Left ventricular non-compaction as a potential source for cryptogenic ischemic stroke in the young: A case-control study

Pöyhönen, Pauli; Kuusisto, Jouni; Järvinen, Vesa; Pirinen, Jani; Räty, Heli; Lehmonen, Lauri; Paakkanen, Riitta; Martinez‐Majander, Nicolas; Putaala, Jukka; Sinisalo, Juha

doi:10.1371/journal.pone.0237228

Cited by 13 publications

(14 citation statements)

References 33 publications

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“…LVNC cardiomyopathy is associated with a higher burden of arrhythmia, and prophylactic placement of automatic implantable cardioverter defibrillators may have a mortality benefit in selected cases [ 18 ]. Patients with LVNC are prone to left ventricular thrombus formation with increased risk of stroke [ 19 ]. However, the presence of LVNC alone is not sufficient for instituting prophylactic anticoagulation.…”

Section: Discussionmentioning

confidence: 99%

An Unusual Presentation of Left Ventricular Non-compaction Cardiomyopathy in an Elderly Patient

Afify¹,

Ghosh²,

Khalil³

et al. 2021

Cureus

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Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy. The true prevalence of LVNC is unclear. The clinical presentation of LVNC varies widely from asymptomatic to end-stage heart failure or sudden cardiac death, and the diagnostic criteria are not standardized. Moreover, there is an increased risk for thromboembolic events with LVNC. We present an unusual case of LVNC first diagnosed in a septuagenarian.

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Section: Discussionmentioning

confidence: 99%

An Unusual Presentation of Left Ventricular Non-compaction Cardiomyopathy in an Elderly Patient

Afify¹,

Ghosh²,

Khalil³

et al. 2021

Cureus

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“…All subjects were imaged with a 1.5-T Avanto fit magnetic resonance scanner (Siemens Healthcare, Erlangen, Germany) using a 32-channel receiver body coil and ECG-gating, as described previously. 15 Specific technical parameters for each sequence type are given in ( online supplemental table S1 ).…”

Section: Methodsmentioning

confidence: 99%

Right atrium and cryptogenic ischaemic stroke in the young: a case–control study

et al. 2021

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BackgroundRecent studies suggest left atrial (LA) dysfunction in cryptogenic stroke. We studied the dynamics of right atrium (RA) and right atrial appendage (RAA) in young adults with cryptogenic stroke. We hypothesised that bi-atrial dysfunction and blood stagnation might contribute to thrombosis formation in patients with patent foramen ovale (PFO), as deep venous thrombosis is detected only in the minority of patients.MethodsThirty patients (aged 18–49) with a first-ever cryptogenic stroke and 30 age-matched and sex-matched stroke-free controls underwent cardiac magnetic resonance (CMR) imaging. An approach to estimate the RAA volume was developed, using crista terminalis and pectinate muscles as anatomical landmarks. Atrial expansion indices were calculated as (maximal volume – minimal volume) ×100%/minimal volume. Total pulmonary to systemic blood flow ratio (Qp/Qs) was based on phase contrast CMR. Right-to-left shunt (RLS) was evaluated with transoesophageal echocardiography in 29 patients and transcranial Doppler in 30 controls, moderate-to-severe RLS considered as clinically significant.ResultsWe found that RA and RAA volumes were similar between patients and controls. Also, RA expansion index was similar, but RAA (95.6%±21.6% vs 108.7%±25.8%, p=0.026) and LA (126.2%±28% vs 144.9%±36.3%, p=0.023) expansion indices were lower in patients compared with controls. Seven (24%) of 29 patients had an RLS compared with 1 (3%) of 30 controls (p=0.012). Among 59 study subjects, RLS was associated with lower RA (81.9%±15.9% vs 98.5%±29.5%, p=0.030), RAA (84.7%±18% vs 105.6%±24.1%, p=0.022), LA (109.8%±18.6% vs 140.1%±33.7%, p=0.017) and LAA (median 102.9% (IQR 65.6%–121.7%) vs 229.1% (151.8%–337.5%], p=0.002) expansion indices and lower Qp/Qs ratio (0.91±0.06 vs 0.98±0.07, p=0.027).ConclusionsThis study suggests bi-atrial dysfunction in young adults with cryptogenic stroke, associated with moderate-to-severe RLS. Dysfunction of the atria and atrial appendages may be an additional mechanism for PFO-related stroke.Trial registration numberNCT01934725.

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“…26,28 A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy A recent case-control study in young adults (age 18-49) presenting with an imaging-positive ischaemic stroke of undetermined aetiology showed that the percentage of non-compacted LV volume was higher in patients with cryptogenic stroke compared with stroke-free controls of similar age and gender. 51 Importantly, a 5% increase in non-compacted ventricular volume at cardiac MRI has been associated with a nine-fold increased risk of cryptogenic ischaemic stroke. 51 Furthermore, several case reports seem to confirm the close relationship between NCCM and juvenile stroke.…”

Section: Noncompaction Cardiomyopathy Per Sementioning

confidence: 99%

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy

et al. 2021

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Noncompaction cardiomyopathy (NCCM) is a rare condition characterized by prominent trabeculae, deep intertrabecular recesses, and a left ventricular myocardium with a two-layered structure, characterized by a spongy endocardial layer and a thinner and compacted epicardial one. NCCM can be isolated or associated with other congenital heart diseases and complex syndromes involving neuromuscular disorders and facial dysmorphisms. To date, more than 40 genes coding for sarcomeric, cytoskeletal, ion channels, and desmosomal proteins have been identified. Clinical presentation is also highly variable, ranging from no symptoms to end-stage heart failure (HF), lethal arrhythmias, sudden cardiac death, or thromboembolic events. In particular, the prevalence of thromboembolism in NCCM patients appears to be higher than that of a similar, age-matched population without NCCM. Thromboembolism has a multifactorial aetiology, which is linked to genetic, as well as traditional cardiovascular risk factors. In previous studies, atrial fibrillation (AF) was observed in approximately 25-30% of adult NCCM patients and embolism had a cardiac source in ~63-69% of cases; therefore, AF represents a strong predictor of adverse events, especially if associated to HF and neuromuscular disorders. Left ventricular dysfunction is another risk factor for thromboembolism, as a result of blood stagnation and local myocardial injury. Moreover, it is not completely clarified if the presence of deep intertrabecular recesses causing stagnant blood flow can constitute per se a thrombogenic substrate even in absence of ventricular dysfunction. For the clinical management of NCCM patients, an appropriate stratification of the thromboembolic risk is of utmost importance for a timely initiation of anticoagulant therapy. The aim of the present study is to review the available literature on NCCM with particular attention on thromboembolic risk stratification and prevention and the current evidence for oral anticoagulation therapy. The use of direct oral anticoagulants vs. vitamin K antagonists is also discussed with important implications for patient treatment and prognosis.

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Left ventricular non-compaction as a potential source for cryptogenic ischemic stroke in the young: A case-control study

Cited by 13 publications

References 33 publications

An Unusual Presentation of Left Ventricular Non-compaction Cardiomyopathy in an Elderly Patient

An Unusual Presentation of Left Ventricular Non-compaction Cardiomyopathy in an Elderly Patient

Right atrium and cryptogenic ischaemic stroke in the young: a case–control study

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy

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