Anesth Analg 2004;99:350-352
© 2004 International Anesthesia Research Society
doi: 10.1213/01.ANE.0000132996.12248.B0
CARDIOVASCULAR ANESTHESIA
Postoperative Death in a Patient with Unrecognized Arrhythmogenic Right Ventricular Dysplasia Syndrome
Khay-Wee Toh, FRCA*,
Kasinathan Nadesan, FRCPath
,
Ming Yao Sie, FRCA*,
Raja Vijeyasingam, FRCS
, and
Patrick Seow Koon Tan, FRCA*
Departments of *Anesthesiology,
Pathology, and
Surgery, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia
Address correspondence and reprint requests to Khay-Wee Toh, FRCA, 27, Jalan Burhanuddin Helmi, Taman Tun Dr. Ismail, 60000 Kuala Lumpur, West Malaysia. Address e-mail to tohkhaywee{at}hotmail.com
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Abstract
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Arrhythmogenic right ventricular dysplasia is an inherited disease causing fatty replacement of heart tissue. This disease often presents as T-wave inversion in the anterior leads of the electrocardiogram (ECG) with life-threatening ventricular arrhythmias. In older patients, progressive right and left ventricular failure can develop. This is a case report of postoperative death occurring in a 59-yr-old woman with undiagnosed arrhythmogenic right ventricular dysplasia after hepatic cystectomy. The patient had T-wave inversion in the inferior ECG leads and no history of arrhythmias. During general anesthesia, cardiovascular collapse occurred in the absence of arrhythmias that was unresponsive to resuscitation.
IMPLICATIONS: Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited disease of the heart resulting in life-threatening arrhythmias and heart failure. The authors describe the case of a 59-yr-old female patient with undiagnosed ARVD. During general anesthesia, cardiovascular collapse unresponsive to resuscitation occurred in the absence of arrhythmias.
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Introduction
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Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited disease affecting 1 in 5,000–10,000 (1). The myocardium becomes replaced by fatty tissue and causes ventricular arrhythmias, sudden death, and heart failure (2–6). In reports of death after general anesthesia in ARVD patients (1,7), cardiovascular collapse has occurred without a preceding arrhythmia and was unresponsive to fluids and inotropes. Although 10% of asymptomatic T-wave inversions can conceal a myocardial condition (8), this may be the sole abnormality observed in patients with ARVD.
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Case Report
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A 59-yr-old woman presented for hepatic cyst excision. She had had a similar operation 10 yr previously. A preoperative electrocardiogram (ECG) showed T-wave inversion in leads III and aVF. Cardiologic evaluation determined that the patient was asymptomatic, without signs of cardiac failure or hepatic or coagulation abnormalities, and no investigations were recommended. Computed tomography revealed healthy liver surrounding a hepatic cyst.
Before anesthesia, the patient had a heart rate (HR) of 105 bpm and arterial blood pressure of 175/80 mm Hg. An epidural catheter was inserted at T10-11; 6 mL of 0.25% bupivacaine was administered, and the arterial blood pressure was subsequently recorded at 145/70 mm Hg. Over the next hour, 1 L of crystalloid was infused.
The patient was anesthetized with propofol 80 mg and succinylcholine 100 mg and was tracheally intubated; anesthesia was maintained with 2% isoflurane in oxygen and nitrous oxide with atracurium. Epidural administration of 6 mL of 0.25% bupivacaine resulted in an HR of 65 bpm, arterial blood pressure of 120/70 mm Hg, and central venous pressure (CVP) of 9 cm H2O. A hepatic cyst containing 5 L of fluid was drained after 2 h. Six liters of IV crystalloid and blood were infused. At 3 h and during dissection of the cyst from the liver, an acute decline in arterial blood pressure from 110/35 to 75/45 mm Hg occurred with an HR of 75 bpm and CVP of 8 cm H2O. Arterial blood gas analysis showed no hypoxemia or electrolyte abnormalities. Over the next 30 min, the patient received 4 L of fluids: five boluses of ephedrine (6 mg) and five boluses of phenylephrine (100 µg). After 4 h, her arterial blood pressure remained at 60/35 mm Hg despite epinephrine and norepinephrine infusions (0.1–0.7 µg · kg–1 · min–1). The blood pressure did not improve when 4 L of IV fluids increased CVP to 20 cm H2O, and pink frothy sputum was present. The operation was terminated because of cardiovascular instability. Fluid loss (5 L of cystic fluid and 3 L of blood) was estimated from suction and swabs. Total IV fluids administered were 15 L. No arrhythmia was observed.
A postoperative echocardiogram showed no tricuspid regurgitation, no ventricular dilation, adequate ventricular loading, and biventricular ejection fractions of 40% with 0.4–0.7 µg · kg–1 · min–1 epinephrine and norepinephrine. A postoperative ECG showed no changes. Over the next 16 h, the patient died from cardiogenic shock unresponsive to inotropes and intravascular fluid loading, with deteriorating renal and coagulation function.
On autopsy, the right ventricle showed fatty replacement of the entire myocardial wall. Myocardial cells were surrounded by fatty fibrous tissue (Fig. 1) compared with normal tissue (Fig. 2); these findings were pathognomonic of ARVD. The left ventricle and coronary vessels were normal. The hepatic cyst was benign, with no parasites.
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Figure 1. Specimen of right ventricular tissue from the patient showing fibrous fatty changes pathognomonic of arrhythmogenic right ventricular dysplasia.
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Discussion
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ARVD is difficult to diagnose when it is asymptomatic. Further investigations are required if there is a history of arrhythmias, syncope, or sudden death in the family.
ECG abnormalities observed in ARVD include T-wave inversion in the anterior leads (V1 to V3), left axis deviation, widened QRS, 
waves, and ventricular tachycardia. T-wave inversion, found in leads III and aVF, as found in our patient, occurs less frequently (9). Although isolated T-wave inversion can be normal, 10% can conceal ischemic heart disease, cardiomyopathies, Wolf-Parkinson-White syndrome, myocarditis, and pericarditis (8,10). Thinning of the right ventricle and wall motion abnormalities were not detected by echocardiography in our patient, possibly because of the large doses of inotropes. Other investigations include magnetic resonance imaging (MRI), ventricular angiography, and myocardial biopsy.
The differential diagnosis considered was myocardial ischemia, anaphylaxis, and pulmonary emboli. The absence of ECG and postmortem findings excluded myocardial ischemia. Anaphylaxis was unlikely because of the absence of hydatidiform cysts, a known cause of anaphylaxis (11), and the absence of new medications administered in the hour preceding collapse. An embolus causing cardiovascular collapse was unlikely, because there was no hypoxemia.
A patient with asymptomatic T-wave inversion should be considered for further evaluation, such as an echocardiogram or MRI. Preoperative optimization and correction of hypovolemia, hypoxia, hypercarbia, acidosis, and electrolyte disturbances prevent arrhythmias (12). Cardiac output monitoring alerts the anesthesiologist to act on changes in preload and contractility (13). Fluid shifts resulting from the release of cystic contents in this case could be avoided by a gradual surgical release. A pulmonary artery catheter was not inserted because of postoperative coagulopathy.
Treatment of ARVD is based on the prevention of arrhythmias with antiarrhythmic drugs such as amiodarone and sotalol. In patients with life-threatening arrhythmias, an implantable cardiac defibrillator can be considered. Relatives of this patient were referred to a cardiologist for possible screening, because this disease is dominant in 30%–50% of patients. A rare variant of this disease called "Naxos disease" is recessively inherited (14).
Eighteen of 50 cases of unexpected death related to anesthesia have been found to have ARVD (15). An international registry of the council on cardiomyopathies has been established to gather information on ARVD and its variants (16). Further study is required to determine why cardiovascular collapse during anesthesia in these patients is unresponsive to resuscitation.
In ARVD, previous uneventful anesthesia does not necessarily predict future perioperative cardiovascular stability. This case highlights the importance of considering investigation of asymptomatic T-wave inversion, because some patients may have a serious cardiac condition.
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References
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Accepted for publication March 3, 2004.
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Abstract
Introduction
