A one-year-old boy with Barth syndrome and severe biventricular heart failure was successfully bridged to heart transplantation using a strategy of emergency biventricular assist device support with strategic right ventricular assist device outflow redirection, followed by conversion to Berlin Heart EXCOR.
Key Findings
Background
Emergency biventricular assist device (BiVAD) support using centrifugal pumps was successfully initiated in a one-year-old boy with Barth syndrome presenting with cardiogenic shock due to severe biventricular heart failure.
The patient was one year old at the time of BiVAD implantation.
The underlying diagnosis was Barth syndrome, a rare X-linked condition associated with cardiomyopathy.
Presentation included cardiogenic shock due to severe biventricular heart failure.
Centrifugal pumps were used for the initial biventricular assist device support.
Results
Under conditions of severe pulmonary hypertension and pulmonary regurgitation, the right ventricular assist device (RVAD) outflow was temporarily redirected to the left atrium to optimize left ventricular preload.
The redirection was necessitated by severe pulmonary hypertension and pulmonary regurgitation present at the time of implantation.
Directing RVAD outflow to the left atrium was used as a strategy to optimize left ventricular assist device (LVAD) performance.
This approach bypassed the pulmonary circulation while pulmonary hemodynamics were unfavorable.
Results
As pulmonary hemodynamics improved, the RVAD outflow was redirected from the left atrium to the pulmonary artery, enabling evaluation of native pulmonary circulation and subsequent RVAD weaning.
The sequential change in RVAD outflow destination allowed for reassessment of native right ventricular and pulmonary vascular function.
RVAD weaning was achieved after successful redirection to the pulmonary artery.
This strategy served as a bridge-to-candidacy by demonstrating recovery of pulmonary hemodynamics.
Results
The LVAD was converted to a Berlin Heart EXCOR 76 days after initial BiVAD implantation, following recovery of end-organ function.
Conversion occurred at 76 days post-BiVAD implantation.
End-organ function recovery was a prerequisite for conversion to the Berlin Heart EXCOR.
The Berlin Heart EXCOR is a paracorporeal pulsatile ventricular assist device designed for pediatric patients.
Results
The patient remained stable on Berlin Heart EXCOR support for 374 days and subsequently underwent successful heart transplantation.
Duration of Berlin Heart EXCOR support was 374 days.
The patient was successfully bridged to heart transplantation after this period.
Total mechanical circulatory support duration was 76 days on BiVAD plus 374 days on Berlin Heart EXCOR before transplantation.
The case demonstrates feasibility of prolonged mechanical circulatory support as a bridge to transplantation in a high-risk pediatric patient.
Discussion
Early RVAD support and strategic management of RVAD outflow were identified as important factors for optimizing LVAD performance in pediatric patients with severe pulmonary hypertension.
The authors highlight that early right ventricular assist device support is critical in biventricular failure with pulmonary hypertension.
Strategic RVAD outflow redirection (left atrium vs. pulmonary artery) was used to manage pulmonary hemodynamics.
This approach was described as potentially facilitating successful bridge-to-candidacy and eventual heart transplantation in high-risk pediatric patients.
Long waiting times for donor organs in pediatric patients were cited as increasing the need for mechanical circulatory support strategies.
What This Means
This research describes the case of a one-year-old boy with Barth syndrome, a rare genetic condition that severely weakens the heart, who went into cardiogenic shock due to failure of both sides of his heart. Because pediatric heart transplants often involve very long waits for a donor organ, the medical team used mechanical heart pumps — one for each ventricle — to keep him alive. A key challenge was that the child also had high blood pressure in his lungs (pulmonary hypertension), which made it difficult for the pumps to work effectively together. The team temporarily redirected the right-sided pump's outflow away from the lungs and into the left side of the heart to stabilize him, then gradually restored normal flow as the lung pressures improved.
After 76 days on the initial pump system and recovery of organ function, the team transitioned the child to a Berlin Heart EXCOR, a specialized portable heart pump designed for children that allowed greater mobility and stability. The child remained on this device for 374 days — over a year — before successfully receiving a heart transplant. This case suggests that creative, stepwise management of mechanical heart pumps, including redirecting blood flow to work around high lung pressures, can keep critically ill infants alive long enough to become eligible for and receive a heart transplant.
This research matters because it demonstrates a practical strategy for managing one of the most difficult scenarios in pediatric heart failure: a very young child with failure of both heart chambers combined with high lung pressures. The approach of temporarily bypassing the pulmonary circulation and then gradually restoring it may offer a roadmap for other centers treating similar high-risk pediatric patients who face lengthy waits for donor hearts.
Arita K, Kido T, Taira M, Watanabe T, Narita J, Ishida H, et al.. (2026). Successful bridge to heart transplantation in a pediatric patient with biventricular heart failure associated with Barth syndrome: a case report.. Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs. https://doi.org/10.1007/s10047-026-01587-2