Sudden Cardiac Arrest during Adenotonsillectomy in a Patient with Subclinical Duchenne's Muscular Dystrophy

1993 ◽  
Vol 72 (2) ◽  
pp. 130-131 ◽  
Author(s):  
Nick C. Benton ◽  
Robert A. Wolgat
Keyword(s):  
Cardiac Arrest ◽  
Muscular Dystrophy ◽  
General Anesthesia ◽  
Case Reports ◽  
Sudden Cardiac Arrest ◽  
Elevated Serum ◽  
Serum Creatine Kinase ◽  
Strong Family History ◽  
History Of ◽  
Duchenne's Muscular Dystrophy

We describe a four-year-old boy of Indian descent who had elective adenotonsillectomy for chronic sore throat and partial airway obstruction. About 10 minutes into the procedure, the patient suddenly developed cardiac asystole. After prolonged cardiac resuscitation, recovery was achieved. No permanent neurologic deficits resulted. The child was later found to have a strong family history of Duchenne's muscular dystrophy (DMD) and an elevated serum creatine kinase level documented since shortly after birth. We reviewed several case reports substantiating the risk for cardiac arrest during general anesthesia in DMD patients, and we concluded that DMD is a little-known risk for cardiac arrest during general anesthesia. The otolaryngologist must be aware of this potential complication, because tonsillectomy and adenoidectomy are commonly indicated for children at an age when DMD may be subclinical.

scholarly journals Becker muscular dystrophy associated with sarcomeric hypertrophic cardiomyopathy in a paediatric patient: a case report

2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Paola Dolader ◽  
Ella Field ◽  
Anna Sarkozy ◽  
Juan Pablo Kaski
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Muscular Dystrophy ◽  
Neuromuscular Disorders ◽  
Elevated Serum ◽  
Becker Muscular Dystrophy ◽  
Neuromuscular Disorder ◽  
Chain Gene ◽  
Septal Hypertrophy ◽  
History Of ◽  
Myocardial Involvement

Abstract Background  Becker muscular dystrophy (BMD) is a neuromuscular disorder associated with myocardial involvement. The most frequent presentation is dilated cardiomyopathy. There have been isolated reports of hypertrophic cardiomyopathy (HCM) in association with BMD, but it is unclear whether these patients had an additional aetiology. Case summary  A 10-year-old boy was diagnosed with BMD having presented with a history of muscular pain during exercise and elevated serum creatine kinase levels. A cardiac screening was arranged and the echocardiogram confirmed an asymmetric septal hypertrophy. Given the unusual finding of HCM in this patient with BMD, we performed genetic testing for HCM-causing mutations and identified a likely pathogenic variant in heterozygosis in the beta-myosin heavy chain gene. Discussion  This case highlights the importance of considering additional aetiologies of cardiac disease in the presence of infrequent phenotypic expressions in neuromuscular disorders.

Sudden cardiac arrest in 15-year-old girl. Could it be avoided?

2018 ◽  
Vol 1 (46) ◽  
pp. 43-48
Author(s):  
Maria Posadowska ◽  
Maria Miszczak-Knecht ◽  
Alicja Mirecka-Rola ◽  
Katarzyna Bieganowska
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Death ◽  
Sudden Cardiac Arrest ◽  
Postmortem Examination ◽  
Cardiac Care ◽  
Related Family ◽  
Asymptomatic Patients ◽  
History Of ◽  
Genetically Determined

We present a case of a 15-year-old girl after sudden cardiac arrest because of ventricular fibrillation. Hypertrophic cardiomyopathy was diagnosed. Family history of patient was positive – her uncle (mother’s brother) died suddenly at the age of 21, postmortem examination showed hypertrophic cardiomyopathy. The deceased man’s family was not under cardiac care. The presented case proves, that due to genetically determined cardiac diseases such as cardiomyopathies and channelopathies, all related family members should be examined cardiologically. Diagnosis of the disease in asymptomatic patients would allow the implementation of treatment and reduce the risk of a sudden cardiac arrest / sudden cardiac death.

Possible GoLytely-Associated Cardiac Arrest: A Case Report and Literature Review

2019 ◽  
Vol 33 (3) ◽  
pp. 364-367 ◽  
Author(s):  
Yoonsun Mo ◽  
Shiv Gandhi ◽  
Jose Orsini
Keyword(s):  
Cardiac Arrest ◽  
Abdominal Pain ◽  
Sudden Cardiac Arrest ◽  
Lower Abdominal Pain ◽  
The Past ◽  
Life Threatening ◽  
History Of ◽  
Artery Disease ◽  
Possible Cause

Purpose: To report a case of sudden cardiac arrest possibly associated with the administration of GoLytely® (polyethylene glycol 3350 and electrolytes). Summary: A 60-year-old male with a history of hypertension, hyperlipidemia, type 2 diabetes, and coronary artery disease presented to the emergency department with complaints of constipation and lower abdominal pain over the past week, and the inability to urinate over the past day. The patient had received GoLytely as treatment to alleviate symptoms of constipation and abdominal pain. However, several hours after administration of the bowel prep solution, the patient suffered an episode of cardiac arrest. After ruling out other possible etiologies, GoLytely was suspected as a possible cause of cardiac arrest. The patient had suffered an anoxic brain injury and remained intubated and unconscious until he eventually expired, 20 days after the event. Conclusion: Although GoLytely appears to be a safe agent with fewer side effects, clinicians need to be mindful of potential life-threatening adverse events following GoLytely administration and monitor patients closely during and after administration.

scholarly journals Early-Onset Infantile Facioscapulohumeral Muscular Dystrophy: A Timely Review

2020 ◽  
Vol 21 (20) ◽  
pp. 7783
Author(s):  
Tai-Heng Chen ◽  
Yan-Zhang Wu ◽  
Yung-Hao Tseng
Keyword(s):  
Muscular Dystrophy ◽  
Early Onset ◽  
Case Reports ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Clinical Severity ◽  
Genetic Diagnostics ◽  
Rapid Decline ◽  
Novel Treatments ◽  
D4z4 Repeat ◽  
History Of

Facioscapulohumeral muscular dystrophy (FSHD)—the worldwide third most common inherited muscular dystrophy caused by the heterozygous contraction of a 3.3 kb tandem repeat (D4Z4) on a chromosome with a 4q35 haplotype—is a progressive genetic myopathy with variable onset of symptoms, distribution of muscle weakness, and clinical severity. While much is known about the clinical course of adult FSHD, data on the early-onset infantile phenotype, especially on the progression of the disease, are relatively scarce. Contrary to the classical form, patients with infantile FSHD more often have a rapid decline in muscle wasting and systemic features with multiple extramuscular involvements. A rough correlation between the phenotypic severity of FSHD and the D4Z4 repeat size has been reported, and the majority of patients with infantile FSHD obtain a very short D4Z4 repeat length (one to three copies, EcoRI size 10–14 kb), in contrast to the classical, slowly progressive, form of FSHD (15–38 kb). With the increasing identifications of case reports and the advance in genetic diagnostics, recent studies have suggested that the infantile variant of FSHD is not a genetically separate entity but a part of the FSHD spectrum. Nevertheless, many questions about the clinical phenotype and natural history of infantile FSHD remain unanswered, limiting evidence-based clinical management. In this review, we summarize the updated research to gain insight into the clinical spectrum of infantile FSHD and raise views to improve recognition and understanding of its underlying pathomechanism, and further, to advance novel treatments and standard care methods.

α-Dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy

FEBS Letters ◽  
1994 ◽  
Vol 350 (2-3) ◽  
pp. 173-176 ◽  
Author(s):  
James M. Ervasti ◽  
Steven L. Roberds ◽  
Richard D. Anderson ◽  
Nicholas J.H. Sharp ◽  
Joe N. Kornegay ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Muscular Dystrophy ◽  
Muscle Contraction ◽  
Elevated Serum ◽  
Serum Creatine Kinase ◽  
Golden Retriever ◽  
Golden Retriever Muscular Dystrophy

scholarly journals SAT-LB87 Golimumab Induced Thyroiditis

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Mo M Mai ◽  
Jocelyne G Karam ◽  
Nyein Lynn ◽  
Gurbaj Singh ◽  
Elizabeth Sedlis Singer
Keyword(s):  
Thyroid Nodules ◽  
Case Reports ◽  
Elevated Serum ◽  
Subacute Thyroiditis ◽  
Abnormal Behavior ◽  
Weight Changes ◽  
Drug Induced ◽  
Thyroid Cells ◽  
Patient Reported ◽  
History Of

Abstract Background: Subacute thyroiditis is caused by an inflammation and a destruction of the thyroid cells, leading to hyperthyroidism due to leakage of thyroid hormones, followed by possible hypothyroidism and/or full recovery of thyroid function. This is a case report describing a rare occurrence of drug-induced thyroiditis secondary to golimumab. Clinical Case: A 79-year-old female with HTN, hyperlipidemia, dementia and rheumatoid arthritis was brought to the ER for abnormal behavior including visual hallucinating and insomnia.Initial ER evaluation showed UTI for which antibiotic therapy was initiated. Dementia workup was performed including a negative head CT, nonreactive RPR, and borderline low vitamin B12 level. TFT obtained showed low TSH of 0.2mlU/L, elevated serum FT4 of 1.72ng/ml (n=0.58-1.64ng/ml) and elevated serum FT3 4.38pg/ml (n=2.5-3.9pg/ml), suggestive of hyperthyroidism. The patient reported no heat intolerance, hyperdefecation, or weight changes, but had intermittent palpitations. She denied any history of thyroid problem and did not take thyroid medication, amiodarone, biotin, or any new drug. She reported no fever or URI symptoms within the few weeks prior to admission. In addition to prednisone and methotrexate, she was taking golimumab 50mg every 30 days for the last 22 months for RA. The patient had a family history of hypothyroidism of two daughters and sister. She denied smoking, alcohol, or any other recreational drug use. Her home medications included prednisone 5mg daily, methotrexate, folic acid, lisinopril, simvastatin, and golimumab. On physical examination, she did not appear thyrotoxic and had no exophthalmos, thyroid tenderness, thyroid enlargement or thyroid nodules. Her HR range was 80bpm.Further analysis revealed normal TSI, TPO, and TgAb levels. The thyroglobulin level was very high at 2505ng/ml (n=1.6-59.9ng/ml). Her thyroid sonogram revealed bilateral thyroid nodules, largest at 1.9cm in the right mid pole. A 24-hr RAIU scan showed very low uptake (1.8%) consistent with thyroiditis (hyperthyroid phase).Endocrinology team did not recommend any antithyroid medications. In addition, she did not warrant NSAIDs or beta blockers as she was not symptomatic or tachycardic. In the absence of an autoimmune or an obvious viral process, her subacute thyroiditis was thought to be induced by golimumab. Conclusion: TNFɑ inhibitors used to treat chronic inflammatory diseases, have been rarely associated with subacute thyroiditis as described in case reports with adalimumab and etanercept use. We report the first subacute thyroiditis associated with golimumab use. We suggest that drug-induced subacute thyroiditis should be one of the differential diagnoses of thyroid dysfunction in patients treated with golimumab.

scholarly journals Predictors of sudden cardiac arrest in adolescents with mitral valve prolapse: an analysis of the nationwide inpatient sample

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Narasimhan ◽  
L Wu ◽  
C.H Lucas ◽  
K Bhatia ◽  
A Shah ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Family History ◽  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Sudden Cardiac Arrest ◽  
Adolescent Patients ◽  
History Of

Abstract Background Mitral valve prolapse (MVP) is the most commonly encountered valvular pathology seen in 2–3% of the general population. Though traditionally regarded as a benign pathology, recent literature suggests that sudden cardiac death is significantly more common in these patients with estimates of 0.2–0.4%/year. The exact underlying mechanism of these higher rates of SCD remain poorly understood. In this study, we aim to identify predictors of sudden cardiac arrest (SCA) in an adolescent population. Methods We conducted a retrospective study using the AHRQ-HCUP National Inpatient Sample 2016-2017 for the years 2016-17. All patients (≤18 years) admitted with Mitral valve prolapse were identified using ICD-10 codes and further sub stratified based on presence or absence of sudden cardiac arrest (SCA). Baseline characteristics were obtained and multivariate regression analysis was utilized to identify potential predictors of SCA. Independent risk factors for in-hospital mortality were identified using a proportional hazards model. Complications were defined as per the Agency for Health Care Research and Quality guideline. Results We screened a total of 71,473,874 admissions in the NIS database to identify a total of 1,372 adolescent patients admitted with MVP in the years 2016–17. These patients were then sub-categorized based on presence or absence of SCA during the hospitalization. Our findings revealed that patients with SCA were generally slightly older (15y vs 13y, p=0.036, OR-1.1, p=0.007) and more likely female (83.3% vs 13%, p=0.227, OR – 3.55, p=0.57)). Interestingly, patients in the SCA cohort were noted to have almost 4 fold higher rates of Mitral regurgitation (66.6% vs 18.35%, p=0.008, OR-8.89, p=0.005) as well as family history of SCD (16.7% vs 4.1%, p=0.145, OR-4.65, p=0.14). Conclusions Presence of Mitral regurgitation and a family history of sudden cardiac death are associated with significantly higher rates of SCA in adolescent patients with mitral valve prolapse. Predictors of SCA in Adolescent MVP Funding Acknowledgement Type of funding source: None

scholarly journals Sudden cardiac arrest risk in young athletes

2013 ◽  
Vol 25 (2) ◽  
pp. 53
Author(s):  
PJ-L Gradidge ◽  
D Constantinou ◽  
L Goldberg
Keyword(s):  
Cardiac Arrest ◽  
Ventricular Tachyarrhythmia ◽  
Sudden Cardiac Arrest ◽  
Previous History ◽  
Premature Death ◽  
Football Player ◽  
Young Athletes ◽  
Unexpected Death ◽  
Cardiac Murmur ◽  
History Of

Underlying cardiac abnormalities are the main cause of unexpected death in athletes on field. These abnormalities have been associated with a previous history of syncope, a family history of sudden cardiac arrest (SCA), cardiac murmur, a history of over-exhaustion post exercise and ventricular  tachyarrhythmia during physical activity. The timely diagnosis of susceptible athletes may assist with an appropriate management plan for these individuals, and allow for the prevention of premature death in sport. A young football player was screened for SCA risk using the fundamental components of the pre-participation examination (PPE) – essentially, a medical history, a resting and stress electrocardiogram, and an echocardiogram to support clinical findings. The case is submitted with consideration of the applicable literature to accentuate the importance of using PPE to prevent SCA in young athletes.

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