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Ann Thorac Surg 1997;63:1489-1491
© 1997 The Society of Thoracic Surgeons

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How To Do It

Technique to Repair Tetralogy of Fallot With Absent Pulmonary Valve

Department of Pediatric Cardiac Surgery, "Marie Lannelongue" Hospital, Paris Sud University, France

Accepted for publication December 2, 1996.

	Abstract

Top Footnotes Abstract Introduction Technique Comment References

 
The syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by aneurysmal dilatation of the pulmonary arteries causing tracheobronchial obstruction of varying degree. Relief of this obstruction is the main goal of the surgical repair and can best be achieved by appropriate pulmonary arterioplasty. We describe our current technique to repair this syndrome in infants and older children including pulmonary arterioplasty, ventricular septal defect closure, and right ventricular outflow tract reconstruction without valve insertion.

	Introduction

Top Footnotes Abstract Introduction Technique Comment References

 
Congenital absence of the pulmonary valve occurs in 3% to 6% of patients with tetralogy of Fallot (TOF). This syndrome includes three main components: lesions of the pulmonary valve (PV) and arteries (PAs), tracheobronchial lesions, and intracardiac lesions. The first component is characterized by PV aplasia or extreme hypoplasia resulting in severe valvular incompetence, mild annular stenosis, and aneurysmal dilatation of the pulmonary trunk and the central portions of the right (more markedly and frequently) and left PAs compressing the tracheobronchial tree. The clinical course of these patients depends mainly on the degree of this compression. The ductus arteriosus is usually absent. The extent of tracheobronchial lesions (obstruction and broncomalacia) is directly related to the degree of the pulmonary vascular lesions. The varying degrees of bronchovascular changes determine the wide clinical spectrum of this syndrome. The cardiac lesions are those typical of TOF: a malalignment type of ventricular septal defect, overriding of the aorta, and varying degrees of right ventricular hypertrophy.

Rarely, an absent pulmonary valve (APV) can also occur as an isolated anomaly (with or without a patent ductus arteriosus) or can be associated with such other cardiac anomalies as double-outlet right ventricle, transposition of the great arteries with ventricular septal defect, common atrioventricular canal, tricuspid atresia, or atrial septal defect.

Patients with TOF and APV syndrome can be divided into two groups depending on age and severity of symptoms [1, 2]. In the older, minimally symptomatic group, elective operation carries a risk similar to that of the common form of TOF. In the younger group, presentation is in early infancy with cardiopulmonary distress because of severe airway obstruction and high pulmonary blood flow. Mortality in this group is high (35% to 100%).

In this article we describe our current technique to repair TOF with APV.

	Technique

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The operation is performed through a median sternotomy with bicaval cannulation, continuous cardiopulmonary bypass, hypothermia to 28°C, and cold blood cardioplegia. The aortic cannula is placed in the distal ascending aorta to allow subsequent aortic transection. After aortic cross-clamping, the right atrium is opened and the atrial septal defect or patent foramen ovale is closed.

A vertical infundibulotomy is then performed and the intracardiac anatomy is defined. Hypertrophied muscle bands in the right ventricular outflow tract are resected and the ventricular septal defect is closed with a Dacron patch and a running 5.0 polypropylene suture. Subsequently, good exposure of the PAs is obtained by transecting the ascending aorta proximal to the clamp. The infundibular incision is extended across the stenotic pulmonary valve annulus up to the pulmonary artery bifurcation. Triangular segments are then resected on the anterior wall of both branch PAs (Fig 1). Resection is extended beyond the hilum of each lung to near the origin of lower lobe arteries. Great care is taken in planning the arterioplasty to avoid obstruction at the PA bifurcation.

View larger version (41K): [in this window] [in a new window]   Fig 1. . After patch closure of the ventricular septal defect through the infundibulotomy, the aorta is transected. The infundibulotomy is extended to the pulmonary artery bifurcation. Triangular segments of the anterior wall of each branch pulmonary artery and part of the anterior wall of the main pulmonary artery are excised, adjusting the pulmonary artery diameter to the appropriate Hegar dilator.

View larger version (34K): [in this window] [in a new window]   Fig 2. . In case of a very large pulmonary artery aneurysm, the anterior wall resection is combined with posterior wall plications of the main, left, and right pulmonary arteries.

View larger version (37K): [in this window] [in a new window]   Fig 3. . After the opposite edges of the pulmonary arteries are sutured, the aorta is reconstructed by direct anastomosis and the right ventricular outflow tract is enlarged with a transannular patch.

	Comment

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A wide variety of operative procedures has been advocated for repair of TOF with APV. Early surgical management is warranted in infants with life-threatening airway obstruction because of the very poor prognosis with medical treatment alone and the development of broncomalacia secondary to prolonged extrinsic vascular compression [4]. In the past, a two-stage approach was preferred in infancy. However, palliative procedures usually have been associated with high mortality, need for further complete repair, and small or no relief of bronchial obstruction. Optimal surgical treatment of TOF with APV remains to be defined. Particularly, there is no consensus with regard to PV insertion and pulmonary arterioplasty.

Successful attempts at primary repair in infancy, including pulmonary arterioplasty and intracardiac repair, have been previously reported by Dunnigan [5], Arensman [6], Stellin [7], and their associates. These procedures, similar to that described herein, did not include PV insertion. Although we believe transannular patch enlargement of the right ventricular outflow tract should be part of the repair, we strongly agree with these authors about the importance of an extensive pulmonary arterioplasty in the immediate relief of airway compression. In our opinion, PA resection should not be too close to the inferior borders of the branch PAs to avoid PA kinking and should be extended distally to the origin of the lower lobe arteries to completely relieve bronchial compression. Posterior wall plications, as proposed by Stellin and associates [7], are necessary for reduction of PA caliber whenever PA aneurysm is excessively large. We recommend this approach in all patients with this syndrome regardless of the age. Good results have also been recently reported [8] in a larger series of infants treated with principles similar to those expressed here.

The role of PV insertion in preventing continued PA dilatation in infancy [2, 4, 9] and allowing a better late outcome in older children [1] has also been emphasized. However, high mortality (37% to 64%) has been reported in infants even when PV insertion was part of the repair [2, 4, 9]. Moreover, the results obtained in our series of infants without PV insertion and the good right ventricular function detected at follow-up in the older patients of this study do not support that theory. The same is true in most patients in whom a transannular patch is part of the repair of TOF without APV. In our experience, the use of PV insertion does not prevent significant pulmonary incompetence at late follow-up [3]. Moreover, there are concerns related to midterm complications of PV insertion, such as development of calcification and stenosis, and to the need for future operations for PV replacement. Finally, compression of the tracheobronchial tree is not obviated with PV insertion. On the contrary, we found the pulmonary arterioplasty to be essential to relieve the bronchial compression in these patients. However, the use of PV insertion in association with pulmonary arterioplasty may be necessary in specific cases such as in severely ill neonates and in patients with high PA pressure.

In conclusion, we have described our current technique to repair TOF with APV in infants and older children including pulmonary arterioplasty, ventricular septal defect closure, and right ventricular outflow tract patch enlargement. Our experience indicates that in most of these patients good results can be obtained without PV insertion.

	Footnotes

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Doctor Conte's current address is Department of Cardiothoracic Surgery (AFSNIT 2152), Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark.

Address reprint requests to Dr Planché, Centre Chirurgical "Marie Lannelongue," 133 Ave de la Resistance, 92350 Le Plessis Robinson, France.

	References

Top Footnotes Abstract Introduction Technique Comment References

 

1. Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, DeLeon SY. Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair? J Thorac Cardiovasc Surg 1981;81:906–15.[Abstract]
2. McCaughan BC, Danielson GK, Driscoll DJ, McGoon DC. Tetralogy of Fallot with absent pulmonary valve. Early and late results of surgical treatment. J Thorac Cardiovasc Surg 1985;89:280–7.[Abstract]
3. Godart F, Houyel L, Lacour-Gayet F, et al. Absent pulmonary valve syndrome: surgical treatment and considerations. Ann Thorac Surg 1996;62:136–42.[Abstract/Free Full Text]
4. Fischer DR, Neches WH, Beerman LB, et al. Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients. Am J Cardiol 1984;53:1433–7.[Medline]
5. Dunnigan A, Oldham HN, Benson DW Jr. Absent pulmonary valve in infancy: surgery reconsidered. Am J Cardiol 1981;48:117–22.[Medline]
6. Arensman FW, Francis PD, Helmsworth JA, et al. Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve. J Thorac Cardiovasc Surg 1982;84:430–6.[Abstract]
7. Stellin G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RBB. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg 1983;36:468–75.[Abstract]
8. Watterson KG, Malm TK, Karl TR, Mee RBB. Absent pulmonary valve syndrome: operation in infants with airway obstruction. Ann Thorac Surg 1992;54:1116–9.[Abstract]
9. Snir E, de Leval MR, Elliott MJ, Stark J. Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg 1991;51:979–82.[Abstract]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Stefano Conte Alain Serraf François Lacour-Gayet Claude Planché Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Conte, S. Articles by Planché, C. Search for Related Content PubMed PubMed Citation Articles by Conte, S. Articles by Planché, C.