At Brussels University Hospital (H.U.B.), this heart transplant in a patient with AL amyloidosis marks a major step forward in the treatment of the disease. Despite achieving a complete haematological response to treatment, the patient’s cardiac damage had become irreversible. Consequently, a heart transplant represented the best treatment option, offering the prospect of improved long-term survival and recovery.
Advances in treatment and a multidisciplinary approach have enabled the H.U.B. to perform its first heart transplant in a patient whose AL amyloidosis had been diagnosed and treated before the procedure. This achievement opens up new possibilities for patients living with this rare and serious disease.
A rare disease that can severely damage the heart
AL amyloidosis is a rare haematological disease associated with the production of abnormal proteins known as light chains. These proteins are produced by certain bone marrow cells called plasma cells. They accumulate in organs in the form of amyloid deposits, progressively impairing their function. The heart and kidneys are particularly affected.
When these deposits accumulate in the heart muscle, the heart becomes stiffer and has difficulty filling with blood. The disease can therefore lead to severe, potentially irreversible heart failure.
For many years, the prognosis was particularly poor when the heart was severely affected. Today, however, innovative treatments for AL amyloidosis offer better control over the production of the proteins responsible for the disease.
A complex, multidisciplinary treatment strategy
In this patient’s case, treatment had achieved a complete haematological response, with no detectable residual disease. However, the damage to the heart was too severe for it to regain sufficient function, despite the administration of standard heart failure treatments.
A heart transplant therefore represented an important treatment option, although it presented a particular challenge: controlling the underlying disease, which is essential to limiting the risk of amyloid deposits recurring in the transplanted heart.
Rigorous patient selection was essential to ensure that no other organs were seriously affected. Close collaboration between the teams specialising in haematology, advanced heart failure, cardiac surgery, anaesthesia and intensive care was vital. The teams also benefited from discussions with cardiac amyloidosis specialists at Henri Mondor Hospital in France.
This case illustrates the importance of a multidisciplinary approach and the new possibilities offered by the latest advances in treatment. When the disease is under control but the damage to the heart is irreversible, a transplant can be an option for carefully selected patients.
Dr Ornella Rizzo, a specialist in plasma cell disorders in the H.U.B.’s Department of Haematology.
An encouraging recovery and new perspectives
Just a few days after surgery, the patient was able to return home. His post-operative recovery has been encouraging. He no longer experiences cardiorespiratory symptoms during the physical activities he is currently able to undertake and continues to recover under close medical supervision.
In addition to restoring cardiac function, the transplant aims to reduce hospital admissions for heart failure and enable the patient to gradually return to a more normal daily life, both personally and within his family and social environment.
A heart transplant aims to improve survival, reduce hospital admissions and enable patients to enjoy a better quality of life. In the specific context of AL amyloidosis, the immunosuppressive treatment designed to prevent rejection of the transplanted heart must be tailored to each patient’s individual circumstances
Professor Jean-Luc Vachiéry, Head of the Clinic for Pulmonary Vascular Diseases and Heart Failure in the H.U.B.’s Department of Cardiology at Erasmus Hospital.
A heart transplant does not, however, cure AL amyloidosis. Regular haematological and cardiological follow-up remains essential to monitor the disease and the functioning of the transplanted heart.
This first at the H.U.B. demonstrates how medical advances and collaboration across specialties can offer new treatment options for patients facing particularly complex medical situations.
