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Ann Thorac Surg 1999;67:519-521
© 1999 The Society of Thoracic Surgeons

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Original Articles

Damus-Kaye-Stansel connections in children with previously transected pulmonary arteries

^a Cardiac Surgical Unit, Royal Children’s Hospital, Melbourne, Australia

Accepted for publication July 9, 1998.

Address reprint requests to Dr Karl, Cardiac Surgical Unit, Royal Children’s Hospital, Flemington Rd, Parkville, 3052, Victoria, Australia

	Abstract

Top Abstract Introduction Patients and methods Comment References

 
Background. In patients with a univentricular arteriovenous connection, transection of the main pulmonary artery may be performed as part of a bidirectional cavopulmonary shunt or Fontan procedure. The proximal stump of the pulmonary artery may remain in the systemic circulation. In cases with a discordant ventriculoarterial connection, subsequent restriction of the bulboventricular foramen may lead to subaortic stenosis. The subaortic stenosis can be corrected in some patients by directing the systemic flow through a combined nonobstructed aortopulmonary outlet, as in the Damus-Kaye-Stansel connection. Previous closure of the pulmonary artery has been considered by some investigators to be a relative contraindication to the Damus-Kaye-Stansel procedure, unless an allograft root can be added to the circuit after excision of the closed pulmonary stump.

Methods. Three patients with previously transected pulmonary arteries underwent a modified Damus-Kaye-Stansel connection using the native pulmonary valve and the proximal pulmonary artery stump.

Results. The native pulmonary valves have functioned well despite thrombus formation in the proximal stump in 2 patients before Damus conversion. All 3 patients are alive and well after 108, 19, and 3 months, with competent nonobstructed ventriculoarterial connections.

Conclusions. If transection and closure of the pulmonary artery as part of a previous palliation has spared the pulmonary valve, then the native pulmonary outlet might be used for a safe Damus-Kaye-Stansel connection.

	Introduction

Top Abstract Introduction Patients and methods Comment References

 
In univentricular hearts with a rudimentary right ventricle, transposed great vessels are common [1]. Some patients with this anatomy may benefit from early pulmonary artery banding to control high pulmonary blood flow [2, 3]. During a subsequent bidirectional cavopulmonary shunt (BCPS) or Fontan operation, the pulmonary artery (PA) may be transected and closed, leaving a blindly ending PA in communication with the systemic circulation [4].

Occasionally, subaortic stenosis (SAS) may develop after BCPS or Fontan operation [5]. The muscular bulboventricular foramen (BVF) becomes restrictive, and the SAS may create an unfavorable hemodynamic situation. This group of patients may benefit from a modified Damus-Kaye-Stansel operation (DKS) [5] to create an unobstructed outlet from both ventricles to the systemic circulation [6–9]. Recently, it has been suggested that an allograft root replacement is appropriate for this situation, after reopening or resection of the native pulmonary orifice [10].

Three patients with double inlet left ventricle and transposition of the great arteries had transection and closure of the main PA at the time of a BCPS or Fontan operation (sparing the valve) and subsequently developed SAS. The native pulmonary valve and the proximal PA remained suitable for a modified DKS connection.

	Patients and methods

Top Abstract Introduction Patients and methods Comment References

 
Patient A presented at 2 days of age with tricuspid atresia, transposition of the great arteries, coarctation of the aorta, and persistent ductus arteriosus (PDA). At 2 days of age he had a coarctation of the aorta repair, PDA ligation, and PA banding. He had a modified Fontan procedure (direct right atrium-to-PA connection with transection and closure of the main PA) at 3 years of age. At 6 years of age he developed SAS with a gradient over the BVF of 45 to 50 mm Hg (Doppler velocity 4 m/s). The mean right atrial pressure was 18 mm Hg and the PA wedge pressure was 14 mm Hg. The proximal PA was filling and emptying, and the valve leaflets were still mobile. The proximal PA was reopened and the pulmonary valve was found to be in excellent condition, with a 3- to 4-mm proximal stump. A modified DKS was performed, and the postoperative course was uneventful. At 108 months later he is asymptomatic, with good ventricular function. There is no pulmonary valve regurgitation and no ventricular outflow tract obstruction. He is in sinus rhythm.

Patient B presented at 8 days of age with double-inlet left ventricle, transposition of the great arteries, coarctation of the aorta, and PDA. At 10 days he had a PA band and coarctation of the aorta repair. At 1.5 years of age he had a BCPS with transection and closure of the main PA. At 3 years of age he had a fenestrated lateral tunnel Fontan operation, closure of the tricuspid valve (for treatment of insufficiency), and enlargement of a restrictive BVF. At 5 years of age he developed SAS with a gradient of 53 mm Hg over the BVF and subaortic infundibulum. There was a tiny defect in the previously closed tricuspid valve. Furthermore, there was a thrombus in the proximal pulmonary artery stump, but the pulmonary leaflets looked normal. During operation we removed a moderately adherent white thrombus from the stump, leaving the pulmonary valve in excellent condition. A modified DKS connection was performed, and the opening in the tricuspid valve was closed. The postoperative course was uneventful. Transthoracic echo after 3 months showed good ventricular function. There was trivial aortic valve regurgitation, no pulmonary valve regurgitation, no atrioventricular valve regurgitation, and no ventricular outflow tract obstruction. The 24-hour Holter monitoring showed continuous sinus rhythm.

Patient C presented at 8 days of age with double-inlet left ventricle, transposition of the great arteries, and hypoplastic right atrioventricular valve. At 12 days of age he had PA banding. At 9 months during a BCPS the PA was transected and closed. After 3 years he developed exertional chest pain. Cardiac catheterization revealed low PA pressure and a well-developed pulmonary arterial tree. There was no gradient between the left ventricle and aorta at rest; however, with the administration of isoproterenol a 50 mm Hg gradient developed. Transesophageal echocardiogram showed movements of the pulmonary valve leaflets although there was a large thrombus in the stump of the PA (Fig 1 ). During operation the PA stump was reopened and an organized thrombus was removed. There was a trileaflet pulmonary valve with thin pliable leaflets, although the anterior leaflet was very hypoplastic. A modified DKS was performed, along with an extracardiac Fontan operation. After bypass the superior vena cava pressure was 13 mm Hg, with common atrial pressure 5 mm Hg. The postoperative period was uneventful. He is now 3 months beyond his DKS, with an arterial oxygen percent saturation of 91%. Transthoracic echocardiogram after 2 weeks showed trivial pulmonary valve regurgitation, no atrioventricular valve regurgitation and no ventricular outflow tract obstruction. He is in sinus rhythm.

View larger version (68K): [in this window] [in a new window]   Fig 1. Transesophageal echocardiogram of patient C. The left atrioventricular valve is opened while the pulmonary leaflets are closed. The thrombus is visible in the stump.

	Comment

Top Abstract Introduction Patients and methods Comment References

The actuarial freedom from stoke after BCPS or Fontan is not clearly defined. The stroke prevalence after a Fontan or BCPS varies according to study group from 2% to 3.6% [13, 14]. The presence of severe polycythemia, an intracardiac right-to-left shunt, coagulation abnormalities, prosthetic material, intracardiac thrombus, and arrhythmias may account, at least in part, for this "background" stroke incidence. The Baltimore and St. Louis teams have described a 4% risk of embolic stroke (3 of 26 patients) after BCPS, Fontan, or both procedures in patients with a blindly ending PA connected to the systemic circulation [15]. Their current practice is to oversew or resect the pulmonary valve leaflets, which makes the pulmonary outlet unsuitable for a modified DKS unless an allograft root is placed in the pulmonary position [10]. If an allograft is ultimately required, this may introduce a new set of potential problems, including risk of damage to the proximal coronary arteries during implantation, and need for future reoperations to replace the allograft should it become incompetent. These risks might be greater than the incremental stroke risk noted for patients with blindly ending pulmonary arteries connected to the systemic circulation. This particular element of stroke potential is an important consideration, although we have not identified the problem in our own practice. Our Fontan patients (and selected BCPS patients) have been maintained on warfarin for life. We believe that this may have some benefit in stroke prevention, although this is unproven. We are currently addressing this problem in a multi-institutional randomized prospective study.

Another possible way to deal with this problem would be to leave a small degree of forward flow through the PA at the time of BCPS, which is our current practice if there is no evidence of SAS and if the superior vena cava pressure is < 12 to 15 mm. This would not be advisable, however, as part of a Fontan operation. A fourth approach would be to perform a DKS connection whenever the PA is divided, even if SAS is not present. This is probably not justified due to the long-term risk of pulmonary insufficiency in patients who may never develop SAS. However, if there is a strong substrate for development of SAS, then we would follow this strategy.

In conclusion, in selected patients with a univentricular heat and discordant ventriculoarterial connection, a DKS connection can be performed even after the PA has been transected and closed at a previous operation. The risk of embolus from the PA stump is small, especially in anticoagulated patients. SAS has become manifest within 3 years of the volume unloading procedure (BCPS or Fontan operation), and the DKS is a good solution to the problem. Therefore, on balance, our intention in future patients of this type is to close the PA without obliterating the valve, especially in patients who have a history of coarctation, and are thus at higher risk of developing SAS. The result of this policy will be closely monitored.

	References

Top Abstract Introduction Patients and methods Comment References

 

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This Article Abstract Full Text (PDF) 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): Christian P.R. Brizard Andrew D. Cochrane Tom R. Karl Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Broekhuis, E. Articles by Karl, T. R. Search for Related Content PubMed PubMed Citation Articles by Broekhuis, E. Articles by Karl, T. R.