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Ann Thorac Surg 1999;68:1344-1348
© 1999 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Surgical management of tetralogy of fallot in the first year of life

^a Division of Cardiothoracic Surgery, Department of Surgery, Louisiana State University, New Orleans, Louisiana, USA
^b Division of Pediatric Cardiology, Department of Pediatrics, Louisiana State University, New Orleans, Louisiana, USA
^c Department of Cardiothoracic Surgery, Soroka Medical Center, Beer-Sheba, Israel

Address reprint requests to Dr Caspi, Children’s Hospital, 200 Henry Clay Ave, New Orleans, LA 70118
e-mail: jcaspi{at}aol.com

Presented at the Thirty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Antonio, TX, Jan 25–27, 1999.

	Abstract

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Background. The surgical approach to tetralogy of Fallot (TOF) continues to evolve and now many centers favor early repair for TOF.

Methods. Our experience includes 82 consecutive patients less than 1 year old with TOF (n = 74) and TOF with pulmonary atresia (n = 8) who were operated on between January 1992 and March 1998. Mean age at repair was 5.2 ± 1.2 months and mean weight was 4.5 ± 0.4 kg. Seven patients (anomalous left anterior descending artery [n = 1], pulmonary atresia with hypoplastic pulmonary arteries [n = 6]), underwent palliative procedures in the neonatal period followed by complete repair. Forty-nine patients (59%) were symptomatic (severe cyanosis or hypoxic spells), and 33 patients (41%) were asymptomatic. A combined transatrial-transpulmonary approach was employed in 28 patients (34%), and transannular patch or conduit for reconstruction of the right ventricular outflow tract (RVOT) was required in 54 patients (66%). The mean Nakata index was 160 ± 25 mm²/m².

Results. There were no hospital deaths. Mean post-repair peak right ventricular/systemic pressure ratio was 0.48 ± 0.1. There were no late deaths or reoperations during a mean follow-up of 23 ± 5 months. All patients are currently asymptomatic and in New York Heart Association class 1. Postoperative evaluation by two-dimensional and Doppler echocardiography or cardiac catheterization showed minimal pulmonary artery stenosis with a mean pressure gradient of 15 ± 6 mm Hg across the RVOT.

Conclusions. Our experience suggests that early repair of TOF can yield excellent results and initial palliation does not preclude early complete repair.

	Introduction

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More than 40 years have passed since the first successful repair of tetralogy of Fallot (TOF) [1], and currently, excellent results for repair of most TOF variants have been achieved. Castañeda and colleagues [2] and Barratt-Boyes and Neutze [3] introduced the concept of repair at early age (less than 1 year old) with low mortality and morbidity. This concept has evolved to early primary repair of TOF in newborns and infants immediately after presentation with no need for initial palliation with a systemic pulmonary shunt. Early repair of TOF has many advantages. It abolishes the secondary effects of increasing cyanosis on vital organs and its adverse effects on cognitive and psychomotor development of the patient. The relief of right ventricular outflow obstruction eliminates the secondary right ventricular hypertrophy and maintains the systolic and diastolic properties of the ventricle when compared with either two-stage repair or repair later in life. The increased blood flow to the pulmonary arteries enables normal development of the pulmonary vasculature and gas exchanging units [4]. The purpose of this retrospective study is to review our surgical experience with complete repair of TOF in patients younger than 1 year of age.

	Material and methods

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This retrospective analysis included 82 patients under 1 year of age who underwent complete repair of TOF (n = 74) or TOF with pulmonary atresia (n = 8) from January 1992 through March 1998. Data from patients’ records, preoperative cardiac catheterization studies (all patients), operative intervention, and preoperative (all patients) and postoperative two-dimensional echocardiographic studies were reviewed. Patients with nonconfluent pulmonary arteries, absent pulmonary valve, or associated atrioventricular canal were excluded from this study. The median age at operation was 4.5 months (range, 1.6 to 12 months), and the median weight was 4.3 kg (range, 3.7 to 7.4 kg). Forty-nine patients (59%) were symptomatic with more than one episode of hypoxic spell or persistent hypoxemia (arterial oxygen saturation less than 80%). Bilateral pulmonary artery cross-sectional area indexed to body surface area as described by Nakata and associated [5] was calculated before surgery in all cases. Primary repair was achieved in 75 patients, and a complete repair followed an initial palliation with systemic to pulmonary shunt in seven patients. Initial palliation with modified Blalock–Taussig systemic to pulmonary shunt with a polytetrafluoroethylene tubular graft (4 mm [n = 5] or 5 mm [n = 2]) was carried out in seven patients: one patient with anomalous left anterior descending artery arising from the right coronary artery, five patients with pulmonary atresia and diminutive pulmonary arteries (Nakata index 90 to 130 mm²/m²), and one patient with body weight less than 2.5 kg. The time interval between palliation and complete repair ranged from 6 to 10 months.

Operative technique
The operative technique was uniform throughout the study period. Continuous low-flow cardiopulmonary bypass with bicaval cannulation, systemic hypothermia (25°C) and blood cardioplegia was employed in all patients. Total circulatory arrest was not used as part of the repair in any case. The protocol for myocardial protection consisted of warm induction with blood cardioplegia (10 cc/kg) and cold blood cardioplegia (20 cc/kg), repeated administration of cold blood cardioplegia (10 cc/kg) every 20 minutes, and terminal warm cardioplegia (10 cc/kg) followed by 2 minutes of warm blood reperfusion (10 cc/kg). Serum ionized calcium was maintained at 0.6 mmol/L before and during the cardiac ischemic period and was normalized after 15 minutes of reperfusion [6]. Total extra corporeal time ranged from 82 to 152 minutes (mean, 104 ± 14 minutes). Aortic cross-clamp time ranged from 38 to 65 minutes (mean, 48 ± 6 minutes). A combined transatrial-transpulmonary approach was employed in 28 patients (34%). In these cases, the ventricular septal defect was closed through the right atrium. The parietal and septal bands were dissected all the way toward the level of the pulmonary valve. Pulmonary valvotomy and, if needed, release of supravalvar tethering was performed through a pulmonary arteriotomy. The size of the right ventricular pulmonary artery junction (annulus) was measured by passing Hegar dilators. The annular size was compared with the mean normal values as predicted by Rowlatt and coworkers [7]. This dimension was transformed to the Z values (standard deviation units) as described previously [8]. In general, a Z value of minus 2 or less was an indication for transannular patch. In those cases, a short incision (5 to 10 mm) was made from the main pulmonary artery across the pulmonary valve annulus via the anterior commisure in order to preserve valvar function. The pulmonary valve was excised only when it was found to be dysplastic. A piece of glutaraldehyde-fixed pericardium was used to augment the right ventricular outflow. The incision from the main pulmonary artery was extended into the orifice of the left pulmonary artery in cases with proximal left pulmonary artery stenosis. The second approach utilized was a right transventricular closure of the ventricular septal defect, division of obstructing hyperthropied infundibular muscle bands, and extended transannular patch augmentation of the right ventricular outflow tract. This technique was used in 54 patients (66%). The indication for the latter approach was based on the finding of long hypoplastic right ventricular-pulmonary junction. A pulmonary artery homograft for reconstruction of the right ventricular outflow tract was required in three patients with TOF-pulmonary atresia, and in one patient with anomalous left coronary artery. A patent foramen ovale was left open to allow right-to-left atrial decompression during the immediate postoperative period.

Associated anomalies, such as ligation of patent ductus arteriosus (n = 3), systemic to pulmonary collaterals (n = 5), and closure of secondum atrial septal defects (n = 4) were performed simultaneously, as indicated.

Modified arterial-venous ultrafiltration was carried out for 10 to 15 minutes following separation from cardiopulmonary bypass in 58 patients in our most recent experience as opposed to conventional ultrafiltration (n = 10) or none (n = 14) early in our experience. Hemodynamic measurements were recorded immediately after separation from cardiopulmonary bypass and repeated before closure of the chest. All patients were sedated with continuous infusion of fentanyl (2 to 8 mcg/kg/hour) and remained on the ventilator for the first 12 to 24 hours after surgery.

Follow-up was complete for the study. The family and physician of each infant were contacted between 3 and 77 months (mean 23 ± 5 months). Late postoperative evaluation included a complete physical examination, an electrocardiogram and two-dimensional and Doppler echocardiography in all patients. Six patients underwent cardiac postoperative catheterization indicated for residual right ventricular outflow pressure gradient.

Statistical analysis
Statistical analyses were performed using the ² test and the Wilcoxon rank sum test for nonparametric variables. A paired t test was used for continuous variables. The results are presented as the mean ± the standard deviation. All statistical analyses were performed using the SAS program [9] (SAS Institute, Cary, NC).

	Results

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The two surgical approaches are compared in Table 1. There were no hospital deaths in either group. The aortic cross-clamp times and cardiopulmonary bypass times were slightly longer in the transventricular group. The mean pressure gradient across the RVOT decreased immediately after repair from 66 ± 5 mm Hg to 24 ± 6 mm Hg (range 0 to 36 mm Hg) (p < 0.05). Ratio of peak pressure in the right ventricle to that of the left ventricle (RV/LV ratio) was 0.48 ± 0.1 for the entire group. This ratio was significantly higher in patients undergoing extended transannular patching; 0.54 ± 0.08 versus 0.32 ± 0.04 (p < 0.05) in patients with no transannular patch, or a short transannular patching.

View larger version (10K): [in this window] [in a new window]   Fig 1. Age distribution of 82 patients undergoing transatrial-transpulmonary (TA-TP) and transventricular (TV) repair of tetralogy of Fallot.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
The outcome after surgical repair of TOF has improved over the last 3 decades, and recently centers have reported an operative mortality ranging from 0% to 7% [10]. The issues that remain unsolved include: (a) the type of surgical procedure (transatrial-tranpulmonary versus transventricular approach); (b) early primary repair as opposed to two-stage repair; (c) the optimal timing for repair in symptomatic and asymptomatic patients; and (d) selection and management of patients requiring initial palliation with early complete repair. Castañeda and colleagues [2] and Barratt-Boyes and Neutze [3] demonstrated that early primary repair of TOF yields excellent results that are comparable with the results obtained in older patients. The classic approach of a two-stage repair with initial shunting and delayed correction at 2 years of age or later has been associated with excellent results, and long-term survival has been shown to be only slightly less than the expected rates for the control population [11]. However, early repair has numerous theoretical and practical advantages: It avoids the risks associated with two operations; it establishes normal pulmonary blood flow resulting in improved development of the pulmonary arteries and lung parenchyma; it avoids the deleterious effects of chronic hypoxemia and right ventricular hypertrophy; it also eliminates the potential serious problems such as branch pulmonary artery stenosis or distortion related to shunts. Borow and associates [12] documented that early repair results in better preservation of left ventricular function, and Walsh and coworkers [13] demonstrated a decreased incidence of ventricular arrhythmias than that documented in studies in which repair was performed at older age.

As such, our surgical approach for the past 7 years has utilized primary repair at an early age. However, we try to defer the operation beyond the first month of age if possible. The indications for initial shunting in this series were anomalous left coronary artery crossing an hypoplastic RVOT, tetralogy of Fallot with pulmonary artesia with no main pulmonary artery, or dimunitive pulmonary arteries and weight less than 2.5 kg. Initial shunting resulted in the growth of the pulmonary arteries as evidenced by greater Nakata index in patients who were palliated, in this study and others [14]. Consequently, patients who were palliated with systemic pulmonary shunt in the first month of life and in whom the pulmonary arteries have developed adequately underwent complete repair in the first year of age, thus still achieving the benefit of early repair. Gro and colleagues [15] have favored early repair in newborns and infants regardless of the size of their pulmonary arteries, because establishment of normal pulmonary blood flow would stimulate their growth. Although we agree with their findings, we still believe that the presence of diminutive branch pulmonary arteries (Nakata index less than 100 mm²/m²) predisposes these patients to a more difficult postoperative course and higher morbidity and mortality as compared with the more conservative approach. Similarly, in the Boston Children’s Hospital and University of Alabama Medical Center in Birmingham (BCH-UAB) study [16], a small ventriculo pulmonary junction (annulus) and distal main pulmonary artery or very young age (less than 3 months) were found to be risk factors for operative mortality. In addition to the size of the pulmonary arteries, an important issue is whether initial shunting could stimulate the growth of the pulmonary annulus. Uva and colleagues [14] and Rittenhouse and associates [17] demonstrated a significant development of the pulmonary annulus in patients who underwent initial palliation. However, we tend to agree with Sade and coworkers [18] and Pacifico and colleagues [19] who demonstrated that systemic aorto-pulmonary shunt does not promote pulmonary annular growth. In our experience, only small size of the distal pulmonary arteries based on angiographic indices was an indication for initial shunting, not the size of the pulmonary annulus.

In this study, younger age was associated with a higher incidence of long transannular patching, which correlated a greater degree of hypoplasia of the right ventricular-pulmonary junction, pulmonary valve annulus, and main pulmonary artery. The high incidence of transannular patching in this study is in accordance with other series [20] which showed an incidence ranging from 57.6% to 85% of transannular patching in this age group, particularly when the body surface area decreases [21]. While it is desirable that a competent pulmonary valve should be preserved in young symptomatic patients, the presence of a severe hypoplastic ventriculo-pulmonary junction often obviates this from a technical point of view. This can be regarded as a drawback when considering early versus late repair. However, in symptomatic patients, early repair results in a lesser degree of secondary right ventricular hypertrophy and fibrosis, and simple division of obstructing muscle bundles and tailoring of a relatively narrow patch to prevent aneurysmal dilation most likely contributes to a better postoperative right ventricular function and fewer arrhythmias. We feel these advantages outweigh the disadvantages of the inability to preserve pulmonary valve competence. A transannular patch has been found to be an independent incremental risk factor following primary repair of tetralogy of Fallot [22]. In this series, we have not observed any short-term adverse effects with the use of a transannular patch on postoperative right ventricular function. Other studies concerned with long-term follow-up showed that mild to moderate pulmonary regurgitation is well tolerated, and the majority of the patients had good functional status following transannular patch repair [23]. We recognize the need for a long-term follow-up in order to determine the effect of pulmonary insufficiency on right ventricular function.

In summary, this study demonstrated that repair of tetralogy of Fallot in infancy can be done with excellent results. Small distal pulmonary arteries, unfavorable coronary anatomy, and weight less than 2.5 kg remain in our view an indication for initial shunting. Limited ventriculotomy, precise tailoring of the outflow patch, improved myocardial preservation techniques, and postoperative management contributed to improved postoperative myocardial function. A longer follow-up is still required to assess the effect of long transannular patching on right ventricular function in this group of patients.

	References

Top Abstract Introduction Material and methods Results Comment References

 

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