Abstract
It is well known that Down’s syndrome is a strong risk factor for mortality after Fontan operations. We performed two lung biopsies in a Down’s syndrome patient who underwent staged Fontan operations. The pathological findings revealed severe pulmonary arterial hypertrophy and a quantitative real-time polymerase chain reaction revealed the overexpression of endothelin and a decline in the eNOS level at the Fontan operation. Although the preoperative hemodynamic studies revealed that all of the criteria for Fontan had been fulfilled, the patient died of acute cardiac insufficiency, 35 days after the Fontan operation.
Similar content being viewed by others
References
Gupta-Malhotra M, Larson VE, Rosengart RM, Guo H, Moller JH. Mortality after total cavopulmonary connection in children with the Down syndrome. Am J Cardiol. 2010;105:865–8.
Furukawa T, Park IS, Yoshikawa T, Nishimura T, Takahashi Y, Ando M, et al. Outcome of univentricular repair in patients with Down syndrome. J Thorac cardiovasc Surg. 2013;146:1349–52.
Wada N, Takahashi Y, Ando M, Park IS, Sasaki T. Single ventricle repair in children with Down’s syndrome. Gen Thorac Caidiovasc Surg. 2008;56:104–8.
Yamaki S, Tezuka F. Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries. Circulation. 1976;54:805–9.
Maeda K, Yamaki S, Kado H, Asou T, Murakami A, Takamoto S. Reevaluation of histomorphometric analysis of lung tissue in decision making for better patient selection for Fontan-type operations. Ann Thorac Surg. 2004;78:1371–81.
Ishida H, Kogaki S, Ichimori H, Narita J, Nawa N, et al. Overexpression of endothelin-1 and endothelin receptors in the pulmonary arteries of failed Fontan patients. Int J Cardiol. 2012;159:34–9.
D’Alto M, Romeo E, Argiento P, D’Andrea A, Sarubbi B, Correra A, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol. 2013;164:323–6.
Saji T. Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int. 2014;56:297–303.
Henaine R, Vergnat M, Bacha EA, Baudet B, Lambert V, Belli E, et al. Effects of lack of pulsatility on pulmonary endothelial function in the Fontan circulation. J Thorac Cardiovasc Surg. 2013;146:522–9.
Henaine R, Vergnat M, Mercier O, Serraf A, Montpreville VD, Ninet J, et al. Hemodynamics and arteriovenous malformations in cavopulmonary anastomosis: the case for residual antegrade pulsatile flow. J Thorac Cardiovasc Surg. 2013;146:1359–65.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Aoki, M., Hirono, K., Higuma, T. et al. Pulmonary vascular disease in a failed Fontan patient with Down’s syndrome. Gen Thorac Cardiovasc Surg 66, 299–302 (2018). https://doi.org/10.1007/s11748-017-0809-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11748-017-0809-6