Arrhythmias in Left Ventricular Noncompaction

Miyake, Christina Y.; Kim, Jeffrey

doi:10.1016/j.ccep.2015.03.007

Cited by 37 publications

(29 citation statements)

References 73 publications

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“…[190] Although cardiac arrhythmias play an important role in the morbidity and mortality of PAH patients, [192] little is known about the arrhythmia phenotypes in the RV, especially compared to existing knowledge of the nature and structure of arrhythmias in LV dysfunction and disease. [125,[193][194][195][196][197] Right-sided cardiac remodeling in response to chronic pressure and volume overload produce an arrhythmogenic substrate in patients with PAH. Factors contributing to arrhythmogenic remodeling include, modulation in autonomic activity, delayed cardiac repolarization and RV myocardial ischemia.…”

Section: Relaxin As a Therapy For Pahmentioning

confidence: 99%

Cardioprotective actions of relaxin

Martin

Romero

Salama

2019

Molecular and Cellular Endocrinology

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Relaxin, a hormone of pregnancy, has shown broad cardioprotective effects including anti-fibrotic (reversed excess TGFβ signaling), anti-arrhythmic (Nav1.5 and INa upregulation and Cx43 phosphorylation and trafficking to intercalated disks) and anti-inflammatory properties (reduced IL-1β and IL-6). While relaxin's anti-fibrotic effects are thought to occur through the SMAD2/3/TGFβ pathway, there is a general lack of understanding of relaxin's mode of action to increase sodium current and alter connexin43 localization to suppress arrhythmias. Based on a rat model of aging, we tested the hypothesis that relaxin acts through activation of Wnt/βcatenin signaling to mediate its effects on Nav1.5 and Cx43 and to regulate collagen expression. We show for the first time that relaxin activates canonical Wnt signaling (increased nuclear βcatenin) to increase Nav1.5 in isolated cardiomyocytes. Block of canonical Wnt signaling (via Dikkopf-1) abrogated relaxin's effect on both Nav1.5 in cardiomyocytes and suppression of excess collagen from fibroblasts. Further, we show that relaxin significantly suppressed expression of Dikkopf-1 in isolated cardiomyocytes and whole LV tissue. In addition, show that relaxin suppressed the age-associated genetic upregulation in inflammatory markers in a sexdependent manner. Finally, we tested the hypothesis that relaxin would be an effective therapy in a rat model of pulmonary arterial hypertension and show that it reduced occlusion of small pulmonary arteries, myocardial and lung fibrosis and reversed the cardiac phenotype of ventricular arrhythmia or arrest. These results suggest that relaxin signals through multiple

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Section: Relaxin As a Therapy For Pahmentioning

confidence: 99%

Cardioprotective actions of relaxin

Martin

Romero

Salama

2019

Molecular and Cellular Endocrinology

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“…La presentación clínica va desde asintomático 15 , falla cardiaca, arritmias 16 , eventos tromboembólicos y muerte súbita. Nuestro paciente se presentó con falla cardiaca más taquicardia supraventricular.…”

Section: Discussionunclassified

“…La radiografía de tórax muestra cardiomegalia e hiperflujo pulmonar, no específica de esta patología. El estudio EKG muestra signos de crecimiento ventricular izquierdo, prolongación del segmento QT, datos que también son reportados en la literatura 16 . La herramienta diagnóstica clave es la ecocardiografía, sin embargo, se debe considerar que ningún parámetro es Gold Estándar ya que sólo valoran parámetros morfológicos.…”

Section: Discussionunclassified

Miocardiopatía no compactada asociada a defectos cardíacos congénitos.

Catalán

Carril²,

Loo³

et al. 2019

RFMH

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El presente caso corresponde a un paciente de un mes de vida, sexo masculino con diagnóstico de miocardiopatía no compactada asociada a defectos cardiacos congénitos. La miocardiopatía no compactada recién es incluida por la American Heart Association (AHA) como entidad propia a partir de la segunda mitad de la década pasada. El diagnóstico principalmente es ecocardiográfico. La sintomatología en menores de un año puede empezar con falla cardiaca. La evolución es variable con tendencia a la mejoría en algunos casos para finalmente en décadas posteriores se hace más pronunciada la falla cardiaca, eventos tromboembólicos, arritmias malignas y muerte súbita. El manejo esta en medicamentos para falla cardiaca, evitar arritmias malignas y eventos tromboembólicos. Palabras clave: Miocardiopatía no compactada; Defectos cardiacos congénitos; Falla cardiaca; Ecocardiografía.

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“…This suggests the possible importance of primary or secondary prevention by ICD implantation. There are no specific guidelines for primary prevention in patients with LVNC; however, current guidelines regarding other types of cardiomyopathies can be extrapolated depending on the phenotype of LVNC (21). Our patient was a candidate for ICD implantation due to recurrent syncope and severe systolic dysfunction.…”

Section: Clinical Presentationmentioning

confidence: 98%

Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review

et al. 2020

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Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct "non-compacted" layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium. Clinical manifestations vary from asymptomatic to heart failure (HF), arrhythmias, or thromboembolic events. We present a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4years-old male patient, diagnosed at 3 months with ILVNC, and NYHA functional class IV HF, was admitted to the Emergency Institute for Cardiovascular Diseases and Transplantation of Targu Mures, Romania, for cardiologic reevaluation, and diagnosis confirmation. ILVNC was confirmed using echocardiography, revealing a non-compaction to compaction (NC/C) ratio of > 2.7. His evolution was stationary until the age of 8 years, when severe pneumonia caused hemodynamic decompensation, and he was listed for heart transplantation (HT). The patient underwent HT at the age of 11 years with favorable postoperative outcome. Meanwhile, a 22-years-old female patient, mother of the aforementioned patient, was also admitted to our institute due to severe fatigue, dyspnea, and recurrent palpitations with multiple implantable cardioverter defibrillator (ICD) shock delivery. Extensive medical history revealed that a presumptive ILVNC diagnosis was established when she was 11 years old. She was asymptomatic until 18 years old, when 3 months post-partum, she developed NYHA functional class III HF, and subsequently underwent ICD implantation. Her diagnosis was confirmed using multi-detector computed tomography angiography, which revealed a NC/C ratio of > 3.3. ICD adjustments were carried out with a favorable evolution under chronic drug therapy. The last evaluation, at 27 years old, revealed that she was in NYHA functional class II HF. In conclusion, ILVNC, even when familial, can present different clinical pictures and therefore requires different medical approaches.

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Arrhythmias in Left Ventricular Noncompaction

Cited by 37 publications

References 73 publications

Cardioprotective actions of relaxin

Cardioprotective actions of relaxin

Miocardiopatía no compactada asociada a defectos cardíacos congénitos.

Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review

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