American Journal of Emergency Medicine 33 (2015) 1840.e1–1840.e2

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Case Report

Cardiac arrest due to airway obstruction in hereditary angioedema☆,☆☆,★,★★ Abstract Hereditary angioedema (HAE) is a rare genetic disease caused by a deficiency of functional C1 esterase inhibitor that causes swelling attacks in various body tissues. We hereby report a case of out-ofhospital cardiac arrest due to airway obstruction in HAE. Cutaneous swelling and abdominal pain attacks caused by gastrointestinal wall swelling are common symptoms in HAE, whereas laryngeal swelling is rare. Emergency physicians may have few chances to experience cases of life-threatening laryngeal edema resulting in a delay from symptom onset to the diagnosis of HAE. Hereditary angioedema is diagnosed by performing complement blood tests. Because safe and effective treatment options are available for the life-threatening swellings in HAE, the diagnosis potentially reduces the risk of asphyxiation in patients and their blood relatives. Hereditary angioedema (HAE) is a rare genetic disease [1,2]. Patients with HAE have a functional C1 esterase inhibitor (C1-INH) deficiency that causes swelling attacks in various body tissues. Here, we report the case of a patient with HAE who had an out-of-hospital cardiac arrest (OHCA) due to asphyxiation caused by laryngeal edema. An 84-year-old man was transferred to the emergency department for OHCA. His medical history included hypertension and chronic kidney disease. He experienced the earliest symptoms of facial swelling 20 hours before the OHCA. He had sudden difficulty breathing during a walk outside and was rushed into a nearby obstetric clinic, where an ambulance was called. When emergency medical services arrived, the patient had a depressed level of consciousness (Glasgow Coma Scale, E4V1M1), blood pressure of 210/120 mm Hg, and heart rate of 124 beats per minute. The initial emergency medical services electrocardiography rhythm was sinus tachycardia; however, it subsequently changed to bradycardia and pulseless electrical activity. Cardiopulmonary resuscitation (CPR) was initiated during transfer. Upon admission to the emergency department, the patient had a pulseless electrical activity cardiac arrest. He had significant swellings of the lips, tongue, pharynx, and larynx. Rapid endotracheal intubation was successful. One cycle of CPR with an intravenous epinephrine successfully achieved return of spontaneous circulation. The interval from the initiation of CPR to return of spontaneous circulation was 14 minutes. Initial arterial blood gas analysis indicated a pH of 6.86, PaCO2 of 77.2 mm ☆ Informed consent: Written informed consent for the publication of their individual details was obtained from the patient whose case is reported here. ☆☆ Funding: There was no funding. ★ Conflict of interest: None. ★★ Presentation at meeting: None.

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Hg, PaO2 of 106 mm Hg, base excess of −20.6 mmol/L, and a lactate level of 6.5 mmol/L. He had no skin symptom of anaphylaxis. There were no remarkable findings to indicate the primary cause of cardiac arrest in clinical laboratory testing, head and thoracic computed tomography, and coronary angiography. Accordingly, the etiology of cardiac arrest was most likely asphyxiation due to severe edema in the upper airway. Subsequently, the patient was admitted to the intensive care unit. Complement blood tests revealed a decreased C1-inhibitor activity (25% [normal, 70%-130%]), decreased C4 level (3 mg/dL [normal, 1745 mg/dL]), and normal C3 level (93 mg/dL [normal, 86-160 mg/dL]). In addition, a detailed medical history including multiple episodes of facial swelling and an episode of OHCA of unknown cause with facial edema at the age of 76 years was obtained from the family. Thus, we diagnosed HAE. On day 3, the patient had persistent facial edema that required continuous endotracheal intubation to secure the airway; a 1500-U dose of intravenous human C1-INH (Berinert; CSL Behring) was administered, which resulted in rapid improvement of the edema in the lips and tongue; the patient was successfully extubated on day 4. On day 33, he was discharged from the hospital with complete neurologic recovery. Subsequent complement blood tests including C1-INH activity identified that 2 of his family members had HAE. Hereditary angioedema is an autosomal dominant disease, and its prevalence is estimated to be 1 in 50000 to 100000 persons [1,2]. Functional C1-INH deficiency potentially causes chronic overactivation and consumption of the complement component C4; thus, decreased C1-INH activity and decreased C4 levels are keys for diagnosis. C1 esterase inhibitor also regulates plasma kallikrein. The deficiency of functional C1-INH potentially causes insufficient kallikrein inhibition, which increases the production of bradykinin, leading to swelling attacks in HAE. The clinical features of HAE are cutaneous swelling, abdominal pain due to edema of the gastrointestinal wall, and laryngeal edema. The frequency of laryngeal edema episodes is low compared with cutaneous swellings or abdominal pain attacks [3]. Furthermore, laryngeal edema is rarer in elderly persons than in young adults [3,4]. Thus, laryngeal edema in the elderly patient reported here is likely uncommon. Most patients with HAE have an initial episode of its clinical symptoms before 40 years [3]. This patient had the initial symptom of cutaneous edema in the hands in his forties. He had recurrent cutaneous swelling in the hands (8 times between the forties and 84 years) but no apparent abdominal pain attacks. He had an episode of OHCA of unknown cause at the age of 76 years with significant facial edema, suggesting he likely had an episode of laryngeal edema 8 years before the current attack. Despite the multiple episodes, he was undiagnosed until this episode. Accordingly, the diagnosis of HAE is often delayed from symptom onset [5]. The average duration of delay reported, more than 10 years [5], highlights the importance of increasing awareness of this rare

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disease among physicians, including emergency physicians, who encounter cutaneous swelling, abdominal attacks, and laryngeal swelling. Screening complement tests for HAE are simple and reliable [1,2,6]. Safe and effective treatment options for HAE-related swelling, including plasma-derived C1-INH [7], kallikrein inhibitor [8], bradykinin B2 receptor antagonist [9], and recombinant C1-INH, were recently approved [10]. Thus, increasing the awareness of HAE potentially shortens the diagnostic delay and increases the chances of effective treatment for acute swelling, thereby improving fatality. Takashi Fuse, MD Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan Taka-aki Nakada, MD, PhD Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan Chiba University Graduate School of Medicine 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan Corresponding author. Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan Tel.: +81 72 469 3111; fax: +81 72 469 7929 E-mail address: [email protected] Masashi Taniguchi, MD Yasuaki Mizushima, MD, PhD Tetsuya Matsuoka, MD, PhD Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan http://dx.doi.org/10.1016/j.ajem.2015.04.003

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Cardiac arrest due to airway obstruction in hereditary angioedema.

Hereditary angioedema (HAE) is a rare genetic disease caused by a deficiency of functional C1 esterase inhibitor that causes swelling attacks in vario...
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