Another success of our pediatric cardiologists in UCC MUG's hospital

4.02.2019

Team of specialists of the MUG’s Department of Paediatric Cardiology and Congenital Heart Defects performed implantation of an atrial flow regulator, a procedure done first time ever in Poland and one of few performed worldwide. The 11-year-old patient was qualified for the procedure by a group of international experts.

The patient, Martyna, suffers from restrictive cardiomyopathy. This heart disease is extremely rare in children and is sometimes referred to as “stiff heart syndrome”. The cardiac muscle does not function properly, with impaired diastolic function leading to congestion of blood in various parts of the body and extreme insufficiency of many organs. Prognoses are not optimistic. In the course of the disease the girl developed an increase in left atrial pressure with resulting secondary pulmonary hypertension. This, in turn, became a contraindication to heart transplantation, which in most such cases is the only chance to prolong life.

The procedure consisted in puncturing of the atrial septum and thus creating communication between the atria. In order to maintain constant interatrial flow, an implant composed of two nitinol discs (photo) was fixed at the puncture site. As a result of the procedure the left atrial pressure was lowered, which in turn led to alleviation of the symptoms of the disease.

- Before the procedure, (2nd of January) the patient was not able to make a few steps on her own. The kidneys and liver were in extremely bad condition. Currently Martyna moves around independently and her parameters of internal organ function are returning to normal. The mechanical function of the heart has also improved – said dr hab. Joanna Kwiatkowska, M.D., Ph.D., Head of the Department of Paediatric Cardiology and Congenital Heart Defects. – The indication for which the procedure was performed, i.e. lowering the left atrial pressure, is a chance to treat patients with impaired left ventricular diastolic function in the course of a range of diseases. In the future, it may become a method for reducing the symptoms of circulatory insufficiency, which occurs in this mechanism.

The procedure was carried out by the team of the Department of Paediatric Cardiology and Congenital Heart Defects, including: dr hab. Robert Sabiniewicz, M.D., Ph.D., dr Piotr Potaż, Ph.D., Sebastian Ciemny, M.D. and dr Grzegorz Żuk, Ph.D., M.D. of the Invasive Cardiology Unit of the University Clinical Centre (UCC). Immediately after the procedure, the patient was transferred to the Paediatric Intensive Care Unit of the Department of Anaesthesiology and Intensive Care, UCC. Martyna was the first patient of this new unit.

The Atrial Flow Regulator device (AFR – Occlutech company) is currently at the stage of clinical testing. So far, few implants have been performed as life-saving procedures, after all pharmacological treatment options have been exhausted, on the basis of individual opinions from a group of experts.