Case Report
A 40-year-old man was admitted to our hospital for treatment of recurrent heart failure. Twelve years prior, he had been diagnosed with dilated cardiomyopathy and Becker muscular dystrophy. After the initial diagnosis, the patient’s heart failure gradually worsened and the patient was referred to our hospital for further treatment. On presentation, the patient’s clinical features were as follows: body temperature of 36.4°C, blood pressure of 82/54mmHg, pulse rate of 80 beats/min, Levine 3/6 systolic heart murmur on auscultation. An electrocardiogram showed normal sinus rhythm and a heart rate of 82 beats/min. Echocardiography showed ventricular dilatation and decreased contractility (ejection fraction; EF, 25%), with severe functional mitral regurgitation due to leaflet tethering. Regarding limb strength, Gowers’ sign was present and he had difficulty crouching.
We performed mitral annuloplasty (28mm SJM Rigid Saddle Ring; Abbott, St. Paul, MN, USA), as well as papillary muscle approximation with posterior left ventriculoplasty. The patient was weaned from cardiopulmonary bypass with high dose of inotropic support as well as intra-aortic balloon pumping (IABP). The cardiopulmonary bypass time was 179min, and the aortic cross-clamp time was 127 min.
On admission to the intensive care unit, the heart rate was around 130-140beats/min, blood pressure was 100/50 mmHg, pulmonary artery pressure was 30/20mmHg, and cardiac index (CI) was 3.0 L/min/m2. On postoperative day (POD) 1, CI was above 3.0 L/min/m2, and IABP was discontinued. Subsequently, the doses of dopamine and milrinone were gradually decreased and completely discontinued on POD 8. After discontinuing these drugs, the patient’s heart rate remained at around 80beats/min under sedation, but when he woke up, he became tachycardic (approximately 120 beats/min). Echocardiography showed decreased contractility (EF, ~30%), without mitral regurgitation.
Following the surgery, the patient developed a continuous fever (39.0°C or higher: Figure 1A). We suspected malignant hyperthermia, however, his serum creatine kinase level peaked at approximately 1500 U/L, and there was no muscular rigidity. Moreover, the patient’s bilirubin level was elevated after the operation and continued to rise (predominantly direct hyperbilirubinemia). During this time, serum transaminase levels (aspartate transaminase and alanine transaminase) were relatively normal (both approximately 30-60 U/L) and alkaline phosphatase levels were only mildly elevated (approximately 350 U/L). Computed tomography and abdominal ultrasonography showed no abnormalities in the hepatobiliary system. It appeared unlikely that the patient had developed hyperbilirubinemia due to liver shock liver given his preserved cardiac output throughout the postoperative course. Of note, the patient continued to have normal kidney function. Therefore, plasmapheresis was performed on POD 10 when total bilirubin reached 20mg/dL. Despite this, his total bilirubin level remained high at approximately 10 mg/dL (Figure 1B). The patient also developed watery diarrhea, but he was negative for Clostridium difficile .
The patient continued to be febrile, produced copious sputum due to pneumonia, and showed signs of congestive heart failure, and we decided to sedate him and put him on ventilator support. When we performed a tracheotomy to do so, we noticed well-developed blood vessels around the thyroid gland. In fact, these vessels resulted in significant bleeding that was difficult to manage.
After the tracheotomy, the patient continued to have delirium, and his mental status did not improve even after sedation was discontinued. At this time, various hormone tests were performed, and abnormal levels of thyroid hormone were observed (thyroid-stimulating hormone, <0.005 μIU/mL; free T4 >7.770 ng/dL; free T3 9.98 pg/mL). Based on these figures, the patient was diagnosed with thyroid storm. Lugol’s iodine and thiamazole (30 mg/day) were administered orally. In addition, a dose of steroid (hydrocortisone sodium succinate, 100 mg) was administered intravenously every 8 hours. As a result, the patient’s mental status immediately improved, his heart rate decreased from 120-140 beats/min to 100beats/min, and his fever subsided (Figure 1). Technetium scintigraphy of the thyroid was performed on POD 37, and showed abnormally low uptake (0.01%: Figure 2), confirming destructive thyrotoxicosis. On POD 42, free T4 (4.080 ng/dL) and free T3 (2.48 pg/mL) were improved and we discontinued Lugol’s iodine, and reduced the dose of thiamazole to 15 mg/day. The tracheostomy was closed, and the patient was transferred to a long-term care facility to continue rehabilitation.