Patients with Congenital Atrial Septal Defects: Effect of Age at Repair and Defect Size on Pulmonary Artery Pressures Prior to Repair

Abstract

Background: Pulmonary arterial hypertension (PAH) is a major complication of atrial septal defect (ASD) and can be responsible for significant functional limitations and early mortality. Various factors have been shown to predispose ASD patients to the development of PAH. Our study aimed to determine the association between the size of the ASD, the age of the patient and the increase in pulmonary artery pressures. Methods: Data from 74 ASD patients was retrospectively reviewed, including the patients' presenting symptoms, vital parameters, comorbidities, as well as their preoperative diagnostic workup. Echocardiography findings were used to determine the type and size of the ASD, and pulmonary artery pressures were evaluated using tricuspid regurgitation velocity as assessed by echocardiography or based on cardiac catheterization data. All patients underwent ASD repair either surgically or via percutaneous repair. Univariate and multivariate linear regression was performed to analyze the effect of age and defect size on pulmonary artery pressures. Model adequacy check was also done for the final model. Postoperative morbidity/mortality was additionally evaluated. Results: The study sample comprised 44.6 % males and 55.4 % females. The most prominent presenting features were shortness of breath (70.3 %), chest pain (43.2 %), and palpitations (33.8 %), and arterial hypertension was the commonest morbidity. Using multiple linear regression analysis, age and size of ASD were found to be independently associated with pulmonary artery pressure. We found that for every 1 mm increase in the size of the ASD, pulmonary artery systolic pressure (PASP) increased by 0.32 mmHg (p ≤ 0.05). Similarly, with every increase of one year in age, pulmonary artery pressure increased by 0.24 mmHg (p ≤ 0.02). No significant postoperative complications were reported following both types of repair. Conclusions: Our study concludes that ASD patients are at greater risk of developing PAH with increasing age and increasing ASD size. This can potentially help to determine which ASD patients are at greater risk and require urgent repair of their defects. The study also shows that early repair is best to prevent complications.

Key words

cardiovascular surgery - atrial septal defect - pulmonary hypertension - ostium secundum atrial septal defect - postoperative complications

References

• 1 Samanek M, Voriskova M. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study.  Pediatr Cardiol. 1999;  20 (6) 411-417
• 2 Campbell M, Neill C, Suzman S. The prognosis of atrial septal defect.  Br Med J. 1957;  1 (5032) 1375-1383
• 3 Markman P, Howitt G, Wade E G. Atrial septal defect in the middle-aged and elderly.  Q J Med. 1965;  34 (136) 409-426
• 4 Perloff J K. Ostium secundum atrial septal defect – survival for 87 and 94 years.  Am J Cardiol. 1984;  53 (2) 388-389
• 5 Rodstein M, Zeman F D, Gerber I E. Atrial septal defect in the aged.  Circulation. 1961;  23 665-674
• 6 Campbell M. Natural history of atrial septal defect.  Br Heart J. 1970;  32 (6) 820-826
• 7 Gatzoulis M A, Freeman M A, Siu S C, Webb G D, Harris L. Atrial arrhythmia after surgical closure of atrial septal defects in adults.  N Engl J Med. 1999;  340 (11) 839-846
• 8 Gatzoulis M A, Redington A N, Somerville J, Shore D F. Should atrial septal defects in adults be closed?.  Ann Thorac Surg. 1996;  61 (2) 657-659
• 9 Shah D, Azhar M, Oakley C M, Cleland J G, Nihoyannopoulos P. Natural history of secundum atrial septal defect in adults after medical or surgical treatment: a historical prospective study.  Br Heart J. 1994;  71 (3) 224-228
• 10 Cherian G, Uthaman C B, Durairaj M et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients.  Am Heart J. 1983;  105 (6) 952-957
• 11 Vogel M, Berger F, Kramer A, Alexi-Meshkishvili V, Lange P E. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects.  Heart. 1999;  82 (1) 30-33
• 12 Morrison G, Macartney F. Effects of oxygen administration, bicarbonate infusions, and brief hyperventilation on patients with pulmonary vascular obstructive disease.  Br Heart J. 1979;  41 (5) 584-593
• 13 Duffels M G, Engelfriet P M, Berger R M et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry.  Int J Cardiol. 2007;  120 (2) 198-204
• 14 McQuillan B M, Picard M H, Leavitt M, Weyman A E. Clinical correlates and reference intervals for pulmonary artery systolic pressure among echocardiographically normal subjects.  Circulation. 2001;  104 (23) 2797-2802
• 15 Schannwell C M, Steiner S, Strauer B E. Diagnostics in pulmonary hypertension.  J Physiol Pharmacol. 2007;  58 (Suppl. 5) (Pt. 2) 591-602
• 16 Sachweh J S, Daebritz S H, Hermanns B et al. Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect.  Ann Thorac Surg. 2006;  81 (1) 207-213
• 17 Balint O H, Samman A, Haberer K et al. Outcomes in patients with pulmonary hypertension undergoing percutaneous atrial septal defect closure.  Heart. 2008;  94 (9) 1189-1193
• 18 Engelfriet P M, Duffels M G, Moller T et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease.  Heart. 2007;  93 (6) 682-687
• 19 Abergel E, Chatellier G, Toussaint P, Dib J C, Menard J, Diebold B. Doppler-derived pulmonary arterial systolic pressure in patients with known systemic arterial pressures.  Am J Cardiol. 1996;  77 (9) 767-769
• 20 Davidson Jr W R, Fee E C. Influence of aging on pulmonary hemodynamics in a population free of coronary artery disease.  Am J Cardiol. 1990;  65 (22) 1454-1458
• 21 Dib J C, Abergel E, Rovani C, Raffoul H, Diebold B. The age of the patient should be taken into account when interpreting Doppler assessed pulmonary artery pressures.  J Am Soc Echocardiogr. 1997;  10 (1) 72-73
• 22 de Divitiis O, Fazio S, Petitto M, Maddalena G, Contaldo F, Mancini M. Obesity and cardiac function.  Circulation. 1981;  64 (3) 477-482
• 23 Abenhaim L, Moride Y, Brenot F et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group.  N Engl J Med. 1996;  335 (9) 609-616
• 24 Rich S, Rubin L, Walker A M, Schneeweiss S, Abenhaim L. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension.  Chest. 2000;  117 (3) 870-874
• 25 John Sutton M G, Tajik A J, McGoon D C. Atrial septal defect in patients ages 60 years or older: operative results and long-term postoperative follow-up.  Circulation. 1981;  64 (2) 402-409
• 26 Popelova J, Hlavacek K, Honek T, Spatenka J, Kolbel F. Atrial septal defect in adults.  Can J Cardiol. 1996;  12 (10) 983-988
• 27 Yalonetsky S, Lorber A. Comparative changes of pulmonary artery pressure values and tricuspid valve regurgitation following transcatheter atrial septal defect closure in adults and the elderly.  Congenit Heart Dis. 2009;  4 (1) 17-20
• 28 Zhao S H, Wang C, Jiang S L et al. [Morphological features of secundum atrial septal defect in adult and implications for transcatheter closure].  Zhonghua Xin Xue Guan Bing Za Zhi. 2006;  34 (11) 987-990
• 29 Swan L, Varma C, Yip J et al. Transcatheter device closure of atrial septal defects in the elderly: technical considerations and short-term outcomes.  Int J Cardiol. 2006;  107 (2) 207-210
• 30 Elshershari H, Cao Q L, Hijazi Z M. Transcatheter device closure of atrial septal defects in patients older than 60 years of age: immediate and follow-up results.  J Invasive Cardiol. 2008;  20 (4) 173-176
• 31 Duffels M, van Loon L, Berger R, Boonstra A, Vonk-Noordegraaf A, Mulder B. Pulmonary arterial hypertension associated with a congenital heart defect: advanced medium-term medical treatment stabilizes clinical condition.  Congenit Heart Dis. 2007;  2 (4) 242-249
• 32 Veldtman G R, Razack V, Siu S et al. Right ventricular form and function after percutaneous atrial septal defect device closure.  J Am Coll Cardiol. 2001;  37 (8) 2108-2113