A 44-year-old man presented to the ED with complaints of numbness and weakness of the right arm. These symptoms persisted for 10 minutes and had resolved upon evaluation 30 minutes later.
The patient had a history of nonischemic cardiomyopathy with an ejection fraction of 10%, obstructive sleep apnea, previous cerebrovascular accident, and persistent atrial fibrillation (AF). Previous attempts at cardioversion and ablation were unsuccessful in maintaining sinus rhythm. Twenty-four days before this presentation, he underwent percutaneous pulmonary vein isolation radiofrequency catheter ablation (RFCA) for treatment of symptomatic persistent AF. Medications included once-daily doses of aspirin 81 mg, atorvastatin 40 mg, eplerenone 25 mg, furosemide 20 mg, metoprolol succinate 200 mg, rivaroxaban 20 mg, and pantoprazole 40 mg. He also took twice-daily sacubitril-valsartan 97 mg/103 mg.
On arrival to the ED, the patient's BP was 141/114 mm Hg; heart rate, 88 beats/minute; respirations, 18; oral temperature, 36.7° C (98.1° F); and SpO2, 100% on room air. He was alert and oriented without neurologic deficits or nuchal rigidity. No wheezing or crackles were auscultated. Cardiac examination revealed no murmurs, gallops, or rubs. Abdominal examination revealed a soft, nontender, and nondistended abdomen.
Based on the differential diagnosis of a transient ischemic attack, the patient had an ECG, which showed normal sinus rhythm with first-degree atrioventricular (AV) block. Laboratory workup showed hemoglobin, 12.1 g/dL (normal range, 13.4 to 17 g/dL); white blood cell count, 8,000 cells/mm3 (normal range, 3,600 to 10,600 cells/mm3); platelet count, 336,000 cells/mm3 (normal range, 150,000 to 450,000 cells/mm3); sodium, 136 mmol/L (normal range, 135 to 145 mmol/L); potassium, 3.7 mmol/L (normal range, 3.5 to 5.5 mmol/L); creatinine, 1.16 mg/dL (normal range, 0.8 to 1.4 mg/dL); glucose, 113 mg/dL (normal range, 70 to 90 mg/dL); calcium, 9.1 mg/dL (normal range, 8.5 to 10.5 mg/dL); and troponin I, less than 0.03 ng/mL (normal range, 0.03 ng/mL or less). Head CT without contrast showed encephalomalacia in the right cerebral hemisphere, corresponding to the previous infarct. He was admitted to the hospitalist service for further evaluation of a transient ischemic attack.
Five hours after arrival to the ED, the patient described new symptoms of blurry vision and headache and was observed to be more lethargic. On examination by the hospitalist team, his vital signs were notable for a BP of 152/106 mm Hg, heart rate of 110 beats/minute, respirations of 23, and oral temperature of 39° C (102.2° F). Right-sided hemiparesis and left upper extremity paralysis were noted. To evaluate new neurologic deficits, a repeat head CT without contrast was obtained, which revealed a small locule of gas in the cortical vein in the right frontoparietal region (Figure 1).
Over the next 2 hours, the patient became increasingly obtunded, with a Glasgow Coma Scale score of 7, denoting coma. He also developed increasing pyrexia with a temperature of 39.3° C (102.7° F). New lateral gaze preference and lateral nystagmus were noted on examination. Laboratory studies were repeated to assess for metabolic causes of rapid deterioration. Chemistries showed a new high anion gap metabolic acidosis with an elevated venous lactate of 9.1 mmol/L (normal range, 0.5 to 1.6 mmol/L).
Given the patient's rapidly deteriorating neurologic status and inability to protect his airway, he was intubated and transferred to the ICU. Lumbar puncture was not performed because of chronic anticoagulant use. Blood cultures were obtained, and meningeal doses of vancomycin, ceftriaxone, and acyclovir were administered. Neurotelemetry was negative for seizure activity. An orogastric tube was placed to suction with return of coffee-ground output. Abdominal and pelvic CT with IV contrast revealed multiple acute splenic infarcts and a right renal infarct. Chest CT with IV contrast (Figure 2) showed a small collection of gas located anterior to the distal esophagus with communication to the left atrium, concerning for an atrioesophageal fistula. Cardiothoracic surgery was consulted for surgical management of the newly diagnosed fistula, which was thought to be related to the recent RFCA. Surgical intervention was deferred pending clinical stability and improvement in the patient's neurologic status. Blood cultures grew multiple Gram-positive cocci, including Streptococcus mitis, Streptococcus salivarius, and Staphylococcus aureus.
The patient continued to decline following his transfer to the ICU, developing hypotension, hypoxia requiring increasing ventilatory support, and worsening mental status. Twenty-four hours after his arrival to the ED, the patient became abruptly bradycardic and developed pulseless electrical activity, and died despite attempts at resuscitation.
The case patient presented with complications related to the development of an atrioesophageal fistula following RFCA. These complications included ischemia related to air emboli resulting in cerebrovascular, splenic, and renal infarctions; upper gastrointestinal (GI) bleeding; and sepsis related to polymicrobial bacteremia.
The incidence of AF is rising as the population ages, and risk factors including hypertension, diabetes, and heart failure become more prevalent. Symptom management and rate and rhythm control of AF often are managed with medications.1 RFCA is a common treatment for drug-refractory symptomatic AF, with more than 50,000 procedures performed in the United States annually.2,3 Although RFCA may improve quality of life and better maintain sinus rhythm than antiarrhythmic drugs, late recurrences of AF are common.1 RFCA carries a periprocedural complication rate of 4.5% to 5%, with an atrioesophageal fistula being one of the most serious and devastating complications (Table 1).3,4 Although the incidence of developing an atrioesophageal fistula is estimated at 0.03% to 0.08% of all catheter ablation procedures, it likely is underestimated related to misdiagnosis and underreporting.4,5 The close proximity of the esophagus to the posterior wall of the left atrium places the esophageal tissue at risk for indirect thermal injury related to high catheter temperatures used during ablation.5,6 Studies have demonstrated distances as small as 5 mm between the left atrium and the esophagus.6 The pathogenesis is not fully understood, but left atrial dilation and small patient body size are considered risk factors.5-7 Procedural risks include the use of general anesthesia, catheter contact force, elevated catheter temperature and power settings, use of naso- or orogastric tubes, and prolonged ablation time over the posterior left atrial wall.4-6
TABLE 1. -
Summary of 45 case studies reporting atrioesophageal fistula following RFCA2,3,6,9,10,12,14-16,18,23-52
||Number of cases reporting variable
Details of presentation
|Days between RFCA and onset of symptoms
|Nausea and/or vomiting
|Shortness of breath
|GI bleeding (hematemesis, melena, coffee-ground emesis)
||Findings consistent with fistula
|CT of the head
|MRI of the brain
Organisms isolated from blood cultures (N = 26)
|Group F Streptococcus
Treatment and outcomes
∗One case report did not report an outcome. Also involves cases where patient died before initiation of planned intervention.
Esophageal injuries after RFCA are common, affecting up to 47% of patients and include erythema, tissue necrosis, and ulcerations.8 Over time, exacerbated by gastric hypomotility, pyloric spasms, esophagitis, and lower esophageal sphincter relaxation causing acid reflux, this esophageal injury may progress, damaging deeper tissues, and eventually forming an atrioesophageal fistula.6 Once a fistula has developed, the elevated esophageal pressures during peristalsis (which can be 10 times greater than intra-atrial pressures) can lead to translocation of bacteria leading to sepsis and air emboli that lead to end-organ ischemia.9
A systematic review by Han and colleagues of 120 cases of atrioesophageal development following RFCA reported that 73% were male, with a median age of 59 years.4 Symptom onset was reported between 0 and 60 days, median 21 days, following RFCA.4 The most common symptoms reported were fever, neurologic deficits, and GI symptoms including hematemesis. Fever was noted in 73% of the cases.4 Although fever is a nonspecific sign, it often has been incorrectly attributed to pericarditis or postpericardiotomy syndrome in the postablation period in patients presenting with an atrioesophageal fistula.10 Other symptoms included confusion, loss of consciousness, seizure, nausea and vomiting, dysphagia, odynophagia, melena, chest pain, and dyspnea. Han and colleagues reported conclusions of multivariable analysis showing independent predictors of mortality, including presentation with neurologic symptoms (P < .001) and GI bleeding (P = .047), stressing the importance of high suspicion of an atrioesophageal fistula in patients presenting with these symptoms within 60 days of RFCA.4
Leukocytosis is a common laboratory finding in patients with an atrioesophageal fistula, and may be related to the underlying bacterial infection and inflammation surrounding the fistula.11 Other nonspecific laboratory findings include elevated troponin and inflammatory markers.12 Bacteremia, when present, typically is caused by translocation of oropharyngeal organisms from the GI tract through the fistula into the left atrium and arterial circulation.10 Of the 27 cases in the Han and colleagues review that reported blood culture results, 23 grew more than one Streptococcus species and 26 grew at least one Gram-positive coccus.4 Organisms isolated included S. mitis, S. oralis, S. sanguinis, S. salivarius, S anginosus, and Mycoplasma salivarium, all which can be found in the oral cavity.4 Other organisms from the GI tract including Enterococcus and Candida albicans were reported.4 Positive blood cultures may mislead clinicians to the diagnosis of bacterial endocarditis, despite the low incidence (0.2%) of postablation endocarditis.10
Multiple modalities to identify an atrioesophageal fistula have been described; however, chest, thoracic, or cardiac CT with IV contrast or chest CT angiography are the most reliable tools for detecting pneumomediastinum or intra-atrial air leading to the diagnosis.10,11,13-16 Han and colleagues reported CT chest as the most common mode of diagnosis, compared with MRI of the chest, endoscopy, transthoracic echocardiogram (TTE), transesophageal echocardiogram (TEE), and CT of the brain.4 Of the 97 cases reporting CT chest results, 95 (98%) showed abnormalities consistent with an atrioesophageal fistula. Most CTs of the chest were performed with IV contrast.4 If suspicion for an atrioesophageal fistula remains high despite negative initial imaging, do not delay further treatment. Han and colleagues reported seven patients with a negative initial CT of the chest; repeat CT imaging 4 to 12 days later ultimately revealed the diagnosis of an atrioesophageal fistula.4
Given the high incidence of neurologic symptoms on presentation, many patients undergo brain imaging, including head CT and brain MRI. Of the 46 patients who underwent CT brain imaging in the review by Han and colleagues, 36 (78%) had abnormalities raising concern for atrioesophageal fistula, including infarct, pneumocephalus or intravascular air, hemorrhage, and edema.4 Twenty-one patients underwent MRI of the brain, with 19 (90%) showing abnormalities including infarction and pneumocephalus or intravascular air.4 In a patient who recently had RFCA, the presence of tiny locules of air in the brain is effectively pathognomonic of an atrioesophageal fistula.14
Esophagogastroduodenoscopy (EGD) is routinely performed in patients who present with hematemesis; however, this procedure can cause catastrophic and fatal outcomes in patients with an atrioesophageal fistula. If atrioesophageal fistula is suspected, avoid insufflation of the esophagus for either EGD or TEE because it can cause air emboli leading to neurologic or cardiovascular injury or death.13,15
Treatment and outcome
The overall mortality for patients with an atrioesophageal fistula is estimated at 55% to 100%, with a systematic review of 65 cases reporting 55.4% mortality.17 This high mortality has been attributed to failure of early recognition, delayed presentation, and complex surgical repair required for treatment.6 Early clinical suspicion, prompt diagnosis, initiation of antibiotic treatment, and immediate surgical intervention is essential for the survival of a patient with an atrioesophageal fistula.10 Patients who underwent definitive surgical repair were 12 times more likely to survive than those treated with esophageal stenting, antibiotic therapy alone, or conservative measures.17 Patients who survive surgical repair may suffer severe neurologic and cerebrovascular morbidity from air and septic emboli and embolic stroke. Hyperbaric oxygen therapy has been shown to improve neurologic deficits in patients with cerebral air emboli; however, it should not be used as definitive treatment in the place of surgical repair of the atrioesophageal fistula.13,16,18
Although no preventive measures for developing an atrioesophageal fistula have been extensively studied, many recommendations are outlined in the literature.3 Suggested measures during the RFCA procedure include limiting power to the posterior wall of the left atrium, employing esophageal temperature monitoring and esophageal cooling, visualization of the esophagus during RFCA, and mechanical displacement of the esophagus.3,16,19 Di Biase and colleagues found that 48% of patients who underwent general anesthesia before RFCA developed an esophageal injury, compared with only 4% of patients who received moderate sedation and analgesia.20 This is thought to be related to reduced peristalsis of the esophagus, lack of swallowing, and lack of pain reception during general anesthesia. Furthermore, esophageal lesions have been seen in 15% and 17% of patients on postablation upper GI endoscopy and capsule endoscopy, respectively.3,5,20 Proton pump inhibitors (PPIs), especially in patients with gastroesophageal reflux, are recommended prophylactically before RFCA to reduce the occurrence of an esophageal injury that could lead to an atrioesophageal fistula.5,13,21 Kapur and colleagues recommend routine prescription of PPIs or H2 blockers directly before or immediately after ablation for 1 to 6 weeks postprocedure.22 Further trials are needed to investigate the efficacy of PPIs in preventing atrioesophageal fistula; however, because of the rarity of this complication, proving benefit may be difficult.21
With the increase in prevalence of AF as the population ages comes the increase in procedures to control symptoms and rhythm. An atrioesophageal fistula is a rare, often fatal, late complication of RFCA. Patients presenting within 60 days of RFCA for AF with fever, neurologic deficits, dysphagia, chest pain, and/or GI bleeding must be assessed for an atrioesophageal fistula. Clinicians must be aware of the risk of an atrioesophageal fistula, as the changeable manifestations can delay diagnosis. Chest CT with contrast or chest CT angiography are the most valuable diagnostic tools. However, a negative imaging study should not delay treatment when there is high clinical suspicion. EGD and TEE are contraindicated in patients with an atrioesophageal fistula because of the risk for air emboli. Early clinical suspicion and diagnosis, initiation of antibiotic treatment, and urgent surgical intervention may improve patient outcomes.
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