Complete Heart Block during Anesthetic Management in a Patient with Mucopolysaccharidosis Type VII

Toda, Yuichiro; Takeuchi, Mamoru; Morita, Kiyoshi; Iwasaki, Tadaaki; Oe, Katsunori; Yokoyama, Masataka; Hirakawa, Masahisa

doi:10.1097/00000542-200110000-00041

Cited by 23 publications

(8 citation statements)

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“…In contrast to a number of other series, there were no significant airway events in this series. Failure to control airway (23), death from a cannot intubate, cannot ventilate scenario (24), postobstructive pulmonary edema (25,26), arrhythmia (27), and intraoperative cardiac arrest (28) have all been described in association with anesthesia for MPS.…”

Section: Discussionmentioning

confidence: 99%

A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses

Frawley

Fuenzalida

Donath

et al. 2012

Pediatric Anesthesia

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Section: Discussionmentioning

confidence: 99%

A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses

Frawley

Fuenzalida

Donath

et al. 2012

Pediatric Anesthesia

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“…Complete AV block can occur in MPS 30 , 31 and may even cause sudden cardiac death in MPS as described by Hishitani et al [31] . In the past, the incidence of sudden death in patients with MPS was reported to be as high as 11%, thus in these patients, careful surveillance with Holter ECG is needed [32] .…”

Section: Discussionmentioning

confidence: 90%

Cardiac disease in children and young adults with various lysosomal storage diseases: Comparison of echocardiographic and ECG changes among clinical groups

Müeller¹,

Jost

Rohrbach

et al. 2013

IJC Heart & Vessels

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BackgroundLysosomal storage disease (LSD) is a rare inherited disease group. Consecutively there are few data on cardiac changes in mucopolysaccharidosis (MPS), Anderson Fabry disease (AFD), and other LSD (oLSD) including Pompe disease (PD) and Danon disease (DD), I-cell disease ICD and mucolipidosis III (ML III).MethodsBetween 1994 and 2011, we identified 39 patients with LSD: 25 with MPS, 8 with AFD, and 6 with oLSD including PD (1), ML III (2), DD (1), and ICD (2) at our institution fulfilling the inclusion criteria of at least one echocardiogram and ECG.ResultsMedian age was 11.4 years (range: 2–27), 22 were females (56%). Normal echocardiograms were present in 12 patients (31%): 4 with MPS (16%), 7 AFD (88%), and 1 oLSD (17%). Valvular heart disease was present in 23 patients (59%) occurring more often in MPS (76%) and oLSD (67%) than in AFD (0%) (p < 0.001). The most common ECG abnormality was a short PR interval in 10 of 35 patients (29%) occurring in all LSD groups. Median follow-up was 5.8 (0.2–22.2) years showing diminished 5-year survival compared to an age-matched group. However, no patient died due to a cardiac cause and no cardiovascular intervention was necessary.ConclusionEchocardiographically detectable cardiovascular involvement in children with LSD is mostly confined to MPS and oLSD. Valve thickening in echo and a short PR interval in the ECG are the most frequent abnormalities. Routine repeat assessment is recommended in LSD. However, significant cardiac disease necessitating cardiac intervention is rare during a short follow-up.

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“…The additional finding of carotid artery intimal-medial hyperplasia confirms the utility of carotid ultrasonography in identifying MPS-related cardiovascular disease in vivo. The pathologic findings (subendocardial myocardial fibrosis, dilated/hypertrophic cardiomyopathy, and anesthesiainduced hemodynamic changes) may all be manifestations A B C of longstanding, ongoing ischemic heart disease and have contributed to the patient's fatal dysrhythmia (Toda et al 2001). While MPS VII cardiovascular lysosomal storage begins prenatally, the progression and pathogenesis of cardiomyopathy, valvular dysfunction, vascular intimal-medial hyperplasia, and elastin fragmentation are being elucidated (Irani et al 1983;Molyneux et al 1997;Geipel et al 2002;Venkat-Raman et al 2006;Delbecque et al 2009).…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular Histopathology of a 11-Year Old with Mucopolysaccharidosis VII Demonstrates Fibrosis, Macrophage Infiltration, and Arterial Luminal Stenosis

Lew

Peña²,

Edwards

et al. 2017

JIMD Reports

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Mucopolysaccharidosis type VII (MPS VII) is caused by β-glucuronidase deficiency, resulting in lysosomal accumulation of glycosaminoglycans (GAGs) and multisystemic disease. We present cardiovascular gross and histopathology findings from a 11-year-old MPS VII male, who expired after developing ventricular fibrillation following anesthesia induction. Gross anatomic observations were made at autopsy; postmortem formalin-fixed paraffin-embedded samples of the carotid artery, aorta, myocardium, and valves were sectioned and stained with hematoxylin-eosin, Verhoeff-Van Gieson, CD68, and trichrome stains. Gross heart findings include an enlarged, dilated heart, mitral valve prolapse with thick, shortened chordae tendinae, and thickened aortic valve cusps. The aorta contained raised intimal plaques mimicking conventional atherosclerosis. Cardiac myocytes included hypertrophic nuclei, subendocardial fibrosis, and increased interfascicular collagen. Coronary lumens were 40-70% stenosed by fibrointimal hyperplasia containing storage material-laden cells, CD68 macrophages, and fragmented elastin laminae. Similar findings were visualized in aortic intimal plaques. We confirm that arterial plaques, elastin fragmentation, and activated CD68 macrophage infiltration occur in human MPS VII, consistent with previously observed findings in murine and canine MPS VII. We also confirm ultrasonographically observed carotid intimal-medial thickening is an in vivo correlate of histopathologic vascular fibrointimal hyperplasia. MPS VII patients should be regularly monitored for cardiac disease, with methods such as Holter monitors and stress testing; MPS VII-directed treatments should effectively address cardiovascular disease.

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Complete Heart Block during Anesthetic Management in a Patient with Mucopolysaccharidosis Type VII

Cited by 23 publications

References 0 publications

A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses

A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses

Cardiac disease in children and young adults with various lysosomal storage diseases: Comparison of echocardiographic and ECG changes among clinical groups

Cardiovascular Histopathology of a 11-Year Old with Mucopolysaccharidosis VII Demonstrates Fibrosis, Macrophage Infiltration, and Arterial Luminal Stenosis

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