Ann Thorac Surg 2004;78:e45-e47
© 2004 The Society of Thoracic Surgeons
Case report
Successful aortic root remodeling for repair of a dilated pulmonary autograft after a ross-Konno procedure in early childhood
Shintaro Nemoto, MD, PhDa,
Catherine Sudarshan, MD, FRCSa,
Christian P. R. Brizard, MDa,*
a Cardiac Surgery Unit, Royal Children's Hospital, Parkville, Victoria, Australia
Accepted for publication February 3, 2004.
* Address reprint requests to Dr Brizard, Department of Cardiac Surgery, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3052, Australia
christian.brizard{at}rch.org.au
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Abstract
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We report a case of successful reoperation for pulmonary autograft root dilatation causing severe regurgitation after a Ross-Konno procedure in an infant. The procedure consisted of reduction of the circumference of the sinotubular junction and the autograft annulus. This technique is an effective alternative to prolong the transition period after a Ross procedure prior to other definitive surgeries, such as valve-sparing aortic root replacement or prosthetic valve replacement, which are preferably avoided at this young age.
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Introduction
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Studies investigating late results after the Ross procedure have shown the emerging risk of pulmonary autograft dilatation [1–3], especially when implanted as a freestanding root replacement. Although reoperation has been successful for autograft dilation causing severe regurgitation in the adult population [1, 4, 6] and in relatively older children [3, 7], those performed in early childhood have rarely been described. Moreover, applying the reported reoperative techniques, such as valve-sparing aortic root replacement or prosthetic valve replacement in children remains controversial. We report the case of a 34-month-old boy in whom progressive pulmonary autograft dilation developed 10 months after a Ross-Konno operation and caused severe regurgitation. Reoperation was successfully performed by aortic root remodeling for reduction of the autograft dilation while preserving the valve leaflets.
A boy, 2 and a half years old, was diagnosed to have interruption of the aortic arch ([IAA] type B), subaortic narrowing, and an infundibular ventricular septal defect (VSD). During his neonatal period, he had undergone a single-stage repair, which consisted of aortic arch repair and transpulmonary patch closure of the VSD by Luciani's method for the subaortic narrowing [5]. Although his postoperative course was straightforward and the pressure gradient across the aortic valve had not been significant in the early postoperative course, the gradient developed rapidly in a few months, reaching approximately 100 mm Hg at 6 months after the surgery. The echocardiography showed a bicuspid aortic valve (6.5 mm in diameter) and tubular subaortic stenosis. An elective Ross-Konno procedure was therefore performed at the age of 6 months. The pulmonary autograft was implanted utilizing the root replacement technique and sutured onto the left ventricular muscle below the aortic valvular ring. The right ventricular tract was reconstructed using a pulmonary homograft (16 mm in diameter). The patient's progress was uneventful, and he was discharged with mild aortic regurgitation documented on echocardiography.
Eleven months after the second operation, clinical signs of aortic regurgitation were noted without symptoms. Echocardiography showed severe aortic regurgitation. The pathophysiology of the regurgitation was attributed to the dilated aortic ring at the nadir of the cusps and dilation of the sinotubular junction (both 21 mm in diameter). The left ventricular end-diastolic dimension (LVEDD) had increased from 3.3 cm to 4.3 cm in the previous 6 months. However, the valve leaflets of the autograft appeared to be slightly thickened and LV function was well preserved. A third operation was therefore indicated.
Under cardioplegic arrest, the aorta was transected along the previous suture line of the pulmonary autograft. There was significant dilatation of the sinotubular junction and minimal thickening of the edges of the left and right coronary cusps. Three triangular wedges of the autograft wall were resected perpendicular to the sinotubular junction directed toward the sinus of Valsalva (Fig 1). The defects created were directly approximated. The area of each triangle resection was designed so as to reduce the diameter of the sinotubular junction to slightly less than the length of the free edge of each opposing cusp.
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Fig 1. Three triangular wedges of the autograft were resected perpendicular to the sinotubular junction directed toward the sinus of Valsalva. Dashed lines represent the suture lines of the coronary artery cuffs at the previous Ross-Konno procedure. (LCA = left coronary artery; RCA = right coronary artery.)
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After completion of the reconstruction and closure of the ascending aorta, the aortic cross-clamp was released, which was followed by spontaneous resumption of sinus rhythm. Although weaning from cardiopulmonary bypass was uneventful, transesophageal echocardiography showed residual moderate aortic regurgitation. The aorta was reopened under cardioplegic arrest on the second pump run. Each commissural interleaflet triangle was plicated at the base by applying a figure-of-eight suture using 3-0 Ticron (Tyco Healthcare Group LP, Norwalk, CT), and the sutures were tied outside the aortic wall (Fig 2). After reconstruction of the ascending aorta, the heart was reperfused and spontaneous sinus rhythm resumed. The patient weaned from the cardiopulmonary bypass uneventfully. Transesophageal echocardiography at this juncture revealed trivial aortic regurgitation with good LV function. His postoperative course was uneventful, and he was discharged on day 7 with no anticoagulation therapy. Six months after the last surgery, his exercise tolerance had dramatically improved. The last echocardiography showed normal LV function with reduced LVDD from 4.3 cm preoperatively to 3.5 cm postoperatively despite mild residual autograft regurgitation.
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Fig 2. Each commissural interleaflet triangle was plicated at the base by applying a figure-of-eight suture using 3-0 Ticron, and the sutures were tied outside the aortic wall (asterisk). Dashed lines represent the suture lines of the coronary artery cuffs at the previous Ross-Konno procedure. (LCA = left coronary artery; RCA = right coronary artery.)
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Comment
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The Ross procedure has been a cornerstone in the surgical management of a small aortic annulus in neonates and infants, especially when combined with the Konno procedure [9]. The pulmonary autograft dilatation is known to occur constantly in adults [1, 2] and older children [3, 7]. Pathologic abnormalities in explanted dilated pulmonary autograft root have been characterized by focal interruption of the media of the autograft wall with total absence of elastin fibers and intimal proliferation with fibrosis [8]. On the other hand, although significant aortic sinus dilation (mean change in z-value, +2.1) occurs within 1 year after the Ross-Konno operation in neonates and infants under 1 year of age [9], very few patients have significant dysfunction of the autograft requiring reoperation in this population. None of reoperative procedures for the autograft dilatation reported in adults and older children (ie, valve-sparing aortic root replacement, simple valve replacement [1, 4, 6], or reduction and fixation of the annulus and sinotubular junction of the autograft) meets the requirements of expectation of aortic annulus growth and avoidance of anticoagulation therapy during the early years of life. Accordingly, we decided to extend the life of the autograft and prolong a transition period to a future definitive repair.
An early reintervention was best indicated in this case to minimize damage to the neoaortic cusps, in turn achieving a successful repair. The technique described here aimed to restore a coaptation surface of the free edges, building a large surface of cusp apposition. However, although LV function was well preserved in this case, the mildly thickened cusps already existed and will never be changed by this technique itself. Consequently, the thickening would have an important role in the long-term outcome because thickening of the cusps, especially the free edge of the leaflets, caused by even small regurgitation often leads to significant consequences, such as increasing stress on the hinge point on each commissure, inadequate apposition of the free edges during the late phase of systole, and inadequate coaptation during early diastole. Therefore, in this case, we should have considered the reintervention earlier before the cusps were thickened. In addition, because the inherent structural difference of the pulmonary autograft resulting in dilation will exist even after this procedure, careful future surveillance of this patient is mandatory.
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References
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- David TE, Omran A, Ivanov J, et al. Dilation of the pulmonary autograft after Ross procedure. J Thorac Cardiovasc Surg. 2000;119:210–220[Abstract/Free Full Text]
- Elkins RC, Lane MM, McCue C. Ross operation in children: late results. J Heart Valve Dis. 2001;10:736–741[Medline]
- Laudito A, Brook MM, Suleman S, et al. The Ross procedure in children and young adults: a word of caution. J Thorac Cardiovasc Surg. 2001;122:147–153[Abstract/Free Full Text]
- Leyh RG, Kofidis T, Fischer S, Kallenbach K, Harringer W, Haverich A. Aortic root reimplantation for successful repair of an insufficient pulmonary autograft valve after the Ross procedure. J Thorac Cardiovasc Surg. 2002;124:1048–1049[Free Full Text]
- Luciani GB, Ackerman RJ, Chang AC, Wells WJ, Starnes VA. One-stage repair of interrupted aortic arch, ventricular septal defect, and subaortic obstruction in the neonate: a novel approach. J Thorac Cardiovasc Surg. 1996;111:348–358[Abstract/Free Full Text]
- Schmidtke C, Stierle U, Sievers HH. Valve sparing aortic root remodeling for pulmonary autograft aneurysm. J Heart Valve Dis. 2002;11:504–505[Medline]
- Lupiretti FM, Duncan BW, Lewin M, Dyamenahalli U, Rosenthal GL. Comparison of autograft and allograft aortic valve replacement in children. J Thorac Cardiovasc Surg. 2003;126:240–246[Abstract/Free Full Text]
- Takkenberg JJM, Zondervan PE, van Herwerden LA. Progres-sive pulmonary autograft root dilation and failure after Ross procedure. Ann Thorac Surg. 1999;67:551–554[Abstract/Free Full Text]
- Ohye RG, Gomez CA, Ohye BJ, Goldberg CS, Bove EL. The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function. Ann Thorac Surg. 2001;72:823–830[Abstract/Free Full Text]
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Abstract
Introduction
