Combined Aortic and Mitral Valve Stenosis in Mucopolysaccharidosis Syndrome Type I-S: A Report of a Rare Case

Mucopolysaccharidosis (MPS) syndrome is an inherited metabolic disorder. In more than half of the patients with MPS syndrome, heart valve involvement is reported; however, combined aortic and mitral valve stenosis in MPS syndrome type I-S is very rare. We describe a 39-year-old man with severe mitral and aortic valve stenosis due to MPS syndrome type I-S. Transthoracic and transesophageal echocardiography revealed severe thickening and calcification in the aortic and mitral valves with severe left ventricular hypertrophy. The coronary arteries were normal in angiography.

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AN UNUSUAL CASE OF MITRAL VALVE CHORDAL RUPTURE

10.22541/au.163251554.48329042/v1 ◽

2021 ◽

Author(s):

Rosa Lillo ◽

Gessica Ingrasciotta ◽

Gabriella Locorotondo ◽

Antonella Lombardo ◽

Francesca Graziani

Keyword(s):

Mitral Valve ◽

Rare Case ◽

Ventricular Hypertrophy ◽

Unusual Case ◽

Left Ventricular ◽

Severe Mitral Regurgitation ◽

Valve Leaflet ◽

Mitral Valve Chordal Rupture ◽

Leaflet Prolapse

In this report, we present a rare case of severe mitral regurgitation due to isolated mitral valve chordal rupture without valve leaflet prolapse in a patient with Fabry cardiomyopathy. This finding could be due to subvalvular apparatus storage of glycosphingolipids rather than fibro-elastic deficiency, underlying how close cardiological follow-up of Fabry patients must be comprehensive and not only focused on left ventricular hypertrophy and arrhythmias. We feel that our finding could be of interest for the readership of your Journal.

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Changes of NT proBNP correlated with the regression of left ventricular hypertrophy in patients with aortic valve replacement

European Journal of Heart Failure Supplements ◽

10.1016/s1567-4215(08)60235-0 ◽

2008 ◽

Vol 7 ◽

pp. 85-85

Author(s):

E JEON ◽

P PARK ◽

K SUNG ◽

D CHOI ◽

J KIM ◽

...

Keyword(s):

Aortic Valve ◽

Left Ventricular Hypertrophy ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Ventricular Hypertrophy ◽

Left Ventricular

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Cornell voltage left ventricular hypertrophy predicts all-cause mortality better than Sokolow-Lyon voltage in patients with and without diabetes – data from 183,749 primary care ECGs

European Heart Journal ◽

10.1093/ehjci/ehaa946.3042 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

S Haxha ◽

U Pedersen-Bjergaard ◽

J.B Nielsen ◽

J Pallisgaard ◽

R.B Devereux ◽

...

Keyword(s):

Primary Care ◽

Left Ventricular Hypertrophy ◽

Predictive Value ◽

Ventricular Hypertrophy ◽

Strong Predictor ◽

Multivariable Analysis ◽

Left Ventricular ◽

Type I ◽

All Cause Mortality ◽

Cornell Voltage

Abstract Background Cornell voltage criteria (CL) and Sokolow-Lyon criteria (SL) for electrocardiographic left ventricular hypertrophy (ECG-LVH) are well known predictors of cardiovascular outcome. However, their predictive value may differ according to patient type and remains to be further tested in diabetic mellitus (DM) patients. Purpose The present study aims to determine the prevalence of each ECG-LVH criteria and their respective predictive value in DM patients. Method A retrospective cohort study of individuals age >40 years with digital ECGs from primary care were collected during 2001 to 2011. Data on medication, comorbidity, and outcomes were collected from Danish nationwide registries. DM was defined if individuals were prescribed oral antidiabetics or insulin, if they were diagnosed with DM type I or II, or had a HbA1c>48 mmol/l. Cox multivariable analysis was used for estimating hazard ratio (HR) and 95% confidence intervals (95% CI) for all-cause mortality during follow-up of up to 17 years. Results Included were 183,749 individuals with a digital ECG collected in primary care. A total of 13,003 (7.1%) individuals had DM, they were older (65.8 vs. 61.3 years), had more myocardial infarction (16.1% vs. 5.2%), stroke (14.4% vs. 6.2%), hypertension (35.1% vs. 13.2%), CL LVH (8.0% vs. 5.6%) and more were males (53.3% vs. 45.3%) compared to the non-DM individuals (all p<0.001). CL identified a larger percentage of LVH in DM compared to non-DM individuals (8.0% vs. 5.6%, p<0.001), whereas SL identified similar percentage LVH in DM and non-DM individuals (8.5% vs. 8.1%, p=0.068). In multivariable adjusted analysis CL LVH remained strongly associated with all-cause mortality [HR 1.45 (95% CI: 1.42–1.48)] compared to SL LVH which found only a modest association [HR 1.06 (95% CI: 1.03–1.10)] (Figure 1). Of note, the association of CL LVH and all-cause mortality was even stronger than DM per se. There was no interaction with DM and either ECG LVH criteria (p>0.45). Conclusion Cornell Voltage Left Ventricular Hypertrophy is a strong predictor of mortality in patients with and without diabetes and an independent risk factor compared to hypertension and diabetes. The predictive value was substantially stronger than Sokolow-Lyon Voltage criteria for hypertrophy. Figure 1. LVH and all-cause mortality Funding Acknowledgement Type of funding source: None

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One-shot transcatheter double valve replacement: six-month follow-up—a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa335 ◽

2020 ◽

Author(s):

Georg Lutter ◽

Mohamed Salem ◽

Derk Frank ◽

Thomas Puehler

Keyword(s):

Mitral Valve ◽

Valve Replacement ◽

Young Patients ◽

Heart Association ◽

Left Ventricular ◽

Mitral Valve Stenosis ◽

Term Outcome ◽

Transcatheter Mitral Valve Replacement ◽

Valve Implantation

Abstract Background Transcatheter aortic valve replacement (TAVR) in combination with a valve-in-valve (V-i-V) transcatheter mitral valve replacement (TMVR) is a rare procedure in comparison to surgical therapy especially in young patients. We report on a young patient at high surgical risk, receiving a double valve implantation with two S3 transcatheter heart valves. Case summary A 59-year-old female patient with two previous mitral valve replacements due to endocarditis and re-endocarditis experienced a new onset of severe mitral valve stenosis in combination with progredient aortic stenosis. She was admitted to the hospital with severe dyspnoea and intermittent non-invasive ventilation [New York Heart Association (NYHA) III–IV]. An interventional transapical transcatheter double valve implantation was planned and carried out due to cardiac decompensation and high comorbidity preoperatively (STS score of 6.92). At 6-month follow-up, the patient presented herself in an improved condition with reduced symptoms (NYHA I–II), a good functional status of both valves and an advanced right and left ventricular function in the echocardiogram. Discussion Even in younger patients at high risk, a combined native TAVR and V-i-V TMVR procedure can be performed. In this case, a transcatheter SAPIEN 3 valve was transapically implanted with good clinical mid-term outcome at 6 months.

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