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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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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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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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Updated: Sep 19, 2025

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Long-Term Survival and Patient-Reported Outcomes After Staged Reconstructive Surgery for Hypoplastic Left Heart

J William Gaynor1, Marlene Mahle1, Richard F Ittenbach2

  • 1Division of Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Philadelphia, and the Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Journal of the American College of Cardiology
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PubMed
Summary

Fewer than one-third of hypoplastic left heart syndrome (HLHS) patients survive 35 years without a transplant. While survival hasn't improved recently, some survivors report excellent long-term quality of life.

Keywords:
Norwood procedureadulthoodhybrid procedurehypoplastic left heart syndromequality of lifesurvival

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Research
  • Surgical Outcomes Analysis

Background:

  • Hypoplastic Left Heart Syndrome (HLHS) survival has improved but remains lower than other congenital heart defects.
  • Long-term outcome data for HLHS patients are limited.

Purpose of the Study:

  • To assess long-term survival rates in HLHS patients.
  • To identify patient factors influencing HLHS survival.
  • To evaluate functional status and quality of life (QOL) in adulthood.

Main Methods:

  • Analysis of 2,012 neonates with HLHS undergoing Norwood or hybrid procedures (1984-2023).
  • Data abstracted from medical records; vital status confirmed via multiple sources.
  • Adult functional outcomes and QOL assessed via patient self-report.

Main Results:

  • 31.0% transplant-free survival at 35 years for HLHS patients.
  • Survival rates have not significantly improved in recent eras.
  • A subset of survivors reported good to excellent general health and QOL.

Conclusions:

  • Less than one-third of HLHS patients survive to 35 years without cardiac transplantation.
  • Long-term survival for HLHS has plateaued, with no recent improvements.
  • A "high-performing" Fontan phenotype is observed in some survivors, reporting good QOL.