Ann Thorac Surg 1997;63:425-428
© 1997 The Society of Thoracic Surgeons
Original Article: Cardiovascular
Allograft Root on Closed Pulmonary Valve for Subaortic Obstruction in Double-Inlet Left Ventricle With Transposition of the Great Arteries
Ad J. J. C. Bogers, MD, PhD,
Wim-Jan van Boven, MD,
Adri H. Cromme-Dijkhuis, MD, PhD
Department of Thoracic Surgery and Pediatric Cardiology, Sophia/Dijkzigt University Hospital, Rotterdam, the Netherlands
Accepted for publication August 19, 1996.
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Abstract
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Background. Until recently closure of the pulmonary valve during staged Fontan-type palliation in the setting of double-inlet left ventricle with an unrestrictive or adequately enlarged ventricular septal defect and transposition of the great arteries with the aorta on a left-sided outflow chamber was regarded as an appropriate part of surgical treatment. Lately, however, an increased incidence of subsequent subaortic obstruction has been described in this regard.
Methods. Allograft root placement on the previously closed pulmonary orifice in combination with a modified Damus-Kaye-Stansel procedure is described to create an unobstructed outflow from the main ventricle to the systemic circulation. This procedure was done in 3 patients. One root placement was combined with the construction of the bidirectional superior cavopulmonary connection, one was done as an intermediate step before completion of the cavopulmonary connection, and one was combined with completion of total cavopulmonary connection.
Results. Immediate relief of the subaortic obstruction was achieved in all 3 patients. Ventricular hypertrophy, echocardiographically assessed by diastolic posterior wall thickness, regressed to normal in all 3 within 6 to 12 months.
Conclusions. Allograft root placement on the reopened pulmonary orifice in double-inlet left ventricle with a ventricular septal defect and transposition of the great arteries appears technically feasible and functionally adequate on short-term follow-up. This procedure should result in regression of ventricular hypertrophy to allow eligibility for a Fontan-type palliation again. To what extent possible failure of the allograft increases the risk of an adverse outcome of this palliation may be a matter of concern.
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Introduction
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Analogous to operation for other cardiac anomalies with univentricular connections, closure of the pulmonary orifice during (staged) Fontan-type [1] palliation in hearts with double-inlet left ventricle (DILV) with a unrestrictive or adequately enlarged ventricular septal defect (VSD) and transposition of the great arteries (TGA) with the aorta on a left-sided outflow chamber was until recently regarded as an appropriate part of the surgical treatment, even in the absence of pulmonary stenosis [2, 3]. Lately, however, it has become evident that in patients with DILV, VSD, and TGA, an originally unrestrictive VSD as well as a surgically enlarged VSD may become restrictive at any stage of palliation in the Fontan-type palliation [4–6]. Previous banding of the pulmonary artery is being reported as an incremental risk factor for this complication [2, 4, 5, 7]. The development of subaortic stenosis may well lead to myocardial hypertrophy, resulting in an unfavorable situation with regard to a Fontan-type operation, either to be completed or already completed [4, 5, 8]. Because the VSD or the outflow chamber is prone to become restrictive, the treatment of either actual or possible subaortic stenosis in this congenital malformation is still a matter of debate, with recent evolution of approaches to early construction of an adequate arterial outlet including the pulmonary valve [5, 6, 9].
In this regard pulmonary-aortic connection as in the Damus-Kaye-Stansel [10–12] or Norwood [13] constructions, or the arterial switch procedure or hemiswitch procedure with transfer of the coronary orifices to the pulmonary artery and closure of the aortic orifice, as part of a staged surgical approach have been advocated [2, 14]. More recently both Damus-Kaye-Stansel construction with an aortopulmonary shunt [15, 16] and the arterial switch operation with atrial septectomy has been described in this regard as primary surgical therapy in young children [8, 16, 17]. All of these options are only feasible when the pulmonary valve and root are still available and adequately functioning.
In this regard we report 3 cases of DILV, VSD, and TGA in which an allograft root placement was done on the previously closed pulmonary orifice in combination with a modified Damus-Kaye-Stansel procedure to create an unobstructed outflow from the main ventricle to the systemic circulation. Each of the root placements was done at a different stage of surgical treatment.
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Material and Methods
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Patients
All three patients presented with central atrial septal defect, DILV, unrestrictive VSD, and TGA. In addition patient A had a hypoplastic tricuspid valve, patient B had a type A interrupted aortic arch with a duct-dependent systemic circulation, and patient C had a straddling tricuspid valve. In patient A a balloon atrioseptostomy was performed at the age of 2 months and a banding of the pulmonary artery at the age of 3 months. Analysis at 13 months of age showed dilatation of the systemic ventricle, normal systemic pressures, and an invasive peak systolic gradient of 10 mm Hg over the VSD and of 70 mm Hg over the banding. A bidirectional superior cavopulmonary anastomosis was constructed. The atrial septal defect was enlarged. The VSD was enlarged by resection of the inferior border of the defect down to the level of the apex of the outlet chamber. The pulmonary valve was closed with division of the pulmonary main stem. The postoperative course was without events. The transcutaneous oxygen saturation (StcO2) was 75% to 80%. At 3 years of age the exercise tolerance decreased. The StcO2 was still 75% to 81%. Analysis showed subaortic obstruction both at the VSD with an invasive systolic gradient of 50 mm Hg and at the systemic outflow tract with a gradient of 55 mm Hg. The left ventricle showed marked hypertrophy with good contractility. In addition, the bidirectional superior cavopulmonary anastomosis was functioning properly and the pulmonary artery pressure was 14 mm Hg. At the age of 3.5 years the patient was reoperated on for allograft root placement.
Patient B was first operated on at neonatal age. The aortic arch was reconstructed with an extended direct anastomosis, using extracorporeal circulation with deep hypothermia and circulatory arrest. During rewarming the VSD and atrial septal defect were enlarged, the VSD by resection of the myocardium inferior to the defect. At the end of the procedure the pulmonary artery was banded. On dismissal the StcO2 was 75% to 80%. At the age of 6 months a bidirectional superior cavopulmonary anastomosis was constructed with reconstruction of the right pulmonary artery and with closure of the pulmonary valve and division of the pulmonary main stem. The VSD was again electively enlarged. At the age of 3 years progressive subaortic stenoses and developing myocardial hypertrophy were diagnosed. The invasive subaortic systolic gradient was 20 mm Hg. Root placement was performed.
Patient C presented at the age of 1 month. A pulmonary artery banding was done at the age of 3 months, which was redone at the age of 6 months because of obstruction of the right pulmonary artery. At the age of 2 years a bidirectional superior cavopulmonary anastomosis was performed. The pulmonary valve was closed, with division of the pulmonary mainstem. The VSD was electively enlarged by resection of the inferior border of the defect. The atrial septal defect was also electively enlarged. The postoperative course was uneventful, with an StcO2 of 75% to 80%. Already at the age of 2.5 years the VSD had become restrictive. Cardiac catheterization showed a systolic gradient of 20 mm Hg. The superior cavopulmonary connection was well functioning. At a later operation the total cavopulmonary connection was completed. The VSD was again enlarged by resection of fibromuscular tissue at the inferior border of the defect. The postoperative course was uneventful. After 1 year, at the age of 3.5 years, signs of subaortic obstruction recurred. Analysis showed an invasive systolic gradient of 50 mm Hg along with progressive ventricular hypertrophy and mild tricuspid incompetence. Root placement was performed.
Operation
The preoperative evaluation did not include selective coronary arteriography. The aortography did not result in detailed anatomy regarding the course of the proximal coronary arteries in relation to the closed pulmonary orifice.
Using cardiopulmonary bypass and cardioplegic arrest, the pulmonary stump was reopened at the suture line. In all 3 patients the remnants of the pulmonary valve inside the annulus as well as the remnants of the pulmonary root on top of the annulus were excised. An aortic allograft 20, 19, and 18 mm in diameter, respectively, was implanted on the pulmonary orifice with running Prolene (Ethicon, Somerville, NJ) sutures. Care was taken to keep the suture line within the borders of the pulmonary orifice and right ventricular outflow tract to avoid the proximal coronary arteries. To prevent distortion or traction, the allograft was in all three procedures oriented with the mitral part at the nonfacing area of the pulmonary orifice, directing the natural curve of the allograft root toward the native aortic root. The ascending aorta was transected proximal to the brachiocephalic artery at a level that resulted in a comparable length of both the native ascending aorta and the newly placed allograft. Both from the adjacent segments of the allograft and the aortic root, a U-shaped segment was resected, and a side-to-side anastomosis was performed (Fig 1
). This double-valved systemic root was anastomosed end-to-end to the ascending aorta (see Fig 1). In patient B the total cavopulmonary connection was completed at the same operation. In patient C the tricuspid valve was closed.
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Fig 1. . Double-inlet left ventricle with ventricular septal defect and transposition of the great arteries with the aorta on a left-sided outflow chamber. (Top) As in patient A, previously a bidirectional superior cavopulmonary anastomosis (CA) had been constructed and the pulmonary orifice (PO) closed. Enlargement of the atrial septal defect and the ventricular septal defect is not shown. (Bottom) The pulmonary orifice was reopened. The allograft root (AL) was placed onto this orifice with its inner curvature oriented toward the native aortic root (AO). The native aortic root was transected. From both roots an adjacent U-shaped segment was removed, and a side-to-side anastomosis is performed. This new double-valved root was reanastomosed to the ascending aorta.
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Intraoperative epicardial echocardiography showed competent allograft and aortic valves without a gradient in all 3 patients. The postoperative courses were uneventful.
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Results
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In patient A StcO2 was 75% to 80%. At 1 year after discharge a clear regression of ventricular hypertrophy was observed by echocardiography. The diastolic posterior wall thickness of the main ventricle decreased from 12 to 5 mm. Cardiac catheterization showed no gradient between the main ventricle and the aorta. Once ventricular wall thickness and dimensions have returned to normal, the patient will be scheduled for total cavopulmonary connection. In patient B there was echocardiographically no gradient over the ventricular outflow tract at 6 months after the last operation. Ventricular hypertrophy was diminished but still present, and the diastolic posterior wall thickness decreased from 8 to 4 mm. Patient C is doing well 9 months after this last operation, with echocardiographic signs of regression of ventricular hypertrophy, the diastolic posterior wall thickness being decreased from 7 to 4 mm.
At 12, 6, and 9 months, respectively, after root placement all three native aortic valves show normal function without signs of obstruction or regurgitation. The allograft valves do not show any obstruction, but in patient A there is slight regurgitation and in patient B moderate regurgitation. In patient C there is no allograft regurgitation.
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Comment
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By now it has become clear that irrespective of the stage of treatment, subaortic stenosis in DILV with VSD and TGA occurs frequently and behaves progressively [4]. Our small series is an additional example of this phenomenon. At present the best way to prevent subaortic stenosis in this setting is to construct an adequate ventricular outflow to the systemic circulation as early as possible in all cases [5, 6, 15, 16]. Especially patients with previous banding of the pulmonary artery are at risk for the development of subaortic stenosis and should be followed up closely to facilitate timely treatment [7, 9, 18]. This is also well demonstrated in the presented patients.
However, some patients have been treated in the past with an operation that included closure of the pulmonary valve, which at the time of the operation seemed appropriate, as was the case in our patients. This precludes conventional reconstructions of the arterial outlet in the subset of these patients in whom restriction of the VSD or the subaortic outlet chamber occurred or recurred. For these patients allograft root placement on the reopened pulmonary orifice appears technically feasible and functionally adequate on short-term follow-up. As shown in our 3 patients, this procedure can be performed at any stage of Fontan-type palliation. In fact, allograft root placement on a reopened pulmonary orifice should allow ventricular function to recover from hypertrophy to allow eligibility for a Fontan-type palliation again. Further follow-up should reveal to what extent possible failure of the allograft increases the risk of an adverse outcome of this palliation.
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Footnotes
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Address reprint requests to Dr Bogers, Thoracic Surgery, Thoraxcentre, Bd 156, Sophia/Dijkzigt University Hospital, Dr Molewaterplein 40, 3015 GD Rotterdam, the Netherlands
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References
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Abstract
Introduction
