COLLECTIVE REVIEW
Concepts and Practices in Surgery for Total Anomalous Pulmonary Venous Connection Nevin M. Katz, M.D., John W. Kirklin, M.D., and Albert D. Pacifico, M.D. ABSTRACT In the last ten years there have been extensive refinements in the surgical approach to total anomalous pulmonary venous connection (TAPVC).This communication reviews determinants of hospital mortality and controversies in surgical management. As examples of current experience with repair of TAPVC, recent results at the University of Alabama and a combined analysis of hospital mortality for Alabama, Children's Hospital Medical Center in Boston, and Green Lane Hospital are reported. From July, 1974, to July, 1977, the Alabama hospital mortality among the 17 infants with supracardiac, cardiac, and infracardiac TAPVC was 12% (2 infants).
Early in the experience with correction of total anomalous pulmonary venous connection (TAPVC), hospital mortality was high, especially in infants [4,13,31,321. In the last ten years a substantial improvement in results has been documented [l,2,7,12,21,24]. This communication reports, as an example of the current experiences of many centers, a recent series of patients who underwent repair of TAPVC at the University of Alabama Medical Center and a combined analysis of hospital mortality based on the Alabama results and those at Children's Hospital Medical Center in Boston and Green Lane Hospital, Auckland, New Zealand. Some determinants of surgical results and current operative approaches are discussed.
Alabama Series Nineteen patients underwent operation during the period July 1, 1974, to July 1, 1977, at the University of Alabama Medical Center. Results prior to July 1,1974, have been reported by ApFrom the Department of Surgery, University of Alabama School of Medicine and Medical Center, Birmingham, AL. We appreciate the editorial and secretarial skills of Ms. Sandy OBrien. Dr. Eugene H . Blackstone provided scientific and statistical advice. Address reprint requests to Dr. Kirklin, Department of Surgery, University Station, Birmingham, AL 35294. 479 0003-4975/78/0025-0521$1.50 @ 1978 by Nevin M. Katz
pelbaum and associates [l].The range of ages at operation in the current series was 10 hours to 10 months with a mean of 2.0 months. The anomalous connection was supracardiac in 10 patients, cardiac in 3, infracardiac in 4, and mixed in 2. The overall hospital mortality for supracardiac, cardiac, and infracardiac TAPVC was 12% (2 patients) (Table 1). The results from September 1, 1971, to July 1, 1977 (excluding those patients with mixed venous drainage) are presented in Table 2. The recent Alabama results as related to preoperative condition are presented in Table 3. Patients designated "critical" are those whose preoperative condition was so poor that emergency operation was done. Two of the 4 patients requiring preoperative emergency endotracheal intubation on admission to the hospital died; there were no deaths among those not requiring intubation preoperatively (p = 0.04).* Pulmonary artery hypertension (systolic pulmonary artery pressure > 60 mm Hg or pulmonarylsystemic systolic pressure ratio > 0.8) was not a determinant of survival (p = 0.4). Cardiopulmonary bypass with profound hypothermia and a period of total circulatory arrest was used in all cases. Surface cooling to 28" to 30" C before the chest was opened and further cooling to 20°C by the pump oxygenator were employed in 7 patients with 2 deaths; 10 patients had cooling by the perfusate alone after cardiopulmonary bypass was started, with no deaths ( p = 0.15). The duration of circulatory arrest ranged from 22 to 62 minutes with a mean of 41 minutes ( f 1 2 minutes). The duration of total circulatory arrest (< or > 45 minutes) was not a determinant of survival (p = 0.3). The last 3 patients in this group had cold cardioplegic myocardial preservation in addition to the other modalities. None received catecholamines; in contrast, catecholamine support was used in 9 of the previous 14 patients without *All p values were determined by Fisher's exact test.
480 The Annals of Thoracic Surgery Vol 25 No 5 May 1978
Table 1 . Mortality among Patients Undergoing Repair of Total Anomalous Pulmonary Venous Connection (Alabama, July, 1974-July, 1977)a*b
1.6 months old required reoperation 24 hours after initial repair because the inferior vena cava had been diverted to the left atrium by closure of the atrial septa1 defect. He survived. Three patients had late reoperations. One infant underwent repair of an anastomotic stricture 1.8 months after the initial correction and died from low cardiac output. This was the only late death in the series. Postmortem examination was not obtained. Another infant underwent reoperation 2.1 months after the initial procedure because of pulmonary venous stenosis, which was relieved by excision of thickened endocardium from the pulmonary vein ostia. The anastomosis itself was not stenotic. One infant underwent late reexploration (at 7.9 months of age, 5.4 months after repair) because of recent onset of fluid retention and hepatomegaly associated with severe pulmonary artery hypertension. The anastomosis and pulmonary veins were not stenotic, and the patient's clinical state was believed to be due to pulmonary vascular disease. One infant underwent recatheterization at 3.5 months of age (1.0 month after operation) because of tachypnea. Pulmonary artery pressure was 32/10 mm Hg, and the anastomosis was not constricted. The left ventricle was moderately large, and the patient was thought to have left ventricular dysfunction. The patient was treated with digoxin. Two patients in the recent Alabama series had TAPVC of the mixed type; neither survived. One 3.5-month-old infant had obstructed anomalous pulmonary venous connections to the superior vena cava, azygos vein, and coro-
Hospital Deaths
No. of Patients
Age fmo)
No.
YO
70 Yo CL
3 >3< 6 >6 l C
aExclusiveof 1 patient 3 weeks old and another 3.5 months old, both with mixed type, both of whom died. Included are 10 with supracardiac,3 with intracardiac,and 4 (no deaths) with infracardiac drainage. bDates start immediately after the series reported by Appelbaum and colleagues [I]. CL = confidence limits.
cardioplegia (p = 0.08). An average of three determinations of cardiac index was made for each patient during the first 6 hours after operation. The average mean value of these indices for the patients who had cold cardioplegic myocardial preservation was 2.21 (f0.37), compared with 1.58 (k0.54) for the patients without cardioplegia ( p = 0.08). The left atrium was enlarged surgically in 10 patients, 1 of whom died. One death occurred among the 7 patients in whom the left atrium was not enlarged (p = 0.7). All the hospital deaths in the recent Alabama series were from low cardiac output. No patient required reoperation for bleeding. One infant
Table 2 . Experience with Repair of Total Anomalous Pulmonary Venous Connection at the University of Alabama since September 1 , 1971a
Age Croup (ma)
1971 (Sept 1)
1972
1973
1974
No. Deaths
No. Deaths
No. Deaths
No. Deaths ~
3 P 3 < 6 P1
Concepts and Practices in Surgery for Total Anomalous Pulmonary Venous Connection Nevin M. Katz, M.D., John W. Kirklin, M.D., and Albert D. Pacifico, M.D. ABSTRACT In the last ten years there have been extensive refinements in the surgical approach to total anomalous pulmonary venous connection (TAPVC).This communication reviews determinants of hospital mortality and controversies in surgical management. As examples of current experience with repair of TAPVC, recent results at the University of Alabama and a combined analysis of hospital mortality for Alabama, Children's Hospital Medical Center in Boston, and Green Lane Hospital are reported. From July, 1974, to July, 1977, the Alabama hospital mortality among the 17 infants with supracardiac, cardiac, and infracardiac TAPVC was 12% (2 infants).
Early in the experience with correction of total anomalous pulmonary venous connection (TAPVC), hospital mortality was high, especially in infants [4,13,31,321. In the last ten years a substantial improvement in results has been documented [l,2,7,12,21,24]. This communication reports, as an example of the current experiences of many centers, a recent series of patients who underwent repair of TAPVC at the University of Alabama Medical Center and a combined analysis of hospital mortality based on the Alabama results and those at Children's Hospital Medical Center in Boston and Green Lane Hospital, Auckland, New Zealand. Some determinants of surgical results and current operative approaches are discussed.
Alabama Series Nineteen patients underwent operation during the period July 1, 1974, to July 1, 1977, at the University of Alabama Medical Center. Results prior to July 1,1974, have been reported by ApFrom the Department of Surgery, University of Alabama School of Medicine and Medical Center, Birmingham, AL. We appreciate the editorial and secretarial skills of Ms. Sandy OBrien. Dr. Eugene H . Blackstone provided scientific and statistical advice. Address reprint requests to Dr. Kirklin, Department of Surgery, University Station, Birmingham, AL 35294. 479 0003-4975/78/0025-0521$1.50 @ 1978 by Nevin M. Katz
pelbaum and associates [l].The range of ages at operation in the current series was 10 hours to 10 months with a mean of 2.0 months. The anomalous connection was supracardiac in 10 patients, cardiac in 3, infracardiac in 4, and mixed in 2. The overall hospital mortality for supracardiac, cardiac, and infracardiac TAPVC was 12% (2 patients) (Table 1). The results from September 1, 1971, to July 1, 1977 (excluding those patients with mixed venous drainage) are presented in Table 2. The recent Alabama results as related to preoperative condition are presented in Table 3. Patients designated "critical" are those whose preoperative condition was so poor that emergency operation was done. Two of the 4 patients requiring preoperative emergency endotracheal intubation on admission to the hospital died; there were no deaths among those not requiring intubation preoperatively (p = 0.04).* Pulmonary artery hypertension (systolic pulmonary artery pressure > 60 mm Hg or pulmonarylsystemic systolic pressure ratio > 0.8) was not a determinant of survival (p = 0.4). Cardiopulmonary bypass with profound hypothermia and a period of total circulatory arrest was used in all cases. Surface cooling to 28" to 30" C before the chest was opened and further cooling to 20°C by the pump oxygenator were employed in 7 patients with 2 deaths; 10 patients had cooling by the perfusate alone after cardiopulmonary bypass was started, with no deaths ( p = 0.15). The duration of circulatory arrest ranged from 22 to 62 minutes with a mean of 41 minutes ( f 1 2 minutes). The duration of total circulatory arrest (< or > 45 minutes) was not a determinant of survival (p = 0.3). The last 3 patients in this group had cold cardioplegic myocardial preservation in addition to the other modalities. None received catecholamines; in contrast, catecholamine support was used in 9 of the previous 14 patients without *All p values were determined by Fisher's exact test.
480 The Annals of Thoracic Surgery Vol 25 No 5 May 1978
Table 1 . Mortality among Patients Undergoing Repair of Total Anomalous Pulmonary Venous Connection (Alabama, July, 1974-July, 1977)a*b
1.6 months old required reoperation 24 hours after initial repair because the inferior vena cava had been diverted to the left atrium by closure of the atrial septa1 defect. He survived. Three patients had late reoperations. One infant underwent repair of an anastomotic stricture 1.8 months after the initial correction and died from low cardiac output. This was the only late death in the series. Postmortem examination was not obtained. Another infant underwent reoperation 2.1 months after the initial procedure because of pulmonary venous stenosis, which was relieved by excision of thickened endocardium from the pulmonary vein ostia. The anastomosis itself was not stenotic. One infant underwent late reexploration (at 7.9 months of age, 5.4 months after repair) because of recent onset of fluid retention and hepatomegaly associated with severe pulmonary artery hypertension. The anastomosis and pulmonary veins were not stenotic, and the patient's clinical state was believed to be due to pulmonary vascular disease. One infant underwent recatheterization at 3.5 months of age (1.0 month after operation) because of tachypnea. Pulmonary artery pressure was 32/10 mm Hg, and the anastomosis was not constricted. The left ventricle was moderately large, and the patient was thought to have left ventricular dysfunction. The patient was treated with digoxin. Two patients in the recent Alabama series had TAPVC of the mixed type; neither survived. One 3.5-month-old infant had obstructed anomalous pulmonary venous connections to the superior vena cava, azygos vein, and coro-
Hospital Deaths
No. of Patients
Age fmo)
No.
YO
70 Yo CL
3 >3< 6 >6 l C
aExclusiveof 1 patient 3 weeks old and another 3.5 months old, both with mixed type, both of whom died. Included are 10 with supracardiac,3 with intracardiac,and 4 (no deaths) with infracardiac drainage. bDates start immediately after the series reported by Appelbaum and colleagues [I]. CL = confidence limits.
cardioplegia (p = 0.08). An average of three determinations of cardiac index was made for each patient during the first 6 hours after operation. The average mean value of these indices for the patients who had cold cardioplegic myocardial preservation was 2.21 (f0.37), compared with 1.58 (k0.54) for the patients without cardioplegia ( p = 0.08). The left atrium was enlarged surgically in 10 patients, 1 of whom died. One death occurred among the 7 patients in whom the left atrium was not enlarged (p = 0.7). All the hospital deaths in the recent Alabama series were from low cardiac output. No patient required reoperation for bleeding. One infant
Table 2 . Experience with Repair of Total Anomalous Pulmonary Venous Connection at the University of Alabama since September 1 , 1971a
Age Croup (ma)
1971 (Sept 1)
1972
1973
1974
No. Deaths
No. Deaths
No. Deaths
No. Deaths ~
3 P 3 < 6 P1
