Von Hippel Lındau Tanılı Hastada Aritmojenik Sağ Ventriküler Kardiyomiyopati

Yıl 2021, Cilt: 13 Sayı: 4, 275 - 282, 11.05.2022

Ugur Ergun , Orcun Can Ali Kırık Cigdem Usul Avsar

Öz

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

Öz

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.

Anahtar Kelimeler

Arrhythmogenic cardiomyopathy, Von Hippel Lindau, cardiomyopathy

Kaynakça

• 1. Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm 2019; 16:e301.
• 2. Gemayel C, Pelliccia A, Thompson PD. Arrhythmogenic right ventricular cardiomyopathy. J Am CollCardiol 2001; 38:1773.
• 3. Sen-Chowdhry S, Lowe MD, Sporton SC, McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med 2004;117:685.
• 4. Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation 1996; 93:841.
• 5. Maher ER, Yates JR, Harries R, et al. Clinical features and natural history of vonHippel-Lindau disease. Q J Med 1990; 77:1151.
• 6. Crespigio, J.,Berbel, L. C. L., Dias, M. A., Berbel, R. F., Pereira, S. S., Pignatelli, D., &Mazzucco, T. L. VonHippel–Lindaudisease: a single gene, several hereditary tumors. Journal of endocrinological investigation. 2018;41(1), 21-31.
• 7. BASSO, Cristina, et al. Arrhythmogenic right ventricular cardiomyopathy. The Lancet, 2009, 373.9671: 1289-1300.
• 8. Tsirlin A, Oo Y, Sharma R, et al. Pheochromocytoma: a review. Maturitas. 2014;77:229–238.
• 9. Lee TW, Lin KH, Chang CJ, et al. Pheochromocytoma mimicking both acute coronary syndrome and sepsis: a case report. MedPrincPract. 2013;22:405–407.