Can a heart transplant be considered in an infant with left ventricular noncompaction?

Heart Transplantation for Infants with Left Ventricular Noncompaction

Heart transplantation is an appropriate and potentially life-saving intervention for infants with left ventricular noncompaction (LVNC) who develop end-stage heart failure, with evidence suggesting it can significantly improve survival despite worse outcomes compared to other cardiomyopathies. 1, 2

Indications for Heart Transplant in LVNC

• Cardiomyopathy, including LVNC, is a primary indication for heart transplantation in infants and children 3
• Specific indications for transplant evaluation in LVNC infants include:
  • Progressive heart failure despite maximal medical therapy (diuretics, ACE inhibitors) 3
  • Growth failure or cardiac cachexia 3
  • Reduced left ventricular ejection fraction (major predictor of poor outcomes) 1
  • Complex ventricular arrhythmias (present in ~40% of children with LVNC) 1

Outcomes and Prognosis

• Long-term outcomes for LVNC with dilated phenotype (most common in infants) show:

  • Freedom from death or transplantation of 48% at 10 years and 45% at 15 years 2
  • Worse prognosis compared to matched children with dilated cardiomyopathy (hazard ratio 2.3 for death or transplantation) 2
  • Only 21% of LVNC patients are alive with normal left ventricular function at 15 years 2

• Heart transplantation can successfully treat LVNC and modify its natural history:

  • Case reports demonstrate successful transplantation in patients with isolated LVNC 4, 5
  • Survival rates for pediatric heart transplant recipients with cardiomyopathy are generally favorable, with some studies showing 100% survival in this group 6

Evaluation Process for Transplant Candidacy

• Comprehensive cardiac evaluation including:

  • Echocardiography to confirm diagnosis (ratio of non-compacted to compacted layer >2) 1
  • Assessment of ventricular function and presence of thrombi 1
  • ECG to detect arrhythmias 1
  • Evaluation for pulmonary hypertension (a potential contraindication) 6

• Exclusion criteria specific to infants:

  • Significant irreversible hepatic or renal dysfunction 3
  • Active infection 3
  • Inadequate pulmonary artery diameter at the hila 3
  • Systemic diseases such as degenerative neuromuscular diseases 3

Special Considerations for Infants

• Family/caregiver evaluation is crucial:

  • Identification of a competent caregiver with legal responsibility 3
  • Assessment of family support system 3
  • Evaluation of potential compliance with post-transplant regimen 3

• Surgical considerations:

  • Complex congenital heart lesions may require creative surgical approaches 3
  • Virtually any anatomic aberration can be addressed during transplantation except inadequate pulmonary arteries 3
  • Fetal diagnosis may allow for coordinated delivery with donor procurement in severe cases 3

Post-Transplant Management

• Regular cardiac follow-up to monitor:

  • Ventricular function 1
  • Development of arrhythmias 1
  • Thromboembolic risk 1
  • Signs of rejection 5

• Immunosuppression management with special attention to compliance in the pediatric population 3

Pitfalls and Caveats

• Pulmonary hypertension is a significant risk factor for operative mortality in pediatric heart transplantation 6
• Rejection and infection remain important causes of late mortality 6
• LVNC with dilated phenotype has worse outcomes than isolated dilated cardiomyopathy, requiring closer monitoring 2
• Careful assessment for potentially reversible conditions contributing to heart failure decompensation should be performed before proceeding to transplantation 3

Heart transplantation represents a definitive therapy for infants with LVNC who develop end-stage heart failure, offering the possibility of long-term survival despite the challenging nature of this congenital cardiomyopathy.