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Right arrow Congenital - cyanotic

Ann Thorac Surg 2001;71:1556-1563
© 2001 The Society of Thoracic Surgeons

Original article: cardiovascular

Tetralogy of Fallot: surgical management individualized to the patient

Charles D. Fraser, Jr, MDa,b, E. Dean McKenzie, MDa,b, Denton A. Cooley, MDb

a Section of Congenital Heart Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas, USA
b Texas Heart Institute, Houston, Texas, USA

Address reprint requests to Dr Fraser, Section of Congenital Heart Surgery, Texas Children’s Hospital, 6621 Fannin, MC1-2285, Houston, TX 77030
e-mail: charlesf{at}bcm.tmc.edu

Presented at the Thirty-sixth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 31–Feb 2, 2000.


   Abstract
Top
 Abstract
Introduction
 Material and methods
 Results
 Comment
 Discussion
 References
 
Background. Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity.

Methods. Based on approximately 40 years’ experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock–Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months.

Results. From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction.

Conclusions. This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 Discussion
 References
 
We are now some 56 years into the surgical treatment for tetralogy of Fallot (TOF). The landmark surgical achievements of Blalock, Lillehei, Kirklin, and their coworkers in treating this disease are well known to all cardiac surgeons [1–4]. The natural history of TOF has been radically altered [5]. Many centers now achieve operative mortality rates for total correction of TOF of less than 5%, with some reporting operative survival approaching 100%.

Are there unanswered questions concerning the surgical management of TOF in the year 2001? The answer to this question must clearly be yes. Despite the noted successes in surgical correction, most of these procedures still must be properly classified as palliative. The present body of knowledge confirms that the life expectancy of patients with corrected TOF is still less than the general population [5]. Late attrition still occurs, predominantly secondary to the effects of right heart failure and ventricular dysrhythmias [5–7]. As Hanley [8] has eloquently stated in a recent editorial, the surgeon is still challenged in determining the best method/strategy for treating the congenitally abnormal right ventricular outflow tract (RVOT).

Controversy persists concerning the optimum management strategy for patients presenting with TOF. Opinions range the spectrum from those who advocate neonatal complete correction in all patients irrespective of symptoms to those preferring to defer total correction until later in life, using palliative shunting if necessary.

In 1954, systemic-pulmonary shunts (Blalock and Potts) were first used to treat TOF at Texas Children’s Hospital. In 1956, intracardiac repair using a pump oxygenator was begun in selected patients. Over the years until 1995, more than 2,175 patients underwent either primary surgical repair or surgical palliation for TOF, and most of these operations were performed by one surgeon (D.A.C.). During that time, surgical technique and strategy evolved substantially, as did outcomes. The trend was for earlier, complete repair, as has been true for most other centers. The patients were referred from all parts of the world, unfortunately making any meaningful follow-up impossible. Nonetheless, this large experience with the development of operations for TOF has proved useful to us in managing patients in the current era (Fig 1).



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  		Fig 1. Surgical experience at Texas Children’s Hospital from 1954 to 1955 in more than 2,175 patients.

 
In 1995, we initiated a surgical management protocol for patients with TOF designed to optimize short- and long-term outcome. This management strategy was based on our own observations over the preceding 40 years, along with the tremendous contributions by Mee, Kirklin, Castenada, and others [9–12]. Our intention was to design a strategy focused on individualizing the surgical procedure to the patient with several basic underlying principles, which included avoidance of circulatory arrest, limitation or avoidance of the right ventriculotomy, preservation of pulmonary valve function, and promotion of pulmonary arterial growth. It was our belief that such a strategy would optimize the patient’s repair and potentially limit long-term morbidity (Table 1).


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  		Table 1. Hypothetical "Ideal" TOF Management Strategy

 

   Material and methods
Top
 Abstract
 Introduction
 Material and methods
Results
 Comment
 Discussion
 References
 
Patients presenting with TOF with pulmonary stenosis (TOF/PS), TOF with complete atrioventricular septal defect (TOF/AVSD), or TOF with pulmonary atresia (TOF/PA) are managed surgically using a strategy based on their anatomy, age (weight at presentation), and symptoms (Fig 2).



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  		Fig 2. Surgical management strategy for patients with symptomatic tetralogy of Fallot.

 
Tetralogy of Fallot with pulmonary stenosis
Neonates and small infants (less than 4 kg) with hypercyanosis are referred for palliative shunting, typically in the form of a modified right Blalock–Taussig shunt (BTS; Fig 2) using either 3.5 or 4.0 mm thin-walled polytetrafluoroethylene. Anatomic definition including pulmonary artery size, presence of major systemic-to-pulmonary collaterals, and associated cardiac malformations is assessed by transthoracic echocardiography and when necessary, cardiac catheterization. Cardiac catheterization is reserved for cases of unclear pulmonary arterial architecture and occasionally, for balloon pulmonary valvotomy. Patients with TOF/PS are then referred for complete repair at 6 to 12 months of life (whenever they weigh more than 4 to 5 kg). Asymptomatic patients or patients presenting for treatment after palliative shunting performed at other institutions are referred for complete repair at 6 to 12 months of life or at time of presentation for older patients.

In neonates with threatened pulmonary artery isolation (usually left pulmonary artery at the level of the ductus), we have occasionally performed left-sided shunting in preference to attempting to patch the pulmonary artery bifurcation in small infants. These patients are also repaired around 6 to 12 months of life.

Tetralogy of Fallot with atrioventricular septal defect
In patients with TOF/AVSD, operation is typically deferred until 6 to 12 months of life. Neonates and infants presenting with hypercyanosis undergo palliative shunting as described for TOF/PS.

Tetralogy of Fallot with pulmonary atresia
Patients with TOF/PA present with ductal-dependent pulmonary blood flow. All such neonates undergo palliative shunting followed by complete repair at 6 to 12 months of life.

Surgical repair
All complete repairs are performed after standard median sternotomy using full-flow (150 to 250 mL · kg-1 · min-1) cardiopulmonary bypass with moderate systemic hypothermia (25°C to 28°C) and blood or crystalloid prime. Circulatory arrest and low-flow cardiopulmonary bypass are avoided. Intermittent, antegrade crystalloid cardioplegia is used for myocardial protection.

The transatrial/transpulmonary method of repair as initially described by Hudspeth and later promoted by Mee, Karl, Pacifico and others [9, 12, 13] is the preferred method of repair. A nontransmural RVOT resection is performed through the tricuspid valve along with patch closure of the ventricular septal defect (VSD). When present, the atrial septal defect is closed. The outflow tract, pulmonary valve and annulus, and branch pulmonary arteries are sized using graded sounds. Adequacy is based on mean expected sizes normalized for body surface. Where necessary, the pulmonary valve and annulus are incised and enlarged to achieve a relaxed dimension of 2 to 3 mm larger than normal. Main and branch pulmonary artery stenoses are patched with untreated autologous pericardium. In selected cases, an autologous pericardial monocusp pulmonary valve is constructed to achieve pulmonary valve competence. Where necessary, a transannular incision is extended onto the infundibulum, but limited to less than 10 mm through the pulmonary annulus. As noted by others, the presence of an anomalous anterior descending coronary artery does not preclude a transatrial/transpulmonary repair [9, 14].

In patients with TOF/AVSD, a two-patch technique is used. In patients with questionable right ventricular function after reconstruction of the atrioventricular valves, we often place a homograft pulmonary valve or monocusp pulmonary valve.

Patients with TOF/PA undergo repair using standard methods. When possible, a RVOT patch (pericardium) ± monocusp valve is used. In other patients, a homograft valved conduit is placed.

At our hospital, all patients undergo intraoperative transesophageal echocardiography before and after repair. We have documented the safety of this technique in even very small children [15]. Criteria for immediate intraoperative reexploration include significant residual VSD, RVOT obstruction (RVOTO), and tricuspid regurgitation.

To assess the efficacy of this strategy, the outcomes of all TOF patients undergoing palliative shunting or complete repair between July 1, 1995, and December 1, 1999, were reviewed. All patients were operated on by 2 surgeons (C.D.F. and E.D.M.). We included patients with TOF as defined by presence of an anterior malaligned VSD (with all degrees of aortic override) and some level of RVOTO. Also included were patients with TOF/AVSD, TOF with absent pulmonary valve syndrome, double-outlet right ventricle (aortomitral discontinuity) with subaortic VSD and pulmonic stenosis, and TOF/PA. Patients with PA, VSD, major aortopulmonary collateral arteries, and miniscule or absent native pulmonary arteries were not included in the study.

Follow-up data were collected using the Hospital Pediatric Cardiology database, inpatient and outpatient medical records, and, where necessary, referring doctor and direct patient/family contact. The study protocol and data collection were approved by the Institutional Review Board of the Hospital.

Statistics
All data are expressed as mean ± standard deviation of the mean and range. The Kaplan–Meier method was used to estimate probability of survival and need for reintervention. Because of the low number of deaths and reoperations, analysis of risk factors for these morbidities was meaningless and not performed. Subgroup comparisons were performed using Fisher’s exact test. A difference was considered significant at a p value of less than 0.05.


   Results
Top
 Abstract
 Introduction
 Material and methods
 Results
Comment
 Discussion
 References
 
From July 1, 1995, through December 1, 1999, 144 patients (82 boys, 62 girls) underwent primary repair or surgical palliation for TOF/PS, TOF/PA, or TOF/AVSD. There were no operative deaths. The patients were followed through December 1, 1999, to ensure maximum follow-up on all shunted patients and to limit the potential for bias in these patients’ assessments. Ninety-four patients underwent primary one-stage repair. Through December 1, 1999, 39 patients underwent complete repair after an initial palliative shunt, and 10 patients are awaiting repair after shunting (1 shunted patient with TOF/AVSD died awaiting repair [see below]).

Tetralogy of Fallot with pulmonary stenosis and tetralogy of Fallot with pulmonary atresia
The mean age and weight for 88 patients undergoing primary TOF/PS repair were 1.0 ± 3 years (2 months to 23.5 years) and 10.5 ± 9 kg (3.3 to 62.3 kg), respectively. For 21 TOF/PS patients undergoing staged repair, the age and weight at complete correction were 18 ± 10 months (6 months to 3.6 years) and 9.6 ± 2.4 kg (5.7 to 14.5 kg), respectively. All patients with TOF/PA were palliated with initial modified BTSs. Of 11 patients with TOF/PA undergoing complete repair, the age and weight at operation were 15 ± 6 months (8 months to 2.4 years) and 9 ± 2 kg (5.4 to 12.7 kg). Nine additional shunted patients with TOF/PS or TOF/PA are awaiting repair.

The details of the operative procedures performed at complete correction for patients with TOF/PS are as follows: 106 of 109 patients (98%) underwent transatrial/transpulmonary repair with 64 patients (68%) requiring minitransannular incision. No patient undergoing a transatrial/transpulmonary repair had a transannular incision longer than 10 mm (mean 5 ± 2 mm). The 3 patients who underwent transventricular repair had difficult VSDs not amenable to transatrial or transpulmonary closure. All 4 patients with anomalous anterior descending coronary arteries underwent transatrial/transpulmonary repair. Ten patients required pericardial monocusp pulmonary valves. Five patients underwent pulmonary homograft valved conduit insertion. Eighteen patients required branch pulmonary artery repair, 8 related to a shunt-induced stenosis. Two patients required VSD enlargement. No patient required circulatory arrest and there were no delayed sternal closures.

For patients with TOF/PA, 7 of 11 patients underwent repair including insertion of a homograft valved conduit. The other 4 patients were managed with outflow tract patches with [2] or without monocusp pericardial pulmonary valves.

To evaluate the effect of staged repair, we compared results of primary versus staged repair in patients with TOF/PS or TOF/PA. Age at correction (1.1 ± 3 years versus 1.5 ± 1.2 years), weight (10.7 ± 9 kg versus 10.2 ± 3.3 kg), pulmonary value z score (0.1 ± 2.4 versus -0.6 ± 1.5), and preoperative saturations (88% ± 10% versus 79% ± 11%) were not different. We found no difference in postoperative outcomes including time to extubation (36 ± 36 hours versus 43 ± 64 hours), intensive care unit length of stay (3.15 ± days versus 3.6 ± 3 days) and hospital length of stay (9.7 ± 11 days versus 11.5 ± 13 days). At late follow-up, no difference was measured in RVOT velocity as assessed by echo Doppler (1.97 ± 0.8 m/s versus 2.5 ± 1.1 m/s).

Tetralogy of Fallot with atrioventricular septal defect
Thirteen patients underwent complete repair of TOF/AVSD including 6 with primary and 7 with staged repairs. Seven of 13 patients underwent repair using a homograft right ventricle to pulmonary artery conduit. Two patients had monocusp pericardial valves created.

Mortality
There were no operative deaths. There were three late deaths (survival at 1,492 days, follow-up 94.8 ± 0.012 days). One neonate with TOF/AVSD underwent palliative shunting because of hypercyanosis. After an initial uneventful hospital stay and recovery, the patient presented in circulatory collapse resulting from sepsis late after the operation and died. Blood cultures and tracheal aspirates were positive in this patient and a shunt-related infection could not be excluded.

There have been two additional late deaths (Fig 3). A patient with CHARGE association, imperforate anus, and colostomy underwent transventricular repair. This patient developed abdominal sepsis late after the operation thought secondary to pancreatitis and died. The other late death occurred in an 8-month-old boy with TOF and absent pulmonary valve syndrome. The patient underwent complete repair at 6 weeks of life because of chronic ventilator dependency and severe air trapping. After a lengthy recovery, the patient was eventually discharged with home respiratory care. He presented to an outlying hospital 6 months after discharge in cardiorespiratory arrest of unclear etiology.



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  		Fig 3. Survival analysis of all patients undergoing repair or palliation of tetralogy of Fallot.

 
Morbidity
There have been no delayed sternal closures after complete repair and no cases of surgical atrioventricular block. Junctional ectopic tachycardia occurred in 8 patients (7 TOF/PS and 1 TOF/AVSD) for an overall incidence of 6%. Two patients developed postoperative mediastinitis.

Primary versus staged repair
To assess the efficacy and safety of palliative shunting as an intermediate step before complete repair, we compared results in TOF/PS patients undergoing primary repair with TOF/PS and TOF/PA patients undergoing staged repair. As previously noted, there were no operative deaths in either group. We observed no differences in intensive care unit or hospital length of stay or time to extubation in the two groups.

Echo follow-up
As previously noted, all patients underwent operative transesophageal echocardiography assessments after complete repair. Early follow-up echo studies were performed either before or after hospital discharge (less than 30 days). Late follow-up echocardiograms were performed at the discretion of the patient’s local or referring cardiologist. At a mean late follow-up of 585 days, the mean RVOT velocity in patients undergoing primary or staged repair was 2.0 ± 0.8 and 2.5 ± 1.0 m/s, respectively (p = NS). Two TOF/PS patients are being followed for RVOT velocities of 3.2 and 3.6 m/s. The latter patient has an anomalous anterior descending coronary artery and underwent transatrial/transpulmonary repair. This patient has good right ventricular function and mild pulmonary valve insufficiency, but will eventually require reoperation.

Reinterventions
Five reinterventions (four surgical, one catheter) have been performed for hemodynamically significant lesions in 4 patients from 2 weeks to 3 years after complete repair. A 16-year-old girl presented for late primary repair of TOF/PS and underwent transatrial/transpulmonary repair. She was readmitted 2 weeks after operation with congestive heart failure because of a large recurrent VSD from a partial patch dehiscence. This defect was repaired uneventfully. A second girl, age 14 years, underwent one-stage repair of TOF/PS, anomalous anterior descending coronary artery, partial anomalous pulmonary venous return from the right lung to the inferior vena cava (scimitar), and multiple muscular VSDs. The patient had a large muscular VSD closed at initial operation, along with transatrial/transpulmonary repair, and partial anomalous pulmonary venous return repair. She was readmitted 6 weeks after initial repair in severe congestive heart failure. Cardiac catheterization revealed large apical muscular VSDs. This patient underwent attempted surgical closure of the VSDs through an apical right ventriculotomy, but had residual shunting. She ultimately required catheter-introduced device closure of the residual VSDs. She is currently well. The other TOF/PS patient who required reoperation was a 14-month-old boy who underwent transatrial/transpulmonary repair at 2 months of life (4 kg). The patient returned 1 year after repair with recurrent RVOTO and a proximal left pulmonary artery stenosis. Both were repaired uneventfully. The final reoperation was in a 17-year-old girl with TOF/AVSD. The patient had undergone a classic BTS as an infant. She was referred and underwent complete repair at 14 years, including a homograft right ventricle to pulmonary artery conduit. She presented 3 years after operation with conduit stenosis. The conduit was changed uneventfully (Fig 4).



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  		Fig 4. Reoperation rate in patients after complete repair of tetralogy of Fallot.

 
Shunt-related complications
As noted, 1 patient with Down syndrome and TOF/AVSD died 9 months after initial palliation. While awaiting complete repair, 2 other patients (both with TOF/PS) developed bacteremia, which was presumed to be shunt-related endocarditis. Both patients were treated with a course of antibiotics and went on to successful complete repair. No cases of phrenic nerve palsy or chylothorax were recorded. Eight of 32 shunted TOF/PS or PA patients who went on to complete repair needed branch pulmonary artery repair; however, 4 of these patients had been shunted elsewhere.


   Comment
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
Discussion
 References
 
Over the last decade, two schools of thought have developed regarding the appropriate timing for complete repair of TOF. Centers focused on neonatal complete repair cite appropriate concerns about potential attrition from palliative procedures, effects of chronic cyanosis, and the effects of surgical delay on right ventricular hypertrophy [16–18]. We believe there is a place for early infant repair, but perhaps not in infants so small that they require circulatory arrest. Furthermore, we believe that our data provide additional support for the notion that appropriate shunting still has a place in the surgical management of this disease.

Many centers now report good early results with neonatal and early infant repair [18–21]. A recent excellent series from Children’s Hospital in Boston documented outcomes in 99 infants less than 90 days old undergoing primary repair of TOF/PS or TOF/PA [21]. In this report, a 3% operative mortality rate was reported with 5 additional late deaths, for an actuarial survival of 91.6% at 5 years. Of note, 22 of 99 patients required reoperation at 5 years. This included a 19% reoperation rate for the 73 patients undergoing repair of TOF/PS. All patients underwent transventricular repair under deep hypothermic circulatory arrest (DHCA) (mean circulatory arrest time longer than 40 minutes). Because of data published on the deleterious effect of this perfusion strategy in neonates with transposition of the great arteries [22], the circulatory arrest time is of concern to us. We contend that avoidance of DHCA should be a central feature in decisions regarding timing of repair of TOF. For programs committed to promotion of all patients for neonatal repair, current techniques will require the use of DHCA in many patients. In our series, no patients required DHCA for complete repair.

The benefits of limiting or avoiding a transmural right ventriculotomy have not been fully determined. We believe, however, that patients undergoing neonatal, transventricular repair with a transannular incision will ultimately develop the same problems with late right ventricular dysfunction seen in the original series of transventricular TOF repairs now followed for almost 50 years [5, 23, 24]. A neonatal transventricular/transannular patch must be relatively large to accommodate the surgeon’s view, at times involving as much as 50% of the length of the right ventricle depending on patient size. Most of these patients will have wide-open, pulmonic insufficiency with a noncontractile infundibulum. The late consequences of this approach will be determined over the next several decades of follow-up.

We are encouraged, however, by the intermediate-term data reported by centers advocating the transatrial/transpulmonary approach. Brizard and colleagues [14] from Royal Children’s Hospital (Melbourne, Australia) reported an actuarial freedom from reoperation of greater than 96% at more than 5 years’ follow-up. Although our length of follow-up is more limited, our freedom from reoperation or catheter intervention seems to be consistent with the Melbourne group’s finding. Our results also compare favorably with the rate of early reoperation reported in a neonatal TOF repair series of 30 patients from C.S. Mott Children’s Hospital. Hennein and associates [25] documented a significant reoperation rate of 27% at 15 months follow-up, including 4 of 16 patients with TOF/PS. They calculated an actuarial freedom from reoperation of only 66% at 5 years. Although the transatrial approach was used for infundibular resection in their series, most patients required periods of DHCA for the repair. We would submit that working under the gun of DHCA in small infants results in a less optimal RVOT resection. Further, the incidence of left pulmonary artery stenosis requiring reoperation was significant, a fact we believe is related to patching small branch pulmonary arteries.

The issue concerning the detrimental effects of palliative shunting on subsequent outcome in patients with TOF remains controversial. The literature has conflicting information concerning the early and late morbidity of shunting. Knott-Craig and colleagues [26], for example, observed a significant mortality rate of 11% for staged repair for TOF. In later years, however, they experienced no mortality from palliative procedures. In addition, at 15 years’ follow-up, the freedom from reintervention after primary or staged complete correction was 85% and 91%, respectively (p = NS). Although some investigators have documented a significant incidence of branch pulmonary artery distortion and mortality related to palliative shunting, others have not [27]. Karl and colleagues [9] (and personal communication, R.B.B. Mee, 2000) documented excellent results for palliative intermediate shunting in patients with TOF, with one mortality related to this procedure in the last 10 years.

In summary, we believe there is a place for judicious shunting in patients with TOF. In our experience, most patients born with TOF/PS will not be symptomatic during the newborn period [28]. Most TOF patients can undergo primary repair in infancy beyond the newborn period (e.g., at 6 to 12 months), which is our current preferred timing.

We believe our management approach represents a rational strategy aimed at optimizing the repair while minimizing the risks of the surgical intervention. Whether or not these results translate into an improved long-term outlook for patients with repaired TOF remains to be elucidated through careful follow-up.


   Discussion
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Discussion
References
 
DR MARSHALL L. JACOBS (Philadelphia, PA): Among the patients who had a palliative shunt, do you have any data or observations regarding any growth of the pulmonary annulus during the palliated period and whether that impacted positively or negatively on the need for a limited transannular incision at the time of definitive repair?

DR FRASER: We have not specifically measured differences in growth of the pulmonary annulus, but I think a palliative shunt probably does not affect the relative growth of the annulus. However, I think most patients in general can be managed with a limited transannular incision irrespective of shunting.

DR RICHARD A. JONAS (Boston, MA): Doctor Fraser, these are certainly spectacular results, and you and your whole team certainly deserve our congratulations.

I wanted to explore with you the weight and age issue, specifically what is it that is critical about 4 kg and 6 months? Do you see that these numbers might evolve in the future? And to give us some perspective, can you tell us your approach to transposition? Is there a weight at which one should palliate rather than correct transposition? Does that also apply to truncus arteriosus or interrupted aortic arch?

And also to give us some perspective, what was your opinion 10 years ago in terms of minimal weight and age before you would correct tetralogy? And where do you estimate you will be in 10 years with respect to minimum weight and age for correction?

DR FRASER: Well, thank you, Dr Jonas. I’ll answer the last question first. Ten years ago I was still in training, so I cannot say where I would have been at that time.

I think there is a minimum in tetralogy in terms of weight at which you can do a transatrial transpulmonary repair and avoid circulatory arrest. Six months or 4 kg is probably a bit arbitrary. My personal preference for doing the transatrial transpulmonary approach is around 5 to 6 kg, which typically coincides, as you know, with around 6 months of life. However, there are patients in our series on whom we operated much earlier because they were bigger infants and we would have a good likelihood of doing the operation without needing circulatory arrest.

About the other conditions you mentioned, we agree with you that most of those conditions should be treated in the neonatal period.

These are our results during the same time. On the right, in the green bar, with the arterial switch operation in neonates, 75 consecutive infants were operated on without a death. So we do agree with you that many conditions can and should be treated in the neonatal period. We also do not use circulatory arrest for most patients with transposition.

There are several points to be emphasized in considering Dr Jonas’ questions:

  1. We believe that based on current technology and surgical methods, there is an optimal age/size for correction of all congenital heart lesions. Just because correcting transposition or interrupted arch in the neonatal period is preferable does not mean that approach is suitable for tetralogy.
  2. The available body of surgical literature suggests that centers preferring neonatal TOF repair often use circulatory arrest and low flow bypass procedures. It seems counterintuitive for Dr Jonas and others to promote the use of DHCA for TOF repair when they have previously documented the significant adverse effects of DHCA for transposition.
  3. We think that the transarterial/transpulmonary method is superior. We believe that a transventricular repair is suboptimal.
  4. DR DENTON A. COOLEY (Houston, TX): In 1954 the late Dr C. Walter Lillehei presented before a national surgical meeting his initial experience with intracardiac repair of TOF and predicted, or implied, that the Blalock–Taussig concept was either obsolete or would become completely obsolete. I believe the point that Dr Fraser has made is an appropriate one, that there is still room for clinical judgment in selecting operation for TOF.
  5. I would like to hear his opinion about the outflow tract in these patients who are managed by a transpulmonary repair of the pulmonary stenosis. I am sure the incidence of outflow tract obstruction is higher than in the previous report we listened to in which they did a more extensive opening of the annulus.
  6. In this large series of cases dating back almost 50 years, has there been an incidence of pulmonary regurgitation requiring surgical correction?
  7. DR FRASER: I think there are probably people in this audience that can speak to the first question better than I. Tom Karl, I noticed here earlier, has probably the world’s largest experience in Melbourne, and now in Philadelphia, with the transatrial/transpulmonary approach. And I think initial concerns about a higher incidence of significant outflow tract gradients, and thereby a need for reoperation, have not been borne out in the Melbourne series. Certainly our follow-up is too short to make a meaningful comment about that, I think.
  8. I do think that there is a substantial difference in terms of how the infundibulum performs when you do a transpulmonary outflow resection as opposed to a transventricular. Leaving a contractile infundibulum seems to be important, at least in my opinion, to the function of the right ventricle, both early and late. But many years will have to pass before that is borne out in terms of late outcome improvement.
  9. DR CHRISTO I. TCHERVENKOV (Montreal, Quebec, Canada): It is with a lot of respect and humility that I rise to discuss this report, recognizing the monumental contributions of Dr Cooley to the entire field of cardiac surgery. I also congratulate Dr Fraser on a superb presentation.
  10. At the Montreal Children’s Hospital, we have taken a different approach since 1987 not unlike that of the Boston Children’s Hospital. In the last 14 years, we have performed primary repair in all patients with TOF regardless of weight, age of the infant, the size of the pulmonary arteries, or the patient’s preoperative status. No patient at our institution has undergone a shunt for TOF since that time. Having said that, I think that your results are outstanding and they contribute to the debate as to the optimal surgical approach in the treatment of TOF. The achievement of a low mortality, with a two-staged approach by you and with the single-stage approach by us and others, indicates that it is time to shift the debate toward other considerations, such as physiologic and anatomic considerations of whether an initial primary repair is optimal or whether a staged repair is more appropriate.
  11. One of these considerations is the impact on the pulmonary circulation. In a neonate with severe TOF, is the development of the pulmonary arterial tree going to be greater after a shunt or after primary intracardiac repair with a transannular patch and pulmonary insufficiency?
  12. A consideration in favor of early primary repair is the ability of the heart in the first few months of life to remodel itself by virtue of myocardial hyperplasia and not only by hypertrophy. Furthermore, there are complications with the use of the shunt, such as branch pulmonary artery stenoses that may lead to severe ipsilateral underdevelopment of the pulmonary circulation, the development of acquired pulmonary atresia after a shunt, et cetera.
  13. What approach leads to a better long-term preservation of myocardial function? Early primary repair with possibly a greater use of a transannular patch and chronic pulmonary insufficiency or delayed intracardiac repair leaving the heart exposed to pressure and volume overload after a shunt in a patient that is still cyanotic? These questions remain to be answered conclusively. Therefore, I think that the debate will have to go beyond the issue of mortality and more carefully assess the physiologic and anatomic measurements associated with one versus the other approach.
  14. I have a couple of questions:

  1. Have you observed in patients with severely hypoplastic pulmonary valve annulus in whom, after a shunt, the annulus has developed adequately, precluding the use of a transannular patch at the time of intracardiac repair with preservation of the pulmonary valve?
  2. How much does your transatrial and transpulmonary surgical approach, as Dr Jonas has alluded to, influence your cut-off point of 4 kg and 6 months in favor of palliation instead of primary repair? Do you think that the transatrial/transpulmonary approach can be applied in much smaller infants such as neonates?
  3. DR FRASER: That was a lot. The second question is I think easily answered. There are not any true neonatal or small infant series of transatrial/transpulmonary repairs. Doctor Bove has published a series in which most patients (neonates) were operated on through the transatrial approach, and there was a 25% reoperation rate in 2 years. I think clearly that rate is high. Therefore, I think it is difficult to see in small infants. And if you happen to believe that circulatory arrest is a good way to go, then you are working under the gun of the clock, and so I do not believe you can carry out as effective an outflow tract reconstruction or resection in those circumstances. Certainly I find it more difficult. I think in patients who are operated on at that size there is a much higher incidence of junctional tachycardia, simply related to the fact that you have to pull a lot harder on the heart to get a good view.
  4. Now, about the question of whether the shunt affects whether we perform a transannular patch. I would not be able to answer that. We had only 3 patients in whom we believed it was necessary to carry out a transventricular closure of the VSD and that would be the only reason that I would do a transventricular repair. In those 3 patients I simply could not get a good closure of the VSD otherwise. The rest of the time I really do make a strong effort to limit the transannular incision to less than 10 mm. And as you saw from our data, the mean length is about 5 mm. That includes shunted and nonshunted patients, but I did not look at that specifically in terms of comparing the two.
  5. DR TCHERVENKOV: A few quick comments. In the last several years, we no longer routinely use circulatory arrest for these patients. They are repaired, at least in our institution, on cardiopulmonary bypass. So this issue is no longer an argument against primary repair. With respect to brain preservation and development, I am not sure what is more detrimental, chronic cyanosis in an unrepaired patient with volume overload and low diastolic pressure after a shunt or a period of circulatory arrest to achieve an early primary repair and a fully saturated patient.
  6. With respect to what constitutes a reoperation, I would also like to raise a question of definition. We have used the reoperation rate to assess outcome after surgical repairs in addition to mortality and long-term survival for or against a particular approach. By definition, all patients subjected to a palliative shunt are reoperated during the intracardiac repair. I therefore believe that the percent of palliated patients should be added to the reoperation rate when comparing the two approaches.
  7. Again, I would like to congratulate you on your outstanding results in Houston with a two-stage approach in the treatment of TOF. I thank the Society for the privilege of discussing this excellent article.


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Discussion
 References
 

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