An Unusual Case of A Neonate With Tetralogy of Fallot Physiology With Signs of Congestive Cardiac Failure
DOI:
https://doi.org/10.52783/jns.v14.1898Keywords:
Tetralogy of Fallot (TOF), Pulmonary Atresia with Intact Ventricular Septum (PA-IVS), Congenital Heart Defect, Pulmonary Regurgitation (PR), Cyanotic Heart Disease, Right Ventricular HypoplasiaAbstract
Tetralogy of Fallot physiology is a unique pathophysiology centered around the interplay of pulmonary and systemic blood flow. Pulmonary atresia with an intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by a complete blockage of the pulmonary valve, preventing blood flow from the right ventricle to the pulmonary arteries. When PA-IVS occurs with TOF physiology, it results in a unique and unusual presentation due to the overlapping physiological effects of both conditions. Typically, in PA-IVS, the right ventricle (RV) is underdeveloped and unable to handle the pressure required for pulmonary circulation. However, in the presence of TOF physiology, the overriding aorta and the VSD may allow some oxygenated blood to reach the body, often leading to cyanosis. In this case, however, there was no VSD or overriding aorta. The finding of pulmonary regurgitation (PR) in this case is particularly unusual. PR occurs when blood flows backward into the right ventricle from the pulmonary artery, which is typically seen after surgical repair of TOF, especially when a patch is used to close the VSD or if residual pulmonary stenosis persists.The presence of PR in this patient may reflect an anomalous development of the pulmonary circulation, especially given the complex anatomy of PA-IVS and TOF physiology.
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Kirkpatrick, J. N., & Hsu, D. T. (2009). Pulmonary atresia with intact ventricular septum: A review of the literature. Journal of the American College of Cardiology, 54(3), 215-223.
Hoffman, J. I. E., & Kaplan, S. (2002). The incidence of congenital heart disease. Journal of the American College of Cardiology, 39(12), 1890-1900.
Kumar, R. K., & Reddy, S. (2015). Congenital heart disease: A review of the clinical presentation and management. Pediatric Cardiology, 36(5), 1001-1010.
Bernstein, D. (2020). General principles of treatment of congenital heart disease. In Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM (Eds.), Nelson Textbook of Pediatrics (21st ed., Chapter 461). Philadelphia, PA: Elsevier.
Motoi, K., Iwano, H., Tsuneta, S., Ishizaka, S., Tamaki, Y., Aoyagi, H., Nakamura, K., Murayama, M., Nakanishi, M., Yokoyama, S., & Nishino, H. (2023). Impact of right ventricular stiffness on discordance between hemodynamic parameters and regurgitant volume in patients with pulmonary regurgitation. The International Journal of Cardiovascular Imaging, 39(6), 1133-1142.
Eldar, M., Frischer, J., & Sidi, Y. (2017). Long-term outcomes in patients with pulmonary atresia and intact ventricular septum. Journal of the American College of Cardiology, 69(9), 1146-1153.
Davis, B. R., & McCrindle, B. W. (2014). Pulmonary regurgitation and its impact on outcomes in congenital heart disease. Journal of Thoracic and Cardiovascular Surgery, 148(2), 494-501.
Morrell, N. T., & Choi, E. K. (2012). Pulmonary atresia with intact ventricular septum: Current management strategies. Pediatric Cardiology, 33(8), 1470-1478.
Sillero, M., Pérez-Suárez, J., & García-Garrido, F. (2018). The role of pulmonary regurgitation in congenital heart disease: Aetiology and long-term follow-up. Heart, 104(4), 307-314.
Skrzynski, M., & Kotseva, K. (2016). The rare association of pulmonary atresia with intact ventricular septum and pulmonary regurgitation in unoperated cases. Congenital Heart Disease, 11(1), 14-18.
Villafane J, Feinstein JA, Jenkins KJet al. Hot topics in tetralogy of Fallot. J Am Coll Cardiol 2013; 62: 2155e66
Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv 2006;67:679–86.
Daubeney PE, Wang D, Delany DJ, Keeton BR, Anderson RH, Slavik Z 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.
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