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Ann Thorac Surg 1999;67:1391-1395
© 1999 The Society of Thoracic Surgeons

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Original Articles

Outcome after repair of tetralogy of Fallot with absent pulmonary valve

^a Division of Pediatric Cardiothoracic Surgery, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Division of Cardiology, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^c Department of Anesthesia and Critical Care, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Gaynor, Department of Cardiac Surgery, Children’s Hospital of Philadelphia, Suite 8527, 34th St and Civic Center Blvd, Philadelphia, PA 19104-4399
e-mail: gaynor{at}email.chop.edu

Presented at the Forty-fifth Annual Meeting of the Southern Thoracic Surgical Association, Orlando, FL, Nov 12–14, 1998.

	Abstract

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Background. Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression.

Methods. Since January 1, 1984, 28 patients with TOF/APV have undergone complete repair (median age 11 days, range 1 day to 16 years).

Results. Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04).

Conclusions. Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

	Introduction

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Absent pulmonary valve syndrome is a rare malformation that is estimated to occur in 3%–6% of patients with tetralogy of Fallot (TOF/APV), and is characterized by rudimentary or nearly absent pulmonary valve tissue with marked dilatation of the pulmonary arteries [1, 2]. Compression of the airways by the dilated pulmonary arteries may result in significant respiratory compromise. Respiratory problems can vary in severity from none to severe distress requiring mechanical ventilation. Mortality for surgical repair in these patients is increased, especially in infants with severe respiratory compromise [3–6] (Table 1). Previous reports have shown improved early outcome when plication of the dilated pulmonary arteries is performed to relieve airway obstruction [4–6]. The role of pulmonary valve implantation, however, is unclear [4–6]. In addition, the incidence of persistent respiratory symptoms and need for reoperation are uncertain. The current study was undertaken to review the early and late outcomes after repair of TOF/APV at our institution.

	Material and methods

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Operative techniques
All patients underwent complete repair incorporating patch closure of the ventricular septal defect (VSD), pulmonary artery plication, and reconstruction of the right ventricular outflow tract (RVOT) according to surgeon preference. The RVOT was reconstructed with a transannular patch in 19 patients (group I), and 9 patients underwent pulmonary valve implantation (group II). Group I patients all underwent surgery before April 1995, and group II patients since that time. A monocusp valve was utilized in 4 patients, and a valved homograft conduit in 5 patients. In 15 patients, combined anterior and posterior plication was performed, anterior plication alone in 11, and no plication in 2 patients. In group I, 6 patients underwent combined anterior and posterior pulmonary artery plication, 11 underwent isolated anterior plication, and in 2 patients, no plication was performed. Combined anterior and posterior plication was performed in all group II patients. Two patients had discontinuous pulmonary arteries; one was associated with a ductus arteriosus. Continuity was established in one case by extensively mobilizing the pulmonary arteries with patch augmentation of the anastomosis. In the other case, after extensive mobilization of the pulmonary arteries, and division of the ductus, a flap of anterior pulmonary artery was created and mobilized to allow for anastomosis to the left pulmonary artery. Currently at our institution, all patients with TOF/APV undergo combined anterior and posterior plication in conjunction with RVOT reconstruction with either a homograft or monocusp valve.

Data analysis
Variables were compared using the Wilcoxon rank sum test. Data are presented as median and range. For survival analysis, the date of surgery was taken as zero time. For survival analysis, the patients were censored at the time of death or withdrawn alive at the time of last follow-up. To assess freedom from death or reoperation, patients were censored at the time of death or reoperation, or withdrawn alive at the time of last follow-up. The Kaplan-Meier survival estimates with 95% confidence limits (CLs) are provided. Survival distributions between groups were compared using the log rank test. Cox’s proportional hazard model was utilized to examine the effects of anatomic and procedural variables on survival time. A univariable analysis was performed, and variables with p < 0.1 were included in the multivariable analysis. Data analysis was performed using STATA version 5 (College Station, TX) and NCSS version 6.0 (Kaysville, UT).

	Results

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Initial procedure
The study population of 28 patients included 18 men. Twenty-six patients had evidence of cyanosis on initial evaluation. Eleven patients, all infants (<1 year of age), required mechanical ventilation before surgery. Three required extracorporeal membrane oxygenation (ECMO). Median age at surgery was 11 days (range 1 day to 16 years). Eighteen patients underwent surgery during infancy and 16 underwent neonatal repair (age <30 days).

There were 6 early deaths, defined as death within 30 days of the procedure or before hospital discharge. One patient was unable to be weaned from cardiopulmonary bypass. The other 5 died of respiratory failure, multiple system failure, or sepsis. All had required preoperative mechanical ventilation and all were infants less than 40 days of age at the time of surgery. Five patients required tracheostomy for failure to wean from mechanical ventilation. Two of these patients survived to hospital discharge.

In group I, 10 of 19 patients were infants (5 neonates). Six patients were intubated at the time of surgery, and ECMO was utilized in 1. The median age of surgery was 5.2 months (range 0.07 to 195 months). In group II, 8 of 9 patients were neonates in the first week of life. Preoperative mechanical ventilation was utilized in 7 group II patients, and ECMO in 2. The median age of surgery was 0.13 months (range 0.03 to 27.2 months). The initial surgery was performed at an earlier age in group II patients compared with group I (p = 0.02), and group II patients were more likely to be intubated preoperatively (p = 0.03). At the time of surgery, group II patients were more likely to undergo combined anterior and posterior pulmonary artery plication (100%), than group I patients (32%) (p = 0.001).

Early mortality was 26% (4/19) in group I patients. However, only 10 group I patients were infants, and thus, the mortality for infants who underwent RVOT reconstruction with a transannular patch was 40% (4/10). Early mortality for group II patients was 22% (2/9). However, all but 1 of group II patients were infants, so the infant mortality was 25% (2/8). This difference in early mortality for infants did not reach significance (p > 0.1).

Follow-up
Twenty-two patients, including 12 infants, survived the initial surgical procedure. For the hospital survivors, follow-up is complete to death or December 31, 1997 in 16 patients, with median follow-up 51 months (range 1 to 162 months). Partial follow-up ranging from 6 to 148 months is available for the remaining patients. One adolescent died after hospital discharge as a result of drug overdose 1.8 years after surgery. One patient lost to follow-up 12.1 years after surgery had evidence of progressive right ventricular enlargement, severe pulmonic insufficiency, and mild tricuspid insufficiency, and is expected to require pulmonic valve placement.

Sixteen patients were followed for at least 1 year after repair, 8 for at least 5 years, and 6 for greater than 10 years. Overall survival was 77% (95% CL 56%, 89%) at 1 year after surgery and 72% (95% CL 50%, 86%) at both 5 and 10 years (Fig 1). Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year, and 52% (95% CL 29%, 71%) at both 5 and 10 years (Fig 2). Freedom from either death or reoperation was significantly worse for patents needing preoperative mechanical ventilation (p = 0.004 by log rank test) (Fig 3).

View larger version (13K): [in this window] [in a new window]   Fig 1. Actuarial survival (Kaplan-Meier) demonstrating outcome for all patients undergoing repair with TOF/APV.

View larger version (13K): [in this window] [in a new window]   Fig 2. Freedom of death or reoperation for all patients undergoing repair of TOF/APV.

View larger version (20K): [in this window] [in a new window]   Fig 3. Comparison of freedom of death or reoperation for patients requiring preoperative ventilation, and patients who did not require preoperative ventilation.

Reoperation
Reoperation was performed in 4 patients for either residual VSD (n = 1) or worsening respiratory symptoms with airway compression confirmed by bronchoscopy (n = 3). The initial repair in all 3 included closure of VSD with anterior and posterior pulmonary artery plication. In 2 patients, RVOT reconstruction was performed with a transannular patch. In the other patient, a monocusp valve was utilized. However, the monocusp valve was severely insufficient in the early postoperative period. The interval to reoperation ranged from 7 to 54 months. At follow-up, all had improvement in their respiratory symptoms, as demonstrated by decreased requirements for bronchodilators, and in one case, successful weaning from mechanical ventilation. One requires occasional bronchodilator therapy, while the other requires no bronchodilator therapy.

	Comment

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
There has been a dramatic reduction in mortality for infants undergoing repair of congenital heart defects in recent years. However, despite improvements in surgical techniques, cardiopulmonary bypass, and critical care, the mortality for infants with TOF/APV remains significantly greater than for infants with simple TOF [4–6]. In addition, there may be significant long-term morbidity secondary to airway compression in infants with TOF/APV. Reports from several centers have demonstrated improved results in recent years (Table 1). Mortality is still increased, however, for neonates who present with serious respiratory compromise due to airway obstruction from dilated pulmonary arteries. Most centers incorporate pulmonary arterioplasty or plication in the repair to diminish the size of the proximal pulmonary arteries and relieve airway obstruction [4–6]. There is less agreement, however, concerning the method of RVOT reconstruction. Some centers have advocated utilization of either a valved homograft conduit or use of a transannular patch incorporating a monocusp valve [4, 5]. In the report by Snir and associates, all patients underwent repair utilizing placement of a valved conduit in the RVOT [5]. Operative mortality for neonatal repair was 25%. Interestingly, there have been no reoperations for airway obstruction. However, a recent study by Godart and associates suggested that pulmonary valve implantation is not necessary [6]. In their study, 37 patients underwent complete repair, including 10 infants (20% mortality) and 27 older children (3.7% mortality). Pulmonary valve implantation was performed in 11 patients, and a transannular patch utilized in the remainder. At a follow-up of approximately 3 years, there has been 1 late death, and no patients required reoperation for recurrent airway obstruction. However, patch reconstruction of the RVOT was utilized in only 2 of 10 neonates.

In the current series, the patient population and type of repair has evolved over time. Before April 1995, the children tended to be older at time of surgery, and only 7 of 19 were neonates (group I). Since 1995, 8 of 9 patients have undergone surgery as neonates (group II). In group I, no plication or only anterior plication was utilized in 13 of 19 patients, while combined anterior and posterior plication was performed in only 6 patients. Pulmonary valve implantation was not performed, and the RVOT was reconstructed with a transannular patch in all patients. Since 1995, all patients have undergone combined anterior and posterior plication and insertion of either a valved homograft conduit or monocusp valve (group II). Overall, 3 patients (2 group I, 1 group II) have required reoperation for persistent pulmonary artery dilatation and severe airway compression. All underwent initial repair as neonates. In 2 patients, the RVOT was reconstructed with a patch. In the other patient, a monocusp valve was utilized but was severely insufficient postoperatively. One child required tracheostomy and prolonged mechanical ventilation before reoperation. Symptoms significantly improved in all patients after repeat plication of the pulmonary arteries and placement of a valved conduit in the RVOT. Overall, a transannular patch was utilized to reconstruct the RVOT in 10 infants, with 4 early deaths and 2 reoperations. In the remaining infants, RVOT reconstruction was performed with a valved conduit or monocusp valve, with 2 early deaths and 1 reoperation in a patient with a dysfunctional monocusp valve.

This study demonstrates that the early mortality for TOF/APV is considerable, especially in infants with significant respiratory compromise. The late survival for patients who survive the initial repair is good, although there is a significant incidence of persistent respiratory symptoms and need for reoperation. These findings are consistent with recent reports [4–6]. The only factor predictive of poor outcome in our study is the need for preoperative intubation and ventilation. These data are suggestive that pulmonary insufficiency may contribute to early mortality and persistent dilatation of the pulmonary artery leading to airway compression. Snir and associates reported good results and a low incidence of reoperation after repair incorporating a pulmonary valve [5]. Godart and colleagues suggest that pulmonary valve implantation is not necessary; however, they utilized pulmonary valve implantation in 80% of the infants in their series [6].

There are little available data concerning the long-term prognosis for children after repair of TOF/APV. In particular, the incidence of late reoperation is not known. In recent reports, the mean duration of follow-up is 3 to 3.5 years. Mean follow-up in the current study is 5.5 years, and 6 patients have been followed for at least 10 years. Three of the 21 long-term survivors have required reoperation for persistent airway compression at a mean of 26 months after the initial repair. Infants who undergo homograft RVOT reconstruction will need conduit replacement as they grow. Use of a transannular patch obviates the need for conduit replacement; however, pulmonary insufficiency is poorly tolerated in these patients, especially in infants. Thus, while pulmonary valve insertion may improve the early outcome and diminish the incidence of persistent pulmonary artery dilatation, it will increase the overall need for reoperation. Use of a monocusp valve may reduce both the severity of the pulmonary insufficiency and the need for conduit replacement.

There are several limitations to this study. It is a retrospective analysis and data are limited by the information available in the medical record. Because of the rarity of TOF/APV, the study population is small and thus statistical power is limited. The patient population has changed in recent years and surgical techniques have evolved. Follow-up is shorter for group II patients than for group I patients, thus, it is difficult to draw conclusions concerning the long-term outcomes.

In conclusion, while early mortality is significant, the long-term outcome for patients who survive the initial repair of TOF/APV is good. There is, however, a significant incidence of persistent respiratory symptoms and need for reoperation. This study suggests that repair of TOF/APV, particularly in infants, should incorporate combined anterior and posterior pulmonary artery plication and placement of a valved homograft or monocusp in the RVOT. Pulmonary insufficiency after repair may result in recurrent dilation of the pulmonary arteries and persistence of airway compression. Reoperation with repeat plication of the pulmonary arteries and insertion of a valved conduit results in significant symptomatic improvement.

	Acknowledgments

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Supported in part by The Daniel M. Tabas Endowed Chair in Cardiothoracic Surgery.

We appreciate the contribution of the many physicians and other personnel who cared for these infants, including William I Norwood, Jr, MD and Marshall L. Jacobs, MD.

	References

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 

1. Lev M., Eckner F.A.O. The pathologic anatomy of tetralogy of Fallot and its variations. Chest 1964;45:251-261.
2. Nagao G.I., Daoud G.I., McAdams A.J., Schwartz D.C., Kaplan S. Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol 1967;20:206-215.[Medline]
3. Lakier J.B., Stanger P., Heymann M.A., Hoffman J.I.E., Rudolph A.M. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations. Circulation 1974;50:167-175.[Abstract/Free Full Text]
4. Watterson K.G., Malm T.K., Karl T.R., Mee R.B. Absent pulmonary valve syndrome: operation in infants with airway obstruction. Ann Thorac Surg 1992;54:1116-1119.[Abstract]
5. Snir E., de Leval M.R., Elliott M.J., Stark J. Current surgical technique to repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg 1991;51:979-982.[Abstract]
6. Godart F., Houyel L., Lacour-Gayet F., et al. Absent pulmonary valve syndrome: surgical treatment and considerations. Ann Thorac Surg 1996;62:136-142.[Abstract/Free Full Text]

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