Abstract
A cardiac involvement in Wilson's disease includes left ventricular hypertrophy, severe atherosclerosis, sudden cardiac death, and various arrhythmias. In this report, a case with Wilson's disease and Wolff-Parkinson-White syndrome is discussed with the relevant literature. A 22-year-old male patient presented to the hospital complaining of chest pain. The patient had the diagnosis of Wilson's disease. He had short periods of palpitations for 2 years but no tachycardic period was detected in his 24-hour electrocardiogram holter monitoring. An electrocardiogram showed a shortened PR interval and prominent delta waves supporting the diagnosis of Wolff-Parkinson-White syndrome. The patient was hospitalized and subsequently transferred to electrophysiologic mapping. The accessory pathway was eliminated with radiofrequency ablation catheter. Patients with Wilson's disease who have palpitations should be evaluated for possible arrhythmias and electrocardiography should be performed. In this report, a case with Wilson's disease and Wolff-Parkinson-White syndrome is discussed with the relevant literatures. Key words: Disorder Of Copper Metabolism; Preexcitation Syndrome; Genetic Diseases.
References
- Kitzberger R, Madl C, Ferenci C. Wilson disease. Metab Brain Dis 2005;20:295-302.
- Mak CM, Lam CW. Diagnosis of Wilson’s disease: A comprehensive review. Crit Rev Clin Lab Sci 2008;45:263-90.
- Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the menkes gene. Nat Genet 1993;5:327-37.
- Hlubocká Z, Mareček Z, Linhart A, Kejková E, Pospísilová L, Martásek P, et al. Cardiac involvement in Wilson disease. J Inherit Metab Dis 2002;25:269-77.
- Factor SM, Cho S, Sternlieb I, Scheinberg IH, Goldfischer S. The cardiomyopathy of Wilson's disease. Myocardial alterations in nine cases. Virchows Arch A Pathol Anat Histol 1982;397:301-11.
- Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med 2001;344:1823-31.
- Kuan P. Cardiac Wilson’s disease. Chest 1987;91:579-83.
- Gollob MH, Seger JJ, Gollob TN, Tapscott T, Gonzales O, Bachinski L, et al. Novel PRKAG2 mutation responsible for the genetic syndrome of ventricular preexcitation and conduction system disease with childhood onset and absence of cardiac hypertrophy. Circulation 2001;104:3030-3.
- Mohan P, Raghavan K, Sagili H. Wolff-Parkinson-White syndrome in association with tuberous sclerosis. N Z Med J 2008;121:71-4.
Abstract
Wilson hastalığının kardiyak tutulumunda sol ventrikül hipertrofisi, aterosklerozis, ani kardiyak ölüm ve çeşitli aritmiler görülebilir. Bu yazıda Wolff-Parkinson-White sendromunun eşlik ettiği Wilson hastalığı literatürler ışığında tartışılmıştır. Wilson hastalığı tanısı olan 22 yaşında erkek hasta göğüs ağrısı şikayeti ile hastaneye başvurdu. 2 yıldır kısa süreli çarpıntı şikayetleri olan hastanın 4 saatlik elektrokardiyografik holter incelemesinde herhangi bir taşikardi saptanmamış. Hastanın elektrokardiyografisinde Wolff-Parkinson-White sendromunu destekleyen kısa PR aralığı ve delta dalgası bulunmaktaydı. Hasta hastaneye yatırıldı; sonrasında elektrofizyolojik haritalama yapıldı ve radyofrekans ablasyon yöntemi ile aksesuar yolak sonlandırıldı. Çarpıntı şikayeti ve Wilson hastalığı olan hastalar olası aritmiler yönünden değerlendirilmeli ve bu hastaların elektrokardiyografileri aritmi açısından incelenmelidir. Bu yazıda Wilson hastalığı ve Wolff-Parkinson-White sendromu olan bir vaka ilgili literatürler eşliğinde sunulmuştur. Anahtar kelimeler: Bakır Metabolizma Bozukluğu; Preeksitasyon Sendromu; Genetik Hastalıklar.
References
- Kitzberger R, Madl C, Ferenci C. Wilson disease. Metab Brain Dis 2005;20:295-302.
- Mak CM, Lam CW. Diagnosis of Wilson’s disease: A comprehensive review. Crit Rev Clin Lab Sci 2008;45:263-90.
- Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the menkes gene. Nat Genet 1993;5:327-37.
- Hlubocká Z, Mareček Z, Linhart A, Kejková E, Pospísilová L, Martásek P, et al. Cardiac involvement in Wilson disease. J Inherit Metab Dis 2002;25:269-77.
- Factor SM, Cho S, Sternlieb I, Scheinberg IH, Goldfischer S. The cardiomyopathy of Wilson's disease. Myocardial alterations in nine cases. Virchows Arch A Pathol Anat Histol 1982;397:301-11.
- Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med 2001;344:1823-31.
- Kuan P. Cardiac Wilson’s disease. Chest 1987;91:579-83.
- Gollob MH, Seger JJ, Gollob TN, Tapscott T, Gonzales O, Bachinski L, et al. Novel PRKAG2 mutation responsible for the genetic syndrome of ventricular preexcitation and conduction system disease with childhood onset and absence of cardiac hypertrophy. Circulation 2001;104:3030-3.
- Mohan P, Raghavan K, Sagili H. Wolff-Parkinson-White syndrome in association with tuberous sclerosis. N Z Med J 2008;121:71-4.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Articles |
| Authors | |
| Publication Date | February 1, 2013 |
| Published in Issue | Year 2013 Volume: 20 Issue: 1 |