As far as we know, this is the first case report describing the anesthetic management of a CS patient with an implanted CRT-D. We accomplished safe anesthetic management by understanding the characteristic features of this disease, implementing the proper settings of the CRT-D device, and preventing progressive heart failure by using the FloTracTM/PreSep/EV1000TM system.
Racial differences in the incidence of CS have been reported, with Japanese patients more frequently reported to have the disease in comparison to Caucasian or African-American patients [2, 14, 15]. Cardiac involvement is reported clinically in 5% of sarcoidosis patients; however, autopsy and cardiovascular magnetic resonance studies have shown a higher percentage of subclinical cardiac involvement, ranging from 15 to 80%, which accounts for the majority of sarcoidosis-related deaths [2, 3, 14]. Clinical management of arrhythmias include the use of antiarrhythmics and/or the placement of an automatic ICD [2, 4]. ICD placement is a class 1 recommendation for CS patients with spontaneous sustained ventricular arrhythmias, a previous cardiac arrest, or a LVEF ≤ 35% despite optimal medical therapy and a period of immunosuppression . CRT placement is also considered for CS patients who are at risk for the development of heart failure [6, 7].
Case reports of anesthetic management for CS patients are limited. In one report, the CS patient had a pacemaker ; in another report, the patient had an ICD ; and in two other reports, the patients did not have any devices [8, 9]. In another case, emergency introduction of a temporary pacemaker via the jugular vein was needed due to a complete atrioventricular block during surgery . However, we could find no reports related to anesthetic management of CS patients with implanted CRT-Ds.
CRTs are used to correct conduction abnormalities by coordinating the pump functions of the left and right ventricles. However, when comparing a cohort of patients with CS with a cohort of patients with dilated cardiomyopathy, the response to CRT was lower, with a higher rate of major adverse cerebral and cardiovascular events (MACCE) . Moreover, among CS patients, the occurrence of ventricular tachycardia/fibrillation (VT/VF) was elevated . These higher complication rates may be attributable to LV dysfunction due to sarcoid lesions and granulomas, as well as widely spreading fibrosis in the myocardium, which exceeded the benefit of CRT in CS patients . In the present case, the patient’s cardiac function had progressively worsened since CRT-D implantation. Thus, clinicians should be aware that there is still a risk of cardiac compromise, even in the presence of a CRT-D, and especially in perioperative settings where many factors, including the stress response, fluid shifts, and hormonal changes, can have substantial influences on the heart and the whole body. Potential intraoperative problems related to CRT-Ds include electromagnetic interference (EMI) caused by surgical devices and the malfunctioning of the defibrillator. We maintained the DDD pacing mode throughout the operation. Shifting the pacing mode to asynchronous pacing, such as VOO, is useful for preventing EMIs; however, the VOO mode of a CRT-D cannot synchronize the left and right ventricles. In order to maintain cardiac output, it is desirable to continue with the synchronous pacing mode if possible.
Anesthetic strategies for patients with sarcoidosis have not clearly been determined due to a limited amount of published research, and no deteriorative anesthetic agents have been reported. In this patient, we chose to use sevoflurane because it has less of an effect on myocardial contractility  and is less likely to cause arrhythmias [18, 19]. Remifentanil, which has a direct negative chronotropic effect , was also safely used in this patient, partially due to the support of the CRT. Nevertheless, the long-term effect of anesthetic drugs on patients with sarcoidosis remains unknown, and further research should be conducted in this field. Continuous epidural anesthesia was used because we believed that reducing the postoperative afterload would be effective in preventing worsening of heart failure. On the other hand, we refrained from bolus administration intraoperatively because the reduction of the preload by epidural anesthesia would disrupt the hemodynamics of this patient.
This patient’s laparoscopic surgery required a significant amount of time in the head-down and head-up positions. The head-down position increases the preload, and the head-up position decreases it. In order to avoid excessive preload and afterload, it was helpful to monitor the stroke volume variation (SVV) and the systemic vascular resistance (SVR) using the FloTrac/PreSep/EV1000TM system. Although the usefulness of dynamic parameters such as SVV in laparoscopic surgery are controversial, anesthesia time in this case was more than 10 h, which increased the importance of sequential hemodynamic monitoring for this patient.
In this patient, the only clinical manifestations of sarcoidosis were in the heart. However, clinicians should be wary about inherent limitations in the sarcoidosis diagnosis, including clinically undetectable inflammation in other organs or cardiac lesions that arise only as an original manifestation . Thus, clinicians should be careful to examine all organs that can be affected by sarcoidosis. Indeed, although this patient did not show any symptoms, the PET-CT demonstrated lung involvement of the sarcoidosis, which can induce both a restrictive and an obstructive pulmonary disorder with wheezing and bronchial hyperreactivity, even in the absence of evident morphological abnormalities of the tracheobronchial tree . Sarcoidosis of the upper respiratory tract, including laryngeal areas, should also be considered because it can occur in up to 5% of patients  and can cause difficult intubations, airway obstruction, and post-extubation croup.
We achieved safe anesthetic management of a patient with cardiac dysfunction caused by CS, who was previously implanted with a CRT-D. Although intraoperative cardiovascular system monitoring contributed to the successful anesthetic management, a detailed understanding of this patient’s condition and his sarcoidosis diagnosis was indispensable for the proper perioperative management of this patient.