Transcatheter closure of atrial septal defect in a patient with Noonan syndrome after corrective surgery

Ljupčo Mangovski; Mihajlo Farkić; Ljiljana Jovović

doi:10.2298/5344

Ljupčo Mangovski "Dedinje" Cardiovascular Institute, Belgrade, Serbia
Mihajlo Farkić "Dedinje" Cardiovascular Institute, Belgrade, Serbia
Ljiljana Jovović "Dedinje" Cardiovascular Institute, Belgrade, Serbia

DOI: https://doi.org/10.2298/5344

Keywords: noonan syndrome, heart septal defects, congenital abnormalities, cardiovascular surgical procedures, treatment outcome,

Abstract

Introduction. Transcatheter atrial septal defect (ASD) closure is considered to be a gold standard for patients with the suitable anatomy as compared to cardiac surgery. Reocurrence of ASD after surgical closure is a very rare late complication which can be successfully managed with transcatheter procedure. Case report. We reported a female patient with Noonan syndrome who presented with hemodinamically significant ASD 37 years after the corrective cardiac surgery. Due to numerous comorbidities which included severe kyphoscoliosis, pectus excavatum and multiple surgeries we decided to perform transcatheter closure of ASD. The procedure itself was very challenging due to the patient’s short stature and heart’s orientation in the chest, but was performed successfully. The subsequent follow-up was uneventful and the patient reported improvement in the symptoms. Conclusion. Transcatheter closure of ASD in a patient with Noonan syndrome with the history of surgically corrected ASD can be performed successfully, despite challenging chest anatomy.

References

Roberts AE, Allanson JE, Tartaglia M, Gelb BD. Noonan syn-drome. Lancet 2013; 381(9863): 333−42.

Opitz JM. The Noonan syndrome. Am J Med Genet 1985; 21(3): 515−8.

Tartaglia M, Mehler EL, Goldberg R, Zampino G, Brunner HG, Kremer H, et al. Mutations in PTPN11, encodes the protein ty-rosine phosphatase SHP-2, cause Noonan syndrome. Nat Genet 2001; 29(4): 465−8.

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(4): 746−59.

Cowley CG, Lloyd TR, Bove EL, Gaffney D, Dietrich M, Rocchini AP. Comparison of results of closure of secundum atrial septal defect by surgery versus Amplatzer septal occluder. Am J Car-diol 2001; 88(5): 589−91.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, edi-tors. Braunwald's Heart Disease: A Textbook of Cardiovascu-lar Medicine. Philadelphia, Pa: Saunders Elsevier; 2011. chap 65. Available from: http://www.nlm.nih.gov/medlineplus/ency/article/001114.htm

Murphy JG, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, et al. Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med 1990; 323(24): 1645−50.