Av Tam Blok Gelișen Duchenne Musküler Distrofi Tanılı Olgunun Anjiografi Laboratuvarındaki Anestezi Yönetimi

Duchenne Musküler Distrofi X’e bağlı resesif kalıtımla aktarılan, 1/3500 sıklıkta gözlenen prog-resif kas dejenerasyonu ve kontraktürler ile karakterize genetik bir hastalıktır. Kas güçsüzlüğü temel problem olmakla birlikte; solunum kaslarının tutulması sonucu öksürme ve ekspektoras-yon güçlüğü nedeni ile solunum yolu enfeksiyonları sıktır. Kardiyomiyopati nedeniyle ventrikü-ler dilatasyon, iletim kusurları, supraventriküler veya ventriküler aritmiler, miyokardiyal nekroz gibi kardiyak problemler görülebileceği için anestezi yönetimi özellik göstermektedir. İnhalas-yon anestezikleri ve depolarizan kas gevșetici ajanlar, malign hipertermi ve rabdomiyoliz gibi mortal komplikasyonlara neden olabilir. Bu makalemizde AV tam blok nedeniyle geçici transve-nöz pacemakerın, kalıcı pacemaker ile değiștirilmesi planlanan Duchenne Musküler Distrofi ta-nılı olguda uyguladığımız anestezi yöntemini sunmayı hedefledik.

Anesthesia Management of Duchenne Muscular Dystrophy with Complete Atrioventricular Block in Angiography Laboratory

Duchenne muscular distrophy is an X-linked, recessive, inherited genetic disorder, which is seen in 1/3500 of the population and characterized by progressive muscle degeneration and cont-ractures. As well as muscle weakness is the main problem; respiratory infections are frequent due to the difficulty of coughing and expectoration caused by the retension of respiratory muscles. Due to the cardiomyopathy, cardiac problems such as ventricular dilatation, conduc-tion defects, supraventricular or ventricular arrhytmias, myocardial necrosis, anesthesia mana-gement is special. Inhalational anesthetics and depolarizing neuromuscular blocking agents may cause fatal complications such as malignant hyperthermia and rhabdomyolysis. In this ar-ticle we aimed to present anesthetical management of implantation of permanent pacemaker to a patient with Duchenne Muscular Dystrophy who had a temporary transvenous pacemaker for complete atrioventricular block.

PDF

___

16. Kocabas S, Yedicocuklu D, Askar F, et al. Anesthetic management of a child with Duchenne muscular dystrophy undergo-ing correction of Fallot’s Tetralogy. Pedi-atric Anesthesia 2008; 18: 448-450.
15. Frankowski GA, Johnson JO, Tobias JD. Rapacuronium administration to two children with Duchenne’s muscular dyst-rophy. Anesth Analg 2000; 91: 27-28.
14. Shin HH, Park DH, Lee C, et al. Anesthe-sia for a 10-year-old boy with Duchenne muscular dystrophy syndrome: A case re-port. Korean J Anesthesiol 2008; 55: 217-220.
13. Girshin M, Mukherjee J, Clowney R, et al. The postoperative cardiovascular arrest of a 5-year-old male: an initial presentation of Duchenne’s muscular dystrophy. Paediatr Anaesth 2006; 16: 170-173.
12. Birnkrat DJ, Panitch HB, Benditt JO, et al. American collage of chest physicians con-sensus statement on the respiratory and re-lated management of patients with Duc-henne muscular dystrophy undergoing anesthesia or sedation. Chest 2007; 132: 1977-1986.
11. Wick S, Muenster T, Schmidt J, et al. On-set and duration of rocuronium-induced neuromuscular blockade in patients with Duchenne muscular dystrophy. Anesthe-siology 2005; 102: 915-919.
10. Epstein AE, DiMarco JP, Ellenbogen KA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/Ameri-can Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2013; 61: e6-75.
9. Finsterer J, Stöllberger C. The heart in hu-man dystrophinopathies. Cardiology 2003; 99: 1-19.
8. Fayssoil A, Nardi O, Orlikowski D, et al. Cardiomyopathy in Duchenne muscular dystrophy: pathogenesis and therapeutics. Heart Fail Rev 2010; 15: 103-107.
7. Obata R, Yasumi Y, Suzuki A, et al. Rhab-domyolysis in association with Duc-henne’s muscular dystrophy. Can J Anesth 1999; 46: 564-566.
6. Mavrogeni S, Markousis-Mavrogenis G, Papavasiliou A, et al. Cardiac involvement in Duchenne and Becker muscular dyst-rophy. World J Cardiol 2015; 7: 410-414.
5. Finder JD, Birnkrant D, Carl J, et al. Res-piratory care of the patient with Duchenne muscular dystrophy: ATS consensus state-ment. Am J Respir Crit Care Med 2004; 170: 456-465.
4. Birnkrant DJ, Bushby KM, Amin RS, et al. The respiratory management of patients with duchenne muscular dystrophy: a DMD care considerations working group specialty article. Pediatr Pulmonol 2010; 45: 739-748.
3. Bartels B, Pangalila RF, Bergen MP, et al. Upper limb function in adults with Duc-henne muscular dystrophy. J Rehabil Med 2011; 43: 770-775.
2. Guglieri M, Bushby K. Recent develop-ments in the management of Duchenne muscular dystrophy. Paediatr Child Health 2011; 21: 501-509.
1. Wicki J, Seto JS, Chamberlain JS. Duc-henne Muscular Dystrophy Brenner's Encyclopedia of Genetics. WA USA. 2013. p. 421e4.