© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12863
Echocardiography
Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment Wojciech Szychta, M.D., Ph.D.,* Gheorghe Cerin, M.D., Ph.D., F.E.S.C.,† Bogdan Adrian Popa, M.D.,† Armienti Felice, M.D.,‡ Guido Lanzillo, M.D., Ph.D.,§ Marco Diena, M.D.,§ and Grzegorz Opolski, M.D., Ph.D., F.E.S.C.* *1st Chair and Department of Cardiology, Medical University of Warsaw, Warsaw, Poland; †Cardiology Division in Cardiac Surgery Dept, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy; ‡Department of Radiology, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy; and §Departments of Cardiac Surgery, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy
Sinus venosus atrial septal defect (SV-ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV-ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio-CT was finally conclusive. This diagnostic approach allowed the surgical planning. (Echocardiography 2015;32:1040–1043) Key words: septal defects, pulmonary arteries/veins, pulmonary vascular resistance/hypertension, echocardiography, Computed tomography Both sinus venosus atrial septal defect (SVASD) and partial anomalous pulmonary vein connection (PAPVC) can cause pulmonary hypertension (PHT), however, the diseases are rare and thus they can be overlooked in diagnosis. Moreover, 87% of patients with SV-ASD have also PAPVC.1 The intracardiac shunt leads to volume overload, right cavities dilation, and PHT.2 PAPVC is a defect, which should be diagnosed by echocardiography. However, having problem with setting such diagnosis prompted us to conduct an analysis of this case. Case Report: A 43-year-old man was referred for a second echocardiographic opinion, having a previous diagnosis of mild PHT (pulmonary artery systolic pressure [PAP] 40 mmHg). Eight years previously, he complained on atypical chest pain and mild right ventricle (RV) dilatation was found by transthoracic echocardiography (TTE). Having systolic murmur in Erbs’ point, widely split, fixed second heart sound, and incomplete right bundle branch block, the clinical suspicion of ASD was set but previous TTE was not conclusive. Address for correspondence and reprint requests: Wojciech Szychta, M.D., Ph.D., Banacha 1A Street, 01-049 Warsaw, Poland. Fax: 0048 22 599-19-57; E-mail: [email protected]
1040
TTE confirmed dilated (40 mm) RV in parasternal long/short axis, moderate tricuspid regurgitation, PAP 40 mmHg, moderate dilated (26 mm) pulmonary artery, however, interatrial septum (IAS) appeared to be uninterrupted in four-chamber and substernal view (Fig. 1). The assessment of pulmonary shunt was evaluated by pulmonary–systemic flow ratio (Qp/Qs = 1.5). Transesophageal echocardiography (TEE) was performed, having high suspicion for ASD. The exam confirmed the high position of ASD, and showed the abnormal outflow track of superior vena cava (SVC) into the right atrium (RA), with malposition of its inflow, which “overrode” the roof of the atria. ASD had diameter of at least 25 mm, but the presence and the drainage of right pulmonary veins (RPV) was not documented. On the left side, only a large common trunk for the pulmonary veins was shown (Figs. 2 and 3). Considering the eccentric position and the dimension of ASD, as well as the overriding SVC, the amount of the shunt and the value of PAP, the case was proposed and discussed for surgical treatment and not for percutaneous closure. A computed tomography angiography (angio-CT) was necessary to confirm the SV-ASD, which was 3 9 3 cm in diameter, with “high-riding” SVC draining both atrial cavities. The coronary arteries were normal. Multi slice angio-CT showed also four pulmonary veins (three of them
Atypical PAPVC With SV-ASD
Figure 1. Transthoracic echocardiography view showing right ventricle (RV) dilatation in parasternal long-axis view.
Figure 3. Transesophageal echocardiography showing color Doppler 72° view: left pulmonary vein trunk.
under observation in Cardiology Outpatient Clinic.
Figure 2. Transesophageal echocardiography showing color Doppler 129° view: sinus venosus atrial septal defect (SV-ASD) with shunt and “high-riding” superior vena cava (SVC).
draining toward the RA and a large common trunk toward the left side). Right upper and middle pulmonary lobe veins were draining into the SVC and the lower pulmonary lobe vein was draining into the RA (Figs. 4 and 5). The patient underwent surgical treatment by reconstruction of IAS with autologous pericardial patch, reconstruction of SVC ostium and transposition of RPV to the left atrium (LA) (Fig. 6). Intraoperative TEE confirmed good surgical result. TTE performed before discharge revealed regression of the RV dimension (34 mm), PAP 27+5 mmHg, and the IAS was without leakage. The patient had an uneventful recovery and was discharged from hospital on the 7th postoperative day. After 1-year follow-up, the patient had no symptoms of heart failure (NYHA class 1) and his exercise capacity is normal. In ECG was found sinus rhythm with incomplete right bundle branch block. TTE confirmed reduction of right cavities and normal pulmonary pressure (27 mmHg). The patient was advised to remain
Comment: We present a rare case of atypical anatomical variation of PAPVC, which to the best of our belief, has never been described before. Until now, were described seven variations of PAPVC, anomaly that can be found in 0.4–0.7% of patients. Most commonly met are as follows: (1) right upper and middle lobe pulmonary vein (PV) to SVC, while the second one to (2) RA. Less commonly met are (3) right PVs draining to inferior vena cava (IVC), (4) right lower PV to IVC, (5) left upper PV to innominate vein, (6) left upper or lower PV(s) to coronary sinus, and (7) finally left lower PV to RA or IVC. In those cases, the right lower lobe PV usually drains to LA.1 The case described showed the combination of two variations: number 1 and 7. Echocardiography is the main diagnostic tool for SV-ASD. However, angio-CT is an alternative diagnostic approach in these patients if TEE is insufficient.3 Some authors described positive diagnosis set in TTE by use of agitated saline solution,4 but TEE is still necessary to confirm both position and dimension of the ASD.3 Sensitivity of TEE to detect the SV-ASD varies between 0 and 100%, however, specificity is 100%. Meanwhile, sensitivity of TTE is 9%, and its specificity is 100%.5,6 On the other hand, angio-CT has 100% sensitivity for diagnosing PAPVR in patients with SV-ASD, 100% specificity, 100% positive predictive value, and 100% negative predictive value,7 what shows importance of this examination. Patients in follow-up after correction of ASD achieve clinical improvement in 77% of cases, however, deterioration is observed in 16% of cases.8 We believe that the patient presented, has good prognosis as his clinical condition improved, his pulmonary pressure decreased and 1041
Szychta, et al.
Figure 4. Three-dimensional volume rendering: partial anomalous pulmonary vein connection (PAPVC) of right upper and middle pulmonary veins to superior vena cava (SVC).
Figure 6. Surgeons view demonstrating right upper and middle pulmonary veins (blue arrow) and sinus venosus atrial septal defect (SV-ASD) (yellow arrow) *abnormal pulmonary venous drainage.
patients are rare they should remain under specialist care for life. This case highlights the weakness of TTE in excluding SV-ASD with PAPVC. In our opinion, proper surgical planning can be achieved with TEE and completed either by angio-CT or MRI.3,10 References
Figure 5. Angio-CT of heart: sinus venosus atrial septal defect (SV-ASD) and superior vena cava (SVC) delivering blood to right atrium (RA) and left atrium (LA).
he had no clinical symptomatic dysrhythmia. However, 6% of such patients have sinus node dysfunction or indications to pacemaker implantation, moreover, 14% of them have atrial fibrillation.8 Moreover, the risk for having arrhythmia is increased by technique of operation, as the surgeon had to perform extended incision nearby cavoatrial junction (Fig. 6), which increases atrial dysrhythmias.9 Although, complications in those 1042
1. Gatzoulis M, Webb G, Daubeney P: Diagnosis and management of adult congenital heart disease (Second Edition). In Ellis A (ed): Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome. London: Churchill Livingstone, 2011, pp. 216–221. 2. Babb J, McGlynn T, Pierce W, et al: Isolated partial anomalous venous connection: A congenital defect with late and serious complications. Ann Thorac Surg 1981;31:540–541. 3. ESC Task Force Guideline: ESC Guidelines for the management of grown-up congenital heart disease. Eur Heart J 2010;31:2915–2957. 4. Dorosz J, Fonseca B, Kay J, et al: A sinus venosus atrial septal defect is diagnosed by echocardiography with an unusual bubble study. Echocardiography 2013;30:E182– E183. 5. Lengyel M, Temesvari A, Villanyi J, et al: The role of transesophageal echocardiography in atrial septal defect detected in adults. Orv Hetil 1991;132:2467– 2471. € gge A, et al: Value of trans6. Hausmann D, Daniel WG, Mu esophageal color Doppler echocardiography for detection of different types of atrial septal defect in adults. J Am Soc Echocardiogr 1992;5:481–488. 7. Amat F, Le Bret E, Sigal-Cinqualbre A, et al: Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact Cardiovasc Thorac Surg 2011;12:179–182.
Atypical PAPVC With SV-ASD
8. Attenhofer Jost CH, Connolly HM, Danielson GK, et al: Sinus venosus atrial septal defect: Long-term postoperative outcome for 115 patients. Circulation 2005;112: 1953–1958. 9. Buz S1, Alexi-Meskishvili V, Villavicencio-Lorini F, et al: Analysis of arrhythmias after correction of partial anoma-
lous pulmonary venous connection. Ann Thorac Surg 2009;87:580–583. 10. Prompona M, Muehling O, Naebauer M, et al: MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects. Int J Cardiovasc Imaging 2011;27:403–412.
1043
Echocardiography
Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment Wojciech Szychta, M.D., Ph.D.,* Gheorghe Cerin, M.D., Ph.D., F.E.S.C.,† Bogdan Adrian Popa, M.D.,† Armienti Felice, M.D.,‡ Guido Lanzillo, M.D., Ph.D.,§ Marco Diena, M.D.,§ and Grzegorz Opolski, M.D., Ph.D., F.E.S.C.* *1st Chair and Department of Cardiology, Medical University of Warsaw, Warsaw, Poland; †Cardiology Division in Cardiac Surgery Dept, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy; ‡Department of Radiology, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy; and §Departments of Cardiac Surgery, Policlinic of Monza, San Gaudenzio Clinic, Novara, Italy
Sinus venosus atrial septal defect (SV-ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV-ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio-CT was finally conclusive. This diagnostic approach allowed the surgical planning. (Echocardiography 2015;32:1040–1043) Key words: septal defects, pulmonary arteries/veins, pulmonary vascular resistance/hypertension, echocardiography, Computed tomography Both sinus venosus atrial septal defect (SVASD) and partial anomalous pulmonary vein connection (PAPVC) can cause pulmonary hypertension (PHT), however, the diseases are rare and thus they can be overlooked in diagnosis. Moreover, 87% of patients with SV-ASD have also PAPVC.1 The intracardiac shunt leads to volume overload, right cavities dilation, and PHT.2 PAPVC is a defect, which should be diagnosed by echocardiography. However, having problem with setting such diagnosis prompted us to conduct an analysis of this case. Case Report: A 43-year-old man was referred for a second echocardiographic opinion, having a previous diagnosis of mild PHT (pulmonary artery systolic pressure [PAP] 40 mmHg). Eight years previously, he complained on atypical chest pain and mild right ventricle (RV) dilatation was found by transthoracic echocardiography (TTE). Having systolic murmur in Erbs’ point, widely split, fixed second heart sound, and incomplete right bundle branch block, the clinical suspicion of ASD was set but previous TTE was not conclusive. Address for correspondence and reprint requests: Wojciech Szychta, M.D., Ph.D., Banacha 1A Street, 01-049 Warsaw, Poland. Fax: 0048 22 599-19-57; E-mail: [email protected]
1040
TTE confirmed dilated (40 mm) RV in parasternal long/short axis, moderate tricuspid regurgitation, PAP 40 mmHg, moderate dilated (26 mm) pulmonary artery, however, interatrial septum (IAS) appeared to be uninterrupted in four-chamber and substernal view (Fig. 1). The assessment of pulmonary shunt was evaluated by pulmonary–systemic flow ratio (Qp/Qs = 1.5). Transesophageal echocardiography (TEE) was performed, having high suspicion for ASD. The exam confirmed the high position of ASD, and showed the abnormal outflow track of superior vena cava (SVC) into the right atrium (RA), with malposition of its inflow, which “overrode” the roof of the atria. ASD had diameter of at least 25 mm, but the presence and the drainage of right pulmonary veins (RPV) was not documented. On the left side, only a large common trunk for the pulmonary veins was shown (Figs. 2 and 3). Considering the eccentric position and the dimension of ASD, as well as the overriding SVC, the amount of the shunt and the value of PAP, the case was proposed and discussed for surgical treatment and not for percutaneous closure. A computed tomography angiography (angio-CT) was necessary to confirm the SV-ASD, which was 3 9 3 cm in diameter, with “high-riding” SVC draining both atrial cavities. The coronary arteries were normal. Multi slice angio-CT showed also four pulmonary veins (three of them
Atypical PAPVC With SV-ASD
Figure 1. Transthoracic echocardiography view showing right ventricle (RV) dilatation in parasternal long-axis view.
Figure 3. Transesophageal echocardiography showing color Doppler 72° view: left pulmonary vein trunk.
under observation in Cardiology Outpatient Clinic.
Figure 2. Transesophageal echocardiography showing color Doppler 129° view: sinus venosus atrial septal defect (SV-ASD) with shunt and “high-riding” superior vena cava (SVC).
draining toward the RA and a large common trunk toward the left side). Right upper and middle pulmonary lobe veins were draining into the SVC and the lower pulmonary lobe vein was draining into the RA (Figs. 4 and 5). The patient underwent surgical treatment by reconstruction of IAS with autologous pericardial patch, reconstruction of SVC ostium and transposition of RPV to the left atrium (LA) (Fig. 6). Intraoperative TEE confirmed good surgical result. TTE performed before discharge revealed regression of the RV dimension (34 mm), PAP 27+5 mmHg, and the IAS was without leakage. The patient had an uneventful recovery and was discharged from hospital on the 7th postoperative day. After 1-year follow-up, the patient had no symptoms of heart failure (NYHA class 1) and his exercise capacity is normal. In ECG was found sinus rhythm with incomplete right bundle branch block. TTE confirmed reduction of right cavities and normal pulmonary pressure (27 mmHg). The patient was advised to remain
Comment: We present a rare case of atypical anatomical variation of PAPVC, which to the best of our belief, has never been described before. Until now, were described seven variations of PAPVC, anomaly that can be found in 0.4–0.7% of patients. Most commonly met are as follows: (1) right upper and middle lobe pulmonary vein (PV) to SVC, while the second one to (2) RA. Less commonly met are (3) right PVs draining to inferior vena cava (IVC), (4) right lower PV to IVC, (5) left upper PV to innominate vein, (6) left upper or lower PV(s) to coronary sinus, and (7) finally left lower PV to RA or IVC. In those cases, the right lower lobe PV usually drains to LA.1 The case described showed the combination of two variations: number 1 and 7. Echocardiography is the main diagnostic tool for SV-ASD. However, angio-CT is an alternative diagnostic approach in these patients if TEE is insufficient.3 Some authors described positive diagnosis set in TTE by use of agitated saline solution,4 but TEE is still necessary to confirm both position and dimension of the ASD.3 Sensitivity of TEE to detect the SV-ASD varies between 0 and 100%, however, specificity is 100%. Meanwhile, sensitivity of TTE is 9%, and its specificity is 100%.5,6 On the other hand, angio-CT has 100% sensitivity for diagnosing PAPVR in patients with SV-ASD, 100% specificity, 100% positive predictive value, and 100% negative predictive value,7 what shows importance of this examination. Patients in follow-up after correction of ASD achieve clinical improvement in 77% of cases, however, deterioration is observed in 16% of cases.8 We believe that the patient presented, has good prognosis as his clinical condition improved, his pulmonary pressure decreased and 1041
Szychta, et al.
Figure 4. Three-dimensional volume rendering: partial anomalous pulmonary vein connection (PAPVC) of right upper and middle pulmonary veins to superior vena cava (SVC).
Figure 6. Surgeons view demonstrating right upper and middle pulmonary veins (blue arrow) and sinus venosus atrial septal defect (SV-ASD) (yellow arrow) *abnormal pulmonary venous drainage.
patients are rare they should remain under specialist care for life. This case highlights the weakness of TTE in excluding SV-ASD with PAPVC. In our opinion, proper surgical planning can be achieved with TEE and completed either by angio-CT or MRI.3,10 References
Figure 5. Angio-CT of heart: sinus venosus atrial septal defect (SV-ASD) and superior vena cava (SVC) delivering blood to right atrium (RA) and left atrium (LA).
he had no clinical symptomatic dysrhythmia. However, 6% of such patients have sinus node dysfunction or indications to pacemaker implantation, moreover, 14% of them have atrial fibrillation.8 Moreover, the risk for having arrhythmia is increased by technique of operation, as the surgeon had to perform extended incision nearby cavoatrial junction (Fig. 6), which increases atrial dysrhythmias.9 Although, complications in those 1042
1. Gatzoulis M, Webb G, Daubeney P: Diagnosis and management of adult congenital heart disease (Second Edition). In Ellis A (ed): Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome. London: Churchill Livingstone, 2011, pp. 216–221. 2. Babb J, McGlynn T, Pierce W, et al: Isolated partial anomalous venous connection: A congenital defect with late and serious complications. Ann Thorac Surg 1981;31:540–541. 3. ESC Task Force Guideline: ESC Guidelines for the management of grown-up congenital heart disease. Eur Heart J 2010;31:2915–2957. 4. Dorosz J, Fonseca B, Kay J, et al: A sinus venosus atrial septal defect is diagnosed by echocardiography with an unusual bubble study. Echocardiography 2013;30:E182– E183. 5. Lengyel M, Temesvari A, Villanyi J, et al: The role of transesophageal echocardiography in atrial septal defect detected in adults. Orv Hetil 1991;132:2467– 2471. € gge A, et al: Value of trans6. Hausmann D, Daniel WG, Mu esophageal color Doppler echocardiography for detection of different types of atrial septal defect in adults. J Am Soc Echocardiogr 1992;5:481–488. 7. Amat F, Le Bret E, Sigal-Cinqualbre A, et al: Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact Cardiovasc Thorac Surg 2011;12:179–182.
Atypical PAPVC With SV-ASD
8. Attenhofer Jost CH, Connolly HM, Danielson GK, et al: Sinus venosus atrial septal defect: Long-term postoperative outcome for 115 patients. Circulation 2005;112: 1953–1958. 9. Buz S1, Alexi-Meskishvili V, Villavicencio-Lorini F, et al: Analysis of arrhythmias after correction of partial anoma-
lous pulmonary venous connection. Ann Thorac Surg 2009;87:580–583. 10. Prompona M, Muehling O, Naebauer M, et al: MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects. Int J Cardiovasc Imaging 2011;27:403–412.
1043
