Hostname: page-component-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-23T09:01:57.292Z Has data issue: false hasContentIssue false
  • English
  • Français

Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?

Published online by Cambridge University Press:  19 August 2008

Carlo Vosa ,
Paolo Arciprete ,
Giuseppe Caianiello  and
Gaetano Palma
Carlo Vosa*
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Paolo Arciprete
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Giuseppe Caianiello
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Gaetano Palma
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
*
Dr Carlo Vosa, Hospital “V. Monaldi”, Naples, Italy. Tel. 35-81-5462091.
Get access Rights & Permissions [Opens in a new window]

Summary

Between February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

Keywords

Pulmonary atresiaintact ventricular septumright ventricletricuspid valvemodified Blalock-Taussig shunt
Type
World Forum for Pediatric Cardiology Symposium on Pulmonary Atresia
Information
Cardiology in the Young , Volume 2 , Issue 4 , October 1992 , pp. 391 - 394
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.Moulton, AL, Bowman, FO, Edie, RN, Hayes, CJ, Ellis, K, Gersony, WM, Malm, JR. Pulmonary atresia with intact ventricular septum. J Thor Cardiovasc Surg 1979; 78: 527–536.Google Scholar
2.de Leval, M, Bull, C, Stark, J, Anderson, RH, Taylor, JFN, Macartney, F. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification. Circulation 1982; 66: 272–280.CrossRefGoogle ScholarPubMed
3.Cobanoglu, A, Metzdorff, MT, Pinson, CW, Grunkemeier, GL, Sunderland, CO, Starr, A. Valvotomy for pulmonary atresia with intact ventricular septum. J Thor Cardiovasc Surg 1985; 89: 482–490.CrossRefGoogle ScholarPubMed
4.Milliken, JC, Laks, H, Hellenbrand, W, George, B, Chin, A, Williams, RG. Early and late results in the treatment of patients with pulmonary atresia and intact ventricular septum. Circulation 1985; 72 (Suppl II): II 61–II 69.Google ScholarPubMed
5.Foker, JE, Braunlin, EA, St. Cyr, JA, Hunter, D, Molina, JE, Moller, JH, Ring, WS. Management of pulmonary atresia with intact ventricular septum. J Thor Cardiovasc Surg 1986; 92: 706–715.CrossRefGoogle ScholarPubMed
6.Coles, JG, Freedom, RM, Lightfoot, NE, Dasmahapatra, HK, Williams, WG, Trusler, GA, Burrows, PE. Long term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1989; 47: 213–217.CrossRefGoogle ScholarPubMed
7.Andrade, JL, Serino, W, de Leval, M, Somerville, J. Two dimensional echocardiographic evaluation of tricuspid hypoplasia in pulmonary atresia. Am J Cardiol 1984; 53: 387–388.CrossRefGoogle ScholarPubMed
8.Marino, B, Franceschini, E, Ballerini, L, Marcelletti, C, Thiene, G. Anatomical-echocardiographic correlation in pulmonary atresia with intact ventricular septum. Use of subcostal cross-sectional views. Int J Cardiol 1986; 11: 103–109.Google Scholar
9.King, DH, Smith, EO, Huhta, JC, Gutgesell, HP. Mitral and tricuspid annular diameter in normal children determined by two-dimensional echocardiography. Am J Cardiol 1985; 55: 787–789.CrossRefGoogle Scholar
10.Bull, C, de Leval, MR, Mercanti, C, Macartney, FJ, Anderson, RH. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation 1982; 66 (Suppl II): II 266–II 271.Google Scholar
11.de Leval, M, Bull, C, Hopkins, R, Rees, P, Deanfield, J, Taylor, JFN, Gersony, W, Stark, J, Macartney, FJ. Decision making in the definitive repair of the heart with a small right ventricle. Circulation 1985; 72 (Suppl II): II 52–II 60.Google Scholar
12.Vosa, C, Renzulli, A, Cotrufo, M. Systemic subclavian pulmonary shunts with PTEE in the first week of life. In: Doyle, EE, Engle, MA, Gersony, WM, Rashkind, WJ, Talner, NJ (eds). Pediatric Cardiology. Springer Verlag Inc., New York, 1986, pp 508–510.CrossRefGoogle Scholar
13.Alboliras, ET, Julsrud, PR, Danielson, GK, Puga, FJ, Schaff, HV, McGoon, DC, Hagler, DJ, Edwards, WD, Driscoll, DJ. Definitive operation for pulmonary atresia with intact ventricular septum. Results in 20 patients. J Thor Cardiovasc Surg 1987; 93: 454–464.CrossRefGoogle Scholar