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Updated: Feb 20, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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ヌーナン症候群 複数のレンチゴインを模倣したノーナン症候群 阻害性多発性心筋症候群 Septal Myectomyで治療

Gabrielle Batista Moreira1, Thaís Baptista Teixeira1, Ana Paula de Morais Rodrigues Barros1

  • 1Instituto do Coração (InCor), Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.

JACC. Case reports
|February 19, 2026
PubMed
まとめ

外科的なセプトミエクトミーは,医療療法が失敗すると,多発性レンチゴイン症候群 (NSML) と阻害性多動性心筋病 (HCM) のヌーナン症候群の患者に安全で効果的な治療法を提供します. この手順は,症状を大幅に改善し,流出管の阻害を軽減することができます.

キーワード:
ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは,ヌーナン症候群 (Noonan syndrome) とは阻害性高縮性心筋症候群 阻害性高縮性心筋症候群セプタルミエクトマイトミー

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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Cardiomyopathy VII: Pre and Post Operative Nursing Management01:28

Cardiomyopathy VII: Pre and Post Operative Nursing Management

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...
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Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
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Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
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