Remimazolam anesthesia for transcatheter mitral valve repair in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome: a case report

Abstract

Background Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is characterized by cardiac depression, respiratory failure, myopathy, and anesthesia for affected patients is challenging. Although several anesthetics have been safely employed, there are no reports on remimazolam used in those patients. Case presentation A 47-year-old male with MELAS syndrome was diagnosed with mitral regurgitation and scheduled for transcatheter mitral valve repair under general anesthesia. Anesthesia was induced with remimazolam and remifentanil (0.3 µg/kg/min). Remimazolam was administered at 12 mg/kg/h until loss of consciousness for approximately 1 min. Anesthesia was maintained with 1.1–1.2 mg/kg/h of remimazolam and 0.1 µg/kg/min of remifentanil without circulatory collapse or severe metabolic acidosis. The tracheal tube was removed in the operating room. Conclusion Remimazolam may be a new option for anesthesia for MELAS syndrome patients with depressed heart function.

Assessing the anesthetic effectiveness of remimazolam in MELAS patients requires careful investigations

“…We do not agree with the statement in the discussion that serum lactate is elevated in MELAS because of metabolic acidosis [ 1 ]. It is the other way around.…”

“…We read with interest the article by Kitaura et al about a 47-year-old male with mitochondrial encephalopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome, who underwent elective transcatheter mitral valve repair because of mitral insufficiency [ 1 ]. MELAS was due to the common variant m.3243A>G and manifested phenotypically with short stature, cerebral and cerebellar atrophy, deafness, hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, heart failure, renal insufficiency requiring hemodialysis, myopathy, and lactic acidosis [ 1 ]. General anesthesia for the procedure was successfully induced and maintained with remimazolam and remifentanil, without circulatory compromise or metabolic acidosis [ 1 ].…”

“…MELAS was due to the common variant m.3243A>G and manifested phenotypically with short stature, cerebral and cerebellar atrophy, deafness, hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, heart failure, renal insufficiency requiring hemodialysis, myopathy, and lactic acidosis [ 1 ]. General anesthesia for the procedure was successfully induced and maintained with remimazolam and remifentanil, without circulatory compromise or metabolic acidosis [ 1 ]. It was concluded that remimazolam may be a new anesthetic option for MELAS [ 1 ].…”

“…General anesthesia for the procedure was successfully induced and maintained with remimazolam and remifentanil, without circulatory compromise or metabolic acidosis [ 1 ]. It was concluded that remimazolam may be a new anesthetic option for MELAS [ 1 ]. The study is attractive but raises concerns that should be discussed.…”

“…The two statements “propofol can be safely used in patients with MELAS” and “it is safer to avoid the continuous administration of propofol in patients with mitochondrial disorders” are contradictory [ 1 ]. This discrepancy should be solved.…”