VENTRICLE-TO-CORONARY SINUS FISTULAS
Ann Thorac Surg 1994;57757-8
usually caused by anomalous communication resulting in high-pressure left-to-right shunt into the coronary sinus. However, in their study this was always the result of a coronary artery-to-coronary sinus fistula. Mantini and colleagues did not describe a left ventricle-to-coronary sinus fistula. Other authors have reported anomalies of the coronary sinus to include coronary artery-coronary sinus fistulas, left superior vena cava, and coronary sinus-left atrial communication [3-51. Fistulas between the left ventricle and the coronary sinus have also been described after mitral valve replacement, but this has been attributed to surgical trauma [6, 71. In a patient with an enlarged coronary sinus, left ventricle-to-coronary sinus fistulas should be suspected when the coronary sinus is unusually large and there is no left superior vena cava. Diagnosis can be made by color Doppler echocardiography and confirmed by angiography with coronary sinus and left ventricular injections. At cardiac catheterization, the oxygen saturation is higher in the coronary sinus than in the right atrium if there is no atrial septal defect. If there are multiple fistulas, as in our patient, a patch to close the communications is recommended. We do not believe it is necessary to excise the coronary sinus aneurysm. Fig 2 . Left ventricular (LV) injection shows the fistulas (F) communicating with the coronary sinus (CS) during ventricular diastole. During systole, the communication was obliterated.
and left ventricular outflow tract obstruction [l].Like our patient, this child also had an atrial septal defect. After a palliative operation, the child had supraventricular tachycardia, which responded to treatment with digoxin, similar to our patient's arrhythmia. At the age of 5 years, the single fistula between the left ventricle and coronary sinus was closed with pledgeted sutures and part of the wall of the coronary sinus was excised to reduce the aneurysm size. The shunt was ligated and a standard Rastelli operation performed, but the child could not be weaned from cardiopulmonary bypass and died. In a comprehensive study of recognized anomalies of the coronary sinus, Mantini and colleagues [2] concluded that in the absence of chronic congestive heart failure or right atrial hypertension, coronary sinus enlargement is
References 1. McGarry CM, Stark J, Macartney FJ. Congenital fistula be2. 3.
4. 5. 6.
7.
tween left ventricle and coronary sinus. Br Heart J 1981;45: 1014 Mantini E, Grondin CM, Lillehei CW,. Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966;33: 317-27. Neufeld HN, Lester RG, Adams 'I Jr, Anderson RC, Lillehei CW, Edwards JC. Congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk ("coronary artery fistula"). Circulation 1961;24:171-9. Lee ME, Sade RM. Coronary sinus septal defect. J Thorac Cardiovasc Surg 1979;78:56>9. Cohle SD, Titus JL, Kim H, Erikson E. Communication of the coronary sinus with both atria. Arch Pathol Lab Med 1981;105: 407-8. Morritt GN, Jamieson MPG, Irving JB, Marquis RM, Walbaum PR. Development of left ventricularqoronary sinus fistula following replacement of mitral valve prosthesis. J Thorac Cardiovasc Surg 1978;76:3814. Miller DC, Schapira JN, Stinson EB, Shumway NE. Left ventricular-coronary sinus fistula following repeated mitral valve replacement. J Thorac Cardiovasc Surg 1978;76:4>5.
9. Wakabayashi A. Expanded applications of diagnostic and therapeutic thoracoscopy. J Thorac Cardiovasc Surg 1991;102: 721-3.
Successful Repair of Congenital Left Ventricle-to-Coronary Sinus Fistulas John E. Fetter, MD, Carl L. Backer, MD, Alexander J. Muster, MD, Thomas J. Weigel, MD, and Constantine Mavroudis, MD Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children’s Memorial Hospital, and Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, Illinois A case of successful repair of congenital fistulas between the left ventricle and coronary sinus is discussed, including the pertinent diagnostic and surgical techniques. (Ann Thorac Surg 1994;57:757-8)
C
ongenital fistula between the left ventricle and the coronary sinus has been previously reported [l]. This is a report of a successful repair of such a congenital fistula between the left ventricle and the coronary sinus. A 22-month-old boy was born prematurely after a 32week gestation and was noted to have a heart murmur as a neonate. He was acyanotic and asymptomatic. Cardiac examination revealed a prominent right ventricular impulse with a 3/6 systolic ejection murmur followed by a loud diastolic rumble. The liver was palpable 2 cm below the costal margin. The chest radiograph showed marked cardiomegaly with increased pulmonary vascular markings. The electrocardiogram showed superior axis, normal sinus rhythm, incomplete right bundle-branch block, and right ventricular hypertrophy. The echocardiogram initially made the diagnosis, revealing a markedly dilated coronary sinus with Doppler color jets from the left ventricle into the coronary sinus. There was no left superior vena cava. A large secundum atrial septal defect with left-to-right shunting was demonstrated. Cardiac catheterization was performed. Right atrial pressures were normal; pulmonary artery pressure was 2318 mm Hg, pulmonary to systemic flow ratio was 1.8:l. Oxygen saturation was 83%in the coronary sinus and 80% in the right atrium. Cineangiography revealed a large secundum type atrial septal defect and a massively dilated coronary sinus, which distorted right atrial, left atrial, and right ventricular anatomy (Fig 1).
CASE REPORT FETTER ET AL LEFT VENTRICLE-TO-CORONARYSINUS FISTULAS
757
There were several fistulas communicating directly from the left ventricle to the coronary sinus (Fig 2). Operative repair was performed through a median sternotomy with routine aortobicaval cannulation, cardiopulmonary bypass, and antegrade aortic cardioplegia. Through a right atriotomy, a 2.0 x 1.5-cm ostium secundum atrial septal defect was identified as well as the large dilated coronary sinus; there were three fistulous openings deep within the sinus, each 2 mm in diameter and 4 to 5 mm apart, connecting to the left ventricle. The fistulous openings were closed with a single 1.0 x 1.5-cm Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) patch anchored with running 5-0 Prolene (Ethicon, Somerville, NJ) suture. The atrial septal defect was then closed with a pericardial patch. After atriorrhaphy, the patient was easily weaned from cardiopulmonary bypass. The postoperative course was complicated by supraventricular tachycardia, atrial fibrillation, and atrial flutter. The child was treated with digoxin and procainamide with eventual control of arrhythmias. He was discharged home in normal sinus rhythm. Follow-up Doppler echocardiographic study demonstrated no residual atrial septal defect or left ventricle to coronary sinus shunting. The arrhythmias have not recurred, and the child is doing well now 1 year postoperatively.
Comment Congenital fistula between the left ventricle and the coronary sinus is a very rare congenital anomaly. One patient was reported in 1981, a child also having transposition of the great arteries with ventricular septal defect
Accepted for publication June 24, 1993. Address reprint requests to Dr Backer, Division of Cardiovascular-Thoracic Surgery, The Children’s Memorial Hospital, 2300 Children’s Plaza, M/C 22, Chicago, IL 60614.
Fig 1. Selective angiography reveals a dilated coronary sinus (CS) communicating with the right atrium (RA) and through multiple fistulas (F) with the left ventricle.
758
CASE REPORT FETTER ET AL LEFT VENTRICLE-TO-CORONARY SINUS FISTULAS
Ann Thorac Surg 1994;57757-8
usually caused by anomalous communication resulting in high-pressure left-to-right shunt into the coronary sinus. However, in their study this was always the result of a coronary artery-to-coronary sinus fistula. Mantini and colleagues did not describe a left ventricle-to-coronary sinus fistula. Other authors have reported anomalies of the coronary sinus to include coronary artery-coronary sinus fistulas, left superior vena cava, and coronary sinus-left atrial communication [3-51. Fistulas between the left ventricle and the coronary sinus have also been described after mitral valve replacement, but this has been attributed to surgical trauma [6, 71. In a patient with an enlarged coronary sinus, left ventricle-to-coronary sinus fistulas should be suspected when the coronary sinus is unusually large and there is no left superior vena cava. Diagnosis can be made by color Doppler echocardiography and confirmed by angiography with coronary sinus and left ventricular injections. At cardiac catheterization, the oxygen saturation is higher in the coronary sinus than in the right atrium if there is no atrial septal defect. If there are multiple fistulas, as in our patient, a patch to close the communications is recommended. We do not believe it is necessary to excise the coronary sinus aneurysm. Fig 2 . Left ventricular (LV) injection shows the fistulas (F) communicating with the coronary sinus (CS) during ventricular diastole. During systole, the communication was obliterated.
and left ventricular outflow tract obstruction [l].Like our patient, this child also had an atrial septal defect. After a palliative operation, the child had supraventricular tachycardia, which responded to treatment with digoxin, similar to our patient's arrhythmia. At the age of 5 years, the single fistula between the left ventricle and coronary sinus was closed with pledgeted sutures and part of the wall of the coronary sinus was excised to reduce the aneurysm size. The shunt was ligated and a standard Rastelli operation performed, but the child could not be weaned from cardiopulmonary bypass and died. In a comprehensive study of recognized anomalies of the coronary sinus, Mantini and colleagues [2] concluded that in the absence of chronic congestive heart failure or right atrial hypertension, coronary sinus enlargement is
References 1. McGarry CM, Stark J, Macartney FJ. Congenital fistula be2. 3.
4. 5. 6.
7.
tween left ventricle and coronary sinus. Br Heart J 1981;45: 1014 Mantini E, Grondin CM, Lillehei CW,. Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966;33: 317-27. Neufeld HN, Lester RG, Adams 'I Jr, Anderson RC, Lillehei CW, Edwards JC. Congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk ("coronary artery fistula"). Circulation 1961;24:171-9. Lee ME, Sade RM. Coronary sinus septal defect. J Thorac Cardiovasc Surg 1979;78:56>9. Cohle SD, Titus JL, Kim H, Erikson E. Communication of the coronary sinus with both atria. Arch Pathol Lab Med 1981;105: 407-8. Morritt GN, Jamieson MPG, Irving JB, Marquis RM, Walbaum PR. Development of left ventricularqoronary sinus fistula following replacement of mitral valve prosthesis. J Thorac Cardiovasc Surg 1978;76:3814. Miller DC, Schapira JN, Stinson EB, Shumway NE. Left ventricular-coronary sinus fistula following repeated mitral valve replacement. J Thorac Cardiovasc Surg 1978;76:4>5.