(21 - Friday) Global recovery, rehabilitation, and Berlin Excor decannulation in permanent junctional reciprocating tachycardia-induced cardiomyopathy with cardiogenic shock

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Abstract:

Introduction:
Permanent junctional reciprocating tachycardia (PJRT) is a rare pediatric tachycardia, associated with cardiomyopathy in up to 20% of patients. Many patients require electrophysiologic catheter ablation due to medication treatment failure. Such patients may require mechanical circulatory support or transplantation. We present a 4-year-old female presenting in cardiogenic shock due to newly diagnosed PJRT, requiring cannulation for mechanical circulatory support and later decannulated after recovery.

Case Description:
A 4-year-old female with asthma presented to the emergency department with dyspnea and severe cardiomegaly on chest radiograph. Electrocardiogram demonstrated PJRT (HR 195 bpm), and echocardiogram revealed severe left ventricular dysfunction (ejection fraction < 10%). Following multidisciplinary discussion, the patient was intubated in the operating room, with multiple support teams on standby. She underwent successful emergent catheter ablation of the PJRT pathway. Poor ventricular function and ventricular ectopy led to temporary left ventricular assist device (VAD) placement, followed by placement of a Berlin Heart 25 ml Excor left VAD. She was listed for cardiac transplantation.
She underwent extensive cardiac rehabilitation over her course. She did not have PJRT recurrence, and ventricular ectopy significantly decreased. She experienced a self-resolved transient ischemic attack 3 weeks prior to decannulation, requiring pump exchange.
With functional and cardiac recovery, VAD decannulation was considered. Criteria included normalized left ventricular end-diastolic diameter, left ventricular ejection fraction, aortic valve opening every beat, trivial atrioventricular valve regurgitation, and sinus rhythm. She underwent weaning studies, modified from Berlin Heart and ACTION resources (Table).
With favorable data, the patient was successfully decannulated 147 days after VAD placement. She was discharged one week later, with normalized left ventricular ejection fraction and left ventricular size, on carvedilol, lisinopril, spironolactone, and aspirin.
Multiple members of the cardiac team enhanced systemic recovery during her critical care course: bedside nursing, dietary, occupational/physical therapy, child life, and neurodevelopmental staff. During this 5-month course the patient did not experience a cardiac arrest or nosocomial infection, maintaining appropriate neurological milestones with sustained growth trajectory.

Discussion:
Berlin heart decannulation in the pediatric population is an infrequent occurrence. Mechanical support provided global recovery, with transition to an outpatient therapy regimen. An integrated multidisciplinary framework was essential to guide this patient through this critical care course. Continued accumulation of experiences is necessary to determine candidacy for Berlin Heart decannulation, relating to patient criteria and timing, weaning protocols, and therapy after decannulation.