Surreal illustration of a child's heart in a surgeon's hands.

Univentricular Palliations: New Hope for Congenital Heart Patients?

Elliot Brynn in Health & Wellness December 2025 • 4 min read.

"Heart transplantation offers a solution for children with complex congenital heart defects that require univentricular palliation, but it comes with significant challenges."

Congenital heart defects (CHDs) are structural abnormalities present at birth, affecting the heart's normal function. In severe cases, where a child is born with a single functional ventricle (univentricular heart), a series of palliative surgeries known as univentricular palliation are performed. These procedures, such as the Fontan procedure, aim to improve blood flow and oxygenation but are not curative.

While univentricular palliation can extend a child's life and improve their quality of life, it is associated with long-term complications, including heart failure, liver dysfunction, and protein-losing enteropathy. In some cases, heart transplantation becomes the only viable option for survival.

Orthotopic heart transplantation, where a diseased heart is replaced with a healthy donor heart, presents a unique set of challenges in patients who have undergone previous univentricular palliation. The altered anatomy and prior surgeries complicate the transplantation procedure and require specialized expertise.

Heart Transplantation After Univentricular Palliation: Addressing the Challenges

Surreal illustration of a child's heart in a surgeon's hands.

A study published in the Journal of Cardiothoracic Surgery in 2015 investigated the outcomes of orthotopic heart transplantation in patients who had previously undergone univentricular palliation. The study, led by María-Teresa González-López and colleagues, reviewed the surgical techniques and results in a cohort of 23 congenital cardiac patients who underwent heart transplantation between 2013 and 2014.

Of the 23 patients, 13 had undergone previous univentricular palliation for conditions such as hypoplastic left heart syndrome, d-transposition of great arteries with criss-cross heart, unbalanced atrioventricular septal defect, pulmonary atresia with intact ventricular septum, and grown-up patients with either double-inlet-left-ventricle or tricuspid atresia. The researchers analyzed the surgical approaches, complications, and outcomes in these patients.

Surgical Techniques: The researchers employed various surgical techniques to address the challenges posed by the altered anatomy and prior surgeries. These included hemiarch repair, pulmonary artery (PA) branches plasty, hilum-to-hilum PA reconstruction, superior venae cavae reconstruction, stent removal from PA, and inferior venae cavae reconstruction.
Outcomes: The average cardiopulmonary-bypass time was 257.6 minutes, and the average total-ischemia-time was 220.7 minutes. One patient required ECMO (extracorporeal membrane oxygenation), a life-support machine that oxygenates the blood outside of the body. Four patients underwent delayed sternal closure, and two patients underwent diaphragm plication, a surgical procedure to correct diaphragmatic paralysis. One patient experienced subacute-humoral-rejection, a type of immune response that can damage the transplanted heart, and was treated with plasmapheresis, a procedure to remove antibodies from the blood. The in-hospital stay averaged 44 days.
Follow-Up: At a follow-up of 14.4 months, the freedom from percutaneous procedures, such as angioplasty or stenting, was 83.3%. There was no 30-day mortality or follow-up mortality. All patients remained with an optimal functional class, indicating that they were able to perform their daily activities without significant limitations.

The study's findings suggest that orthotopic heart transplantation following univentricular palliation is technically demanding but can yield excellent short-term results. The researchers emphasized the importance of extensive surgical reconstruction using donor or heterologous tissues to improve outcomes. They also noted that further follow-up is necessary to evaluate the long-term results in this patient population.

Hope and the Future

Heart transplantation following univentricular palliation offers hope for children with complex congenital heart defects. While the procedure is technically challenging, advancements in surgical techniques and immunosuppression have improved outcomes. Further research and long-term follow-up are needed to optimize the management of these patients and ensure the best possible quality of life.

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This article is based on research published under:

DOI-LINK: 10.1186/1749-8090-10-s1-a92, Alternate LINK

Title: Orthotopic Heart Transplantation Following Univentricular Palliation: New Challenges For The Congenital Cardiac Surgeon

Subject: Cardiology and Cardiovascular Medicine

Journal: Journal of Cardiothoracic Surgery

Publisher: Springer Science and Business Media LLC

Authors: María-Teresa González-López, Juan-Miguel Gil-Jaurena, Ramón Pérez-Caballero-Martínez, Ana-María Pita-Fernández, Manuela Camino-López, Nuria Gil-Villanueva, José-Luis Zunzunegui-Martínez, Constancio Medrano-López

Published: 2015-12-01

Everything You Need To Know

What are congenital heart defects?

Congenital heart defects (CHDs) are structural abnormalities of the heart present at birth. These defects can interfere with the heart's ability to function properly. In severe cases, like when a child has only one functional ventricle, univentricular palliation is performed to improve blood flow. These palliative surgeries aim to enhance the patient's quality of life. However, these procedures are not curative and can lead to long-term complications.

What is univentricular palliation?

Univentricular palliation is a series of surgical procedures, like the Fontan procedure, performed on children born with a single functional ventricle. These procedures aim to improve blood flow and oxygenation throughout the body. While univentricular palliation can extend a child's life and improve their quality of life, it is not a cure and is associated with long-term complications such as heart failure and liver dysfunction. These complications can impact the child's overall health and well-being.

What does orthotopic heart transplantation involve?

Orthotopic heart transplantation is a surgical procedure where a diseased heart is replaced with a healthy donor heart. In the context of patients who have undergone univentricular palliation, orthotopic heart transplantation presents unique challenges due to the altered anatomy and prior surgeries. This makes the transplantation procedure more complex and requires specialized surgical expertise. However, it can be a viable option for survival when univentricular palliation is no longer sufficient.

What outcomes are monitored after a heart transplant?

After orthotopic heart transplantation, several outcomes are monitored to assess the success of the procedure. These include cardiopulmonary-bypass time, total-ischemia-time, and potential complications like the need for ECMO (extracorporeal membrane oxygenation), delayed sternal closure, diaphragm plication, and subacute-humoral-rejection. Follow-up assessments also track the patient's functional class and freedom from percutaneous procedures, such as angioplasty or stenting. Monitoring these outcomes helps to ensure the transplanted heart is functioning optimally and the patient is recovering well.

What surgical techniques are used in heart transplants after univentricular palliation?

Surgical techniques employed during orthotopic heart transplantation following univentricular palliation include hemiarch repair, pulmonary artery (PA) branches plasty, hilum-to-hilum PA reconstruction, superior venae cavae reconstruction, stent removal from PA, and inferior venae cavae reconstruction. These techniques are crucial for addressing the challenges posed by the altered anatomy and prior surgeries, ensuring the successful integration of the donor heart.