The Blalock-Hanlon Procedure

Transcription

1 The Blalock-Hanlon Procedure A New Look at an Old Operation Douglas M. Behrendt, M.D., Marvin M. Kirsh, M.D., Mark B. Orringer, M.D., Burton Perry, M.D., Joan Sigmann, M.D., Aaron Stern, M.D., and Herbert Sloan, M.D. ABSTRACT The Blalock-Hanlon procedure has been carried out in 48 neonates with early deaths (2%). This compares favorably with the reported early mortality for balloon atrial septostomy. Most striking have been the length of palliation, freedom from complications, and low incidence of late death among these patients. In contrast, reported series of balloon septostomies have shown a high incidence of strokes, repeat hospitalization, inadequate palliation, and late deaths. Since palliation is reliable following a Blalock-Hanlon procedure, we do not favor early correction with hypothermic arrest but recommend it at 2 to 8 months of age, when conventional perfusion can be easily utilized. T ransposition of the great arteries (TGA) was an essentially untreatable, rapidly fatal anomaly until Blalock and Hanlon devised the atrial septectomy operation in 95 to improve intracardiac mixing [2]. This operation for the first time allowed newborns with transposition and poor mixing to survive infancy and become candidates for later correction. Subsequently, other palliative operations have been devised, such as open atrial septectomy with inflow occlusion with [7] or without [8] hyperbaric oxygenation. In spite of its successes, the Blalock-Hanlon procedure (BH) and other types of surgical atrial septectomy have been criticized on several counts: () the mortality rate is excessive; (2) the pericardial space develops adhesions postoperatively that lead to excessive bleeding and thicken the pericardium, making it less suitable for use as a patch in the final repair; (3) the atrial conduction pathways are disturbed, leading to a high incidence of postoperative arrhythmias; (4) infants who survive continue to face the risk of cerebral thrombosis or embolus; and (5) many infants die in the interval between septectomy and ultimate correction. The Rashkind balloon atrial septectomy (BAS)[9 has been recommended as a way of reducing the mortality rate, pericardial adhesions, and arrhythmias. Early correction is being advocated to reduce the number of patients lost in the interval between palliation and correction. In order to assess the validity of these criticisms, we have reviewed the results obtained with BH at the University of Michigan since 965 and have compared them with the results others have obtained using BAS and early correction. From the Department of Surgery, Section of Thoracic Surgery, and the Department of Pediatrics, University of Michigan Medical Center, Ann Arbor, Mich. Presented at the Eleventh Annual Meeting of The Society of Thoracic Surgeons, Montreal, Que., Canada, Jan. 2-22, 975. Address reprint requests to Dr. Behrendt, C-775 University Hospital, Ann Arbor, Mich THE ANNALS OF THORACIC SURGERY

2 The Blalock-Hanlon Procedure Clirikal Material Between 965 and 974, BH was carried out in 52 infants with TGA. The operation was an isolated procedure in 48 patients who form the basis for this report. It was combined with pulmonary artery banding in 2 patients and with systemic-to-pulmonary artery anastomosis in 2 others, who are not included. These constitute all patients with TGA whom we have treated palliatively during this interval except for 4 who had BAS. The ages at operation ranged from day to 2 months. Two-thirds were less than month old at the time of operation and half were in the first week of life. Hypoxia was the main indication for operation in the majority of patients. In 5 individuals refractory congestive heart failure secondary to elevated left atrial pressure was an additional reason. Metabolic acidosis was frequent, the average ph of the group being 7.28 and the lowest recording 6.8. Four patients experienced or more cardiac arrests prior to operation, and an additional 5 patients were moribund. Technique After induction of anesthesia, catheters were inserted into the brachial artery and vein for central arterial and venous pressure monitoring. Arterial blood samples were taken for blood gas analyses every 5 to 3 minutes throughout the procedure. Severe metabolic acidosis was treated appropriately, but bicarbonate was not given prophylactically. An atrial septa defect was created by the BH technique through a lateral thoracotomy in the right fifth intercostal space. The pericardium was incised longitudinally posterior to the phrenic nerve, and the right pulmonary artery and pulmonary veins were encircled and occluded with heavy silk ligatures. Then a curved vascular clamp was placed with one blade anterior to the right atrium and the other posterior to the left atrium. Parallel incisions were made in the right atrium and the anterior surface of the right pulmonary vein confluence, and the atrial septum was grasped, withdrawn, and excised. The color of the right atrium generally improved as soon as the incision was closed and the encircling ligatures and vascular clamps were released. Following operation the endotracheal tube was removed if the infant exhibited vigorous respiration and the arterial blood gases were satisfactory. If not, mechanical ventilation with a respirator was maintained for as long as deemed necessary. Blood gas values were determined frequently and appropriate measures taken to correct any abnormal findings. Digitalis, diuretics, or both were administered postoperatively if congestive heart failure occurred. Results Thirty-eight of the 48 patients survived BH (Fig. ). Not unexpectedly, 9 of the deaths occurred in patients less than month old, and half of these were consequent to preoperative complications such as septicemia, intracerebral VOL. 2, NO. 4, OCTOBER,

3 BEHRENDT ET AL SURVIVORS NONSURVIVORS \ FIG. I. Age at operation of 48 patients Week Weeks Months Months Months undergoing the Blalock-Hanlon p-oce- Age at Operation dure, , hemorrhage, or cardiac arrest (Table ). Interestingly, the mortality rate was higher among patients with simple TGA than among those with TGA complicated by ventricular septa defect, pulmonary stenosis, or both (Fig. 2). Nine (24%) of the 38 survivors experienced complications early postoperatively (Table 2). Transient disturbances in atrial conduction and pulmonary problems were most frequent. Ventilatory support was required in 8 patients. Only 4 patients needed catecholamine infusions postoperatively. The systemic arterial oxygen saturation increased in 79% of the patients from a mean of 49% preoperatively to 62% postoperatively (Fig. 3). Systemic arterial oxygen saturations were obtained in 2 of these patients one to four years later at the time of final correction (Fig. 4). In 4 the saturation remained at 7% or better, and there was no tendency for oxygen saturation to diminish with time. Only 2 patients had saturations of less than 6%; of these had pulmonary hypertension, and the other had severe subpulmonary stenosis. There was a gradual rise in the serum hematocrit as the duration of follow-up increased, even in patients with arterial oxygen saturations above 7% (Fig. 5). Almost all survivors experienced very satisfactory palliation, becoming normally active even though retarded in physical growth. Palliation proved to be long lasting: 24 patients were followed more than 2 months - and of these, more TABLE. CAUSES OF HOSPITAL DEATH IN 48 PATIENTS UNDERGOING THE BLALOCK-HANLON OPERATION Cause No. of Patients Intraoperative hemorrhage * Septicemia 2* Intracerebral hemorrhage Infarction of right lung Respiratory insufficiency 3 Inadequate atrial communication Unrecognized cardiac defect *Two patients had experienced cardiac arrest preoperatively. 426 THE ANNALS OF THORACIC SURGERY

4 The Blalock-Hanlon Procedure FIG. 2..\lortnlitj rdr of Blnlork-Hnriloti 5 procediire iii siittph trnitspositioii (left) coinpored mith coirrplex trnitspositioii ioith associated iletittiwlnr srptnl deject or pulmo * L 22- * SIMPLE SURVIVORS NONSURVIVORS than 24 months - prior to final correction (Table 3). Three patients were not improved by operation, 2 because of pulmonary vascular disease (they died late) and for unknown reasons. Only 3 patients required hospitalization for treatment of pneumonia, and there was just equivocal thromboembolic complication among the entire group of patients. There have been 3 late deaths among the survivors. One child with a ventricular septal defect remained chronically ill with pulmonary hypertension and marked cyanosis and died at the age of 2 months while awaiting correction. The second patient, who had an intact ventricular septum, died at 5 months of age of severe pulmonary hypertension. The remaining patient died at home at age month of acute laryngotracheobronchitis. Thirty-one of the 38 survivors have undergone correction with 8 deaths. None of these deaths were attributable to the previous BH procedure. However, in 6 patients adhesions were considered to be moderate or severe, and 5 required reexploration for bleeding, pericardial tamponade, or both. The atrial septal patency was described as large in 2 of the 24 operative notes in which ajudgment was recorded. TABLE 2. EARLY POSTOPERATIVE COMPLICATIONS IN 38 SURVIVORS OF THE BLALOCK-HANLON OPERATION Complication ~~ Arrhythmias Low cardiac output Pulmonary problems Metabolic abnormalities Inadequate palliation Phrenic nerve paralysis Seizures Burn Congestive heart failure Wound infection Pneumothorax Pericardial effusion No. of Patients VOL. 2, NO. 4, OCTOBER,

5 BEHRENDT ET AL. 9 r Preoperative Postoperative FIG. 3. Immediate preoperative and postoperative arterial oxygen saturations in patients having the Blalock-Hanlon procedure. Not all patients are included because not all had reliable data both before and - after operation. Comment In its uncomplicated form, TGA is now correctable with a low operative risk and good long-term functional results. Children with this anomaly usually are severely hypoxic early in infancy, at an age when total correction presents difficult technical demands. Thus, an initial palliative procedure is usually required. The problem becomes one of choosing the approach that will yield the highest number of ultimate survivors following final correction. One must consider not only the operative mortality of the palliative procedure but also deaths in the interval between palliation and correction and any complications that mightjeopardize the ultimate overall results. A high operative mortality (3 to 5%) has been attributed to surgical atrial septectomy [3-6, and BAS has been advocated as a safer alternative [lo, 23. Examination of the data used to condemn surgical septectomy reveals that usually the surgical results are from an earlier series of patients and the BAS results from a later series. Our results indicate that the operative mortality for BH need be no higher than for BAS. Bernhard and his group [8], using hyperbaric oxygenation with open septectomy, Trusler and Mustard [, and Castaneda's group [ 4 have also achieved low operative mortalities (9,, and %, respectively). 9 r s - 7- In C r i FIG. 4. Arterial oxygen saturations obtained just prior to final correction, demonstrating no tendency to diminish with time following the Blalock-Hanlon Qrocedure. 4' -\ >:8 Age in Months 428 THE ANNALS OF THORACIC SURGERY

6 The Blalock-Hanlon Procedure - + '5 6 E 2 5- FIG. S. Hematocrit obtained just prior to final correction, demonstrating a gradual b -! 8 I I I I I Reported BAS series have had early mortality rates of 2 to 26%, with an appreciable failure rate requiring emergency surgical septectomy [3,, 2, 4. The most recent data available from the New England Regional Infant Cardiac Program* reveal an immediate mortality of 25% with a one-year mortality of 4% for BAS in TGA with intact ventricular septum. Thus, it seems clear that the surgical and nonsurgical approaches have about the same immediate mortality in skilled hands and that the risk depends to a large extent on the condition of the patients treated. Most striking have been the quality and longevity of palliation experienced by our patients. Atrial arrhythmias have not been a problem. No patient has required repeat septectomy or early correction because of inadequate interatrial mixing. Their arterial oxygen saturations have been well maintained. Only 3 have required hospitalization prior to final correction, all for pneumonia. Atrial arrhythmias have been transient and of no clinical importance. Only child has suffered a cerebrovascular accident (of questionable nature), which left no residua. In contrast, virtually all BAS series report a relatively high incidence of strokes, repeated hospitalization, and secondary surgical septectomies [,, 2, 3. Postoperative studies have documented the frequently inadequate size of the defect produced by BAS with resulting low arterial oxygen saturations [. One certain advantage of BAS is that no pericardial adhesions are produced and thus the pericardium to be used in the final correction is unscarred. We are careful to irrigate blood from the pericardial cavity during a BH procedure in an attempt to minimize this problem. Nevertheless, half of our patients had dense pericardial adhesions and 5 required reexploration for bleeding. The thickened pericardium has not created a problem. In fact, one could speculate that it might have greater strength than undamaged pericardium. One of the most compelling arguments in favor of early correction, i.e., within the first 6 months of life, is the high mortality said to occur among patients awaiting correction following palliation. In contrast to the high incidence of late deaths following BAS (7 to 26%) [ - 2, only 3 of our surviving patients died prior to surgical correction. Two had severe pulmonary hypertension when first *D. Fyler, personal communication, 974. VOL. 2, NO. 4, OCTOBER,

7 BEHRENDT ET AL. TABLE 3. DURATION OF PALLIATION IN 38 SURVIVORS OF THE BLALOCK-HANLON OPERATION* Length of Palliation None <2 mo 3-24 mo 2-5 yr >5 yr No. of Patients *A longer period of palliation might have been observed had not elective correction been performed. seen. One died of laryngotracheobronchitis. Castaneda and colleagues [ 4 reported only late death during the interval between BH and final correction in their series. Current information suggests that the Mustard procedure carried out in the first 6 months of life is likely to have a high incidence of technical problems such as caval obstruction. Hypothermic circulatory arrest facilitates operations on small babies considerably, but we continue to have reservations about its long-term neurological effects and reserve it for situations in which it is clearly needed. Since we have lost so few patients in the waiting period between the BH and Mustard procedures, we prefer to perform final correction at about year of age, when it can easily be carried out using conventional perfusion. References. Baker, F., Baker, L., Zoltun, R., and Zuberbuhler, J. R. Effectiveness ofthe Rashkind procedure on the great arteries in infants. Circulation 43, 44 (Suppl I):l, Blalock, A., and Hanlon, C. R. The surgical treatment of complete transposition of the aorta and the pulmonary artery. Surg Gynecol Obstet 9:, Clarkson, P. M., Barratt-Boyes, B. G., Neutze, J. M., and Lowe, J. B. Results over a ten-year period of palliation followed by corrective surgery for complete transposition of the great arteries. Circulation 45:25, Cooley, D. A., Hallman, G. L., Bloodwell, R. D., and Leachman, R. D. Two-stage surgical treatment of complete transposition of the great vessels. Arch Surg 93:74, Cornell, W. P., Maxwell, R. E., Haller, J. A., and Sabiston, D. C. Results of the Blalock-Hanlon operation in 9 patients with transposition of the great vessels. J Thorac Cardiouasc Surg 52:525, Deverall, P. B., Tynan, M. J., Carr, I., Panagopoulos, P., Aberdeen, E., Bonham- Carter, R. E., and Waterston, D. J. Palliative surgery in children with transposition of the great arteries. J Thoruc Cardiouasc Surg 58:72, Lindesmith, G. G., Meyer, B. W., Stanton, R. E., Gallaher, M. E., Stiles, Q. R., and Jones, J. C. Surgical treatment of transposition of the great vessels. Ann Thorac Surg 8: Plauth, W. H., Jr., Nadas, A. S., Bernhard, W. F., and Gross, R. E. Transposition of the great arteries: Clinical and physiological observations on 74 patients treated by palliative surgery. Circulation 37:36, Rashkind, W. J., and Miller, W. W. Creation of an atrial septa defect without thoracotomy: A palliative approach to complete transposition of the great arteries. JAMA 96:99, Rashkind, W. J., and Miller, W. W. Transposition of the great vessels: Results of palliation by balloon atrioseptostomy in thirty-one infants. Circulation 38:453, THE ANNALS OF THORACIC SURGERY

8 Th Blalock-Hanlon Procedure. Trusler, G. A., and Mustard, W. T. Palliative and reparative procedures for transposition of the great arteries. Ann Thorac Surg 7:4, Tynan, M. Survival of infants with transposition of great arteries after balloon atrial septostomy. Lancet :62, Venables, A. W. Balloon atrial septostomy in complete transposition of great arteries in infancy. Br Heart J 32:6, Zamora, R., Moller, J. H., Lucas, R. V., Jr., and Castaneda, A. R. Complete transposition of the great vessels: Surgical results of emergency Blalock-Hanlon operation in infants. Surgq 67:76, 97. Discussion DR. ROBERT L. REPLOCLE (Chicago, Ill.): We all admire the excellent results that the Michigan group, the fountainhead of thoracic surgery, always gives us, and this paper is no exception. I might leave my laudatory comments at that except for a letter that the chairman of the Program Committee circulated a few weeks ago suggesting the discussants be more provocative. Since I m not instinctively a combative sort of person, I ll screw up my courage and try to be so today. Dr. Behrendt has ably pointed out that surgical treatment of TGA in the palliative sense can be done quite well. He didn t have a chance to say, but it does come out in his final manuscript, that palliation is necessary very early in the course of the disease. Patients with an intact septum are in trouble very early. These are the cyanosed babies who go into failure within the first few days of life, and it is at this point that something has to be done. There is an advantage to doing a palliative procedure at the time ofcatheterization and improving these children physiologically. On the other hand, if it is necessary to transport them to the operating room and prep the team in the middle of the night, it can be a drawback. The decision may ultimately depend on whether the cardiologists are willing to rouse themselves in the middle of the night to do the balloon septostomy or whether they would prefer to have the surgeons get up and do a BH procedure. The size of the septal defect produced by the catheter is related to the size of the balloon inserted, and this is directly related to the heart of the cardiologist: the more courage he has, the bigger the balloon he will use and the stronger his pull will be. That may in some respects be responsible for the results I am about to describe, because we have very courageous cardiologists. I looked up our results from 97 to 974: 23 Rashkind procedures, 2 of which were successful with no mortality. There were 2 late failures, at 4 months in a child who had a BH procedure and waited 2 months for correction, and at 4 months in a child who had the Mustard procedure. For the 2 Rashkind procedures that failed we did BH operations without mortality. You can see that my personal experience with this operation is not very great. One child a year after the Rashkind procedure had a good-sized atrial septal defect. He showed pulmonary compliance changes, improvement in systemic oxygen saturation and pulmonary compliance, and reduction of the average respiratory wait after the Rashkind procedure. Dr. Behrendt pointed out that adhesions can be a problem. One child who had a BH developed some adhesions to the right atrium but they were not very extensive, and I don t think this problem is really a major deterrent to the operation. In summary, I suppose there are advantages to both procedures. DR. JAROSLAV F. STARK (London, England): I would like to congratulate Dr. Behrendt for his excellent presentation and just say a few words about our own policy. At the Hospital for Sick Children, Great Ormond Street, we prefer balloon septostomy at the time of the original investigation. After that, we plan an early Mustard operation at the age of about 8 to 2 months. This policy was adopted for several reasons. We have seen several patients who failed to improve after a BH septectomy. At the time of an emergency Mustard operation we found a good interatrial communication, but the mixing remained inadequate. Waiting for the second operation was an additional strain for the patient s family. Finally, the adhesions caused by the first operation were sometimes cumbersome. In the last four years we have performed 4 primary Mustard operations in infants VOL. 2, NO. 4, OCTOBER,

9 BEHRENDT ET AL. with simple TGA with hospital death. Five of these were younger than 3 months, were 3 to 6 months, and 25 were 6 to 2 months old. Rashkind septostomy should be performed by an experienced cardiologist; the child should be followed carefully and Mustard s operation planned early. Should any serious deterioration occur, Mustard s operation can be performed irrespective of age or weight. DR. DONALD B. DOTY (Iowa City, Iowa): Dr. Behrendt has presented an experience with the BH procedure in a large number of patients with remarkable and enviable results, a mortality rate of approximately 2%. At the University of Iowa the results of this procedure during the same period were not as good. Overall mortality was 48%. This figure is similar to rates in other previously reported series. The results seemed worse the earlier surgical intervention was required, especially in those patients seen during the first week of life after Rashkind BAS has failed. We had hoped direct interatrial repair would benefit this special group of patients. We had 8 infants with TGA and intact ventricular septum requiring operation in the first week of life. Seven of them had Rashkind procedures with temporary improvement. But within a few hours, profound arterial hypoxemia (Pao, of 7 to 26 mm Hg) recurred and necessitated surgical intervention. Four had atrial septectomy and 4 had Mustard s operation under deep hypothermia and circulatory arrest. Here are the results. Three of the 4 died within a few days after atrial septectomy. Two of4 patients survived the intraatrial baffle procedure, but had persistent heart failure and died 6 weeks after the operation. The other survivor returned with pulmonary venous obstruction 8 months later. Reoperation to relieve the obstruction was performed, but the patient died at one year. Obviously, the overall results are discouraging. Can Dr. Behrendt provide more detailed information regarding method and results in this special group of patients who require operation for TGA soon after birth? I also noted in the presentation that only 6 patients had a PaOz less than 4 mm Hg. Can the authors provide correlations between survival and low arterial oxygen levels? DR. GEORGE A. TRUSLER (Toronto, Ont., Canada): I enjoyed Dr. Behrendt s thoughtful presentation very much, and I support much of what he has said. Over the past eight years, following rather disastrous earlier results, we have had improved survival with the BH procedure. We have operated on 33 infants in whom a BAS was either impossible or inadequate. This is a selected group in that only one-third were operated on in the first month of life; the other two-thirds were slightly older. We are grateful to Drs. Cooley and Aberdeen and others for showing us how to do this procedure, because a special technique is necessary. Two of these children died at operation, a mortality of 6%. There was only late death, 7 months later, showing how prolonged the benefit is in most patients. Nine children are still awaiting repair. Twenty underwent transposition repair an average of 22 months later, and required a Blalock shunt 6 months later. Thus, the BH operation carries a low risk and provides relatively prolonged palliation. Nevertheless, we agree with Dr. Stark that repair can be carried out at an earlier age, and we usually choose to repair if the child is 3 months or older. If the child is less than 3 months and the problem is lack of mixing between the two circulations despite balloon septostomy, we prefer the BH procedure. DR. BEHRENDT: I thank all the discussants. We really did not mean to suggest that anybody throw out his balloon and begin to do BH operations. If the Rashkind procedure works well in an individual institution and if that institution has good results with early correction, then this approach should be continued. I think only time will tell whether the long-term results of the Mustard operation carried out early in infancy warrant this policy. On the other hand, if this approach has not proved satisfactory, we merely wanted to point out that the BH procedure offers a rational alternative. We believe the mortality rate stated in the literature for this operation is grossly overestimated. The details of the operative technique requested by Dr. Doty are contained in the full manuscript. 432 THE ANNALS OF THORACIC SURGERY