Life-threatening stress-induced cardiomyopathy combined with acute adrenal crisis.

Stress-induced cardiomyopathy, also known as Takotsubo cardiomyopathy and left ventricular apical ballooning syndrome, is characterized by transient systolic dysfunction of the apical and mid-segments of the left ventricle with the abscence of coronary occlusion.[1] This disease typically occurs in postmenopausal women of an advanced age after emotional or physical stress. Although the prognosis is generally favorable, acute fatal complications have been reported in a small subset of patients.[2]–[4] Some patients died with acute cardiac complications, on the other hand, other patients died with underlying critical illness which triggered stress-induced cardiomyopathy.[4],[5] It was noted that underlying diseases can determine the short-term prognosis of stress-induced cardiomyopathy rather than cardiac death.[4],[6] We present a case of life-threatening stress-induced cardiomyopathy combined with acute adrenal crisis that was successfully recovered with hydrocortisone replacement therapy.

An 81-year old woman with a history of hypertension, diabetes mellitus, and stable angina presented to the emergency department with severe chest discomfort and dyspnea (NYHA FC IV). Her daughter told that she had suffered from chest discomfort because of herpes zoster for several weeks and had been complaining of general weakness and loss of appetite for about five months. The patient had undergone percutaneous coronary intervention at right coronary artery (RCA) due to unstable angina 1.5 years ago. On admission, blood pressure was 130/80 mmHg and heart rate was 140 beats/min. Electrocardiography (ECG) showed sinus tachycardia with negative T waves in leads I, II, III, aVF and V4-6. However, 2 hours later, she looked pale and her mental status was confused. Thereafter, she was needed to intubate mechanically for severe dyspnea and pulmonary edema. Her blood pressure was dropped up to 80/60 mmHg and heart rate was 120 beats/min. Her blood gas analysis and serum electrolyte showed severe metabolic acidosis (pH 7.1, pCO2 32.8 mmHg, HCO3 10.4 mmol/L, Na 137.0 mEq/L, K 7.0 mEq/L, Cl 105.0 mEq/L). Her serum creatinine kinase MB (CK-MB) [7.8 (0–5.0) ng/mL] and troponin I [2.2 (0–0.8) ng/mL] levels were elevated. While waiting for transferring to the cardiac catheterization laboratory, her emergent transthoracic echocardiography revealed akinesia of the mid-left ventricular segments and apex and low left ventricular ejection fraction (LVEF) (28%). Her coronary angiography showed no significant stenosis and no in-stent restenosis at RCA (Figure 1A–B). Ergonovine spasm provocation test was also negative. These findings were compatible with stress-induced cardiomyopathy. Due to unstable hemodynamic status and past medical history concerning chronic steroid injection due to osteoarthritis of the knee, we performed additional diagnostic tests such as measurement of basal serum cortisol and serum adrenocorticotropic hormone (ACTH) levels with a rapid ACTH (250 µg corticotropin) test. The results showed that basal serum cortisol level was 8.7 µg/dL (< 4 µg/dL is highly suggestive of adrenal insufficiency and ≥ 18 µg/dL can rule out the possibility of adrenal insufficiency) and serum ACTH level was low-normal [13.2 (0–50) pg/mL).[7] After rapid ACTH test, peak serum cortisol level was 15.8 µg/dL (< 18 µg/dL for the diagnosis of adrenal insufficiency). Abdominal computed tomography showed no evidence of pheochromocytoma. Therefore, we diagnosed her status as acute adrenal crisis with chronic adrenal insufficiency and we started glucocorticoid hormone replacement therapy. In the first place, we administrated 100 mg of hydrocortisone bolus injection via intravenous route and then 200 mg of hydrocortisone was infused intravenously. On the sixth hospital day, her vital signs became stable and her ventilator weaning was successful. The total dose of infused steroid was tapered and changed to hydrocortisone 20 mg/day. On the tenth hospital day, her follow-up echocardiography showed no regional wall motion abnormalities and fully improved LVEF (76%).

Figure 1.

Coronary angiography in patient with stress-induced cardiomyopathy combined with acute adrenal crisis.

(A): The left coronary angiography shows no significant stenosis at left anterior descending and left circumflex artery; (B): The right coronary angiography reveals no significant stenosis and no in-stent restenosis of previous stent at proximal RCA (black arrows).

Typically stress-induced cardiomyopathy occurs in older women and is triggered by emotionally or physically stressful circumstances. It is characterized by transient systolic dysfunction of the apical and mid-segments of the left ventricle without significant coronary artery disease.[1] Although the majority of patients rapidly recover and the prognosis is generally favorable, some patients died with underlying critical illness which triggered stress-induced cardiomyopathy.[4],[5] Therefore, the management of underlying disease can determine prognosis such as this event is very important. In this case, the patient was shown to have acute adrenal crisis with chronic adrenal insufficiency during acute illness or stressful circumstance, such as herpes zoster. Subsequently, hydrocortisone replacement therapy could fully recover her cardiomyopathy. Like our case report, the authors think that prompt diagnosis and earlier rapid glucocorticoid theraphy are very important in patients with life-threatening stress-induced cardiomyopathy combined with acute adrenal crisis.

Although the precise cause of left ventricular dysfunction in stress-induced cardiomyopathy is remain unclarified, some investigations suggest that this acute cardiomyopathy may result from intense sympathetic activation with heterogeneity of myocardial autonomic innervation, diffuse microvascular spasm, and/or direct catecholamine toxicity.[8],[9] In this patient with stress-induced cardiomyopathy combined with adrenal crisis, it is possible that catecholamine-mediated myocardial stunning that results from a combination of myocardial ischemia related to microvascular dysfunction and catecholamines can directly injure the myocardium.

In conclusion, stress-induced cardiomyopathy may be a fatal disorder according to underlying critical illness such as acute adrenal crisis. Prompt diagnosis and early management of underlying casues of stress-induced cardiomyopathy are very important for improving the prognosis of this disorder.