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Three Unusual Complications Resulting from Attempted Repair of Partial Anomalous Pulmonary Venous Drainage
Three Unusual Complications Resulting from Attempted Repair of Partial Anomalous Pulmonary V·enous Drainage* LTC Paul D. Anderson, MC, USA;• • Stephen P. Glasser, M.D.;t Stephen Czarnecki, M.D.;t and Alan R. Hopeman, M.D., F.C.C.P.§
The correction of shunts resulting from partial anom• lous pulmonary venous drainage has become an accepted surgical procedure. Surgical compUcations, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual compUcations resulting from this type of surgery. Unilateral pulmonary venous obstruction andre-
J arge volume left-to-right shunts can occur in pa-
tients with partial anomalous pulmonary venous drainage whether or not there is an associated atrial septal defect. 1 Correction of partial anomalous pulmonary venous drainage has become an accepted and frequently attempted surgical procedure.2.3 Reported surgical complications have been rare and were usually postoperative. Tachyarrhythmias, temporary and permanent heart block, wound infection, pleural effusion, air embolism, bronchopleural fistula, and cardiac arrest have been noted. 4 A report by Friedli et al5 suggests that asymptomatic anatomic complications may be more common than previously recognized. The present report details three patients with unusual complications resulting from this type of surgery. In one patient, there were multiple pulmonary infections after surgery. Unilateral obstruction of the pulmonary veins was demonstrated, with apparent resultant pulmonary hypertension. In the second case a breakdown of the patch tunnel placed at the time of surgery ultimately resulted in obstruction to ~
•From the Cardiology and Thoracic Surgery Services, Walter Reed Army Medical Center, Washington, DC. · • 0 Presently Chief, Internal Medicine Service, Gorgas Hospital, Balboa Heights, Canal Zone. tPresently Associate Professor of Medicine and Chief, Cardiology Section, Louisiana State University Medical Center, Shreveport. !Presently Director of Cardiology, Edgewater Hospital, Chicago. §Presently Chief, Division of Cardiothoracic Surgery, Denver General Hospital, Denver. Manuscript received June 26; revision accepted September 19. Reprint requests: Dr. GIDsser, LSU School of Medicine, PO Box 3932, Shreveport 71130
384 ANDERSON ET AL
peated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an inadeqnate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.
the superior vena cava and diversion of its blood flow into the left atrium Exertional venous hypertension in the upper half of the body resulted. In the third patient, failure to recognize partial anomalous pulmonary venous drainage into the coronary sinus at the time of corrective surgery for "atrial septal defect" resulted in an inadequate operation necessitating further surgery. CASE REPORTS CASE
1
A seven-year-old boy was first seen when he was two years of age for the evaluation of a cardiac murmur. In the following year, cardiac catheterization revealed partial anomalous pulmonary venous drainage from the right lung with an intact atrial septum and modest pulmonary hypertension. When the patient was four years of age, a pericardia] tunnel was fashioned within the right atrium to divert blood from the anomalous pulmonary vein through a surgically induced atrial septal defect into the left atrium. The boy felt good during the following year and showed some resolution of the physical and electrocardiographic evidence of right ventricular hypertrophy; however, four episodes of right lower and middle lobe pneumonia were reported during his sixth year. Physical examination revealed the recurrence of findings of pulmonary hypertension. A second cardiac catheterization demonstrated both pulmonary hypertension and pulmonary venous obstruction of the right lung with no residual left-to-right shunt. At the time of additional corrective surgery, the entrance of the right pulmi:mary vein into the pericardia] tunnel (right atrium ) was found to be reduced to a 3 mm opening. The vein itself appeared to have been affected with thrombosis and recanalized. An attempt was made to enlarge this opening. The boy was seen six months later after having had further episodes of pneumonia. A third cardiac catheterization showed persistence of pulmo-
CHEST, 69: 3, MARCH, 1976
nary venous obstruction and little improvement in the pulmonary hypertension. Pulmonary angiographic studies showed delayed clearance of contrast media from the right lung, with normal clearance on the left.
Comment. In this case, obstruction to venous return occurred after surgery at the site of entrance of the partial anomalous pulmonary venous drainage into the right atrium. Apparently this was the result of exuberant scar-tissue formation in the area of attachment between the pericardia} patch and the atrial wall or of greater than normal shrinkage of the autogenous pericardium. It suggests that when this form of surgery is utilized, there should be a generous margin, if possible, between the patch and the entrance of the anomalous vein. Because the entrance of the right superior pulmonary vein is frequently near the sinus node, this may be impossible, in which case extra care should be taken to use a patch large enough to prevent tensional deformity of the venous orifice. CASE
2
A 38-year-old woman had symptoms of fatigue and headache on mild exertion. These symptoms were not progressive but had limited her physical activity for several years. At the age of 14 years, the patient's complaints of shortness of breath during effort had led to the discovery of a cardiac murmur. At 18 years of age, the patient underwent partial
right cardiac catheterization, which suggested the presence of an atrial septal defect. At the ages of 22 and 26 years, the patient carried successful pregnancies to term without difficulty. By age 27, the patient's dyspnea on exertion had worsened, and there were occasional episodes of hemoptysis. A repeat right cardiac catheterization documented the presence of partial anomalous pulmonary venous drainage. The pulmonary blood flow was twice as great as the systemic. At the time of surgical correction, partial anomalous pulmonary venous drainage of the right upper and middle lobes into the low superior vena cava was found, in addition to an ostium secundum atrial septal defect that was 2.5 em in diameter. A Teflon-patch tunnel was created to drain the partial anomalous pulmonary venous drainage from its entrance into the right atrium through the atrial septal defect into the left atrium. The atrial septal defect was slightly enlarged (Fig 1 ). The patient did well for approximately six months after surgery, at which time there was an abrupt return of exertional dyspnea and the onset of exertional headache. The electrocardiographic findings, which had shown a diastolic type of right ventricular hypertrophy, had resolved toward normal. Ten years later, at age 38 years, because of the persistence of the patient's symptoms, she was hospitalized for further study. Her physical examination revealed normal blood pressure and normal cervical venous pulse pressure. Cardiac examination revealed a grade 2/6 midsystolic ejection murmur along the left sternal border, as well as a questionable apical diastolic rumble. The electrocardiogram and chest xray film were believed to be normal. Cardiac catheterization revealed the initial mean pressure of the superior vena cava
FIGURE 1. Drawings represent cutaway view of right atrium from right side. (a) Before placement of patch, atrial septal defect ( ASD) was enlarged (E). Anomalous vein (AV) enters superior vena cava ( SVC) near the right atrium. Suturing ( S) patch to SVC creates tunnel separating flow of SVC from that of AV. Inferior vena cava (IVC) is normal. TV, Tricuspid valve. (b) Completely placed Teflon patch ( P) directs anomalous vein flow into widened ASD. ( c) Proposed mechanism of complication showing obstruction to superior vena caval blood flow by loosening of distal ( SVC) end with elevation of patch ( P) and thrombus ( T), so that blood flow from both SVC and anomalous veins is directed into left atrium. Area of obstruction probably exists at level of junction of SVC with right atrium.
CHEST, 69: 3, MARCH, 1976
ATIEMPTED REPAIR OF PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE 385
to be normal ( 4.5 mm Hg). The advancing catheter then directly entered the left atrium and left ventricle. The catheter was returned to the superior vena cava, and a pulmonary vein was entered. Only with great difficulty could the catheter finally be manipulated into the right atrium and then into the pulmonary artery. The right-sided pressures were normal. Pulmonary arterial hydrogen appearance time was normal. On pullback, there was a gradient of 3 to 4 mm Hg between the right atrium and the superior vena cava. The hydrogenappearance time in the superior vena cava was rapid, confirming a left-to-right shunt. There was no resting gradient between left ventricular end-diastolic pressure, left atrial pressure, and superior vena caval pressure. With exercise, there was a slight increase in the left ventricular end-diastolic pressure and left atrial pressure, and a gradient of 4 mm Hg now existed between them and the superior vena cava. The superior vena caval pressure had risen from 4.5 to 12 mm Hg. f:ineangiographic studies demonstrated that contrast material injected into the high superior vena cava entered into the left atrium through the Teflon tunnel. A filling defect, presumably the di.~placed patch or thrombosis at the base of the superior vena cava, was noted. Two large dilated azygos veins carried contrast material cephalocaudad below the diaphragm to enter the inferior vena cava. It was apparent that the normal direct entrance of the superior vena cava into the right atrium was functionally completely closed and that the superior vena caval drainage was largely into the left atrium although some collateral How to the inferior vena cava existed. The exertional headaches were evidently related to the elevation of the cephalic venous pressure with effort. The patient declined further surgical correction.
Comment. In this case the partial anomalous pulmonary venous drainage was into the superior vena cava and required atrial plication and a Teflon patch tunnel to direct the flow from the anomalous pulmonary vein into the left atrium. Apparently, loosening and buckling or thrombosis of the superior vena cava end of the patch occurred, so that superior vena caval blood Bow was excluded from the right atrium except by collateral channels. Figure 1 demonstrates the now-abandoned technique and proposed mechanism of complications. In one report, significant narrowing or occlusion of the superior vena cava occurred in four of five patients studied after surgery with similar anatomic types of partial anomalous pulmonary venous return, in spite of the fact that all patients were asymptomatic at the time of study. 5 A patch plus plication almost always results in a patch that is too small when the plicative sutures come loose, an event which is common since these sutures are under tension. As mentioned, we have abandoned the use of plication of the superior vena cava for correction of partial anomalous pulmonary venous drainage because of technical problems. Several modified techniques are now utilized in the correction of this defect. 6•7 Another interesting feature in this case was that some degree of obstruction was also present between the superior vena cava and the left atrium. Although there were equal pressures at rest, mild
386 ANDERSON ET AL
exercise produced a measurable gradient. Furthermore, the patient noted the typical onset of headache during maximal venous pressure. CASE
3
A 23-year-old active-duty infantry officer in excellent health was found to have a heart murmur during evaluation of an upper respiratory infection. A cardiac consultant recommended cardiac catheterization after finding a persistently split second sound, a right ventricular ejection murmur, and delayed activation of the right ventricle on the ECG. Catheterization at another institution demonstrated a 2:1 leftto-right shunt at the atrial level. The absence o£ evidence of anomalous pulmonary venous drainage was specifically mentioned. At surgery, closure of an atrial septal defect that was 1 em in diameter within the fossa ovalis was accomplished by direct suture. At the time of surgery, a large posterior defect resembling a third atrial chamber was found in the atrial septum. In retrospect, this was a giant coronary-sinus ostium. The decision not to close this defect was reached at the surgical table. Bypass time was 75 minutes. A stormy postoperative course included coma, seizures, left hemiparesis, transient organic psychosis, and permanent personality changes. Two months later, because of a residual murmur and limited exercise ability, the patient underwent a repeat catheterization. It revealed a persistent 1.7/1.0 left-to-right shunt at the atrial level. A recurrent or residual atrial septal defect was postulated, and repeat surgery was advised. The patient then transferred to Walter Reed Army Medical Center, Washington, D.C. A third catheterization was performed in order to have more complete hemodynamic and angiographic information prior to the proposed surgery. Oxygen saturations confirmed a 1.6/1.0 left-to-right shunt at the atrial level. Dye curves with indocyanine green ( Cardio-Green) suggested intact atrial and ventricular septa with the shunt occurring entirely through the left lung. The venous drainage of the right lung appeared to be normal. Cut-film angiocardiography performed within the greatly enlarged coronary sinus demonstrated the connections of the partial anomalous pulmonary venous drainage from the entire left lung. Further surgery was performed using a pericardia) patch to redirect the entire coronary-sinus blood How through an artificial atrial septal defect into the left atrium. The site of the previously closed atrial septal defect was noted. Unfortunately, the permanent residual neurologic defects have terminated this previously asymptomatic young officer's activeduty career, even though findings from his present physical examination are normal in regard to the cardiovascular system.
Comment. Failure to recognize partial anomalous pulmonary drainage through a giant coronary sinus resulted in failure of correction at the time of surgery, a prolonged bypass time, and a need for a second operation. A previously reported patient with this type of defect had an even more tragic course. 8 The enlarged coronary sinus was mistaken for the atrial septal defect, and suture closure resulted in an operative failure. The presence of a greatly enlarged coronary sinus in the setting of surgery for atrial septal defect should suggest the possibility of partial anomalous CHEST, 69: 3, MARCH, 1976
pulmonary venous drainage. At the time of repairing an atrial septal defect, most cardiac surgeons explore the right atrium, particularly the right upper posterior margin, for partial anomalous pulmonary venous drainage. The necessity of considering the anatomy of the coronary sinus is less well appreciated. In general, about 90 percent of partial anomalous pulmonary venous drainage is solely or largely from the right lung, particularly the upper and middle lobes. Of the 10 percent of partial anomalous pulmonary venous drainage from the left lung, most is through a form of persistent cardinal system ( vertical vein) to the left innominate vein and superior vena cava. The pattern of partial anomalous pulmonary venous drainage into the coronary sinus is quite unusual, even though it represents the pattern in 20 percent of patients with total anomalous pulmonary venous retum. 9 One of the few fatalities reported in the medical literature relating to correction of partial anomalous pulmonary venous drainage resulted from failure to recognize this anatomic variant. 8 CoNCLUSION
While the literature is replete with description of the various technical maneuvers to correct the assorted types of partial anomalous pulmonary venous drainage, little is mentioned of the surgical complications. This report describes the complications resulting from this type of surgery in three patients seen at one institution in a two-year period. The
conditions represent significant and disabling complications resulting from repair of what is considered by some to be a minor congenital cardiac abnormality that is easily repaired. Nonetheless, the variety and degree of postoperative complications were unusual. The indications for surgical repair of partial anomalous pulmonary venous drainage must be balanced against these and other potential postoperative complications. REFERENCES
.1 Sepulveda G, Lukas DS, Steinberg I: Anomalous drainage of pulmonary veins. Am J Med 18:883-899, 1955 2 Blake HA, Hall RJ, Manion WC: Anomalous pulmonary venous return. Circulation 32:406-414, 1965 3 Friedberg CF: Disease of the Heart (3rd ed). Philadelphia, WB Saunders Co, 1966, p 1215 :' Kalke BR, Carlson RG, Ferlic RM, et al: Partial anomalous pulmonary venous connections. Am J Cardiol 20:91-101, 1967 5 Friedli 8, Guerin R, Davignon A, et al: Surgical treatment of partial anomalous pulmonary venous drainage. Circulation 45:159-170, 1972 6 Puig-Massana M, Murtra M, Revuelta JM: A new technique in the correction of partial anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 64:108-113, 1972 7 Robicsek F, Sanger PW, Daugherty HK: Surgical treatment of partial anomalous pulmonary venous return into the superior vena cava: A technical modification. Am Surg 169:305-307, 1969 8 Sherman FE: An Atlas of Congenital Heart Disease. Philadephia, Lea and Febiger, 1963, p 42 9 Gathman GE, Nadas AS: Total anomalous pulmonary venous connection. Circulation 42:143-154, 1970
ANNOUNCEMENT 15th Inhalation and Respiratory Therapy Course Under the direction of Dr. Maurice S. Segal, Tufts University School of Medicine will present the 15th Inhalation and Respiratory Therapy Course at Howard Johnson's Motor Hotel, 57 Park Plaza, Boston,
CHEST, 69: 3, MARCH, 1976
April 19-23. For information contact Course Coordinator for Continuing Education, Box 72, 136 Harrison Avenue, Boston 02111.
ATTEMPTED REPAIR OF PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE 387
The correction of shunts resulting from partial anom• lous pulmonary venous drainage has become an accepted surgical procedure. Surgical compUcations, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual compUcations resulting from this type of surgery. Unilateral pulmonary venous obstruction andre-
J arge volume left-to-right shunts can occur in pa-
tients with partial anomalous pulmonary venous drainage whether or not there is an associated atrial septal defect. 1 Correction of partial anomalous pulmonary venous drainage has become an accepted and frequently attempted surgical procedure.2.3 Reported surgical complications have been rare and were usually postoperative. Tachyarrhythmias, temporary and permanent heart block, wound infection, pleural effusion, air embolism, bronchopleural fistula, and cardiac arrest have been noted. 4 A report by Friedli et al5 suggests that asymptomatic anatomic complications may be more common than previously recognized. The present report details three patients with unusual complications resulting from this type of surgery. In one patient, there were multiple pulmonary infections after surgery. Unilateral obstruction of the pulmonary veins was demonstrated, with apparent resultant pulmonary hypertension. In the second case a breakdown of the patch tunnel placed at the time of surgery ultimately resulted in obstruction to ~
•From the Cardiology and Thoracic Surgery Services, Walter Reed Army Medical Center, Washington, DC. · • 0 Presently Chief, Internal Medicine Service, Gorgas Hospital, Balboa Heights, Canal Zone. tPresently Associate Professor of Medicine and Chief, Cardiology Section, Louisiana State University Medical Center, Shreveport. !Presently Director of Cardiology, Edgewater Hospital, Chicago. §Presently Chief, Division of Cardiothoracic Surgery, Denver General Hospital, Denver. Manuscript received June 26; revision accepted September 19. Reprint requests: Dr. GIDsser, LSU School of Medicine, PO Box 3932, Shreveport 71130
384 ANDERSON ET AL
peated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an inadeqnate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.
the superior vena cava and diversion of its blood flow into the left atrium Exertional venous hypertension in the upper half of the body resulted. In the third patient, failure to recognize partial anomalous pulmonary venous drainage into the coronary sinus at the time of corrective surgery for "atrial septal defect" resulted in an inadequate operation necessitating further surgery. CASE REPORTS CASE
1
A seven-year-old boy was first seen when he was two years of age for the evaluation of a cardiac murmur. In the following year, cardiac catheterization revealed partial anomalous pulmonary venous drainage from the right lung with an intact atrial septum and modest pulmonary hypertension. When the patient was four years of age, a pericardia] tunnel was fashioned within the right atrium to divert blood from the anomalous pulmonary vein through a surgically induced atrial septal defect into the left atrium. The boy felt good during the following year and showed some resolution of the physical and electrocardiographic evidence of right ventricular hypertrophy; however, four episodes of right lower and middle lobe pneumonia were reported during his sixth year. Physical examination revealed the recurrence of findings of pulmonary hypertension. A second cardiac catheterization demonstrated both pulmonary hypertension and pulmonary venous obstruction of the right lung with no residual left-to-right shunt. At the time of additional corrective surgery, the entrance of the right pulmi:mary vein into the pericardia] tunnel (right atrium ) was found to be reduced to a 3 mm opening. The vein itself appeared to have been affected with thrombosis and recanalized. An attempt was made to enlarge this opening. The boy was seen six months later after having had further episodes of pneumonia. A third cardiac catheterization showed persistence of pulmo-
CHEST, 69: 3, MARCH, 1976
nary venous obstruction and little improvement in the pulmonary hypertension. Pulmonary angiographic studies showed delayed clearance of contrast media from the right lung, with normal clearance on the left.
Comment. In this case, obstruction to venous return occurred after surgery at the site of entrance of the partial anomalous pulmonary venous drainage into the right atrium. Apparently this was the result of exuberant scar-tissue formation in the area of attachment between the pericardia} patch and the atrial wall or of greater than normal shrinkage of the autogenous pericardium. It suggests that when this form of surgery is utilized, there should be a generous margin, if possible, between the patch and the entrance of the anomalous vein. Because the entrance of the right superior pulmonary vein is frequently near the sinus node, this may be impossible, in which case extra care should be taken to use a patch large enough to prevent tensional deformity of the venous orifice. CASE
2
A 38-year-old woman had symptoms of fatigue and headache on mild exertion. These symptoms were not progressive but had limited her physical activity for several years. At the age of 14 years, the patient's complaints of shortness of breath during effort had led to the discovery of a cardiac murmur. At 18 years of age, the patient underwent partial
right cardiac catheterization, which suggested the presence of an atrial septal defect. At the ages of 22 and 26 years, the patient carried successful pregnancies to term without difficulty. By age 27, the patient's dyspnea on exertion had worsened, and there were occasional episodes of hemoptysis. A repeat right cardiac catheterization documented the presence of partial anomalous pulmonary venous drainage. The pulmonary blood flow was twice as great as the systemic. At the time of surgical correction, partial anomalous pulmonary venous drainage of the right upper and middle lobes into the low superior vena cava was found, in addition to an ostium secundum atrial septal defect that was 2.5 em in diameter. A Teflon-patch tunnel was created to drain the partial anomalous pulmonary venous drainage from its entrance into the right atrium through the atrial septal defect into the left atrium. The atrial septal defect was slightly enlarged (Fig 1 ). The patient did well for approximately six months after surgery, at which time there was an abrupt return of exertional dyspnea and the onset of exertional headache. The electrocardiographic findings, which had shown a diastolic type of right ventricular hypertrophy, had resolved toward normal. Ten years later, at age 38 years, because of the persistence of the patient's symptoms, she was hospitalized for further study. Her physical examination revealed normal blood pressure and normal cervical venous pulse pressure. Cardiac examination revealed a grade 2/6 midsystolic ejection murmur along the left sternal border, as well as a questionable apical diastolic rumble. The electrocardiogram and chest xray film were believed to be normal. Cardiac catheterization revealed the initial mean pressure of the superior vena cava
FIGURE 1. Drawings represent cutaway view of right atrium from right side. (a) Before placement of patch, atrial septal defect ( ASD) was enlarged (E). Anomalous vein (AV) enters superior vena cava ( SVC) near the right atrium. Suturing ( S) patch to SVC creates tunnel separating flow of SVC from that of AV. Inferior vena cava (IVC) is normal. TV, Tricuspid valve. (b) Completely placed Teflon patch ( P) directs anomalous vein flow into widened ASD. ( c) Proposed mechanism of complication showing obstruction to superior vena caval blood flow by loosening of distal ( SVC) end with elevation of patch ( P) and thrombus ( T), so that blood flow from both SVC and anomalous veins is directed into left atrium. Area of obstruction probably exists at level of junction of SVC with right atrium.
CHEST, 69: 3, MARCH, 1976
ATIEMPTED REPAIR OF PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE 385
to be normal ( 4.5 mm Hg). The advancing catheter then directly entered the left atrium and left ventricle. The catheter was returned to the superior vena cava, and a pulmonary vein was entered. Only with great difficulty could the catheter finally be manipulated into the right atrium and then into the pulmonary artery. The right-sided pressures were normal. Pulmonary arterial hydrogen appearance time was normal. On pullback, there was a gradient of 3 to 4 mm Hg between the right atrium and the superior vena cava. The hydrogenappearance time in the superior vena cava was rapid, confirming a left-to-right shunt. There was no resting gradient between left ventricular end-diastolic pressure, left atrial pressure, and superior vena caval pressure. With exercise, there was a slight increase in the left ventricular end-diastolic pressure and left atrial pressure, and a gradient of 4 mm Hg now existed between them and the superior vena cava. The superior vena caval pressure had risen from 4.5 to 12 mm Hg. f:ineangiographic studies demonstrated that contrast material injected into the high superior vena cava entered into the left atrium through the Teflon tunnel. A filling defect, presumably the di.~placed patch or thrombosis at the base of the superior vena cava, was noted. Two large dilated azygos veins carried contrast material cephalocaudad below the diaphragm to enter the inferior vena cava. It was apparent that the normal direct entrance of the superior vena cava into the right atrium was functionally completely closed and that the superior vena caval drainage was largely into the left atrium although some collateral How to the inferior vena cava existed. The exertional headaches were evidently related to the elevation of the cephalic venous pressure with effort. The patient declined further surgical correction.
Comment. In this case the partial anomalous pulmonary venous drainage was into the superior vena cava and required atrial plication and a Teflon patch tunnel to direct the flow from the anomalous pulmonary vein into the left atrium. Apparently, loosening and buckling or thrombosis of the superior vena cava end of the patch occurred, so that superior vena caval blood Bow was excluded from the right atrium except by collateral channels. Figure 1 demonstrates the now-abandoned technique and proposed mechanism of complications. In one report, significant narrowing or occlusion of the superior vena cava occurred in four of five patients studied after surgery with similar anatomic types of partial anomalous pulmonary venous return, in spite of the fact that all patients were asymptomatic at the time of study. 5 A patch plus plication almost always results in a patch that is too small when the plicative sutures come loose, an event which is common since these sutures are under tension. As mentioned, we have abandoned the use of plication of the superior vena cava for correction of partial anomalous pulmonary venous drainage because of technical problems. Several modified techniques are now utilized in the correction of this defect. 6•7 Another interesting feature in this case was that some degree of obstruction was also present between the superior vena cava and the left atrium. Although there were equal pressures at rest, mild
386 ANDERSON ET AL
exercise produced a measurable gradient. Furthermore, the patient noted the typical onset of headache during maximal venous pressure. CASE
3
A 23-year-old active-duty infantry officer in excellent health was found to have a heart murmur during evaluation of an upper respiratory infection. A cardiac consultant recommended cardiac catheterization after finding a persistently split second sound, a right ventricular ejection murmur, and delayed activation of the right ventricle on the ECG. Catheterization at another institution demonstrated a 2:1 leftto-right shunt at the atrial level. The absence o£ evidence of anomalous pulmonary venous drainage was specifically mentioned. At surgery, closure of an atrial septal defect that was 1 em in diameter within the fossa ovalis was accomplished by direct suture. At the time of surgery, a large posterior defect resembling a third atrial chamber was found in the atrial septum. In retrospect, this was a giant coronary-sinus ostium. The decision not to close this defect was reached at the surgical table. Bypass time was 75 minutes. A stormy postoperative course included coma, seizures, left hemiparesis, transient organic psychosis, and permanent personality changes. Two months later, because of a residual murmur and limited exercise ability, the patient underwent a repeat catheterization. It revealed a persistent 1.7/1.0 left-to-right shunt at the atrial level. A recurrent or residual atrial septal defect was postulated, and repeat surgery was advised. The patient then transferred to Walter Reed Army Medical Center, Washington, D.C. A third catheterization was performed in order to have more complete hemodynamic and angiographic information prior to the proposed surgery. Oxygen saturations confirmed a 1.6/1.0 left-to-right shunt at the atrial level. Dye curves with indocyanine green ( Cardio-Green) suggested intact atrial and ventricular septa with the shunt occurring entirely through the left lung. The venous drainage of the right lung appeared to be normal. Cut-film angiocardiography performed within the greatly enlarged coronary sinus demonstrated the connections of the partial anomalous pulmonary venous drainage from the entire left lung. Further surgery was performed using a pericardia) patch to redirect the entire coronary-sinus blood How through an artificial atrial septal defect into the left atrium. The site of the previously closed atrial septal defect was noted. Unfortunately, the permanent residual neurologic defects have terminated this previously asymptomatic young officer's activeduty career, even though findings from his present physical examination are normal in regard to the cardiovascular system.
Comment. Failure to recognize partial anomalous pulmonary drainage through a giant coronary sinus resulted in failure of correction at the time of surgery, a prolonged bypass time, and a need for a second operation. A previously reported patient with this type of defect had an even more tragic course. 8 The enlarged coronary sinus was mistaken for the atrial septal defect, and suture closure resulted in an operative failure. The presence of a greatly enlarged coronary sinus in the setting of surgery for atrial septal defect should suggest the possibility of partial anomalous CHEST, 69: 3, MARCH, 1976
pulmonary venous drainage. At the time of repairing an atrial septal defect, most cardiac surgeons explore the right atrium, particularly the right upper posterior margin, for partial anomalous pulmonary venous drainage. The necessity of considering the anatomy of the coronary sinus is less well appreciated. In general, about 90 percent of partial anomalous pulmonary venous drainage is solely or largely from the right lung, particularly the upper and middle lobes. Of the 10 percent of partial anomalous pulmonary venous drainage from the left lung, most is through a form of persistent cardinal system ( vertical vein) to the left innominate vein and superior vena cava. The pattern of partial anomalous pulmonary venous drainage into the coronary sinus is quite unusual, even though it represents the pattern in 20 percent of patients with total anomalous pulmonary venous retum. 9 One of the few fatalities reported in the medical literature relating to correction of partial anomalous pulmonary venous drainage resulted from failure to recognize this anatomic variant. 8 CoNCLUSION
While the literature is replete with description of the various technical maneuvers to correct the assorted types of partial anomalous pulmonary venous drainage, little is mentioned of the surgical complications. This report describes the complications resulting from this type of surgery in three patients seen at one institution in a two-year period. The
conditions represent significant and disabling complications resulting from repair of what is considered by some to be a minor congenital cardiac abnormality that is easily repaired. Nonetheless, the variety and degree of postoperative complications were unusual. The indications for surgical repair of partial anomalous pulmonary venous drainage must be balanced against these and other potential postoperative complications. REFERENCES
.1 Sepulveda G, Lukas DS, Steinberg I: Anomalous drainage of pulmonary veins. Am J Med 18:883-899, 1955 2 Blake HA, Hall RJ, Manion WC: Anomalous pulmonary venous return. Circulation 32:406-414, 1965 3 Friedberg CF: Disease of the Heart (3rd ed). Philadelphia, WB Saunders Co, 1966, p 1215 :' Kalke BR, Carlson RG, Ferlic RM, et al: Partial anomalous pulmonary venous connections. Am J Cardiol 20:91-101, 1967 5 Friedli 8, Guerin R, Davignon A, et al: Surgical treatment of partial anomalous pulmonary venous drainage. Circulation 45:159-170, 1972 6 Puig-Massana M, Murtra M, Revuelta JM: A new technique in the correction of partial anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 64:108-113, 1972 7 Robicsek F, Sanger PW, Daugherty HK: Surgical treatment of partial anomalous pulmonary venous return into the superior vena cava: A technical modification. Am Surg 169:305-307, 1969 8 Sherman FE: An Atlas of Congenital Heart Disease. Philadephia, Lea and Febiger, 1963, p 42 9 Gathman GE, Nadas AS: Total anomalous pulmonary venous connection. Circulation 42:143-154, 1970
ANNOUNCEMENT 15th Inhalation and Respiratory Therapy Course Under the direction of Dr. Maurice S. Segal, Tufts University School of Medicine will present the 15th Inhalation and Respiratory Therapy Course at Howard Johnson's Motor Hotel, 57 Park Plaza, Boston,
CHEST, 69: 3, MARCH, 1976
April 19-23. For information contact Course Coordinator for Continuing Education, Box 72, 136 Harrison Avenue, Boston 02111.
ATTEMPTED REPAIR OF PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE 387