Abstract

Background: Tetralogy of Fallot (TOF) is recognized as the predominant form of cyanotic congenital heart disease beyond infancy. Grasping the dynamic factors that either exacerbate or ameliorate the shunt represents the cornerstone for managing critically ill patients with TOF in an emergency department (ED). Case description: We herein document a 29-year-old uncorrected TOF patient who manifested symptoms of acute gastroenteritis & respiratory failure. A provisional diagnosis was then formulated сonnectіng aсute gastroenteritis to a provoked hyрoxіс spell with uncorrected TOF. Suррortіve сare was provided that led to positive outcome. Clinical relevance: This case study elucidates the clinical dilemmas in managing a critically ill, uncorrected TOF individual dealing with the complex interplay of physiology and biochemical changes. Attention to comprehensive management of a Tet spell is thereby underscored. Information suggests this situation represents a rare case of a few surviving uncorrected TOF patients documented in the Indian population.

• 1.   Sharieff G.Q., McCollough M. The nightmare neonate: life-threatening events in the first month of life. Emerg Med Pract, 5 (2003), p. 1.
• 2.   Kirklin J.W., Barratt-Boyes B.G. Ventricular septal defect and pulmonary stenosis or atresia. In: ed. J.W., Kirklin B.G., Barratt-Boyes ed. Cardiac Surgery. New York: John Wiley and Sons; 1993. pp. 749–82.
• 3.   Bailliard F., Anderson R.H. Tetralogy of Fallot. Orphanet J Rare Dis. 2009; 4:2. doi:10.1186/1750-1172-4-2.
• 4.   Nottestad S.Y., Slife D.M., Rubal B.J., Moody J.M. Tetralogy of Fallot in a 71-year-old patient with new-onset hypoxemia. Cathet Cardiovasc Diagn 1993; 28(4): 335–38 DOI: 10.1002/ ccd.1810280413.
• 5.   Dickinson CJ, Martin JF. Megakaryocytes and platelet clumps as the cause of finger clubbing. Lancet. 1987; 2(8559): 1434–1435.
• 6.   Rao PS. Pathophysiology of cyanotic congenital heart disease in children and adults. Indian Heart J. 2017; 69(4): 532–543.
• 7.   Freed M.D., Rosenthal A., Bernhard W.F., Nadas A.S. Exercise-induced pulmonary hypertension in patients with tetralogy of Fallot. Circulation. 1973; 48(2): 306–314.
• 8.   Andropoulos D.B., Stayer S.A., Skjonsby B., Fraser C.D. Anesthesia for congenital heart disease: management of tet spells. Anesth Analg. 2004; 98(6): 1512–1521.
• 9.   Montero J.V., Nieto E.M., Vallejo I.R., Montero S.V. (2015) Intranasal midazolam for the Emergency management of hypercyanotic spells in tetralogy of Fallot. Pediatr Emerg Care 31(4): 269-271.
• 10.   Tse D.S., Vitberg Y.M., Berezow J., et al: Treatment of tetralogy of Fallot hypoxic spell with intranasal fentanyl. Pediatrics 134(1): e266-e269, 2014.
• 11.   Tetralogy of Fallot: Perioperative Anesthetic Management of Children and Adults. Mark D. Twite, M.B., BChir, FRCP, and Richard J. Ing (SA)Volume 16, Issue 2. https://doi. org/10.1177/1089253211434749.
• 12.   Goyal R., Singh S., Bangi A., et al: case series: dexmedetomidine and ketamine for anesthesia in patients with uncorrected congenital cyanotic heart disease presenting for non cardiac surgery. J Anaesthesiol Clin Pharmacol 29 (4): 543-546, 2013.
• 13.   Elkomy M.H., Drover D.R., Hammer G.B., A Population pharmacokinetics of ketamine in children with heart disease. Int J Pharm 478(1): 223-231, 2014.
• 14.   Dimopoulos K., Diller G.P., Koltsida E., et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008; 117(18): 23202328.
• 15.   Herson V.C., Raye J.R., Rowe J.C., Philipps A.F. Acute renal failure associated with polycythemia in a neonate. J Pediatr 1982; 100: 137-9.
• 16.   Perloff J.K. Erythrocytosis and cyanotic congenital heart disease: The hematologic adaptations and sequelae. Am J Med Sci. 1994; 307(4):316–326.
• 17.   Hida K., Wada J., Yamasaki H., Nagake Y., Zhang H., Sugiyama H., et al. Cyanotic Congenital heart disease associated with glomerulomegaly and focal segmental glomerulosclerosis: Remission of nephrotic syndrome with angiotensin-converting enzyme inhibitor. Nephrol Dial Transplant 2002; 172
• 18.   Weng Y.M., Chang Y.C., Chiu T.F., Weng C.S. Tet spell in an adult. Am J Emerg Med 2009; 27: 130.e3-5

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