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Related Concept Videos

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...
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...
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,...
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...
Heart Failure V: Medical Management01:30

Heart Failure V: Medical Management

Medical Management of Acute Decompensated Heart Failure (ADHF)The primary goals of therapy for patients hospitalized with acute decompensated heart failure (ADHF) include:Relieving symptomsOptimizing volume statusSupporting oxygenation and ventilationMaintaining cardiac output (CO) and end-organ perfusionIdentifying and addressing the cause of ADHFPreventing complicationsProviding patient education on factors precipitating HF exacerbationPlanning for dischargeOngoing monitoring and assessment...
Cardiomyopathy VI: Nursing Management01:29

Cardiomyopathy VI: Nursing Management

Assessment: Nursing management of patients with cardiomyopathy begins with a thorough assessment of the patient's history, including a family history of cardiomyopathy or sudden cardiac death, personal history of heart disease, hypertension, diabetes, and any alcohol consumption or drug use.During the physical examination, assess vital signs, look for signs of heart failure (such as edema, jugular venous distention, and cyanosis), auscultate for abnormal heart sounds (like murmurs and gallops),...

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Left Atrial Ligation in the Avian Embryo as a Model for Altered Hemodynamic Loading During Early Vascular Development
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Hypoplastic left heart syndrome: current considerations and expectations.

Jeffrey A Feinstein1, D Woodrow Benson, Anne M Dubin

  • 1Department of Pediatrics, Stanford University School of Medicine, Lucile Salter Packard Children's Hospital, Palo Alto, California 94304, USA. jeff.feinstein@stanford.edu

Journal of the American College of Cardiology
|December 24, 2011
PubMed
Summary

Hypoplastic Left Heart Syndrome (HLHS) management has dramatically improved, with 70% of newborns now expected to reach adulthood. This paper details the current state of HLHS care across all stages.

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Neonatal Care

Background:

  • Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect with historically poor outcomes.
  • Significant advancements in diagnosis and treatment have transformed HLHS management and survival rates.
  • Current survival expectations indicate that approximately 70% of newborns with HLHS may reach adulthood.

Purpose of the Study:

  • To present the current state of the art in understanding and treating Hypoplastic Left Heart Syndrome (HLHS).
  • To outline the comprehensive management strategies across all stages of HLHS care.
  • To address critical aspects including genetics, developmental outcomes, and quality of life for HLHS patients.

Main Methods:

  • Review of current diagnostic approaches and management strategies for HLHS.
  • Detailed description of the multi-stage treatment protocol: pre-Stage I, Stage I, Stage II, and Stage III (Fontan surgery).
  • Inclusion of long-term follow-up considerations, genetics, developmental outcomes, and quality of life.

Main Results:

  • Survival rates to age 5 have improved, with expectations for 70% of newborns with HLHS to reach adulthood.
  • A well-established 3-stage treatment approach exists, though center-specific variations are noted.
  • Comprehensive care strategies encompass fetal assessment through long-term follow-up.

Conclusions:

  • Hypoplastic Left Heart Syndrome (HLHS) management has seen a dramatic positive shift in outcomes.
  • A standardized, multi-stage approach is crucial for optimizing HLHS patient care.
  • Holistic management, including genetics and quality of life, is essential for complex HLHS patients.