Diagnostic Radiology
Anomalous Pulmonary Venous Connection of Entire Left Lung with Intact Atrial Septum Radiological Features and Clinical Implications 1 Stephen W. Miller, M.D.,2 Robert E. Dinsmore, M.D., Richard R. Liberthson, M.D., Allan Goldblatt, M.D., Willard M. Daggett, M.D., and Mortimer J. Buckley, M.D.
Three cases oftotal unilateral pulmonary venous connection of the leftlung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein inthe superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar towhat isseen incases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the leftpulmonary veins and documented the absence of an atrial septal defect. INDEX TERMS: Atrial septal defect. Pulmonary angiography, indications • Pulmonary arteries, abnormalities. (Supracardiac partial anomalous pulmonary venous connection, 5 [65]. 1581) • Veins, pulmonary
Radiology 122:591-595, March 1977
murmurs discovered during routine medical examinations. The physical examination of all 3 patients suggested an atrial septal defect with a Grade 2-3 (of 6) systolic ejection murmur at the upper left sternal border. The second sound was physiologically split in 1 and widely split in 2 patients throughout the respiratory cycle. The electrocardiogram revealed an incomplete right bundle branch block with right axis deviation in 2 and left axis deviation in 1 patient (TABLE
ARIATIONS in pulmonary venous connections are uncommon, occurring in 0.4% in an unselected autopsy series (10). Anomalous pulmonary veins are occasionally associated with other cardiac defects, particularly atrial septal defect, and may therefore present a difficult problem in diagnosis. We wish to describe an unusual variant of anomalous pulmonary venous connection in the absence of other cardiac abnormalities, and to discuss the angiographic diagnosis and clinical management of this anomaly.
V
1). At cardiac catheterization, all 3 patients had a pulmonary to systemic blood flow rate of 2:1 and normal pulmonary arterial pressures. An anomalous vertical vein connecting the left pulmonary veins to the innominate vein was identified in 2 patients by retrograde injection of
CLINICAL PRESENTATION
Two adults and 1 infant underwent evaluation of heart
Table I:
Summary of Clinical and Electrocardiographic Data and Results of Cardiac Catheterization
Patient Sex Age Murmur Second Heart Stound Electrocardiogram QRS axis Interventricu lar conduction Qp/Qst Pulmonary Artery Pressure (mm Hg) Foramen Ovale Other Cardiac Defects
L.B.
S.H.
S.W. Female at upper left sternum Physiologically split
Male 8 months Grade 3/6 systolic ejection at upper left sternum Fixed, split
90
-40
normal
I RBBB* 1.8
25 years Grade 2/6 systolic ejection
2.4 14/7
22/9
Probe patent at surgery
None identified at catheterization None
None
Female
20 years Grade 2/6 systolic ejection at upper left sternum Fixed, split
+70 IRBBB
1.9 21/5 None None
*Incomplete right bundle branch block t Ratio of pulmonary to systemic blood flow
1 From the Departments of Radiology, Medicine, and Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Mass. Accepted for publication inOctober, 1976. ss 2 Scholar of the James Picker Foundation.
591
592
STEPHEN
W.
MILLER AND OTHERS
Table II: Radiologic Interpretation of Chest Radiographs in Postero-anterior and Lateral Projections Patient -
---.-~
S.H.
S.W.
L.B.
March 1977
contrast agent into the vertical vein. No atrial septal defect was identified during catheterization, although 1 patient had a patent foramen ovale discovered during surgery.
~------_._---------------_._~---_.-
Age Superior Vena Cava Right Atrium Right Ventricle Main Pulmonary Artery Peripheral Pulmonary Arteries Left Atrium Left Ventricle Anomalous Vertic Ie Vein --------
25 years
8 months
0 0 + ++
++ + + ++
+
+
+
0
0
0 0
+
++
+
20 years
0
RADIOGRAPHIC FEAlURES
0 + ++
.!:
_._--------_._---
Degree of enlargement: slight; ++ = moderate.
0
=
none; ±
= equivocal;
+
=
A tabulation of chest radiographic features is presented in TABLE II. The main pulmonary arteries and peripheral pulmonary branches were enlarged, consistent with a left-to-right shunt. In all 3 patients, there was no difference between the degree of enlargement of the right and left pulmonary vessels. The right ventricle was slightly enlarged in all 3 patients. The left atrium was equivocally
,
-,
Fig. 1. Postero-anterior chest radiographs of patients S.W. (A), L.B. (B), and S.H. (C) show the anomalous vein (arrow) on the left side of the mediastinum and the slightly large central pulmonary arteries.
Vol. 122
ANOMALOUS PULMONARY VENOUS CONNECTION
enlarged in 2, and the right atrium was enlarged in 1 patient. The right superior vena cava was visible only in the child, and appeared to be separate from the thymus. The anomalous vein connecting the left common pulmonary vein to the left innominate vein was clearly visible in all 3 patients on the left side of the mediastinum (Fig. 1). The large central and peripheral pulmonary arteries, and the visibly large anomalous vein in the superior mediastinum, were, therefore, the major radiological findings. Pulmonary angiography was performed to locate the pulmonary veins and to establish the completeness of the atrial septum. A main pulmonary artery injection was made in 1 patient, and selective right and left pulmonary artery injections were made in 2. The entire venous drainage from the left lung entered a common pulmonary vein and ascended to the left innominate vein (Fig. 2). The veins from the right lung drained normally. In those 2 patients undergoing selective pulmonary angiography, injection into the right pulmonary artery did not re-opacify the right atrium; an atrial septal defect was thereby excluded angiographically (Fig. 3).
593
Diagnostic Radiology
Fig. 2. Venous phase of pulmonary angiogram of patient L.B. demonstrates that the veins from the left lung drain into an anomalous vein (arrows) which connects to the innominate vein (arrowheads). The right pulmonary veins connect normally.
SURGICAL CORRECTION
The 2 adults underwent surgical correction of the anomalous pulmonary connection. (The surgical aspects of the care of patient L.B. have been discussed previously in Reference 3). After sternal splitting and the institution of cardiopulmonary bypass, the anomalous pulmonary vein behind the pericardium was dissected free. In one patient, the left atrial appendage was amputated and an anastomosis constructed between the side of the anomalous vein and the stump of the atrial appendage; then the anomalous vein was divided near its junction with the left innominate vein. In the other patient, a side-to-side anastomosis was constructed between the anomalous pulmonary venous trunk and the posterior aspect of the left atrium at the base of its appendage; the anomalous vein was then ligated, rather than divided, in order not to kink the anastomosis. DISCUSSION
Anomalies of pulmonary venous connection which were previously considered rare are being recognized clinically with increasing frequency, partly because of refinements in cardiac catheterization and surgery. The first to report a case of partial anomalous pulmonary venous connection was Winslow in 1739 (25). The first angiographic documentation of partial anomalous pulmonary venous return is credited to Dotter (6) who, in 1949, used a large bolus of contrast material injected into a peripheral vein. His 2 patients had anomalous pulmonary veins in the right lung which drained into the inferior vena cava. Several reviews (2,3,9,10,12,14-16,19,20,22,23)havetabulatedthe variety of abnormal pulmonary venous connections and their associated cardiac defects. The most common sites of drainage (3) are into the superior vena cava, right atrium,
and left innominate vein, with less frequent involvement of the coronary sinus, inferior vena cava, subclavian vein, and azygous vein. The more frequent pulmonary venous anomalies are lobar, usually occurring in the right lung. Unilateral total anomalous pulmonary venous connection (APVC) from either lung is quite rare. In a search of the literature on APVC in which the status of the atrial septum was reported, we found 26 cases (7, 14,21,22,24) of total unilateral right APVC, half of which included an intact atrial septum. There have been 6 cases of total APVC from the left lung, 5 with intact atrial septum (3, 4, 5, 19, 22) and 1 with an atrial septal defect (8). Healey (10) discussed 33 patients with APVC from 1 lung (22 from the right and 11 from the left lung), but did not record the status of the atrial septum. Thus, this report adds 3 more cases of total APVC of the left lung with intact atrial septum to those previously documented. Multiple cardiac abnormalities may occur with partial anomalous pulmonary venous connection. Frequently, an atrial septal defect of either the sinus venosus, ostium secundum, or ostium primum type is present (22). Rarely associated cardiac malformations occurring with APVC include single atrium, corrected transposition, ventricular septal defect, endocardial cushion defect, tetralogy of Fallot, cor triatrium, and the heterotaxy syndrome (16, 22). With the exception of 1 patient with atrial septal defect (8), these cardiac malformations do not appear to be associated with APVC of the left lung. Since the development of the pulmonary vein occurs concomitantly with the formation of the atria, it is not surprising that defects in both of these structures may be associated. In the 3 cases discussed here, and in the 5 previously described, no atrial septal defect was found, suggesting that the embryologic defect involves only the left anterior cardinal vein (22).
594
STEPHEN
W. MILLER AND OTHERS
The history, physical examination, and electrocardiogram of patients with anomalous pulmonary veins do not distinguish these patients from those with an isolated atrial septal defect (5, 11, 14). The electrocardiographic findings are usually those of right ventricular overload (right axis deviation and incomplete right bundle branch block). One of our patients had left axis deviation, which is unusual in the absence of other cardiac defects. In Kalke's series (12), 2 of 57 patients with APVC also had left axis deviation, but both of these patients also had mitral valve disease. Our patient with left axis deviation had normal pulmonary artery pressure and no clinical or angiographic evidence of mitral valve disease. The technique of surgical treatment of partial anomalous pulmonary veins depends upon the number and size of the veins, as well as the presence of an atrial septal defect. Digital intracardiac palpation of the atrial septum and exploration of both pulmonary hila is therefore usually carried out after the standard midline sternotomy. In 1 of the 2 cases reported here that involved surgical correction, the repair was similar to that reported by Kirklin (13) and Cooley (5), with the construction of an anastomosis from the side of the anomalous vertical vein to the end of the amputated left atrial appendage stump. The large pulmonary venous anastomosis was constructed to decrease the tendency of stricture formation and thrombosis at the suture line. In patient S.W., the anastomosis was constructed to the left atrium rather than to the appendage; this technique allows less displacement of the anomalous pulmonary venous trunk. An angiographic method of identifying an atrial septal defect in the presence of anomalous pulmonary veins was recently described by Sos (23), who used separate pulmonary artery injections. His method involves selective injection of the ipsilateral pulmonary artery to demonstrate the anomalous vein, and selective injection of the contralateral pulmonary artery to establish the presence of an atrial septal defect. This method was successful in identifying the anomalous vein and in excluding an atrial septal
March 1977
defect in both patients (S.W. and S.H.) for whom it was used. The principal differential diagnostic consideration of unilateral left APVC on the plain chest radiograph is total anomalous pulmonary venous connection to an anomalous vertical vein. Both entities may have large pulmonary arteries with aortas of normal size, suggesting an intracardiac shunt which, in the absence of left atrial enlargement, is usually at the atrial level. The additional finding of a vertical vascular structure on the left side of the mediastinum could be either an anomalous vertical vein communicating with the pulmonary veins or a persistent left superior vena cava connecting the left innominate vein with the coronary sinus. Rarely does a left superior vena cava connect with the left atrium, left pulmonary veins, and the left innominate vein (22). Clinically, the ausculatory findings and electrocardiogram are similar in cases of unilateral left APVC and total APVC. Cyanosis has not been found to be associated with unilateral left APVC. With this set of radiographic findings, therefore, cyanosis appears to be diagnostic of total APVC. Patients with total APVC may have a larger pulmonary blood flow, depending upon the size of the atrial septal defect and the pulmonary vascular resistence. These patients therefore tend to have larger pulmonary arteries, vertical veins, and superior vena cavae than do those with left lung APVC. In contrast, patients with lesser degrees of APVC, i.e., lobar or segmental, have normal-sized pulmonary arteries, because the pulmonary-to-systemic flow ratio is less than 2: 1; in these minor shunts, the only possibility of diagnosis by plain chest radiograph is visualization of the anomalous vein.
REFERENCES 1. Bello AG, Daggett WM, Hutter AM Jr, et al: Surgical correction of partial anomalous venous drainage with intact atrial septum: association with acquired valve lesions. J Cardiovasc Surg 14:357-364, Jul-Aug 1973
Fig. 3. Frames from a cJne pulmonary angiogram of patient S.W. show the venous return from selective injection into the right and left pulmonary arteries. A. After right pulmonary artery injection, only the left atrium (arrows) is opacified, suggesting the absence of an atrial septal defect. B. The venous phase of the left pulmonary artery injection shows the anomalous vein (arrows).
Vol. 122
ANOMALOUS PULMONARY VENOUS CONNECTION
2. Blake HA, Hall RJ, Manion WC: Anomalous pulmonary venous return. Circulation 32:406-414, Sep 1965 3. Brody H: Drainage of the pulmonary veins into the right side of the heart. Arch Pathol 33:221-240, Feb 1942 4. Conant JS, Kurland LT, Dale G: Pulmonary tuberculosis associated with anomalous common left pulmonary vein entering the left innominate vein. J Thorac Surg 16:422-426, Aug 1947 5. Cooley DA, Mahaffey DE: Anomalous pulmonary venous drainage of entire left lung: report of a case with surgical correction. Ann Surg 142:986-991, Dec 1955 6. Dotter CT, Hardisty NM, Steinberg I: Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosedduring life by angiocardiography and cardiac catheterization. Am J Med Sci 218:31-36, Jul 1949 7. , Ehrenhaft JL, Theilen EO, Lawrence MS: The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 148:249-258, Aug 1958 8. Geraci JE, Kirklin JW: Transplantation of left anomalous pulmonary vein to left atrium: report of case. Proc Staff Meet Mayo Clin 28:472-475,26 Aug 1953 9. Gilman RA, Skowron CA, Musser BG, et al: Partial anomalous venous drainage. Am J Surg 94:688-694, Nov 1957 10. Healey JE Jr: An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg 23:433-444, May 1952 11. Hickie JB, Gimlette TMD, Bacon APC: Anomalous pulmonary venous drainage. Br Heart J 18:365-377, Jul 1956 12. Kalke BR, Carlson RG, Ferlic RM, et al: Partial anomalous pulmonary venous connections. Am J CardioI20:91-101, Jul 1967 13. Kirklin JW: Surgical.treatment of anomalous pulmonary venous connection (partial anomalous pulmonary venous drainage). Proc Staff Meet Mayo Clin 28:476-479,26 Aug 1953 14. Morrow AG, Awe WC, Aygen MM: Total unilateral anomalous pulmonary venous connection with intact atrial septum. Am J Cardiol 9:933-937, Jun 1962
595
Diagnostic Radiology
15. Muir AR: Anomalous pulmonary venous drainage. Thorax 8:65-68, Mar 1953 16. Nakib A, Moller JH, Kanjuh VI, et al: Anomalies of the pulmonary veins. Am J Cardiol 20:77-90, Jul 1967 17. Neirotti R, Gonzalez-Lavin L, Ross DN: Anomalous pulmonary venous connexions of left lung associated with valvular heart disease. Report of two cases. Br Heart J 34:969-973, Sep 1972 18. Samet P, Fierer EM, Berstein WH: Anomalous pulmonary venous drainage: diagnostic value of bronchospirometry. Am J Med 26:654-658, Oct 1958 19. Sepulveda G, Lukas DS, Steinberg I: Anomalous drainage of pulmonary veins; clinical, physiologic, and angiocardiographic features. Am J Med 18:883-899, Jun 1955 20. Shumacker HB Jr, Judd D: Partial anomalous pulmonary venous return with reference to drainage into the inferior vena cava and to an intact atrial septum. J Cardiovasc Surg 5:271-278, Jul-Aug 1964 21. Snellen HA, Albers FH: The clinical diagnosis of anomalous pulmonary venous drainage. Circulation 6:80 1-816, D~c 1952 22. Snellen HA, Bruins C: Anomalies of venous return. [In] Watson H, ed: Paediatric Cardiology. St Louis, Mosby, 1968, pp 416-426 23. Sos TA, Tay D, Levin AR, et al: Angiographic demonstration of the absence of an atrial septal defect in the presence of partial anomalous pulmonary venous connection. Am J Roentgenol 121: 591-596, Jul 1974 24. TaussigHB: Congenital malformations of the heart. New York, Harvard University, 2d ed, 1960, pp 536-580 25. Winslow J: cited by Brody
Department of Radiology Massachusetts General Hospital Boston, Mass. 02114
Anomalous Pulmonary Venous Connection of Entire Left Lung with Intact Atrial Septum Radiological Features and Clinical Implications 1 Stephen W. Miller, M.D.,2 Robert E. Dinsmore, M.D., Richard R. Liberthson, M.D., Allan Goldblatt, M.D., Willard M. Daggett, M.D., and Mortimer J. Buckley, M.D.
Three cases oftotal unilateral pulmonary venous connection of the leftlung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein inthe superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar towhat isseen incases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the leftpulmonary veins and documented the absence of an atrial septal defect. INDEX TERMS: Atrial septal defect. Pulmonary angiography, indications • Pulmonary arteries, abnormalities. (Supracardiac partial anomalous pulmonary venous connection, 5 [65]. 1581) • Veins, pulmonary
Radiology 122:591-595, March 1977
murmurs discovered during routine medical examinations. The physical examination of all 3 patients suggested an atrial septal defect with a Grade 2-3 (of 6) systolic ejection murmur at the upper left sternal border. The second sound was physiologically split in 1 and widely split in 2 patients throughout the respiratory cycle. The electrocardiogram revealed an incomplete right bundle branch block with right axis deviation in 2 and left axis deviation in 1 patient (TABLE
ARIATIONS in pulmonary venous connections are uncommon, occurring in 0.4% in an unselected autopsy series (10). Anomalous pulmonary veins are occasionally associated with other cardiac defects, particularly atrial septal defect, and may therefore present a difficult problem in diagnosis. We wish to describe an unusual variant of anomalous pulmonary venous connection in the absence of other cardiac abnormalities, and to discuss the angiographic diagnosis and clinical management of this anomaly.
V
1). At cardiac catheterization, all 3 patients had a pulmonary to systemic blood flow rate of 2:1 and normal pulmonary arterial pressures. An anomalous vertical vein connecting the left pulmonary veins to the innominate vein was identified in 2 patients by retrograde injection of
CLINICAL PRESENTATION
Two adults and 1 infant underwent evaluation of heart
Table I:
Summary of Clinical and Electrocardiographic Data and Results of Cardiac Catheterization
Patient Sex Age Murmur Second Heart Stound Electrocardiogram QRS axis Interventricu lar conduction Qp/Qst Pulmonary Artery Pressure (mm Hg) Foramen Ovale Other Cardiac Defects
L.B.
S.H.
S.W. Female at upper left sternum Physiologically split
Male 8 months Grade 3/6 systolic ejection at upper left sternum Fixed, split
90
-40
normal
I RBBB* 1.8
25 years Grade 2/6 systolic ejection
2.4 14/7
22/9
Probe patent at surgery
None identified at catheterization None
None
Female
20 years Grade 2/6 systolic ejection at upper left sternum Fixed, split
+70 IRBBB
1.9 21/5 None None
*Incomplete right bundle branch block t Ratio of pulmonary to systemic blood flow
1 From the Departments of Radiology, Medicine, and Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Mass. Accepted for publication inOctober, 1976. ss 2 Scholar of the James Picker Foundation.
591
592
STEPHEN
W.
MILLER AND OTHERS
Table II: Radiologic Interpretation of Chest Radiographs in Postero-anterior and Lateral Projections Patient -
---.-~
S.H.
S.W.
L.B.
March 1977
contrast agent into the vertical vein. No atrial septal defect was identified during catheterization, although 1 patient had a patent foramen ovale discovered during surgery.
~------_._---------------_._~---_.-
Age Superior Vena Cava Right Atrium Right Ventricle Main Pulmonary Artery Peripheral Pulmonary Arteries Left Atrium Left Ventricle Anomalous Vertic Ie Vein --------
25 years
8 months
0 0 + ++
++ + + ++
+
+
+
0
0
0 0
+
++
+
20 years
0
RADIOGRAPHIC FEAlURES
0 + ++
.!:
_._--------_._---
Degree of enlargement: slight; ++ = moderate.
0
=
none; ±
= equivocal;
+
=
A tabulation of chest radiographic features is presented in TABLE II. The main pulmonary arteries and peripheral pulmonary branches were enlarged, consistent with a left-to-right shunt. In all 3 patients, there was no difference between the degree of enlargement of the right and left pulmonary vessels. The right ventricle was slightly enlarged in all 3 patients. The left atrium was equivocally
,
-,
Fig. 1. Postero-anterior chest radiographs of patients S.W. (A), L.B. (B), and S.H. (C) show the anomalous vein (arrow) on the left side of the mediastinum and the slightly large central pulmonary arteries.
Vol. 122
ANOMALOUS PULMONARY VENOUS CONNECTION
enlarged in 2, and the right atrium was enlarged in 1 patient. The right superior vena cava was visible only in the child, and appeared to be separate from the thymus. The anomalous vein connecting the left common pulmonary vein to the left innominate vein was clearly visible in all 3 patients on the left side of the mediastinum (Fig. 1). The large central and peripheral pulmonary arteries, and the visibly large anomalous vein in the superior mediastinum, were, therefore, the major radiological findings. Pulmonary angiography was performed to locate the pulmonary veins and to establish the completeness of the atrial septum. A main pulmonary artery injection was made in 1 patient, and selective right and left pulmonary artery injections were made in 2. The entire venous drainage from the left lung entered a common pulmonary vein and ascended to the left innominate vein (Fig. 2). The veins from the right lung drained normally. In those 2 patients undergoing selective pulmonary angiography, injection into the right pulmonary artery did not re-opacify the right atrium; an atrial septal defect was thereby excluded angiographically (Fig. 3).
593
Diagnostic Radiology
Fig. 2. Venous phase of pulmonary angiogram of patient L.B. demonstrates that the veins from the left lung drain into an anomalous vein (arrows) which connects to the innominate vein (arrowheads). The right pulmonary veins connect normally.
SURGICAL CORRECTION
The 2 adults underwent surgical correction of the anomalous pulmonary connection. (The surgical aspects of the care of patient L.B. have been discussed previously in Reference 3). After sternal splitting and the institution of cardiopulmonary bypass, the anomalous pulmonary vein behind the pericardium was dissected free. In one patient, the left atrial appendage was amputated and an anastomosis constructed between the side of the anomalous vein and the stump of the atrial appendage; then the anomalous vein was divided near its junction with the left innominate vein. In the other patient, a side-to-side anastomosis was constructed between the anomalous pulmonary venous trunk and the posterior aspect of the left atrium at the base of its appendage; the anomalous vein was then ligated, rather than divided, in order not to kink the anastomosis. DISCUSSION
Anomalies of pulmonary venous connection which were previously considered rare are being recognized clinically with increasing frequency, partly because of refinements in cardiac catheterization and surgery. The first to report a case of partial anomalous pulmonary venous connection was Winslow in 1739 (25). The first angiographic documentation of partial anomalous pulmonary venous return is credited to Dotter (6) who, in 1949, used a large bolus of contrast material injected into a peripheral vein. His 2 patients had anomalous pulmonary veins in the right lung which drained into the inferior vena cava. Several reviews (2,3,9,10,12,14-16,19,20,22,23)havetabulatedthe variety of abnormal pulmonary venous connections and their associated cardiac defects. The most common sites of drainage (3) are into the superior vena cava, right atrium,
and left innominate vein, with less frequent involvement of the coronary sinus, inferior vena cava, subclavian vein, and azygous vein. The more frequent pulmonary venous anomalies are lobar, usually occurring in the right lung. Unilateral total anomalous pulmonary venous connection (APVC) from either lung is quite rare. In a search of the literature on APVC in which the status of the atrial septum was reported, we found 26 cases (7, 14,21,22,24) of total unilateral right APVC, half of which included an intact atrial septum. There have been 6 cases of total APVC from the left lung, 5 with intact atrial septum (3, 4, 5, 19, 22) and 1 with an atrial septal defect (8). Healey (10) discussed 33 patients with APVC from 1 lung (22 from the right and 11 from the left lung), but did not record the status of the atrial septum. Thus, this report adds 3 more cases of total APVC of the left lung with intact atrial septum to those previously documented. Multiple cardiac abnormalities may occur with partial anomalous pulmonary venous connection. Frequently, an atrial septal defect of either the sinus venosus, ostium secundum, or ostium primum type is present (22). Rarely associated cardiac malformations occurring with APVC include single atrium, corrected transposition, ventricular septal defect, endocardial cushion defect, tetralogy of Fallot, cor triatrium, and the heterotaxy syndrome (16, 22). With the exception of 1 patient with atrial septal defect (8), these cardiac malformations do not appear to be associated with APVC of the left lung. Since the development of the pulmonary vein occurs concomitantly with the formation of the atria, it is not surprising that defects in both of these structures may be associated. In the 3 cases discussed here, and in the 5 previously described, no atrial septal defect was found, suggesting that the embryologic defect involves only the left anterior cardinal vein (22).
594
STEPHEN
W. MILLER AND OTHERS
The history, physical examination, and electrocardiogram of patients with anomalous pulmonary veins do not distinguish these patients from those with an isolated atrial septal defect (5, 11, 14). The electrocardiographic findings are usually those of right ventricular overload (right axis deviation and incomplete right bundle branch block). One of our patients had left axis deviation, which is unusual in the absence of other cardiac defects. In Kalke's series (12), 2 of 57 patients with APVC also had left axis deviation, but both of these patients also had mitral valve disease. Our patient with left axis deviation had normal pulmonary artery pressure and no clinical or angiographic evidence of mitral valve disease. The technique of surgical treatment of partial anomalous pulmonary veins depends upon the number and size of the veins, as well as the presence of an atrial septal defect. Digital intracardiac palpation of the atrial septum and exploration of both pulmonary hila is therefore usually carried out after the standard midline sternotomy. In 1 of the 2 cases reported here that involved surgical correction, the repair was similar to that reported by Kirklin (13) and Cooley (5), with the construction of an anastomosis from the side of the anomalous vertical vein to the end of the amputated left atrial appendage stump. The large pulmonary venous anastomosis was constructed to decrease the tendency of stricture formation and thrombosis at the suture line. In patient S.W., the anastomosis was constructed to the left atrium rather than to the appendage; this technique allows less displacement of the anomalous pulmonary venous trunk. An angiographic method of identifying an atrial septal defect in the presence of anomalous pulmonary veins was recently described by Sos (23), who used separate pulmonary artery injections. His method involves selective injection of the ipsilateral pulmonary artery to demonstrate the anomalous vein, and selective injection of the contralateral pulmonary artery to establish the presence of an atrial septal defect. This method was successful in identifying the anomalous vein and in excluding an atrial septal
March 1977
defect in both patients (S.W. and S.H.) for whom it was used. The principal differential diagnostic consideration of unilateral left APVC on the plain chest radiograph is total anomalous pulmonary venous connection to an anomalous vertical vein. Both entities may have large pulmonary arteries with aortas of normal size, suggesting an intracardiac shunt which, in the absence of left atrial enlargement, is usually at the atrial level. The additional finding of a vertical vascular structure on the left side of the mediastinum could be either an anomalous vertical vein communicating with the pulmonary veins or a persistent left superior vena cava connecting the left innominate vein with the coronary sinus. Rarely does a left superior vena cava connect with the left atrium, left pulmonary veins, and the left innominate vein (22). Clinically, the ausculatory findings and electrocardiogram are similar in cases of unilateral left APVC and total APVC. Cyanosis has not been found to be associated with unilateral left APVC. With this set of radiographic findings, therefore, cyanosis appears to be diagnostic of total APVC. Patients with total APVC may have a larger pulmonary blood flow, depending upon the size of the atrial septal defect and the pulmonary vascular resistence. These patients therefore tend to have larger pulmonary arteries, vertical veins, and superior vena cavae than do those with left lung APVC. In contrast, patients with lesser degrees of APVC, i.e., lobar or segmental, have normal-sized pulmonary arteries, because the pulmonary-to-systemic flow ratio is less than 2: 1; in these minor shunts, the only possibility of diagnosis by plain chest radiograph is visualization of the anomalous vein.
REFERENCES 1. Bello AG, Daggett WM, Hutter AM Jr, et al: Surgical correction of partial anomalous venous drainage with intact atrial septum: association with acquired valve lesions. J Cardiovasc Surg 14:357-364, Jul-Aug 1973
Fig. 3. Frames from a cJne pulmonary angiogram of patient S.W. show the venous return from selective injection into the right and left pulmonary arteries. A. After right pulmonary artery injection, only the left atrium (arrows) is opacified, suggesting the absence of an atrial septal defect. B. The venous phase of the left pulmonary artery injection shows the anomalous vein (arrows).
Vol. 122
ANOMALOUS PULMONARY VENOUS CONNECTION
2. Blake HA, Hall RJ, Manion WC: Anomalous pulmonary venous return. Circulation 32:406-414, Sep 1965 3. Brody H: Drainage of the pulmonary veins into the right side of the heart. Arch Pathol 33:221-240, Feb 1942 4. Conant JS, Kurland LT, Dale G: Pulmonary tuberculosis associated with anomalous common left pulmonary vein entering the left innominate vein. J Thorac Surg 16:422-426, Aug 1947 5. Cooley DA, Mahaffey DE: Anomalous pulmonary venous drainage of entire left lung: report of a case with surgical correction. Ann Surg 142:986-991, Dec 1955 6. Dotter CT, Hardisty NM, Steinberg I: Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosedduring life by angiocardiography and cardiac catheterization. Am J Med Sci 218:31-36, Jul 1949 7. , Ehrenhaft JL, Theilen EO, Lawrence MS: The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 148:249-258, Aug 1958 8. Geraci JE, Kirklin JW: Transplantation of left anomalous pulmonary vein to left atrium: report of case. Proc Staff Meet Mayo Clin 28:472-475,26 Aug 1953 9. Gilman RA, Skowron CA, Musser BG, et al: Partial anomalous venous drainage. Am J Surg 94:688-694, Nov 1957 10. Healey JE Jr: An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg 23:433-444, May 1952 11. Hickie JB, Gimlette TMD, Bacon APC: Anomalous pulmonary venous drainage. Br Heart J 18:365-377, Jul 1956 12. Kalke BR, Carlson RG, Ferlic RM, et al: Partial anomalous pulmonary venous connections. Am J CardioI20:91-101, Jul 1967 13. Kirklin JW: Surgical.treatment of anomalous pulmonary venous connection (partial anomalous pulmonary venous drainage). Proc Staff Meet Mayo Clin 28:476-479,26 Aug 1953 14. Morrow AG, Awe WC, Aygen MM: Total unilateral anomalous pulmonary venous connection with intact atrial septum. Am J Cardiol 9:933-937, Jun 1962
595
Diagnostic Radiology
15. Muir AR: Anomalous pulmonary venous drainage. Thorax 8:65-68, Mar 1953 16. Nakib A, Moller JH, Kanjuh VI, et al: Anomalies of the pulmonary veins. Am J Cardiol 20:77-90, Jul 1967 17. Neirotti R, Gonzalez-Lavin L, Ross DN: Anomalous pulmonary venous connexions of left lung associated with valvular heart disease. Report of two cases. Br Heart J 34:969-973, Sep 1972 18. Samet P, Fierer EM, Berstein WH: Anomalous pulmonary venous drainage: diagnostic value of bronchospirometry. Am J Med 26:654-658, Oct 1958 19. Sepulveda G, Lukas DS, Steinberg I: Anomalous drainage of pulmonary veins; clinical, physiologic, and angiocardiographic features. Am J Med 18:883-899, Jun 1955 20. Shumacker HB Jr, Judd D: Partial anomalous pulmonary venous return with reference to drainage into the inferior vena cava and to an intact atrial septum. J Cardiovasc Surg 5:271-278, Jul-Aug 1964 21. Snellen HA, Albers FH: The clinical diagnosis of anomalous pulmonary venous drainage. Circulation 6:80 1-816, D~c 1952 22. Snellen HA, Bruins C: Anomalies of venous return. [In] Watson H, ed: Paediatric Cardiology. St Louis, Mosby, 1968, pp 416-426 23. Sos TA, Tay D, Levin AR, et al: Angiographic demonstration of the absence of an atrial septal defect in the presence of partial anomalous pulmonary venous connection. Am J Roentgenol 121: 591-596, Jul 1974 24. TaussigHB: Congenital malformations of the heart. New York, Harvard University, 2d ed, 1960, pp 536-580 25. Winslow J: cited by Brody
Department of Radiology Massachusetts General Hospital Boston, Mass. 02114
