Noonan Sendromlu Bir Hastada Nadir Olarak Saptanan Kardiyak Anomalilerin Tedavisi

Abstract

Noonan sendromu farklı kardiyak anomalilerin de eşlik ettiği nadir görülen bir hastalıktır. Sunulan olguda Noonan sendromlu bir hastada kardiyak anomalilerin daha önce bildirilmemiş bir kombinasyonu bildirilmiştir. Eşlik eden kardiyak anomaliler atriyal septal defekt, atriyal fibrilasyon ve ventrikül miyokardında noncompaction idi. Atriyal septal defekt cerrahi olarak fenestre biçimde kapatılırken maze prosedürü uygulandı. Sendromlu hastalarda saptanan konjenital kalp defektlerinin cerrahi tedavisinde sendromu olmayan hastalara göre farklı güçlükler ile karşılaşılmaktadır. Bu nedenle sendromlu vakalarda özellikli bir yaklaşım ihtiyacı vardır.

Keywords

• Noonan sendromu,atriyal fibrilasyon,atriyal septal defekt,noncompaction,hasta sinüs sendromu

Management of Rare Combination of Cardiac Anomalies in A Patient with Noonan Syndrome

Abstract

Noonan syndrome is a rare disorder in which different cardiac abnormalities can exist. This report describes the combination of cardiac abnormalities in a patient with Noonan syndrome, which has not been reported previously. Coexisting cardiac abnormalities were: atrial septal defect, atrial fibrillation and noncompaction of the ventricular myocardium. Surgical fenestrated closure of atrial septal defect and concomitant maze procedure were performed. Surgery of congenital heart defects in syndromic patients has different difficulties than those in nonsyndromic patients. Thus, specific approach should be taken in these patients.

Keywords

• Noonan syndrome,atrial fibrillation,atrial septal defect,noncompaction,sick sinus syndrome

References

1. 1. Romano AA, Allanson JE, Dahlgren J, Gelb BD, Hall B, Pierpont ME, et al. Noonan syndrome: clinical features, diagnosis, and management guidelines. Pediatrics 2010;126:746-59.
2. 2. van der Burgt I. Noonan syndrome. Orphanet J Rare Dis 2007;2:4.
3. 3. Burch M, Sharland M, Shinebourne E, Smith G, Patton M, McKenna W. Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 1993;22:1189-92.
4. 4. Marino B, Digilio MC, Toscano A, Giannotti A, Dallapiccola B. Congenital heart diseases in children with Noonan syndrome: an expanded cardiac spectrum with high prevalence of atrioventricular canal. J Pediatr 1999;135:703-6.
5. 5. Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J 2011;32:1446-56.
6. 6. Spirito P, Autore C. Apical hypertrophic cardiomyopathy or left ventricular non-compaction? A difficult differential diagnosis. Eur Heart J 2007;28:1923-4.
7. 7. Hickey EJ, Mehta R, Elmi M, Asoh K, McCrindle BW, Williams WG, et al. Survival implications: Hypertrophic cardiomyopathy in Noonan syndrome. Congenit Heart Dis 2011;6:41-7.
8. 8. Cho YH, Jun TG, Yang JH, Park PW, Huh J, Kang IS, et al. Surgical strategy in patients with atrial septal defect and severe pulmonary hypertension. Heart Surg Forum 2012;15:E111-5.

9. 9. Pasic M, Musci M, Siniawski H, Edelmann B, Tedoriya T, Hetzer R. Transient sinus node dysfunction after the Cox-maze III procedure in patients with organic heart disease and chronic fixed atrial fibrillation. J Am Coll Cardiol 1998;32:1040-7.
10. 10. Masuda M, Inoue K, Iwakura K, Okamura A, Koyama Y, Kimura R, et al. Preprocedural ventricular rate predicts subsequent sick sinus syndrome after ablation for long-standing persistent atrial fibrillation. Pacing Clin Electrophysiol 2012;35:1074-80.
11. 11. Dretzke J, Toff WD, Lip GY, Raftery J, Fry-Smith A, Taylor R. Dual chamber versus single chamber ventricular pacemakers for sick sinus syndrome and atrioventricular block. Cochrane Database Syst Rev 2004:CD003710.
12. 12. Sharland M, Patton MA, Talbot S, Chitolie A, Bevan DH. Coagulation-factor deficiencies and abnormal bleeding in Noonan’s syndrome. Lancet 1992;339:19-21.
13. 13. Campbell RM, Benson LN, Williams WW, Adatia I. Chylopericardium after cardiac operations in children. Ann Thorac Surg 2001;72:193-6.