Identification and assessment of atrial septal defects in adults
- Martin G St. John Sutton, MBBS, FRCP, FACC, FASE
Martin G St. John Sutton, MBBS, FRCP, FACC, FASE
- John Bryfogle Professor of Medicine
- University of Pennsylvania School of Medicine
Atrial septal defects (ASDs) are the most common congenital lesion in adults after bicuspid aortic valves. Although persons with ASDs are often asymptomatic until adulthood, potential complications of an undetected lesion include irreversible pulmonary hypertension, right ventricular failure, progressive tricuspid valve regurgitation from annular dilatation, atrial arrhythmias, paradoxical embolization, and cerebral abscess. (See "Clinical manifestations and diagnosis of atrial septal defects in adults".)
This topic will review the methods used for diagnosis and assessment of ASDs, including identification of the precise anatomy of the defect and associated anomalies, evaluation of right ventricular overload, evaluation of pulmonary artery pressures, and estimation of the pulmonary-to-systemic flow ratio [1,2]. In most cases, echocardiography provides all the required information. Other modalities such as cardiovascular magnetic resonance and cardiac computed tomography may be helpful in selected cases, especially when echocardiography is technically suboptimal or inconclusive. Cardiac catheterization is rarely required to diagnose and assess an ASD because the requisite information including hemodynamics can be obtained noninvasively in the majority of individuals.
The pathophysiology, clinical features, and management of ASDs in adults and issues related to ASD in children are discussed separately. (See "Clinical manifestations and diagnosis of atrial septal defects in adults" and "Indications for closure and medical management of atrial septal defects in adults" and "Classification of atrial septal defects (ASDs), and clinical features and diagnosis of isolated ASDs in children" and "Management and outcome of isolated atrial septal defects in children".)
IDENTIFICATION OF ASD AND ASSOCIATED ANOMALIES
Overview — Patients with unexplained right ventricular volume overload should be referred for evaluation of possible atrial communication, partial anomalous venous connection, or unroofed coronary sinus/coronary sinus atrial septal defect (ASD) . Imaging of an ASD should include demonstration of the presence and direction of shunting across the defect and evaluation of associated anomalies .
In most patients, echocardiography identifies the ASD, its size and direction of flow, associated abnormalities, and complications. Partial anomalous pulmonary venous drainage of the right upper and middle pulmonary veins frequently accompanies superior sinus venosus defects and less frequently occurs with secundum ASDs. Primum ASDs are generally accompanied by cleft anterior mitral valve leaflet. Since the sensitivity of echocardiography varies with technology, acoustic windows, and operator/patient factors, prior negative suboptimal echocardiograms do not definitively exclude an ASD.
- Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
- Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31:2915.
- Shub C, Dimopoulos IN, Seward JB, et al. Sensitivity of two-dimensional echocardiography in the direct visualization of atrial septal defect utilizing the subcostal approach: experience with 154 patients. J Am Coll Cardiol 1983; 2:127.
- Pollick C, Sullivan H, Cujec B, Wilansky S. Doppler color-flow imaging assessment of shunt size in atrial septal defect. Circulation 1988; 78:522.
- Vick GW 3rd, Murphy DJ Jr, Ludomirsky A, et al. Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography. J Am Coll Cardiol 1987; 9:580.
- Konstantinides S, Kasper W, Geibel A, et al. Detection of left-to-right shunt in atrial septal defect by negative contrast echocardiography: a comparison of transthoracic and transesophageal approach. Am Heart J 1993; 126:909.
- Suzuki Y, Kambara H, Kadota K, et al. Detection of intracardiac shunt flow in atrial septal defect using a real-time two-dimensional color-coded Doppler flow imaging system and comparison with contrast two-dimensional echocardiography. Am J Cardiol 1985; 56:347.
- Roberson DA, Cui W, Patel D, et al. Three-dimensional transesophageal echocardiography of atrial septal defect: a qualitative and quantitative anatomic study. J Am Soc Echocardiogr 2011; 24:600.
- Faletra F, Scarpini S, Moreo A, et al. Color Doppler echocardiographic assessment of atrial septal defect size: correlation with surgical measurements. J Am Soc Echocardiogr 1991; 4:429.
- Roberson DA, Muhiudeen IA, Silverman NH, et al. Intraoperative transesophageal echocardiography of atrioventricular septal defect. J Am Coll Cardiol 1991; 18:537.
- Seo JS, Song JM, Kim YH, et al. Effect of atrial septal defect shape evaluated using three-dimensional transesophageal echocardiography on size measurements for percutaneous closure. J Am Soc Echocardiogr 2012; 25:1031.
- Watanabe N, Taniguchi M, Akagi T, et al. Usefulness of the right parasternal approach to evaluate the morphology of atrial septal defect for transcatheter closure using two-dimensional and three-dimensional transthoracic echocardiography. J Am Soc Echocardiogr 2012; 25:376.
- Debl K, Djavidani B, Buchner S, et al. Quantification of left-to-right shunting in adult congenital heart disease: phase-contrast cine MRI compared with invasive oximetry. Br J Radiol 2009; 82:386.
- Holmvang G, Palacios IF, Vlahakes GJ, et al. Imaging and sizing of atrial septal defects by magnetic resonance. Circulation 1995; 92:3473.
- Brenner LD, Caputo GR, Mostbeck G, et al. Quantification of left to right atrial shunts with velocity-encoded cine nuclear magnetic resonance imaging. J Am Coll Cardiol 1992; 20:1246.
- Powell AJ, Tsai-Goodman B, Prakash A, et al. Comparison between phase-velocity cine magnetic resonance imaging and invasive oximetry for quantification of atrial shunts. Am J Cardiol 2003; 91:1523.
- Beerbaum P, Körperich H, Esdorn H, et al. Atrial septal defects in pediatric patients: noninvasive sizing with cardiovascular MR imaging. Radiology 2003; 228:361.
- Dittmann H, Jacksch R, Voelker W, et al. Accuracy of Doppler echocardiography in quantification of left to right shunts in adult patients with atrial septal defect. J Am Coll Cardiol 1988; 11:338.
- Mehta RH, Helmcke F, Nanda NC, et al. Transesophageal Doppler color flow mapping assessment of atrial septal defect. J Am Coll Cardiol 1990; 16:1010.
- Hofmann T, Franzen O, Koschyk DH, et al. Three-dimensional color Doppler echocardiography for assessing shunt volume in atrial septal defects. J Am Soc Echocardiogr 2004; 17:1173.
- IDENTIFICATION OF ASD AND ASSOCIATED ANOMALIES
- - Transthoracic echocardiography
- Two-dimensional TTE
- - Agitated saline contrast
- - TEE
- - Three-dimensional
- CMR and CT
- Cardiac catheterization
- EVALUATION FOR RV OVERLOAD
- EVALUATION OF PULMONARY ARTERY PRESSURES
- ESTIMATION OF THE Qp/Qs
- Doppler echocardiography
- Cardiac catheterization
- SUMMARY AND RECOMMENDATIONS