Ugur ERGUN, Orcun CAN, Ali KIRIK, Cigdem USUL AVSAR

Von Hippel Lındau Tanılı Hastada Aritmojenik Sağ Ventriküler Kardiyomiyopati

Aritmojenik kardiyomiyopati, aritmi ve miyokardiyal yapısal anormalliklerin eşlik ettiği bir klinik tablo olarak tanımlanmaktadır. Aritmojenik kardiyomiyopati, çarpıntı ve ventriküler aritmiler gibi şüpheli klinik semptomları olan hastalarda düşünülmesi gereken, birden fazla test gerektiren bir hastalıktır. Von Hippel Lindau hastalarının %20'sinin feokromositoma ile ilişkili olduğu bildirilmiştir, ancak feokromositoma durumunda dilate kardiyomiyopati veya akut kalp yetmezliği gibi kardiyak etkiler yaygın değildir. Bu yazımızda literatürde yer almayan, olası birliktelik ve klinik antite bağlamında tartışmak amacıyla Von Hippel Lindau tanısı almış bir hastada Aritmojenik kardiyomiyopati olgusu sunulmaktadır.

Anahtar Kelimeler:

Aritmojenik kardiyomyopati, Von Hippel Lindau, kardiyomyopati

Arrhythmogenic Right Ventricular Cardiomyopathy in a Patient Wıth Von Hippel Lindau Disease

Arrhythmogenic cardiomyopathy is defined as a clinical manifestation of arrhythmia and myocardial structuralab normalities. Arrhythmogenic cardiomyopathyis difficult to diagnose disease that requires multipletests, which should be considered in patients with suspicious clinical symptoms such as palpitations and ventricular arrhythmias. It has been reported the %20 of Von Hippel Lindau patients are related to pheochromocytoma, but cardiac effects such as dilated cardiomyopathy or acute heart failure are not common in the case of pheochromocytoma. In this study, we present a case of Arrhythmogenic cardiomyopathy in a patient who was diagnosed with Von Hippel Lindau in order to discuss two rare diseases in the context of possible association and the clinical entity that mayac company the absence in the literature.

Keywords:

Arrhythmogenic cardiomyopathy, Von Hippel Lindau, cardiomyopathy,

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