Ann Thorac Surg 2004;77:734-736
© 2004 The Society of Thoracic Surgeons
How to do it
Autologous repair of supravalvar pulmonic stenosis
Emile A. Bacha, MDa*,
Robert Kalimi, MDa,
Joanne P. Starr, MDa,
Jose Quinones, MDb,
Peter Koenig, MDb
a Section of Cardiothoracic Surgery, Pediatric and Congenital Cardiac Surgery, The University of Chicago Children's Hospital, Chicago, Illinois, USA
b Section of Pediatric Cardiology, The University of Chicago Children's Hospital, Chicago, Illinois, USA
Accepted for publication April 8, 2003.
* Address reprint requests to Dr Bacha, Pediatric and Congenital Cardiac Surgery, The University of Chicago Children's Hospital, 5841 S. Maryland Ave, MC 5040, Chicago, IL 60637, USA.
e-mail: ebacha{at}surgery.bsd.uchicago.edu
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Abstract
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Native supravalvar pulmonary stenosis is a rare anomaly, but iatrogenic supravalvar pulmonary stenosis occurs after various repairs for congenital heart disease with relative frequency. Surgical techniques such as patching carry the risk of restenosis. We describe a technique of repair using only autologous tissues that can be applied to both native and iatrogenic supravalvar pulmonary stenosis. There were no complications and no patient developed restenosis at follow-up. Autologous repair of supravalvar pulmonary stenosis is an effective technique.
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Introduction
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Isolated native supravalvar pulmonary stenosis (SVPS) occurs with a very low incidence [1, 2] and is the rarest form of pulmonary stenosis [3]. It is characterized by a stenosing ring of hypertrophic tissue located at the sinotubular junction of the main pulmonary artery (MPA). Other features are shortening of the free edge of the dysplastic cusps with tethering to the sinotubular constriction [3], creating various degrees of valvar stenosis, similar to what is found in supravalvar aortic stenosis [4]. Poststenotic dilatation is often present. Whether by clinical exam or echocardiogram, it is often mistaken for pulmonary valvar stenosis. Various techniques have been proposed to deal with this problem [1–3]. Balloon dilation, often attempted because of a presumptive diagnosis of valvar stenosis, is unsuccessful because of the elasticity and recoil of the sinotubular constrictive ring. The proximity of the pulmonary valve prohibits the use of a stent. In contrast, iatrogenic SVPS can occur after procedures involving the MPA such as repair of tetralogy of Fallot (TOF), the arterial switch operation (ASO), or pulmonary artery band (PAB) placement and removal [5]. Surgical repairs usually entail either simple resection of the stenotic area and reanastomosis, closing a longitudinal incision made across the stenotic area horizontally or patching the stenotic area with either a single patch (sometimes Y-shaped), or multiple patches [2, 5]. This report describes a patchless technique, using circumferential incisions and counterincisions, that is analogous to a technique described for repair of supravalvar aortic stenosis [4, 6].
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Technique
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Under standard cardiopulmonary bypass techniques and mild hypothermia (34°C), the branch PAs are mobilized extensively. Cardioplegic arrest is often necessary for adequate visualization, but is not an absolute requirement. The MPA is transected at the sinotubular junction in native SVPS, or at the site of the tightest stenosis in iatrogenic SVPS (Fig 1).
Branch PA ostia are inspected to exclude stenosis. The hypertrophic stenotic segment is excised. Each sinus is assessed as to its relative size, and a longitudinal incision carried into each one of them, taking it close to the pulmonary valve annulus. After the incisions into each sinus have been made, the pulmonary valve is easily exposed. The pulmonary cups are released from any attachments and commissurotomies performed as needed. Three incisions are then made into the (usually poststenotically dilated) distal MPA, opposite to each commissural post (Fig 2).
Both ends are then sutured together with a running polypropelene suture, paying precise attention to align "peaks" and "valleys" with each other (Fig 3).
Because the suture line is complicated, it helps to place marking stitches. Transesophageal echocardiographic (TEE) monitoring and right ventricle to left ventricle (RV/LV) pressure ratio as well as RV/PA gradients were obtained by direct measurements in all patients.
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Fig 2. Incisions are made into each sinus and into the distal main pulmonary artery at each commissural post.
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Results
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Between Jan 1, 2000 and Dec 31, 2002, eight patients underwent repair of SVPS using the described technique, either alone or as part of further cardiac repairs (see Table 1 for clinical details) at The University of Chicago Children's Hospital.
For patients with isolated SVPS, mean bypass and cross clamp time were 72 minutes (range, 50 to 108) and 42 minutes (range, 30 to 56), respectively.
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Table 1. Patients with Supravalvar Pulmonary Stenosis (SVPS) Treated With the Patchless Autologous Technique (Gradients Are Systolic in mm Hg)
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All patients did well. Prolonged intubation (2 weeks) was needed in a 2-month-old, formerly premature child with severe bronchopulmonary dysplasia and a large VSD who had a PA band placed at 1.2 kg because of hemodynamic instability during a septic episode. Each patient was contacted for this study. At a mean follow-up of 22 months (range, 6 to 34), all are asymptomatic and are thriving. By echocardiogram, no patient has developed either recurrent pulmonary stenosis or pulmonary valve regurgitation.
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Comment
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Recurrent stenosis after PA debanding, TOF repair, or any surgical intervention on the MPA occurs with low but persistent frequency [5]. Patches can be difficult to fashion correctly, do not address a posterior ridge, can distort the PA, or can shrink and calcify. Simple resection and end-end anastomosis results in a circumferential suture line that in itself can cause restenosis, as illustrated by the arterial switch experience. The present technique's main advantage is that it uses only native tissues. It is well-recognized that an all-autologous tissue repair offers the best potential for growth of the repair site in children. Because the flaps created from the poststenotically dilated MPA augment the sinuses in a physiologic manner, the reconstructed sinuses look much more "normal" than if they were patched. As long as there is an adequate distal MPA segment, this technique can also be used for SVPS occurring after banding, TOF repair, or other procedures. An important maneuver is mobilization of the branch PAs, in order to decrease tension on the repair. Drawbacks include a complicated suture line and a longer bypass time than for simple patching. In addition, it cannot be used for diffuse stenosis of the MPA or for branch PA stenosis. One such patient in our series had stenting of the distal left PA in conjunction with this repair. In conclusion, the described autologous repair is an effective technique to deal with native or iatrogenic supravalvar pulmonary stenosis.
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References
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- Roberts N., Moes C.A. Supravalvar pulmonary stenosis. J Pediatr 1973;82:838-841.[Medline]
- Mavroudis C., Backer C.L. Right ventricular outflow tract obstruction. In: Mavroudis C., Backer C.L., eds. Pediatric cardiac surgery. Philadelphia: Mosby, 1994:268-275.
- Milo S., Fiegel A., Shem-Tov A., Neufeld H.N., Goor D.A. Hour-glass deformity of the pulmonary valve: a third type of pulmonary stenosis. Br Heart J 1988;60:128-133.[Abstract/Free Full Text]
- Myers J.L., Waldhausen J.A., Cyran S.E., Gleason M.M., Weber H.S., Baylen B.G. Results of surgical repair of congenital supravalvar aortic stenosis. J Thorac Cardiovasc Surg 1993;105:281-288.[Abstract]
- Kirklin J.W., Appelbaum A., Bargeron L.M., Jr Primary repair versus banding for ventricular septal defects in infants. In: Kidd B.S.L., Rowe R.D., eds. The child with congenital heart disease after surgery. Mount Kisco, NY: Futura, 1976:3-5.
- Brom A.G. Obstruction of the left ventricular outflow tract. In: Khonsari S., ed. Cardiac surgery: safeguards and pitfalls in operative technique. Rockville, MD: Aspen, 1988:276-280.
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Abstract
Introduction
