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Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy

Published online by Cambridge University Press:  19 August 2008

Doff B. McElhinney ,
V. Mohan Reddy ,
Norman H. Silverman ,
Michael M. Brook  and
Frank L. Hanley
Doff B. McElhinney
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric CardiologyUniversity of California, San Francisco, San Francisco, CA, USA
V. Mohan Reddy*
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric CardiologyUniversity of California, San Francisco, San Francisco, CA, USA
Norman H. Silverman
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric CardiologyUniversity of California, San Francisco, San Francisco, CA, USA
Michael M. Brook
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric CardiologyUniversity of California, San Francisco, San Francisco, CA, USA
Frank L. Hanley
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric CardiologyUniversity of California, San Francisco, San Francisco, CA, USA
*
V Mohan Reddy, MD, University of California, San Francisco, Division of Cardiothoracic Surgery, 505 Parnassus Avenue, M593, San Francisco, CA 94143-0118. Tel: (415) 476-3501; Fax: (415) 476-9678
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Abstract

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpul-monary stenosis. A single patch technique, with closure of the zone of apposition (‘cleft’) in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosu-perior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

Type
Original Articles
Information
Cardiology in the Young , Volume 8 , Issue 4 , October 1998 , pp. 455 - 461
Copyright
Copyright © Cambridge University Press 1998

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References

1.Uretzky, G, Puga, FJ, Danielson, GK, Feldt, RH, Julsrud, PR, Seward, JB, Edwards, WD, McGoon, DC. Complete atrioven-tricular canal associated with tetralogy of Fallot: Morphologic and surgical considerations. J Thorac Cardiovasc Surg 1984;87:756–766.CrossRefGoogle ScholarPubMed
2.Pacifico, AD, Kirklin, JW, Bargeron, LM. Repair of complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle: Report of 10 patients. Ann Thorac Surg 1980;29:351–356.CrossRefGoogle ScholarPubMed
3.Vargas, FJ, Coto, EO, Mayer, JE, Jonas, RA, Castaneda, AR. Complete atrioventricular canal and tetralogy of Fallot: Surgical considerations. Ann Thorac Surg 1986;42:258–263.CrossRefGoogle ScholarPubMed
4.Guo-wei, H, Mee, RBB. Complete atrioventricular canal associ ated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: A report of successful surgical treatment. Ann Thorac Surg 1986;41:612–615.CrossRefGoogle Scholar
5.Ilbawi, M, Cua, C, DeLeon, S, Muster, A, PAul, M, Cutilletta, Arcilla R, Idriss, F. Repair of complete atrioventricular septal defect with tetralogy of Fallot. Ann Thorac Surg 1990;50:407–412.CrossRefGoogle ScholarPubMed
6.Fisher, RD, Bone, DK, Rowe, RD, Gott, VL. Complete atrioven tricular canal associated with tetralogy of Fallot: Clinical expe rience and operative methods. J Thorac Cardiovasc Surg 1975;70:265–271.CrossRefGoogle Scholar
7.Arciniegas, E, Hakimi, M, Farooki, ZQ, Green, EWResults of total correction of tetralogy of Fallot with complete atrioven tricular canal. J Thorac Cardiovasc Surg 1981;81:768–773.CrossRefGoogle Scholar
8.Mack, JW, Rogers, J, Wheller, J. Early total repair of tetralogy of Fallot associated with complete atrioventricular canal. J Cardiovasc Surg 1985;26:585–588.Google ScholarPubMed
9.Reddy, VM, Liddicoat, JR, McElhinney, DB, Brook, MM, Stanger, P, Hanley, FL. Routine primary repair of tetralogy of Fallot in neonates and infants under three months of age. Ann Thorac Surg 1995;60:S592-S596.CrossRefGoogle Scholar
10.Reddy, VM, McElhinney, DB, Parry, AJ, Brook, MM, Hanley, FL. Atrioventricular valvar function after primary repair of atri oventricular septal defects by the single patch technique in infancy: when should we repair? J Thorac Cardiovasc Surg (In press)Google Scholar
11.Rastelli, GC, Kirklin, JW, Titus, JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc 1966;41:296–308.Google ScholarPubMed
12.Silverman, NH. Pediatric Echocardiography. Baltimore: Williams & Wilkins, 1993: 143–166.Google Scholar
13.Hanley, FL, Fenton, KN, Jonas, RA, Mayer, JE, Cook, NR, Wernovsky, G, Castaneda, AR. Surgical repair of complete atri oventricular canal defects in infancy: Twenty-year trends. J Thorac Cardiovasc Surg 1993;106:387–397.CrossRefGoogle Scholar
14.Bando, K, Turrentine, MW, Sun, K, Sharp, TG, Ensing, GJ, Miller, AP, Kesler, KA, Binford, RS, Carlos, GN, Hurwitz, RA, Caldwell, RL, Darragh, RK, Hubbard, J, Cordes, TM, Girod, DA, King, H, Brown, JWSurgical management of complete atrioventricular septal defects: A twenty-year experience. J Thorac Cardiovasc Surg 1995;110:1543–1554.CrossRefGoogle ScholarPubMed
15.Michielon, G, Stellin, G, Rizzoli, G, Milanesi, O, Rubino, M, Moreolo, GS, Casarotto, D. Left atrioventricular valve incompe tence after repair of common atrioventricular canal defects. Ann Thorac Surg 1995;60:S604–S609.CrossRefGoogle Scholar
16.Hennein, HA, Mosca, RS, Urcelay, G, Crowley, DC, Bove, EL. Intermediate results after complete repair of tetralogy of Fallot in neonates. J Thorac Cardiovasc Surg 1995;109:332–344.CrossRefGoogle ScholarPubMed
17.Suzuki, K, Ho, SY, Anderson, RH, Becker, AE, Neches, WH, Devine, WA, Tatsuno, K, Minori, S. Morphometric analysis of atrioventricular septal defect with common valve orifice. J Am CollCardiol 1998;31:217–223.CrossRefGoogle ScholarPubMed
18.Zellers, TM, Zehr, R, Weinstein, E, Leonard, S, Ring, WS, Nikaidoh, H. Two-dimensional and Doppler echocardiography alone can adequately define preoperative anatomy and hemo-dynamic status before repair of complete atrioventricular sep tal defect in infants < 1 year old. J Am Coll Cardiol 1994;24:1565–1570.CrossRefGoogle Scholar
19.Capouya, ER, Laks, H, Drinkwater, DC, Pearl, JM, Milgalter, E. Management of the the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg 1992; 104:196–203.CrossRefGoogle ScholarPubMed
20.Backer, CL, Mavroudis, C, Alboliras, ET, Zales, VR. Repair of complete atrioventricular canal defects: Results with the two-patch technique. Ann Thorac Surg 1995;60:530–537.CrossRefGoogle ScholarPubMed
21.Tlaskal, T, Hucin, B, Kostelka, Chaloupecky V, Marek, J, Tax, P, Janouaek, J, Hruda, J, Reich, O, Skovranek, J. Repair of tetralogy of Fallot associated with atrioventricular septal defect. Cardiol Young 1998;8:105–112.CrossRefGoogle ScholarPubMed
22.Clapp, S, Perry, BL, Farooki, ZQ, Jackson, WL, Karpawich, PP, Hakimi, M, Arcinegas, E, Green, EW, Pinsky, WWDown’s syn drome, complete atrioventricular canal, and pulmonary vascu lar obstructive disease. J Thorac Cardiovasc Surg 1990;100:115–121.CrossRefGoogle Scholar