Novel diagnostic procedure
CASE REPORT
Persistent left superior vena cava in association with sinus venosus defect type of atrial septal defect and partial pulmonary venous return on 64-MDCT Bansal Disha,1 Koteshwara Prakashini,1 Ranjan K Shetty2 1
Department of Radiology and Imaging, Kasturba Medical College, Manipal, Karnataka, India 2 Department of Cardiology, Kasturba Medical College, Manipal, Karnataka, India Correspondence to Dr Koteshwara Prakashini, [email protected] Accepted 23 April 2014
SUMMARY The most common venous abnormality of the thorax is persistent left superior vena cava (PLSVC), incidence being less than 0.5%. However, with congenital heart disease, it is about 6.1%. When the coronary sinus is dilated always search for PLSVC. The coronary sinus may communicate with the left atrium. This is known as an unroofed coronary sinus (UCS) and preoperatively documenting it is important. Of all the congenital cardiac anomalies, the sinus venosus defect (SVD) type of atrial septal defect (ASD) is most commonly associated with PLSVC and accounts for 4–11% of all ASDs. Multidetector CT can easily show all these abnormalities along with haemodynamics. On transoesophageal echocardiography it is difficult to characterise SVD and visualise a coronary sinus because of a limited window, contrast resolution and poor patient compliance. The complex of UCS and PLSVC is one such abnormality and its treatment requires careful assessment of other concomitant cardiac abnormalities to prevent post-treatment haemodynamic complications.
BACKGROUND Few difficulties faced with transthoracic and transoesophageal echocardiography can be easily solved on cardiac CT and can be presented in threedimensional (3D) view. Partial anomalous pulmonary venous connection (PAPVC) which will not show on ECHO can be very well demonstrated on contrast-enhanced CT; it is also an indirect sign of sinus venosus defect (SVD) type of atrial septal defect (ASD). An SVD type of defect can be misdiagnosed on transthoracic ECHO as a high septum secundum type of defect due to a ‘false atrial septum’.1–5
coronary sinus due to persistent left superior vena cava (PLSVC). Retrospective ECG-gated cardiac CT was performed on a Philips Brilliance 64 slice multidetector CT (MDCT) scanner. The findings on CT were cardiomegaly with an SVD type of ASD (figure 1). The right upper and middle lobar pulmonary veins were seen draining aberrantly into the right superior vena cava (SVC) suggestive of PAPVC (figure 2 The left-sided superior vena cava was seen draining into the coronary sinus which was grossly dilated. Pulmonary arterial hypertension with dilation of the inferior vena cava (IVC) and early opacification of hepatic veins was noted. Pericardial effusion was present. Our patient was managed conservatively.
INVESTIGATIONS ▸ ECG showed features of AF with CVR. ▸ Transthoracic and transoesophageal ECHO cardiography with contrast was suggestive of high ostium secundum ASD from left to right shunt and a markedly dilated coronary sinus due to PLSVA. ▸ Retrospective ECG-gated cardiac CT was performed on a Philips Brilliance 64 slice MDCT scanner using a bolus tracking technique. Nonionic iodinated contrast (Ultravist 370 mg/mL,
CASE PRESENTATION
To cite: Disha B, Prakashini K, Shetty RK. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/ bcr-2013-202999
A 66-year-old man, recently diagnosed to have congenital heart disease with chronic obstructive pulmonary disease (COPD) and a history of stroke, presented to the emergency room with a history of dyspnoea on exertion with chest pain and palpitations over the past 1 month. Physical examination revealed an irregular pulse. Basic laboratory investigations were unremarkable. ECG showed atrial fibrillation (AF) with controlled ventricular response (CVR). ECHO was suggestive of high ostium secundum ASD from left to right shunt, dilated right atrium and ventricle, severe tricuspid regurgitation, moderate pulmonary arterial hypertension, mild mitral regurgitation and a markedly dilated
Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
Figure 1 Sinus venosus defect type of atrial septal defect seen as superior vena cava (star) opening into the right and left atrium forming a single chamber (triangle) with absent inter-atrial septum (RA, right atrium; LA, left atrium). 1
Novel diagnostic procedure
Figure 2 Maximum intensity projection (MIP) reformatted image showing upper lobe pulmonary veins (arrow) draining to superior vena cava (star) instead to right atrium suggestive of partial anomalous pulmonary venous connection. 80 mL) injection was given at the rate of 5 mL/s using a 12-gauge cannula followed by 40 mL of saline chaser at 5 mL/s. Postprocessing such as curved reformation and maximum intensity projection images were prepared to evaluate and demonstrate anomalies. The findings on CT were cardiomegaly with dilated right atrium and ventricle. A defect measuring 2.6 cm was seen in the high inter-atrial septum with the opening of the right superior vena cava into both atria forming a single chamber suggestive of an SVD type of ASD (figure 1). This defect was leading to communication between the right atrium, left atrium and the right superior vena cava. The right upper and middle lobar pulmonary veins were seen draining aberrantly into the right SVC suggestive of PAPVC (figure 2) with normal left pulmonary veins and right lower lobar vein draining to the left atrium. Left-sided persistent superior vena cava was seen draining into the coronary sinus which was grossly dilated measuring 4.8 cm. A small but patent intercommunicating left brachiocephalic vein and right SVC of normal calibre were seen (figure 3A–C). Pulmonary arterial hypertension was present with dilation of IVC and early opacification of hepatic veins. Pericardial effusion was present. ▸ Chest radiograph showed mild cardiomegaly. ▸ Catheter angiography and surgery was refused by the patient hence Qp/Qs ratio could not be assessed.
TREATMENT The patient was conservatively managed. Although the patient was advised to undergo catheter angiography followed by surgery, confounding comorbid conditions such as COPD and old age compelled the patient to refuse surgical treatment.
OUTCOME AND FOLLOW-UP The patient was conservatively managed and stable on medication. 2
Figure 3 (A) Axial maximum intensity projection (MIP) image showing the right and left superior vena cava (SVC; star) and its relationship with the left main bronchus. (B) Persistent left superior vena cava (PLSVC) is joining the coronary sinus (CS) which is grossly dilated in calibre and draining into right atrium. (C) PLSVC showed in coronal MIP image.
DISCUSSION In the embryo, systemic venous drainage is by a symmetrical system of cardinal veins. A portion of the right anterior cardinal vein and corresponding common cardinal vein forms the SVC on the right side. The left anterior cardinal vein regresses to Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
Novel diagnostic procedure
Figure 4 Echocardiography image showing interatrial septal defect, however presence of a small septum-like structure at the superior part made diagnoses os primum type of atrial septal defect. form a ligament of the left vena cava whereas the left common cardinal vein and left horn of the sinus venosus persist to form the coronary sinus. The anastomosis between the two anterior cardinal veins develops into the left brachiocephalic vein.6 In some cases (0.5%), the left anterior cardinal and left common cardinal veins can persist and develop into PLSVC, which is an asymptomatic anomaly. This PLSVC drains into the coronary sinus and finally into the right atrium. Most cases of PLSVC present as a part of a double SVC and the singular left SVC is a rare occurrence.7 The PLSVC and PAPVC of the left upper lobe can be confused due to similar appearance at the level of the aortic arch. However, both can be easily differentiated on CT as there is only one vessel, the left superior pulmonary vein which is located ventral to the left main bronchus. In PLSVC there are two vessels: the normal left superior pulmonary vein and the left SVC, whereas in a case of PAPVC of the left upper lobe there are no vessels. Also, by tracing the drainage of the pulmonary veins into the SVC or brachiocephalic veins, a diagnosis of PAPVC can be performed. Other clues pointing to a diagnosis of PLSVC are focal superior mediastinal widening to the left of the aortic knob seen on a radiograph and dense opacification of enlarged coronary sinus on CTwhen contrast is injected into the left arm. In around 10% of cases there can be a communication of PLSVC with the left atrium forming a left to right shunt.1 Rarely there can be a communication between the left atrium and the coronary sinus which is known as an ‘unroofed coronary sinus,’ a rare type of ASD. The importance of unroofing of the coronary sinus syndrome is that a right to left shunt can lead to cerebral embolism or a brain abscess.7 8 Also, before certain operations or placement of a cavopulmonary shunt, it is important to know the haemodynamics like right to left shunt which is associated with desaturation of oxygenated blood. Whenever PLSVC and ASD exist together, we need to rule out unroofing of coronary sinus preoperatively because this also needs to be closed in order to prevent a persistence of cyanosis.2 PLSVC, per se, is a benign condition but important for pacemaker implantation and canulation in cardiac surgery.9 Along with PLSVC the patient also had a SVD type of ASD with its common association, PAPVC. Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
SVD develops due to absence or maldevelopment of atrial infolding that normally separates the two atria in the region between SVC and right pulmonary veins. This also explains the common association of SVD with right PAPVC as evidenced by our case. Transthoracic ultrasound has low sensitivity; however, transoesophageal echocardiography should be the ideal method. 3 4 For flow dynamics, ultrasound contrast material has to be used and needs experienced hands.5 Probe position in the high oesophageal level is discomforting to the patient, which limits in obtaining a plane at the SVC-atrium junction. At a slightly lower level, a thin echogenic septum can be seen, the so-called false atrial septum which can be confused with an interatrial septum. According to Nicola et al,5 it probably represents a prominent Eustachian Valve or it could be part of the Chiari network, or it could simply be generated by the right atrial extreme distortion and enlargement. This can lead to false categorisation of ASD on ECHO (figure 4). Owing to nonspecific clinical signs and symptoms, an unroofed coronary sinus is difficult to diagnose clinically. MRI and MDCT demonstrate these defects very well because of multiplanar capability. MRI is non-invasive and without injection of contrast medium, using spin echo and cine MRI, the walls of cardiovascular structure and deeper structures of the heart can be visualised with excellent contrast resolution.3 Flow information can be made out using phase-contrast velocity encoded cine technique. According to the study by Kim et al,10 CT and MRI facilitated definite diagnosis by direct visualisation of the defects and haemodynamics in whom an enlarged coronary sinus was found on echocardiography. MDCT scores over MRI in terms of better image resolution, capability of curved planar reconstruction and less time consumption, and is hence helpful in uncooperative patients.4 Also by injecting diluted contrast medium in the left arm and visualisation of contrast in the left atrium, a diagnosis of unroofed coronary sinus with right-to-left shunt can be easily made. Although in our case there was no unroofing of the coronary sinus. MRI and MDCT help in preoperative surgical planning by providing detailed 3D anatomy of the heart and demonstrating other associated anomalies like PAPVC which may not be demonstrable on ECHO. Our case report highlights this and also the importance of coexisting lesions which should be carefully looked for.
Learning points ▸ Our case report highlights that multiple anomalies can coexist; we have tried discussing a brief embryological background of each of these. ▸ Whenever there is a dilated coronary sinus look for persistent left superior vena cava . At the same time also look for unroofing of the coronary sinus, a rare type of atrial septal defect (ASD) which needs to be closed. Use of CT is one non-invasive modality. ▸ Partial anomalous pulmonary venous connection is indirect evidence of a sinus venosus defect type of ASD which is easily appreciated on CT. ▸ Three-dimensional images act as a roadmap and help in guiding surgeons. Contributors BD wrote the case discussion part. KP performed CT scan and reported the findings and added case details. EKS performed ECHO and clinical case report collected and prepared the write up. Competing interests None. Patient consent Obtained. 3
Novel diagnostic procedure Provenance and peer review Not commissioned; externally peer reviewed.
6
REFERENCES
7
1 2 3 4 5
Kula S, Cevik A, Sanli C, et al. Persistent left superior vena cava: experience of a tertiary health-care center. Pediatr Int 2011;53:1066–9. Quaegebeur J, Kirklin JW, Pacifico AD, et al. Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418–25. Hahm JK, Park YW, Lee JK, et al. Magnetic resonance imaging of unroofed coronary sinus: three cases. Pediatr Cardiol 2000;21:382–7. Brancaccio G, Miraldi F, Ventriglia F, et al. Multidetector-row helical computed tomography imaging of unroofed coronary sinus. Int J Cardiol 2003;91:251–3. Nicola G, Graziano M, Poeta ML. Case report Anatomy of a wrong diagnosis: false Sinus Venosus Atrial Septal Defect. Cardiovasc Ultrasound 2003;1:15.
8
9
10
Sadler TW. Langman’s medical embryology. 7th edn. Baltimore, MD: Williams and Wilkins, 1995:221–3. Ootaki Y, Yamaguchi M, Yoshimura N, et al. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 2003;126:1655–6. Troost E, Gewillig M, Budts W. Percutaneous closure of a persistent left superior vena cava connected to the left atrium. Int J Cardiol 2006;106: 365–6. Rodríguez-Fernández JA, Almazán-Soo A. A Pacemaker lead implant via the persistent left superior vena cava. Arch Cardiol Mex 2005;75(Suppl 3): S3-106–12. Kim H, Choe YH, Park SW, et al. Partially unroofed coronary sinus: MDCT and MRI findings. AJR 2010;195:W331–6.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
4
Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
CASE REPORT
Persistent left superior vena cava in association with sinus venosus defect type of atrial septal defect and partial pulmonary venous return on 64-MDCT Bansal Disha,1 Koteshwara Prakashini,1 Ranjan K Shetty2 1
Department of Radiology and Imaging, Kasturba Medical College, Manipal, Karnataka, India 2 Department of Cardiology, Kasturba Medical College, Manipal, Karnataka, India Correspondence to Dr Koteshwara Prakashini, [email protected] Accepted 23 April 2014
SUMMARY The most common venous abnormality of the thorax is persistent left superior vena cava (PLSVC), incidence being less than 0.5%. However, with congenital heart disease, it is about 6.1%. When the coronary sinus is dilated always search for PLSVC. The coronary sinus may communicate with the left atrium. This is known as an unroofed coronary sinus (UCS) and preoperatively documenting it is important. Of all the congenital cardiac anomalies, the sinus venosus defect (SVD) type of atrial septal defect (ASD) is most commonly associated with PLSVC and accounts for 4–11% of all ASDs. Multidetector CT can easily show all these abnormalities along with haemodynamics. On transoesophageal echocardiography it is difficult to characterise SVD and visualise a coronary sinus because of a limited window, contrast resolution and poor patient compliance. The complex of UCS and PLSVC is one such abnormality and its treatment requires careful assessment of other concomitant cardiac abnormalities to prevent post-treatment haemodynamic complications.
BACKGROUND Few difficulties faced with transthoracic and transoesophageal echocardiography can be easily solved on cardiac CT and can be presented in threedimensional (3D) view. Partial anomalous pulmonary venous connection (PAPVC) which will not show on ECHO can be very well demonstrated on contrast-enhanced CT; it is also an indirect sign of sinus venosus defect (SVD) type of atrial septal defect (ASD). An SVD type of defect can be misdiagnosed on transthoracic ECHO as a high septum secundum type of defect due to a ‘false atrial septum’.1–5
coronary sinus due to persistent left superior vena cava (PLSVC). Retrospective ECG-gated cardiac CT was performed on a Philips Brilliance 64 slice multidetector CT (MDCT) scanner. The findings on CT were cardiomegaly with an SVD type of ASD (figure 1). The right upper and middle lobar pulmonary veins were seen draining aberrantly into the right superior vena cava (SVC) suggestive of PAPVC (figure 2 The left-sided superior vena cava was seen draining into the coronary sinus which was grossly dilated. Pulmonary arterial hypertension with dilation of the inferior vena cava (IVC) and early opacification of hepatic veins was noted. Pericardial effusion was present. Our patient was managed conservatively.
INVESTIGATIONS ▸ ECG showed features of AF with CVR. ▸ Transthoracic and transoesophageal ECHO cardiography with contrast was suggestive of high ostium secundum ASD from left to right shunt and a markedly dilated coronary sinus due to PLSVA. ▸ Retrospective ECG-gated cardiac CT was performed on a Philips Brilliance 64 slice MDCT scanner using a bolus tracking technique. Nonionic iodinated contrast (Ultravist 370 mg/mL,
CASE PRESENTATION
To cite: Disha B, Prakashini K, Shetty RK. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/ bcr-2013-202999
A 66-year-old man, recently diagnosed to have congenital heart disease with chronic obstructive pulmonary disease (COPD) and a history of stroke, presented to the emergency room with a history of dyspnoea on exertion with chest pain and palpitations over the past 1 month. Physical examination revealed an irregular pulse. Basic laboratory investigations were unremarkable. ECG showed atrial fibrillation (AF) with controlled ventricular response (CVR). ECHO was suggestive of high ostium secundum ASD from left to right shunt, dilated right atrium and ventricle, severe tricuspid regurgitation, moderate pulmonary arterial hypertension, mild mitral regurgitation and a markedly dilated
Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
Figure 1 Sinus venosus defect type of atrial septal defect seen as superior vena cava (star) opening into the right and left atrium forming a single chamber (triangle) with absent inter-atrial septum (RA, right atrium; LA, left atrium). 1
Novel diagnostic procedure
Figure 2 Maximum intensity projection (MIP) reformatted image showing upper lobe pulmonary veins (arrow) draining to superior vena cava (star) instead to right atrium suggestive of partial anomalous pulmonary venous connection. 80 mL) injection was given at the rate of 5 mL/s using a 12-gauge cannula followed by 40 mL of saline chaser at 5 mL/s. Postprocessing such as curved reformation and maximum intensity projection images were prepared to evaluate and demonstrate anomalies. The findings on CT were cardiomegaly with dilated right atrium and ventricle. A defect measuring 2.6 cm was seen in the high inter-atrial septum with the opening of the right superior vena cava into both atria forming a single chamber suggestive of an SVD type of ASD (figure 1). This defect was leading to communication between the right atrium, left atrium and the right superior vena cava. The right upper and middle lobar pulmonary veins were seen draining aberrantly into the right SVC suggestive of PAPVC (figure 2) with normal left pulmonary veins and right lower lobar vein draining to the left atrium. Left-sided persistent superior vena cava was seen draining into the coronary sinus which was grossly dilated measuring 4.8 cm. A small but patent intercommunicating left brachiocephalic vein and right SVC of normal calibre were seen (figure 3A–C). Pulmonary arterial hypertension was present with dilation of IVC and early opacification of hepatic veins. Pericardial effusion was present. ▸ Chest radiograph showed mild cardiomegaly. ▸ Catheter angiography and surgery was refused by the patient hence Qp/Qs ratio could not be assessed.
TREATMENT The patient was conservatively managed. Although the patient was advised to undergo catheter angiography followed by surgery, confounding comorbid conditions such as COPD and old age compelled the patient to refuse surgical treatment.
OUTCOME AND FOLLOW-UP The patient was conservatively managed and stable on medication. 2
Figure 3 (A) Axial maximum intensity projection (MIP) image showing the right and left superior vena cava (SVC; star) and its relationship with the left main bronchus. (B) Persistent left superior vena cava (PLSVC) is joining the coronary sinus (CS) which is grossly dilated in calibre and draining into right atrium. (C) PLSVC showed in coronal MIP image.
DISCUSSION In the embryo, systemic venous drainage is by a symmetrical system of cardinal veins. A portion of the right anterior cardinal vein and corresponding common cardinal vein forms the SVC on the right side. The left anterior cardinal vein regresses to Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
Novel diagnostic procedure
Figure 4 Echocardiography image showing interatrial septal defect, however presence of a small septum-like structure at the superior part made diagnoses os primum type of atrial septal defect. form a ligament of the left vena cava whereas the left common cardinal vein and left horn of the sinus venosus persist to form the coronary sinus. The anastomosis between the two anterior cardinal veins develops into the left brachiocephalic vein.6 In some cases (0.5%), the left anterior cardinal and left common cardinal veins can persist and develop into PLSVC, which is an asymptomatic anomaly. This PLSVC drains into the coronary sinus and finally into the right atrium. Most cases of PLSVC present as a part of a double SVC and the singular left SVC is a rare occurrence.7 The PLSVC and PAPVC of the left upper lobe can be confused due to similar appearance at the level of the aortic arch. However, both can be easily differentiated on CT as there is only one vessel, the left superior pulmonary vein which is located ventral to the left main bronchus. In PLSVC there are two vessels: the normal left superior pulmonary vein and the left SVC, whereas in a case of PAPVC of the left upper lobe there are no vessels. Also, by tracing the drainage of the pulmonary veins into the SVC or brachiocephalic veins, a diagnosis of PAPVC can be performed. Other clues pointing to a diagnosis of PLSVC are focal superior mediastinal widening to the left of the aortic knob seen on a radiograph and dense opacification of enlarged coronary sinus on CTwhen contrast is injected into the left arm. In around 10% of cases there can be a communication of PLSVC with the left atrium forming a left to right shunt.1 Rarely there can be a communication between the left atrium and the coronary sinus which is known as an ‘unroofed coronary sinus,’ a rare type of ASD. The importance of unroofing of the coronary sinus syndrome is that a right to left shunt can lead to cerebral embolism or a brain abscess.7 8 Also, before certain operations or placement of a cavopulmonary shunt, it is important to know the haemodynamics like right to left shunt which is associated with desaturation of oxygenated blood. Whenever PLSVC and ASD exist together, we need to rule out unroofing of coronary sinus preoperatively because this also needs to be closed in order to prevent a persistence of cyanosis.2 PLSVC, per se, is a benign condition but important for pacemaker implantation and canulation in cardiac surgery.9 Along with PLSVC the patient also had a SVD type of ASD with its common association, PAPVC. Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
SVD develops due to absence or maldevelopment of atrial infolding that normally separates the two atria in the region between SVC and right pulmonary veins. This also explains the common association of SVD with right PAPVC as evidenced by our case. Transthoracic ultrasound has low sensitivity; however, transoesophageal echocardiography should be the ideal method. 3 4 For flow dynamics, ultrasound contrast material has to be used and needs experienced hands.5 Probe position in the high oesophageal level is discomforting to the patient, which limits in obtaining a plane at the SVC-atrium junction. At a slightly lower level, a thin echogenic septum can be seen, the so-called false atrial septum which can be confused with an interatrial septum. According to Nicola et al,5 it probably represents a prominent Eustachian Valve or it could be part of the Chiari network, or it could simply be generated by the right atrial extreme distortion and enlargement. This can lead to false categorisation of ASD on ECHO (figure 4). Owing to nonspecific clinical signs and symptoms, an unroofed coronary sinus is difficult to diagnose clinically. MRI and MDCT demonstrate these defects very well because of multiplanar capability. MRI is non-invasive and without injection of contrast medium, using spin echo and cine MRI, the walls of cardiovascular structure and deeper structures of the heart can be visualised with excellent contrast resolution.3 Flow information can be made out using phase-contrast velocity encoded cine technique. According to the study by Kim et al,10 CT and MRI facilitated definite diagnosis by direct visualisation of the defects and haemodynamics in whom an enlarged coronary sinus was found on echocardiography. MDCT scores over MRI in terms of better image resolution, capability of curved planar reconstruction and less time consumption, and is hence helpful in uncooperative patients.4 Also by injecting diluted contrast medium in the left arm and visualisation of contrast in the left atrium, a diagnosis of unroofed coronary sinus with right-to-left shunt can be easily made. Although in our case there was no unroofing of the coronary sinus. MRI and MDCT help in preoperative surgical planning by providing detailed 3D anatomy of the heart and demonstrating other associated anomalies like PAPVC which may not be demonstrable on ECHO. Our case report highlights this and also the importance of coexisting lesions which should be carefully looked for.
Learning points ▸ Our case report highlights that multiple anomalies can coexist; we have tried discussing a brief embryological background of each of these. ▸ Whenever there is a dilated coronary sinus look for persistent left superior vena cava . At the same time also look for unroofing of the coronary sinus, a rare type of atrial septal defect (ASD) which needs to be closed. Use of CT is one non-invasive modality. ▸ Partial anomalous pulmonary venous connection is indirect evidence of a sinus venosus defect type of ASD which is easily appreciated on CT. ▸ Three-dimensional images act as a roadmap and help in guiding surgeons. Contributors BD wrote the case discussion part. KP performed CT scan and reported the findings and added case details. EKS performed ECHO and clinical case report collected and prepared the write up. Competing interests None. Patient consent Obtained. 3
Novel diagnostic procedure Provenance and peer review Not commissioned; externally peer reviewed.
6
REFERENCES
7
1 2 3 4 5
Kula S, Cevik A, Sanli C, et al. Persistent left superior vena cava: experience of a tertiary health-care center. Pediatr Int 2011;53:1066–9. Quaegebeur J, Kirklin JW, Pacifico AD, et al. Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418–25. Hahm JK, Park YW, Lee JK, et al. Magnetic resonance imaging of unroofed coronary sinus: three cases. Pediatr Cardiol 2000;21:382–7. Brancaccio G, Miraldi F, Ventriglia F, et al. Multidetector-row helical computed tomography imaging of unroofed coronary sinus. Int J Cardiol 2003;91:251–3. Nicola G, Graziano M, Poeta ML. Case report Anatomy of a wrong diagnosis: false Sinus Venosus Atrial Septal Defect. Cardiovasc Ultrasound 2003;1:15.
8
9
10
Sadler TW. Langman’s medical embryology. 7th edn. Baltimore, MD: Williams and Wilkins, 1995:221–3. Ootaki Y, Yamaguchi M, Yoshimura N, et al. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 2003;126:1655–6. Troost E, Gewillig M, Budts W. Percutaneous closure of a persistent left superior vena cava connected to the left atrium. Int J Cardiol 2006;106: 365–6. Rodríguez-Fernández JA, Almazán-Soo A. A Pacemaker lead implant via the persistent left superior vena cava. Arch Cardiol Mex 2005;75(Suppl 3): S3-106–12. Kim H, Choe YH, Park SW, et al. Partially unroofed coronary sinus: MDCT and MRI findings. AJR 2010;195:W331–6.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
4
Disha B, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-202999
