Hostname: page-component-77c89778f8-gq7q9 Total loading time: 0 Render date: 2024-07-24T13:06:46.057Z Has data issue: false hasContentIssue false
  • English
  • Français

Atrioventricular septal defects with severe left ventricular hypoplasia—clinical findings and surgical options

Published online by Cambridge University Press:  19 August 2008

Carlos J. Troconis ,
Roberto M. Di Donato ,
Bruno Marino ,
Ugo Vairo  and
Carlo Marcelletti
Carlos J. Troconis
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Roberto M. Di Donato*
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Bruno Marino
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Ugo Vairo
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Carlo Marcelletti
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
*
Dr. Roberto Di Donato, Department of Cardiac Surgery, Bambino Gesu Hospital, Piazza Sant' Onofrio 4, 00165 Rome, Italy. Tel. 6–65191, Fax 6–65192296
Get access Rights & Permissions [Opens in a new window]

Summary

Right ventricular dominance was observed in 22 (6.6%) out of 332 patients with atrioventricular septal defects. Their mean age was 2.24 ± 2.73 months. Chromosomal anomalies were seen in only three patients (Down's syndrome in two and trisomy nine in one). Seventeen patients underwent surgical treatment. A pulmonary artery banding was performed in eight of them with one long-term survivor who underwent a successful staged orthoterminal repair. A Norwood-type of palliation was attempted in four without success. Biventricular repair was performed in four additional patients with two mid-term survivors, in one of whom the patch used for closure of the atrial septal defect was fenestrated. One patient underwent successful heart transplantation at seven months of age. Pathophysiological problems and surgical options are discussed.

Keywords

Atrioventricular septal defectright ventricular dominance
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 1 , January 1992 , pp. 53 - 55
Copyright
Copyright © Cambridge University Press 1992

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Quero Jiménez, M, Pérez Martínez, VM, Maitre Azcarate, MJ, Merino Batres, G, Moreno Granados, F. Exaggerated displace ment of the atrioventricular canal towards the bulbus cordis (rightward displacement of the mitral valve). Br Heart J 1973; 35: 453–460.CrossRefGoogle Scholar
2.Freedom, RM, Bini, RM, Rowe, RD. Endocardial cushion defect and significant hypoplasia of the left ventricle: a distinct clinical and pathological entity. Eur J Cardiol 1978; 7: 262–281.Google ScholarPubMed
3.Bharati, S, Lev, M. The spectrum of common atrioventricular orifice (canal). Am Heart J 1973; 86: 553–561.CrossRefGoogle ScholarPubMed
4.Corno, A, Marino, B, Catena, G, Marcelletti, C. Atrioventricular septal defects with severe left ventricular hypoplasia. Staged palliation. J Thorac Cardiovasc Surg 1988; 96: 249–252.CrossRefGoogle ScholarPubMed
5.Marino, B, Vairo, U, Corno, A, Nava, S, Guccione, P, Calabró, R, Marcelletti, C. Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome. Am J Dis Child 1990; 144: 1120–1122.CrossRefGoogle ScholarPubMed
6.Studer, M, Blackstone, EH, Kirklin, JW. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg 1982; 84: 523–542.CrossRefGoogle ScholarPubMed
7.Chin, AJ, Keane, JF, Norwood, WI, Castańeda, AR. Repair of complete common atrioventricular canal in infancy. J Thorac Cardiovasc Surg 1982; 84: 437–445.CrossRefGoogle ScholarPubMed
8.Marino, B, Digilio, MC, Giannotti, A, Dallapiccola, B. Atrioventricular canal associated with trisomy 9. Chest 1989; 96: 1420–1421.CrossRefGoogle ScholarPubMed
9.Piccoli, GP, Ho, SY, Wilkinson, JL, Macartney, FJ, Gerlis, LM, Anderson, RH. Left-sided obstructive lesions in atrioventricu lar septal defects. An anatomic study. J Thorac Cardiovasc Surg 1982; 83: 453–460.CrossRefGoogle Scholar
10.Chang, CI, Becker, AE. Surgical anatomy of left ventricular outflow tract obstruction in complete atrioventricular septal defect. A concept for operative repair. J Thorac Cardiovasc Surg 1987; 94: 897–903.CrossRefGoogle ScholarPubMed
11.Draulans-Noe, HAY, Wenink, ACG. Anterolateral muscle banding of the left ventricle in atrioventricular septal defect: left ventricular outflow tract and subaortic stenosis. Pediatr Cardiol 1991; 12: 83–88.CrossRefGoogle Scholar
12.De Biase, L, Di Ciommo, V, Ballerini, L, Bevilacqua, M, Marcelletti, C, Marino, B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down's syndrome. J Thorac Cardiovasc Surg 1986; 91: 467–469.CrossRefGoogle ScholarPubMed
13.Thies, WR, Bargeron, LM, Bini, RM, Colvin, EV, Soto, B. Spectrum of hearts with one underdeveloped and one dominant ventricle. Pediatr Cardiol 1986; 7: 129–139.CrossRefGoogle ScholarPubMed