Symptomatic complete heart block leading to a diagnosis of Kearns–Sayre syndrome

Puri, Aniket; Pradhan, Akshyaya; Chaudhary, Gaurav; Singh, Vikas; Sethi, Rishi; Narain, Varun Shankar

doi:10.1016/j.ihj.2012.07.010

Cited by 6 publications

(5 citation statements)

References 7 publications

(4 reference statements)

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“…Most KSS patients have been diagnosed in Europe, the Middle East, and North America. [4][5][6][7] In Korea, one case involving the cardiac conduction system has been reported. That patient had a permanent pacemaker implanted, but the diagnosis was not confirmed by muscle biopsy.…”

Section: Discussionmentioning

confidence: 99%

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

Park

Her

et al. 2017

KMJ

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Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. KSS can lead to a risk of sudden death because of the potential progression of conduction abnormalities such as right or left bundle branch block or complete atrioventricular (AV) block. Here we describe the case of a KSS patient with type I diabetes who experienced syncope in the presence of complete AV block, confirmed by muscular biopsy.

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Section: Discussionmentioning

confidence: 99%

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

Park

Her

et al. 2017

KMJ

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“…These exacerbations have been noted to be associated with high blood eosinophil count which in turn is associated with coronary artery vasoconstriction 25,26 . In extreme cases, coronary vasospasm can cause complete coronary occlusion leading to acute coronary syndrome and myocardial infarction 27,28 or even fulminant heart failure 29 .…”

Section: Eosinophilia Coronary Spasm and Role Of Aspirinmentioning

confidence: 99%

Recurrent coronary spasm presenting as acute STEMI; To stent or not to stent?

Ullah¹,

Keshavarzi

Fraser

2022

Pak. j. Cardio vas. int. (print/Online)

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Coronary artery spasm is a relatively uncommon cause of ST segment elevation myocardial infarction. However, the clinical presentation and electrocardiographic findings are remarkably similar to STEMI secondary to plaque rupture. Recurrent presentation with coronary vasospasm as STEMI presents a unique clinical dilemma especially if they present to different centers where the previous history is not well known. Management in the acute settings continue to pose significant challenges in these acutely unwell patients. We describe and explore a similar clinical situation in which a patient with severe recurrent STEMI presentations was found to have coronary artery vasospasms. The case was twice misinterpreted as having plaque rupture STEMI leading to percutaneous coronary intervention and then further difficulties in long term management in view of need for long term antiplatelet and suspected association of eosinophilia with coronary vasospasm which in turn can be caused by Aspirin.

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“…This disorder is very rare, effecting only 1–3 per 100,000 individuals and are caused by large deletions in mtDNA ranging from 1000 to 10,000 base pairs that results in loss of genes involved in oxidative phosphorylation (Table 2) [216]. For cardiac manifestations, AV block is a common complication, with case reports of cardiac arrest and conduction abnormalities including complete heart block requiring permanent cardiac pacemaker implantation (Table 1) [217,218,219,220]. All patients with KSS or KSS spectrum disorders should be regularly screened with ECGs to monitor for progressive AV block, and there should be low threshold for monitoring in any KSS patient presenting with syncope.…”

Section: Primary Mitochondrial Disordersmentioning

confidence: 99%

Cardiovascular Manifestations of Mitochondrial Disease

Duran

Martínez

Adler

2019

Biology

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Genetic mitochondrial cardiomyopathies are uncommon causes of heart failure that may not be seen by most physicians. However, the prevalence of mitochondrial DNA mutations and somatic mutations affecting mitochondrial function are more common than previously thought. In this review, the pathogenesis of genetic mitochondrial disorders causing cardiovascular disease is reviewed. Treatment options are presently limited to mostly symptomatic support, but preclinical research is starting to reveal novel approaches that may lead to better and more targeted therapies in the future. With better understanding and clinician education, we hope to improve clinician recognition and diagnosis of these rare disorders in order to improve ongoing care of patients with these diseases and advance research towards discovering new therapeutic strategies to help treat these diseases.

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Symptomatic complete heart block leading to a diagnosis of Kearns–Sayre syndrome

Cited by 6 publications

References 7 publications

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

Recurrent coronary spasm presenting as acute STEMI; To stent or not to stent?

Cardiovascular Manifestations of Mitochondrial Disease

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