The Heart Function, Transplant and Ventricular Assist Device Program at Children’s Hospital of Philadelphia (CHOP) is redefining what’s possible for children with advanced heart failure. By combining next-generation ventricular assist devices with structured ventricular recovery strategies and multidisciplinary expertise, the team is advancing new pathways beyond transplant — including the potential for meaningful myocardial recovery. The following case highlights how CHOP’s innovative, recovery-focused approach is transforming outcomes for even the youngest and most critically ill patients.
The case
A previously healthy 3-month-old female presented with progressive feeding intolerance, respiratory distress and signs of cardiogenic shock. An initial evaluation revealed severe dilated cardiomyopathy with profoundly depressed left ventricular systolic function. The electrocardiogram demonstrated a left bundle branch block (LBBB) with a markedly prolonged QRS duration. The echocardiography showed severe left ventricular dilation, global systolic dysfunction and paradoxical septal motion consistent with mechanical dyssynchrony.
Diagnostic findings:
- Echocardiography: Severely dilated left ventricle with biplane ejection fraction of 11% and marked septal dyskinesis
- Electrocardiogram: Left bundle branch block with QRS duration of 144 ms
- Etiologic evaluation: Genetic and viral testing were unrevealing; coronary anatomy was normal
- Hemodynamics: Consistent with low-output heart failure without fixed pulmonary hypertension
Medical therapy was escalated, but the patient developed worsening heart failure and required intubation and inotropic support. Given her ongoing clinical deterioration, the patient was referred to CHOP’s Heart Function, Transplant and Ventricular Assist Device Program.
Clinical course and recovery-focused management
Given her refractory heart failure, the patient underwent implantation of a paracorporeal left ventricular assist device (Berlin Heart EXCOR®) as a bridge to decision, with the explicit goal of myocardial recovery.
Following mechanical unloading, she was rapidly stabilized, extubated and weaned from inotropic support. A structured ventricular recovery strategy was implemented, including maximally tolerated guideline-directed medical therapy, aggressive nutritional rehabilitation and early physical and developmental therapy. Over several months of support, the patient demonstrated steady somatic growth and progressive improvement in ventricular systolic function.
Serial recovery assessments – including echocardiography, hemodynamic testing during LVAD turn-down trials, and invasive catheterization – demonstrated normalization of cardiac output and filling pressures with improvement of left ventricular ejection fraction to >50%. However, despite favorable hemodynamics, persistent electrical and mechanical dyssynchrony related to LBBB was noted, with ongoing septal dyskinesis raising concern for incomplete myocardial recovery and durability following device explantation.
In the context of this recovery plateau, the team identified cardiac dyssynchrony as a potentially modifiable barrier to durable recovery. As part of the recovery program’s individualized optimization strategy, epicardial biventricular pacing was incorporated at the time of LVAD explantation to address residual dyssynchrony and support long-term ventricular remodeling.
Outcome
Following LVAD explantation and the initiation of resynchronization therapy, the patient demonstrated normalization of left ventricular systolic function with resolution of septal dyskinesis and marked narrowing of the QRS complex. She was discharged home without recurrent heart failure symptoms and has remained clinically stable as an outpatient with sustained ventricular function at long-term follow-up.
The CHOP difference
This case illustrates how intentional, protocolized ventricular recovery strategies can enable successful explantation of mechanical circulatory support even in infants with severe cardiomyopathy. Mechanical unloading alone may be insufficient to achieve durable recovery; recovery often requires the systematic identification and treatment of residual barriers such as electrical dyssynchrony.
While cardiac resynchronization therapy served as an important adjunct in this patient, its role was embedded within a broader recovery framework emphasizing serial physiologic assessment, multidisciplinary decision-making and individualized optimization strategies. A second infant treated within the same recovery program demonstrated a similar course, reinforcing the reproducibility of this approach.
Key takeaways
- Ventricular recovery in pediatric patients supported with mechanical circulatory support is an active, intentional process, not a passive outcome.
- Serial physiologic assessment is essential to identify modifiable barriers to durable myocardial recovery.
- Adjunctive therapies, including cardiac resynchronization, may play a role in select patients when recovery plateaus.
- Programmatic recovery strategies can expand opportunities for LVAD explantation and reduce reliance on transplantation in carefully selected pediatric patients.
Jon Edelson, MD, is the Associate Medical Director of the Heart Function, Transplant and Ventricular Assist Device Program at Children’s Hospital of Philadelphia.
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The Heart Function, Transplant and Ventricular Assist Device Program at Children’s Hospital of Philadelphia (CHOP) is redefining what’s possible for children with advanced heart failure. By combining next-generation ventricular assist devices with structured ventricular recovery strategies and multidisciplinary expertise, the team is advancing new pathways beyond transplant — including the potential for meaningful myocardial recovery. The following case highlights how CHOP’s innovative, recovery-focused approach is transforming outcomes for even the youngest and most critically ill patients.
The case
A previously healthy 3-month-old female presented with progressive feeding intolerance, respiratory distress and signs of cardiogenic shock. An initial evaluation revealed severe dilated cardiomyopathy with profoundly depressed left ventricular systolic function. The electrocardiogram demonstrated a left bundle branch block (LBBB) with a markedly prolonged QRS duration. The echocardiography showed severe left ventricular dilation, global systolic dysfunction and paradoxical septal motion consistent with mechanical dyssynchrony.
Diagnostic findings:
- Echocardiography: Severely dilated left ventricle with biplane ejection fraction of 11% and marked septal dyskinesis
- Electrocardiogram: Left bundle branch block with QRS duration of 144 ms
- Etiologic evaluation: Genetic and viral testing were unrevealing; coronary anatomy was normal
- Hemodynamics: Consistent with low-output heart failure without fixed pulmonary hypertension
Medical therapy was escalated, but the patient developed worsening heart failure and required intubation and inotropic support. Given her ongoing clinical deterioration, the patient was referred to CHOP’s Heart Function, Transplant and Ventricular Assist Device Program.
Clinical course and recovery-focused management
Given her refractory heart failure, the patient underwent implantation of a paracorporeal left ventricular assist device (Berlin Heart EXCOR®) as a bridge to decision, with the explicit goal of myocardial recovery.
Following mechanical unloading, she was rapidly stabilized, extubated and weaned from inotropic support. A structured ventricular recovery strategy was implemented, including maximally tolerated guideline-directed medical therapy, aggressive nutritional rehabilitation and early physical and developmental therapy. Over several months of support, the patient demonstrated steady somatic growth and progressive improvement in ventricular systolic function.
Serial recovery assessments – including echocardiography, hemodynamic testing during LVAD turn-down trials, and invasive catheterization – demonstrated normalization of cardiac output and filling pressures with improvement of left ventricular ejection fraction to >50%. However, despite favorable hemodynamics, persistent electrical and mechanical dyssynchrony related to LBBB was noted, with ongoing septal dyskinesis raising concern for incomplete myocardial recovery and durability following device explantation.
In the context of this recovery plateau, the team identified cardiac dyssynchrony as a potentially modifiable barrier to durable recovery. As part of the recovery program’s individualized optimization strategy, epicardial biventricular pacing was incorporated at the time of LVAD explantation to address residual dyssynchrony and support long-term ventricular remodeling.
Outcome
Following LVAD explantation and the initiation of resynchronization therapy, the patient demonstrated normalization of left ventricular systolic function with resolution of septal dyskinesis and marked narrowing of the QRS complex. She was discharged home without recurrent heart failure symptoms and has remained clinically stable as an outpatient with sustained ventricular function at long-term follow-up.
The CHOP difference
This case illustrates how intentional, protocolized ventricular recovery strategies can enable successful explantation of mechanical circulatory support even in infants with severe cardiomyopathy. Mechanical unloading alone may be insufficient to achieve durable recovery; recovery often requires the systematic identification and treatment of residual barriers such as electrical dyssynchrony.
While cardiac resynchronization therapy served as an important adjunct in this patient, its role was embedded within a broader recovery framework emphasizing serial physiologic assessment, multidisciplinary decision-making and individualized optimization strategies. A second infant treated within the same recovery program demonstrated a similar course, reinforcing the reproducibility of this approach.
Key takeaways
- Ventricular recovery in pediatric patients supported with mechanical circulatory support is an active, intentional process, not a passive outcome.
- Serial physiologic assessment is essential to identify modifiable barriers to durable myocardial recovery.
- Adjunctive therapies, including cardiac resynchronization, may play a role in select patients when recovery plateaus.
- Programmatic recovery strategies can expand opportunities for LVAD explantation and reduce reliance on transplantation in carefully selected pediatric patients.
Jon Edelson, MD, is the Associate Medical Director of the Heart Function, Transplant and Ventricular Assist Device Program at Children’s Hospital of Philadelphia.
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