BACKGROUND: Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD. METHODS: A retrospective cohort study was performed on 427 patients with ASD (median age at diagnosis 38 years, IQR 18-56) out of which 186 patients (44%) manifested PH. ASD closure in patients with PH was only considered in patients without Eisenmenger syndrome with pulmonary vascular resistance < 5 WU. Median follow-up duration was 18 years (IQR 9-31 years). Kaplan-Meier and Cox proportional hazards survival analyses were performed to evaluate 12 potential predictors of survival. RESULTS: Defect closure was associated with improved long-term survival in ASD patients both with (P < 0.001) and without PH (P = 0.01) and this association was present also in patients over 40 years. The 20-year survival since diagnosis was significantly higher in patients with PH and closed ASD compared to those with PH and open ASD (65% vs. 41%). ASD closure was a significant independent predictor of long-term survival (P = 0.003) after accounting for age at diagnosis, PH, NYHA class, Eisenmenger syndrome, and mitral regurgitation. Significant negative independent predictors of survival were older age at diagnosis (P < 0.001), Eisenmenger syndrome (P < 0.001), and PH (P = 0.03). CONCLUSION: ASD closure appears to be associated with improved long-term survival independently of age, PH, and other clinical variables.

Department of Biochemistry and Molecular Medicine School of Medicine University of California Davis Davis CA United States

Department of Cardiac Surgery Na Homolce Hospital Prague Czechia

Department of Pharmacology School of Medicine University of California Davis Davis CA United States

Faculty Hospital Motol Pediatric Heart Centre Prague Czechia

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Opotowsky AR. Clinical evaluation and management of pulmonary hypertension in the adult with congenital heart disease. Circulation. (2015) 131:200–10. 10.1161/CIRCULATIONAHA.114.006976 PubMed DOI

Cuypers JAAE, Opiæ P, Menting ME, Utens EMWJ, Witsenburg M, Helbing WA, et al. The unnatural history of an atrial septal defect: longitudinal 35 year follow up after surgical closure at young age. Heart. (2013) 99:1346–52. 10.1136/heartjnl-2013-304225 PubMed DOI

Ranard LS, Mallah WE, Awerbach JD, Abernethy A, Halane M, Qureshi AM, et al. Impact of pulmonary hypertension on survival following device closure of atrial septal defects. Am J Cardiol. (2019) 124:1460–4. 10.1016/j.amjcard.2019.07.042 PubMed DOI

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) 323:1645–50. 10.1056/NEJM199012133232401 PubMed DOI

Baumgartner H, Bonhoeffer P, De Groot NMSS, de Haan F, Deanfield JE, Galie N, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. (2010) 31:2915–57. 10.1093/eurheartj/ehq249 PubMed DOI

Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller G-P, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. (2020) 42:1–83. 10.1093/eurheartj/ehaa554 PubMed DOI

Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. (2016) 37:67–119. 10.1093/eurheartj/ehv317 PubMed DOI

Parasuraman S, Walker S, Loudon BL, Gollop ND, Wilson AM, Lowery C, et al. Assessment of pulmonary artery pressure by echocardiography-a comprehensive review. Int J Cardiol Hear Vasc. (2016) 12:45–51. 10.1016/j.ijcha.2016.05.011 PubMed DOI PMC

Humenberger M, Rosenhek R, Gabriel H, Rader F, Heger M, Klaar U, et al. Benefit of atrial septal defect closure in adults: Impact of age. Eur Heart J. (2011) 32:553–60. 10.1093/eurheartj/ehq352 PubMed DOI PMC

Grambsch PM, Therneau TM. Proportional hazards tests and diagnostics based on weighted residuals. Biometrika. (1994) 81:515. 10.2307/2337123 DOI

Kaemmerer H, Apitz C, Brockmeier K, Eicken A, Gorenflo M, Hager A, et al. Pulmonary hypertension in adults with congenital heart disease: updated recommendations from the cologne consensus conference 2018. Int J Cardiol. (2018) 272:79–88. 10.1016/j.ijcard.2018.08.078 PubMed DOI

Lammers AE, Bauer LJ, Diller GP, Helm PC, Abdul-Khaliq H, Bauer UMM, et al. Pulmonary hypertension after shunt closure in patients with simple congenital heart defects. Int J Cardiol. (2020) 308:28–32. 10.1016/j.ijcard.2019.12.070 PubMed DOI

Yong G, Khairy P, De Guise P, Dore A, Marcotte F, Mercier LA, et al. Pulmonary arterial hypertension in patients with transcatheter closure of secundum atrial septal defects a longitudinal study. Circ Cardiovasc Interv. (2009) 2:455–62. 10.1161/CIRCINTERVENTIONS.108.826560 PubMed DOI

Zwijnenburg RD, Baggen VJM, Geenen LW, Voigt KR, Roos-Hesselink JW, van den Bosch AE. The prevalence of pulmonary arterial hypertension before and after atrial septal defect closure at adult age: a systematic review. Am Heart J. (2018) 201:63–71. 10.1016/j.ahj.2018.03.020 PubMed DOI

Lammers AE, Stegger J, Koerten MA, Helm PC, Bauer UM, Baumgartner H, et al. Secundum type atrial septal defect in patients with trisomy 21—therapeutic strategies, outcome, and survival: a nationwide study of the German national registry for congenital heart defects. J Clin Med. (2021) 10:3807. 10.3390/jcm10173807 PubMed DOI PMC

Nyboe C, Karunanithi Z, Nielsen-Kudsk JE, Hjortdal VE. Long-term mortality in patients with atrial septal defect: a nationwide cohort-study. Eur Heart J. (2018) 39:993–8. 10.1093/eurheartj/ehx687 PubMed DOI PMC

D’Alto M, Romeo E, Argiento P, Correra A, Santoro G, Gaio G, et al. Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure. Int J Cardiol. (2013) 168:3797–801. 10.1016/j.ijcard.2013.06.036 PubMed DOI

Kim Y-H, Yu JJ, Yun T-J, Lee Y, Kim YB, Choi HS, et al. Repair of atrial septal defect with Eisenmenger syndrome after long-term sildenafil therapy. Ann Thorac Surg. (2010) 89:1629–30. 10.1016/j.athoracsur.2009.10.027 PubMed DOI

Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. (2014) 35:716–24. 10.1093/eurheartj/eht072 PubMed DOI

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: executive summary: a report of the american college of cardiology/american heart association task force on clinical practice guidelines. J Am Coll Cardiol. (2019) 73:1494–563. 10.1016/j.jacc.2018.08.1028 PubMed DOI

Steele PM, Fuster V, Cohen M, Ritter DG, McGoon DC. Isolated atrial septal defect with pulmonary vascular obstructive disease - long-term follow-up and prediction of outcome after surgical correction. Circulation. (1987) 76:1037–42. 10.1161/01.CIR.76.5.1037 PubMed DOI

Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation. (2009) 120:1679–86. 10.1161/CIRCULATIONAHA.109.866319 PubMed DOI

Nyboe C, Olsen MS, Nielsen-Kudsk JE, Johnsen SP, Hjortdal VE. Risk of pneumonia in adults with closed versus unclosed atrial septal defect (from a Nationwide Cohort Study). Am J Cardiol. (2014) 114:105–10. 10.1016/j.amjcard.2014.03.063 PubMed DOI

Nyboe C, Olsen MS, Nielsen-Kudsk JE, Hjortdal VE. Atrial fibrillation and stroke in adult patients with atrial septal defect and the long-term effect of closure. Heart. (2015) 101:706–11. 10.1136/heartjnl-2014-306552 PubMed DOI

Akseer S, Abrahamyan L, Lee DS, Huszti E, Meier LM, Osten M, et al. Long-Term outcomes in adult patients with pulmonary hypertension after percutaneous closure of atrial septal defects. Circ Cardiovasc Interv. (2022) 15:e011110. 10.1161/CIRCINTERVENTIONS.121.011110 PubMed DOI

Normalization of Four Different Types of Pulmonary Hypertension After Atrial Septal Defect Closure