Partial Anomalous Pulmonary Venous Connection to the Azygos Vein With Atrial Septa1 Defect John C. Mullen, MD, Anees J. Razzouk, MD, William G. Williams, MD, C. A. Frederic Moes, MD, and Robert M. Freedom, MD Departments of Surgery, Radiology, and Pediatrics, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
A patient undergoing successful repair of partial anomalous pulmonary venous connection to the azygos vein associated with a secundum atrial septal defect is presented. (Ann Thorac Surg 1991;52:116&5)
P
artial anomalous pulmonary venous connection (PAPVC) is frequently associated with a sinus venosus type of atrial septal defect and right upper lobe pulmonary venous drainage to the superior vena cava [ 11. Three patients with PAPVC to the azygos vein with an intact atrial septum have been reported [ 2 4 ] . We report here a patient with PAPVC to the azygos vein and an associated atrial septal defect who was managed successfully by complete surgical correction. A 16-year-old girl was referred for evaluation of increasing fatigue and shortness of breath on exertion. The child had previously been well but had been noted to have a heart murmur in infancy. Physical examination revealed a grade 2/6 systolic ejection murmur heard maximally at the upper left sternal border, and loud fixed splitting of the second heart sound. A chest roentgenogram (Fig 1) showed moderate cardiomegaly, mild pulmonary plethora, and a prominent sharply circumscribed density at the level of the right tracheobronchial bifurcation. Twodimensional echocardiography outlined a 17-mm secundum-type atrial septal defect with left to right shunting, right ventricular enlargement, and PAPVC near the superior vena caval-right atrial junction. A transesophageal echocardiogram confirmed these findings. Selective pul-
hypothermia was employed. The atrial septal defect was enlarged by incising the superior rim of the septum as far as the foramen ovale, and autologous pericardium was employed to baffle the flow from the enlarged azygos vein to the atrial septal defect. The right pleural cavity was opened and the azygos vein ligated inferior to the right superior pulmonary venous connection. The supreme intercostal vein draining into the azygos vein was also ligated. After repair, venous oxygen saturations were similar in the superior vena cava cephalad to the azygos vein, right atrium, and pulmonary artery. The postoperative course was uncomplicated. A pwfusion lung scan performed 1 week postoperatively sholwed 71% of the flow going to the left lung and 29"/0to the right. Cardiac catherization was repeated and showed unobstructed flow from the right superior pulmonary vein through the azygos and baffle and into the left atrium. Flow through the superior vena cava was also unobstructed. Follow-up 6 months postoperatively revealed she had remained asymptomatic and she was not taking any medications.
m o n a r y arterial angiograms demonstrated t h e right supe-
rior pulmonary vein entering the azygos vein. There was then flow of contrast material into an enlarged right superior vena cava and right atrium (Fig 2A). The left pulmonary veins entered the left atrium (Fig 2B), as did the right inferior pulmonary vein. She underwent operation on July 18, 1990. Through a median sternotomy incision, cardiopulmonary bypass was established using bicaval cannulation. The heart was arrested with warm induction blood cardioplegia followed by myocardial cooling to 15"C, and moderate systemic Accepted for publication March 26, 1991. Address reprint requests to Dr Williams, The Hospital for Sick Children, 555 University Ave, #1525, Toronto, Ont M5G 1x8, Canada.
0 1991 by The Society of Thoracic Surgeons
Fig I . Chest roentgenogram showing moderate cardiac enlargement, increased pulmona y vascularity, and prominence of the azygos vein (arrow). 0003-4975/91/$3.50
Ann Thorac Surg 1991:521164-5
A
CASE REPORT MULLEN ET AL PARTIAL ANOMALOUS PULMONARY CONNECTION
1165
intact atrial septum have previously been reported [ 2 4 ] , and in 2 of these surgical repair was successfully performed [2, 31. Cases exhibiting bilateral total anomalous pulmonary connection have previously been described [5]. The present case is unusual, having unilateral partial pulmonary venous connection to the azygos vein plus an atrial septal defect. The diagnosis of PAPVC to the azygos vein is difficult to make by transthoracic or transesophageal echocardiography. The atypical appearance of the PAPVC in the region of the superior vena caval-right atrial junction prompted selective cardiac catheterization, which was critical in making an accurate diagnosis. Surgical correction was similar to the procedure used in patients with PAPVC to the superior vena cava [6, 71. The atrial septal defect was first enlarged and then a pericardial baffle was constructed to tunnel the blood flow from the azygos vein to the left atrium. The other systemic venous tributaries of the enlarged azygos vein were divided to prevent a subsequent right to left shunt and possible "paradoxical" emboli. In the 2 previous surgically corrected cases [2, 31 this was not done, but difficulties from the resultant small shunt were not reported. Partial anomalous pulmonary venous connection to the azygos vein requires an index of suspicion and cardiac catheterization to diagnose. The condition may or may not be associated with an atrial septal defect. Complete repair is feasible and can be accomplished safely.
References
B
Fig 2. Selective cardiac angiograms (levophase) with catheter in the right (A) and left (B) pulmonary arteries. The right superior pulmonary vein (RPV) drains superiorly into the azygos vein (AZ) and then inferiorly into the superior vena cava (SVC). The normal left pulmonary veins are seen entering the left atrium (LA).
Comment Partial anomalous pulmonary Venous connection to the azygos vein is uncommon. Three cases of PAPVC and
1. Healey JE Jr. An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Cardiovasc Surg 1952;23:43?4. 2. Yabek SM, Akl BF, Berman W. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum. Chest 1979;76:48&7. 3. Makino S, Yamazaki N, Okabe M, et al. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum [in Japanese]. Kyobu Geka 1986;39(2):13H3. 4. Jennings JG, Serwer GA. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum. Pediatr Cardiol 1986;7:115-7. 5. Moes CAF, Fowler RS, Trusler GA. Total anomalous pulmonary venous drainage into the azygos vein. Am J Roentgen01 1966;98:37%87. 6 . Cooley DA, Ellis PR, Bellizzi ME. Atrial septal defects of the sinus venosus type: surgical considerations. Dis Chest 1961; 39:l-8. 7. Shumacker HB Jr. Partial anomalous pulmonary venous return. Dis Chest 1966;49:309-16.
A patient undergoing successful repair of partial anomalous pulmonary venous connection to the azygos vein associated with a secundum atrial septal defect is presented. (Ann Thorac Surg 1991;52:116&5)
P
artial anomalous pulmonary venous connection (PAPVC) is frequently associated with a sinus venosus type of atrial septal defect and right upper lobe pulmonary venous drainage to the superior vena cava [ 11. Three patients with PAPVC to the azygos vein with an intact atrial septum have been reported [ 2 4 ] . We report here a patient with PAPVC to the azygos vein and an associated atrial septal defect who was managed successfully by complete surgical correction. A 16-year-old girl was referred for evaluation of increasing fatigue and shortness of breath on exertion. The child had previously been well but had been noted to have a heart murmur in infancy. Physical examination revealed a grade 2/6 systolic ejection murmur heard maximally at the upper left sternal border, and loud fixed splitting of the second heart sound. A chest roentgenogram (Fig 1) showed moderate cardiomegaly, mild pulmonary plethora, and a prominent sharply circumscribed density at the level of the right tracheobronchial bifurcation. Twodimensional echocardiography outlined a 17-mm secundum-type atrial septal defect with left to right shunting, right ventricular enlargement, and PAPVC near the superior vena caval-right atrial junction. A transesophageal echocardiogram confirmed these findings. Selective pul-
hypothermia was employed. The atrial septal defect was enlarged by incising the superior rim of the septum as far as the foramen ovale, and autologous pericardium was employed to baffle the flow from the enlarged azygos vein to the atrial septal defect. The right pleural cavity was opened and the azygos vein ligated inferior to the right superior pulmonary venous connection. The supreme intercostal vein draining into the azygos vein was also ligated. After repair, venous oxygen saturations were similar in the superior vena cava cephalad to the azygos vein, right atrium, and pulmonary artery. The postoperative course was uncomplicated. A pwfusion lung scan performed 1 week postoperatively sholwed 71% of the flow going to the left lung and 29"/0to the right. Cardiac catherization was repeated and showed unobstructed flow from the right superior pulmonary vein through the azygos and baffle and into the left atrium. Flow through the superior vena cava was also unobstructed. Follow-up 6 months postoperatively revealed she had remained asymptomatic and she was not taking any medications.
m o n a r y arterial angiograms demonstrated t h e right supe-
rior pulmonary vein entering the azygos vein. There was then flow of contrast material into an enlarged right superior vena cava and right atrium (Fig 2A). The left pulmonary veins entered the left atrium (Fig 2B), as did the right inferior pulmonary vein. She underwent operation on July 18, 1990. Through a median sternotomy incision, cardiopulmonary bypass was established using bicaval cannulation. The heart was arrested with warm induction blood cardioplegia followed by myocardial cooling to 15"C, and moderate systemic Accepted for publication March 26, 1991. Address reprint requests to Dr Williams, The Hospital for Sick Children, 555 University Ave, #1525, Toronto, Ont M5G 1x8, Canada.
0 1991 by The Society of Thoracic Surgeons
Fig I . Chest roentgenogram showing moderate cardiac enlargement, increased pulmona y vascularity, and prominence of the azygos vein (arrow). 0003-4975/91/$3.50
Ann Thorac Surg 1991:521164-5
A
CASE REPORT MULLEN ET AL PARTIAL ANOMALOUS PULMONARY CONNECTION
1165
intact atrial septum have previously been reported [ 2 4 ] , and in 2 of these surgical repair was successfully performed [2, 31. Cases exhibiting bilateral total anomalous pulmonary connection have previously been described [5]. The present case is unusual, having unilateral partial pulmonary venous connection to the azygos vein plus an atrial septal defect. The diagnosis of PAPVC to the azygos vein is difficult to make by transthoracic or transesophageal echocardiography. The atypical appearance of the PAPVC in the region of the superior vena caval-right atrial junction prompted selective cardiac catheterization, which was critical in making an accurate diagnosis. Surgical correction was similar to the procedure used in patients with PAPVC to the superior vena cava [6, 71. The atrial septal defect was first enlarged and then a pericardial baffle was constructed to tunnel the blood flow from the azygos vein to the left atrium. The other systemic venous tributaries of the enlarged azygos vein were divided to prevent a subsequent right to left shunt and possible "paradoxical" emboli. In the 2 previous surgically corrected cases [2, 31 this was not done, but difficulties from the resultant small shunt were not reported. Partial anomalous pulmonary venous connection to the azygos vein requires an index of suspicion and cardiac catheterization to diagnose. The condition may or may not be associated with an atrial septal defect. Complete repair is feasible and can be accomplished safely.
References
B
Fig 2. Selective cardiac angiograms (levophase) with catheter in the right (A) and left (B) pulmonary arteries. The right superior pulmonary vein (RPV) drains superiorly into the azygos vein (AZ) and then inferiorly into the superior vena cava (SVC). The normal left pulmonary veins are seen entering the left atrium (LA).
Comment Partial anomalous pulmonary Venous connection to the azygos vein is uncommon. Three cases of PAPVC and
1. Healey JE Jr. An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Cardiovasc Surg 1952;23:43?4. 2. Yabek SM, Akl BF, Berman W. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum. Chest 1979;76:48&7. 3. Makino S, Yamazaki N, Okabe M, et al. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum [in Japanese]. Kyobu Geka 1986;39(2):13H3. 4. Jennings JG, Serwer GA. Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum. Pediatr Cardiol 1986;7:115-7. 5. Moes CAF, Fowler RS, Trusler GA. Total anomalous pulmonary venous drainage into the azygos vein. Am J Roentgen01 1966;98:37%87. 6 . Cooley DA, Ellis PR, Bellizzi ME. Atrial septal defects of the sinus venosus type: surgical considerations. Dis Chest 1961; 39:l-8. 7. Shumacker HB Jr. Partial anomalous pulmonary venous return. Dis Chest 1966;49:309-16.
