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Heart transplantation in a child with Ebstein's Anomaly and primary cardiomyopathy

Session Poster Session 4

Speaker Kseniia Chueva

Event : Heart Failure 2019

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Dilative Cardiomyopathy
  • Session type : Poster Session

Authors : K Chueva (Saint Petersburg,RU), T Loevets (Saint Petersburg,RU), T Pervunina (Saint Petersburg,RU), T Vershinina (Saint Petersburg,RU), P Fedotov (Saint Petersburg,RU), E Vasichkina (Saint Petersburg,RU), R Tatarskii (Saint Petersburg,RU), D Lebedev (Saint Petersburg,RU)

K Chueva1 , T Loevets1 , T Pervunina1 , T Vershinina1 , P Fedotov1 , E Vasichkina1 , R Tatarskii1 , D Lebedev1 , 1Federal Almazov Medical Research Centre - Saint Petersburg - Russian Federation ,


The patient was diagnosed with the congenital heart desease: Ebstein's  Anomaly and Wolf-Parkinson-White Syndrome at the age of 2 days. For several years reicuspidal regurgitation was II grade, myocardial contractility of left ventricular was > 53 % and she have been asymptomatic. The first symptoms of heart failure such as decline of exercise capacity, cyanosis and dyspnea by physical activity  appeared only at the age of 14 years. Because of the progression of symptoms, it was decided to perform tricuspid valve replacement with bioprostetic vale Carpentier Edwards ?31.  Until that time the patient had already underwent 3 catheter ablations of multiple right-sided accessory AV connections. But by admission a girl was presented with significant heart failure (Class III NYHA) and reduced cardiac contractility with ejection fraction of left ventricular 25%. Such rapid heart failure progression was considered not only as end stage of congenital heart disease, but as a result of  myocarditis or primary cardiac cardiomyopathy. Due to anamnesis, laboratory findings and further diagnostic procedures no evidence of inflammatory cardiomyopathy was revealed,  but  in our patient detected novel missense mutation in gene MYPNc.458A>G (p.Lys153Arg), wich  have been linked to the pathogenesis of dilated cardiomyopathy. In that case correction of Ebstein's Anomaly was associated with a poor prognosis.For this reason she was listed for heart transplantation, wich was performed after 12 months on the waiting list. Since two years after transplatation the patient have had normal heart function, she have not got limitation in physical activity and herpsychological status have become good.

Each patient with congenital heart disease and significant heart failureneed of personified approach in management. Among patients with congenital heart disease associated with primary cardiomyopathy heart transplantation could be procedure of choice and show better prognosis in comparison with surgical correction of heart lesion.

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