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Critical care outcomes in pulmonary atresia and intact ventricular septum undergoing single-ventricle palliation

  • Mark A. Walsh (a1), Kentaro Asoh (a1), Glen S. Van Arsdell (a2) and Tilman Humpl (a1) (a3)

Abstract

Objective

To examine early outcomes for pulmonary atresia with intact ventricular septum undergoing single-ventricle palliation and to determine risk factors for mortality.

Design

Retrospective observational study.

Setting

Tertiary paediatric critical care unit.

Intervention

Risk factors for mortality were sought for infants after the primary intervention whether surgical shunt or ductal stent.

Measurements and main results

We reviewed outcomes of 19 infants with pulmonary atresia with intact ventricular septum undergoing single-ventricle palliation between July, 2000 and July, 2008. Echocardiograms, cardiac catheterisation findings, anaesthesia, and critical care management, as well as autopsy reports were reviewed. We modelled survival after surgery and looked for predictors of early mortality. A total of 19 infants underwent single-ventricle palliation and seven of these died. The risk of death was increased by a lower arterial pH at induction of anaesthesia (p = 0.01), a lower systolic blood pressure (p = 0.01), and technical problems during surgery (p = 0.03). On admission to the critical care unit, a lower mixed venous saturation (p = 0.02) and presence of tachyarrhythmia (p = 0.02) were associated with the need for mechanical support within the first 48 hours.

Conclusions

There is a high early mortality for those who undergo single-ventricle palliation. It is higher for those who are haemodynamically compromised before surgery; technical problems, and haemodynamic instability during surgery also increase mortality.

Copyright

Corresponding author

Correspondence to: Tilman Humpl, MD, Division of Cardiac Critical Care Medicine, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada. Tel: +1 416 813 6477; Fax: +1 416 813 7299; E-mail: tilman.humpl@sickkids.ca

References

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1. Zuberbuhler, JR, Anderson, RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281288.
2. Freedom, RM. The morphologic variations of pulmonary atresia with intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983; 4: 183188.
3. Alwi, M, Geetha, K, Bilkis, AA, et al. Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt. J Am Coll Cardiol 2000; 35: 468476.
4. Hausdorf, G, Schulze-Neick, I, Lange, PE. Radiofrequency-assisted “reconstruction” of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect. Br Heart J 1993; 69: 343346.
5. Humpl, T, Söderberg, B, McCrindle, BW, et al. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum: impact on patient care. Circulation 2003; 108: 826832.
6. Kreutzer, C, Mayorquim, RC, Kreutzer, GO, et al. Experience with one and a half ventricle repair. J Thorac Cardiovasc Surg 1999; 117: 662668.
7. Daubeney, PE, Delany, DJ, Anderson, RH, et al. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol 2002; 39: 16701679.
8. Daubeney, PE, Wang, D, Delany, DJ, et al. Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg 2005; 130: 1071.
9. Freedom, RM, Anderson, RH, Perrin, D. The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum. Cardiol Young 2005; 15: 447468.
10. Hwang, MS, Taylor, GP, Freedom, RM. Decreased left ventricular coronary artery density in pulmonary atresia and intact ventricular septum. Cardiology 2008; 109: 1014.
11. Sanghavi, DM, Flanagan, M, Powell, AJ, Curran, T, Picard, S, Rhodes, J. Determinants of exercise function following univentricular versus biventricular repair for pulmonary atresia/intact ventricular septum. Am J Cardiol 2006; 97: 16381643.
12. Hanley, FL, Sade, RM, Blackstone, EH, Kirklin, JW, Freedom, RM, Nanda, NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993; 105: 406423; 424–427; discussion 423–424.
13. Jahangiri, M, Zurakowski, D, Bichell, D, Mayer, JE, del Nido, PJ, Jonas, RA. Improved results with selective management in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1999; 118: 10461055.
14. Schneider, M, Zartner, P, Sidiropoulos, A, Konertz, W, Hausdorf, G. Stent implantation of the arterial duct in newborns with duct-dependent circulation. Eur Heart J 1998; 19: 14011409.
15. Coles, JG, Freedom, RM, Lightfoot, NE, et al. Long-term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1989; 47: 213217.
16. Guleserian, KJ, Armsby, LB, Thiagarajan, RR, del Nido, PJ, Mayer, JE Jr. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg 2006; 81: 22502257; discussion 2258.
17. Tanoue, Y, Kado, H, Maeda, T, Shiokawa, Y, Fusazaki, N, Ishikawa, S. Left ventricular performance of pulmonary atresia with intact ventricular septum after right heart bypass surgery. J Thorac Cardiovasc Surg 2004; 128: 710717.
18. Ekman-Joelsson, BM, Berggren, H, Boll, AB, Sixt, R, Sunnegardh, J. Abnormalities in myocardial perfusion after surgical correction of pulmonary atresia with intact ventricular septum. Cardiol Young 2008; 18: 8995.
19. Allan, CK, Thiagarajan, RR, del Nido, PJ, Roth, SJ, Almodovar, MC, Laussen, PC. Indication for initiation of mechanical circulatory support impacts survival of infants with shunted single-ventricle circulation supported with extracorporeal membrane oxygenation. J Thorac Cardiovasc Surg 2007; 133: 660667.

Keywords

Critical care outcomes in pulmonary atresia and intact ventricular septum undergoing single-ventricle palliation

  • Mark A. Walsh (a1), Kentaro Asoh (a1), Glen S. Van Arsdell (a2) and Tilman Humpl (a1) (a3)

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