Case Report

Tet spell in an adult Abstract

Cyanosis is a bluish skin due to deoxygenated hemoglobin or methomoglobin in the arterial blood. central cyanosis mostly results from cardiovascular or pulmonary shunts. Tet spell is an episodic central cyanosis due to total occlusion of right ventricle outflow in a patient with a congenital heart disease, such as Tetralogy of Fallot (TOF). There are limited cases of patients who lived with untreated TOF until adulthood. Tet spell in an adult is rarely seen in an emergency department. Early recognition of this condition, along with proper disposition and management, can prevent major morbidity or mortality. We report a 29-year-old man who presented with cyanotic spell and in whom a diagnosis of TOF was later confirmed. This case reminds us of TOF as a differential diagnosis of hypercyanotic crises and its unique manifestations and management.

Cyanosis is a bluish skin and mucosa because of more than 5 g of reduced hemoglobin or methomoglobin in the arterial blood [1]. It can be classified as central or peripheral cyanosis. Central cyanosis involves the lips, tongue, and mucous membranes and mostly results from cardiovascular or pulmonary shunts [2].

Tet or hypoxic spell is an episodic central cyanosis due to total occlusion of right ventricle (RV) outflow. It typically occurs in a crying infant with a congenital heart disease such as Tetralogy of Fallot (TOF). However, an unrepaired TOF with mild to moderate RV outflow obstruction may be clinically silent until adulthood.

Tetralogy of Fallot with Tet spell in an adult is rarely seen in an emergency department (ED) [3,4]. We report a 29-year- old man who presented with Tet spell and in whom the diagnosis of TOF was later confirmed.

A 29-year-old man was found squatting at the front door of our pharmacy with shortness of breath and bluish skin and lips. He was immediately brought by guards to our ED. At our ED, the patient was unable to give any of his past history because of severe dyspnea and nearly fainting with diaphoresis. Besides, he was irritable and unwilling to lie down. On physical examinations, he was afebrile but had tachypnea with undetectable oxygen saturation by Pulse oximetry monitoring. Bluish discoloration of distal limbs and

lips with clubbing fingers was disclosed (Figs. 1 and 2). The chest breathing sound was clear without audible heart murmur by auscultation. The complete electric cardiography revealed sinus rhythm with a heart rate of 90 beats/min, Right axis deviation, right Atrial enlargement, and RV hypertrophy (Fig. 3). Hypoxic spell with congenital heart disease and right-to-left shunt of this patient were suspected.

We kept the patient in knee-chest position, and nonreb- reathing mask with 100% oxygen was also given. The patient recovered with improving symptoms gradually. Endotracheal intubation of medications such as morphine or propranolol was not infused because of clinical improvement. Laboratory test results disclosed a red cell count of 8.41 million/cumm and a hematocrit of 60.7%. The cardiac enzymes were within normal limits. The chest plain film showed decreased lung markings with prominence of RV (Fig. 4). The echocardio- graphy and medical records retrieved using the patient’s identification number further confirmed the diagnosis of TOF with pulmonary atresia and a large ventricular septal defect. The patient revealed having symptoms of upper respiratory tract infection recently, which may act as a precipitating factor of this episode. He was then admitted and waited for heart- lung transplantation after cardiovascular evaluation.

Tetralogy of Fallot was first described by Etienne-Louis Arthur Fallot in 1888 [5]. The prevalence of TOF is about 3.9 per 10000 live births [6]. It is composed of ventricular septal

Fig. 1 Cyanotic lips.

0735-6757/$ – see front matter (C) 2009

130.e4 Case Report

Fig. 2 Distal cyanosis and clubbing fingers.

defect, overriding aorta, right ventricular outflow obstruc- tion, and RV hypertrophy due to deviated infundibular septum during development. The clinical presentations of systemic hypoxemia correlate with the degree of RVoutflow obstruction, which also indicates the timing of Surgical repair. An unrepaired TOF with mild to moderate RVoutflow obstruction may be clinically silent until adulthood.

In our case, medical history was unavailable because of an emergent situation of hypoxemia and compromised ventilation- perfusion state. However, the diagnosis could be approached by

Fig. 4 The chest plain film showed decreased lung markings with prominence of RV.

careful physical examinations, which disclosed central cyanosis with clubbing fingers. Besides, a minimal improvement with supplemental oxygen also suggests a Cardiac origin. A nonaudible heart murmur of this patient may be due to decreased blood flow through the right ventricular obstruction. Hypoxic spell of chronic hypoxemic state due to congenital

Fig. 3 The complete electric cardiography revealed sinus rhythm with a heart rate of 90 beats/min, right axis deviation, right atrial enlargement, and Right ventricular hypertrophy.

Case Report 130.e5

heart disease was suspected. Keeping the patient in the knee- chest position instead of the supine position increases the systemic resistance and aids the pulmonary flow. In addition to supplemental oxygen, morphine and ?-blocker agents, which release the obstruction of RVoutflow, were considered [4,7]. ?– Agonist inhalation therapy and unopposed ?-agonist agents may increase the heart rate and precipitate the right-to-left shunt and should be avoided. Delayed or missed diagnosis of this condition may lead to wrong decision making and inappropriate management, which will result in major morbidity or even mortality. Our patient responded promptly to proper positioning and supplemental oxygen. The patient was not amenable to surgical repair after cardiovascular evaluation. Therefore, heart- lung transplantation was indicated.

There are limited cases of patients who lived with untreated TOF until adulthood. Tet spell in an adult is rarely seen in an ED. This case reminds us of TOF as a differential diagnosis of hypercyanotic crises and its unique manifesta- tions and management.

Yi-Ming Weng MD Yu-Che Chang MD Te-Fa Chiu MD

Department of Emergency Medicine Chang Gung Memorial Hospital and

Chang Gung University College of Medicine

Tao-Yuan, Taiwan E-mail addresses: changyuche@adm.cgmh.org.tw

changyuche@yahoo.com.tw

Chia-Sui Weng MD

Department of Obstetrics and Gynecology

Mackay Memorial Hospital

Taipei, Taiwan

doi:10.1016/j.ajem.2008.04.023

References

1. Woolridge DP, Love JC. Congenital heart disease in the pediatric emergency department. Part I: pathophysiology and clinical char- acteristics. Pediatr Emerg Med Rep 2002;7:69.
2. Sharieff GQ, McCollough M. The nightmare neonate: life- threatening events in the first month of life. Emerg Med Pract 2003;5:1.
3. Ghavidel AA, Javadpour H, Tabatabaei MB, Adambeig A, Raeisi K, Noohi F. Complete surgical repair of Tetralogy of Fallot in adults, is it ever too late? J Card Surg 2008;23(1):23-6.
4. Kaemmerer H, Bauer U, Pensl U, Oechslin E, Gravenhorst V, Franke A, et al. Management of emergencies in adults with congenital cardiac disease. Am J Cardiol 2008;101(4):521-5.
5. Fallot E. Contribution a l’anatomie pathologique de la maladie bleu (cyanose cardiaque). Mars Med 1888;25:418.
6. Centers for Disease Control and Prevention. Improved national preva- lence estimates for 18 selected major birth defects–United States, 1999-2001. MMWR Morb Mortal Wkly Rep 2006;54:1301.
7. Gewitz MH, Vetter V. Cardiac emergencies. In: Fleisher GR, Luwig S, editors. Textbook of Pediatric Emergency Medicine. 4th ed. Baltimore: Lippincott Williams & Wilkins; 2000. p. 659-700.