Patient suspected susceptibility to malignant hyperthermia: impact of the disease<sup/>

Rodrigues, Gislene; Andrade, Pamela Vieira; Santos, Joilson Moura dos; Castro, Isac de; Amaral, José Luiz Gomes do; Silva, Helga Cristina Almeida da

doi:10.1016/j.bjane.2021.10.021

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Brazilian Journal of Anesthesiology

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Sumário

Original Investigation • para Braz. J. Anesthesiol. 73 (2) • Mar-Apr 2023 • https://doi.org/10.1016/j.bjane.2021.10.021 copy

Patient suspected susceptibility to malignant hyperthermia: impact of the disease^☆ ☆ This manuscript is part of the MSc dissertation entitled “Patients with Malignant Hyperthermia: Study of the Impact of the Disease and Preliminary Development of a Guide”

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Introduction

Malignant Hyperthermia (MH) is an inherited hypermetabolic syndrome triggered by exposure to halogenated anesthetics/succinylcholine. The lack of knowledge regarding this condition might be associated with the rare occurrence of MH reaction and symptoms.

Methods

This observational study evaluated 68 patients from 48 families with confirmed or suspected MH susceptibility due to medical history of MH reaction or idiopathic increase of creatine kinase or MH-related myopathies. Participants were assessed by a standardized questionnaire and submitted to physical/neurological examination to assess the characteristics of patients with MH, their knowledge about the disease, and the impact suspected MH had on their daily lives.

Results

Suspected MH impacted the daily life of 50% of patients, creating difficulties in performing surgical/clinical/dental treatment and problems related to their family life/working/practicing sports. The questionnaire on MH revealed a correct answer score of 62.1 ± 20.8 (mean ± standard deviation) on a scale 0 to 100. Abnormal physical/neurological examination findings were detected in 92.6% of susceptible patients.

Conclusions

Suspected MH had impacted the daily lives of most patients, with patients reporting problems even before MH investigation with IVCT. Patients showed a moderate level of knowledge about MH, suggesting the need to implement continuing education programs. MH susceptible patients require regular follow-up by a health team to detect abnormalities during physical and neurological examination.

KEYWORDS
Anesthesia; Health education; Malignant hyperthermia

48 Families	MH susceptible (IVCT+): n = 29 (60.4%)	MH Non-susceptible (IVCT-): n = 8 (16.6%)	Did not perform IVCT: n = 11 (22.9%)
Anesthetic MH reaction (31 Families - 64.6%)
12 Families (12 MH reaction survivors tested) ^a a Father was tested for one survivor < 10 years.	9 (75%)	1 (8.3%)	2 (16.6%)
19 Families (37 first degree relatives tested) ^b b 16 MH deaths (11 families) and 8 not tested survivors (8 families).	9 (47.4%)	5 (26.3%)	5 (26.3%)
CK level increase 7 Families - 14.6%	6 (85.7%)	1 (14.3%)	0 (0%)
Myopathies 10 Families - 20.8%	5 (50%)	2 (20%)	3 (30%)

Surgery	n	%
Gastrointestinal	7	20.6
Urological	7	20.6
Orthopedic	4	11.8
Thyroidectomy	4	11.8
Adenoidectomy and/or tonsillectomy	3	8.8
Oral and Maxillofacial	3	8.8
Nodulectomy (two cases in the neck and one in the lung)	3	8.8
Eye surgery	2	5.9
Neurosurgery	1	2.7
Total	34	100.0

Item	Question	Correct answer n (%)	Incorrect answer n (%)	Not answered n (%)
A	What are signs of an MH reaction?	25 (36.77%)	15 (22.05%)	28 (41.18%)
B	What is the cause of MH?	56 (82.36%)	8 (11.76%)	4 (5.88%)
C	When does MH reaction occur?	55 (80.89%)	6 (8.82%)	7 (10.29%)
D	How is MH treated?	40 (58.83%)	9 (13.23%)	19 (27.94%)
E	Who is at risk for MH?	46 (67.65%)	13 (19.12%)	9 (13.23%)
F	How can MH be avoided?	32 (47.06%)	27 (39.71%)	9 (13.23%)
G	Can a MH susceptible patient undergo surgery?	64 (94.12%)	3 (4.41%)	1 (1.47%)
H	Can a patient with MH receive local anesthesia during dental treatment?	61 (89.71%)	2 (2.94%)	5 (7.35%)
I	Does a MH susceptible individual have any restrictions regarding medication?	24 (35.29%)	28 (41.18%)	16 (23.53%)
J	Does a MH susceptible individual have any restrictions in daily or professional activities?	19 (27.94%)	43 (63.24%)	6 (8.82%)

Clinical/Neurological findings	MH susceptible IVCT+:n = 41	MH negative IVCT-:n = 11
BMI < 18.5	1 (2.4%)	0 (0%)
BMI > 24.9	22 (53.6%)	5 (45.4%)
Hypertension	9 (21.9%)	4 (36.3%)
Neurological findings
Dysmorphisms	19 (46.3%)	6 (54.5%)
Muscle hypertrophy	17 (41.5%)	3 (27.2%)
Motor unit syndrome	15 (36.6%)	5 (45.4%)
Cranial nerve abnormalities	5 (12.2%)	1 (9.1%)
Tactile hypoesthesia^a a Compression neuropathies related to anatomical deformities or previous surgeries.	5 (12.2%)	0 (0%)

Sociedade Brasileira de Anestesiologia (SBA) Rua Professor Alfredo Gomes, 36, Botafogo , cep: 22251-080 - Rio de Janeiro - RJ / Brasil , tel: +55 (21) 97977-0024 - Rio de Janeiro - RJ - Brazil
E-mail: editor.bjan@sbahq.org

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[1] ⁎ Corresponding author: H.C.A. Silva E-mail:halsilva@uol.com.br (H.C. Silva).

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Patient suspected susceptibility to malignant hyperthermia: impact of the disease☆ ☆ This manuscript is part of the MSc dissertation entitled “Patients with Malignant Hyperthermia: Study of the Impact of the Disease and Preliminary Development of a Guide”

Abstract

Introduction

Methods

Results

Conclusions

Patient suspected susceptibility to malignant hyperthermia: impact of the disease^☆ ☆ This manuscript is part of the MSc dissertation entitled “Patients with Malignant Hyperthermia: Study of the Impact of the Disease and Preliminary Development of a Guide”