Pediatr Cardiol (2015) 36:1100–1101 DOI 10.1007/s00246-015-1137-1
IMAGES IN PEDIATRIC CARDIOLOGY
Tetralogy of Fallot with Left Superior Vena Cava and Coronary Sinus Atrial Septal Defect: A Rare Association Kiran K. Mallula1 • Neil D. Patel1 • Ra-id Abdulla1 • John W. Bokowski1
Received: 24 January 2015 / Accepted: 4 March 2015 / Published online: 12 March 2015 Ó Springer Science+Business Media New York 2015
Abstract This report describes a rare case of Tetralogy of Fallot with associated left superior vena cava and coronary sinus atrial septal defect. The initial diagnosis was made by echocardiography. The patient underwent complete repair at 2 months of age. Her postoperative course was complicated by low cardiac output requiring ECMO. She was subsequently weaned off of ECMO and discharged home. She continues to do well on serial follow-up. Keywords Coronary sinus ASD Left SVC Tetralogy of Fallot Raghib Left superior vena cava (LSVC) is the most common form of anomalous systemic venous connection and is reported to occur in 2.1–4.3 % of patients with congenital cardiac lesions [1]. Raghib and his associates [5] in 1965 described a rare developmental complex consisting of an atrial septal defect (ASD), termination of a LSVC in the left atrium, and absence of the coronary sinus. Failure of the left atriovenous fold and left cardinal vein to participate in the formation of the postero-inferior portion of the atrial septum and coronary vein results in the ASD and absent coronary sinus [2]. Quaegebeur et al. [4] reported one case of tetralogy of Fallot (TOF) with associated Raghib’s complex in their review of unroofed coronary, and Jian et al. [3] had Electronic supplementary material The online version of this article (doi:10.1007/s00246-015-1137-1) contains supplementary material, which is available to authorized users. & Neil D. Patel [email protected] 1
4650 Sunset Blvd, Mailstop #34, Los Angeles, CA 90027, USA
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reported another case with associated dextrocardia. We report this rare case of Raghib’s complex with associated TOF from our experience. The patient was diagnosed postnatally after a murmur was auscultated, which prompted further evaluation. A transthoracic echocardiogram revealed a LSVC draining into the supero-anterior aspect of the left atrium into an unroofed coronary sinus (Fig. 1). There was atrial situs solitus with levocardia and normal drainage of the inferior vena cava into the right atrium (RA). There was a small right superior vena cava draining into the RA along with a large 10 mm coronary sinus atrial septal defect with no bridging vein between the superior vena cavae (Fig. 2). The LSVC passed anterior to the ascending aorta and left pulmonary artery. The ASD was postero-inferior to the fossa ovalis. The tricuspid and mitral valves were coplanar with no evidence of insufficiency. There was a large ventricular septal defect (VSD) with overriding of the aortic valve and predominantly left to right shunting (Fig. 3). The aortic valve was trileaflet with normal origin of the coronary arteries. The aortic arch was leftward. At 2 months of age, she underwent repair of the VSD, right ventricular outflow tract augmentation with a transannular patch and monocusp pulmonary valve, repair of the unroofed coronary sinus, and repair of the ASD. Intraoperatively, there was a small foramen of Morgagni hernia noted that was repaired. She was transferred to an outside hospital 1 week after surgery for ECMO secondary to low cardiac output. She was weaned to decannulation over 1 week and then discharged home the following week. Serial follow-ups in the clinic showed good biventricular systolic function with no residual shunts, mild but stable pulmonary insufficiency, and no pulmonary valve stenosis. She continues to thrive with decent weight gain.
Pediatr Cardiol (2015) 36:1100–1101
Fig. 1 Modified high parasternal short axis view (crab view): still image demonstrating LSVC draining directly into the LA, indicating an unroofed coronary sinus. LUPV (arrow) is seen draining into the LA in the vicinity of the LSVC
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Fig. 3 Subcostal right anterior oblique view: still image demonstrating narrow RVOT, large VSD, and anterior displacement of the aorta. RA right atrium, RV right ventricle, RVOT right ventricular outflow tract, VSD ventricular septal defect
References
Fig. 2 Subcostal long axis view: still image demonstrating the coronary sinus ASD (arrow). RA right atrium, LA left atrium
1. de Leval MR, Ritter DG, McGoon DC, Danielson GK (1975) Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc 50:599–610 2. Foster ED, Baeza OR, Farina MF, Shaher RM (1978) Atrial septal defect associated with drainage of left superior vena cava to left atrium and absence of the coronary sinus. J Thorac Cardiovasc Surg 76(5):718–720 3. Jian Z, Li J, Xiao Y (2010) Rare association of tetralogy of Fallot with partially unroofed coronary sinus and PLSVC: case report. Thorac Cardiovasc Surg 58(2):117–119 Epub 23 Mar 2010 4. Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr (1979) Surgical experience with unroofed coronary sinus. Ann Thorac Surg 27(5):418–425 5. Raghib G, Ruttenberg HD, Anderson RC et al (1965) Termination of left superior vena cava in left atrium, atrial septal defect and absence of coronary sinus: a developmental complex. Circulation 31:906
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IMAGES IN PEDIATRIC CARDIOLOGY
Tetralogy of Fallot with Left Superior Vena Cava and Coronary Sinus Atrial Septal Defect: A Rare Association Kiran K. Mallula1 • Neil D. Patel1 • Ra-id Abdulla1 • John W. Bokowski1
Received: 24 January 2015 / Accepted: 4 March 2015 / Published online: 12 March 2015 Ó Springer Science+Business Media New York 2015
Abstract This report describes a rare case of Tetralogy of Fallot with associated left superior vena cava and coronary sinus atrial septal defect. The initial diagnosis was made by echocardiography. The patient underwent complete repair at 2 months of age. Her postoperative course was complicated by low cardiac output requiring ECMO. She was subsequently weaned off of ECMO and discharged home. She continues to do well on serial follow-up. Keywords Coronary sinus ASD Left SVC Tetralogy of Fallot Raghib Left superior vena cava (LSVC) is the most common form of anomalous systemic venous connection and is reported to occur in 2.1–4.3 % of patients with congenital cardiac lesions [1]. Raghib and his associates [5] in 1965 described a rare developmental complex consisting of an atrial septal defect (ASD), termination of a LSVC in the left atrium, and absence of the coronary sinus. Failure of the left atriovenous fold and left cardinal vein to participate in the formation of the postero-inferior portion of the atrial septum and coronary vein results in the ASD and absent coronary sinus [2]. Quaegebeur et al. [4] reported one case of tetralogy of Fallot (TOF) with associated Raghib’s complex in their review of unroofed coronary, and Jian et al. [3] had Electronic supplementary material The online version of this article (doi:10.1007/s00246-015-1137-1) contains supplementary material, which is available to authorized users. & Neil D. Patel [email protected] 1
4650 Sunset Blvd, Mailstop #34, Los Angeles, CA 90027, USA
123
reported another case with associated dextrocardia. We report this rare case of Raghib’s complex with associated TOF from our experience. The patient was diagnosed postnatally after a murmur was auscultated, which prompted further evaluation. A transthoracic echocardiogram revealed a LSVC draining into the supero-anterior aspect of the left atrium into an unroofed coronary sinus (Fig. 1). There was atrial situs solitus with levocardia and normal drainage of the inferior vena cava into the right atrium (RA). There was a small right superior vena cava draining into the RA along with a large 10 mm coronary sinus atrial septal defect with no bridging vein between the superior vena cavae (Fig. 2). The LSVC passed anterior to the ascending aorta and left pulmonary artery. The ASD was postero-inferior to the fossa ovalis. The tricuspid and mitral valves were coplanar with no evidence of insufficiency. There was a large ventricular septal defect (VSD) with overriding of the aortic valve and predominantly left to right shunting (Fig. 3). The aortic valve was trileaflet with normal origin of the coronary arteries. The aortic arch was leftward. At 2 months of age, she underwent repair of the VSD, right ventricular outflow tract augmentation with a transannular patch and monocusp pulmonary valve, repair of the unroofed coronary sinus, and repair of the ASD. Intraoperatively, there was a small foramen of Morgagni hernia noted that was repaired. She was transferred to an outside hospital 1 week after surgery for ECMO secondary to low cardiac output. She was weaned to decannulation over 1 week and then discharged home the following week. Serial follow-ups in the clinic showed good biventricular systolic function with no residual shunts, mild but stable pulmonary insufficiency, and no pulmonary valve stenosis. She continues to thrive with decent weight gain.
Pediatr Cardiol (2015) 36:1100–1101
Fig. 1 Modified high parasternal short axis view (crab view): still image demonstrating LSVC draining directly into the LA, indicating an unroofed coronary sinus. LUPV (arrow) is seen draining into the LA in the vicinity of the LSVC
1101
Fig. 3 Subcostal right anterior oblique view: still image demonstrating narrow RVOT, large VSD, and anterior displacement of the aorta. RA right atrium, RV right ventricle, RVOT right ventricular outflow tract, VSD ventricular septal defect
References
Fig. 2 Subcostal long axis view: still image demonstrating the coronary sinus ASD (arrow). RA right atrium, LA left atrium
1. de Leval MR, Ritter DG, McGoon DC, Danielson GK (1975) Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc 50:599–610 2. Foster ED, Baeza OR, Farina MF, Shaher RM (1978) Atrial septal defect associated with drainage of left superior vena cava to left atrium and absence of the coronary sinus. J Thorac Cardiovasc Surg 76(5):718–720 3. Jian Z, Li J, Xiao Y (2010) Rare association of tetralogy of Fallot with partially unroofed coronary sinus and PLSVC: case report. Thorac Cardiovasc Surg 58(2):117–119 Epub 23 Mar 2010 4. Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr (1979) Surgical experience with unroofed coronary sinus. Ann Thorac Surg 27(5):418–425 5. Raghib G, Ruttenberg HD, Anderson RC et al (1965) Termination of left superior vena cava in left atrium, atrial septal defect and absence of coronary sinus: a developmental complex. Circulation 31:906
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