Sinus Venosus Atrial Septal Defects

No Results

No Results


In simple terms, an atrial septal defect (ASD) is a deficiency of the atrial septum. Atrial septal defects account for about 10-15% of all congenital cardiac anomalies and are the most common congenital cardiac lesion presenting in children and adults. [1] Sinus venosus atrial septal defects account for only 10% of atrial septal defects. The remaining atrial septal defects are ostium secundum type (70%), ostium primum type (20%), and unroofed coronary sinus, or coronary sinus septal defects, (< 1%). Most children with sinus venosus atrial septal defects are asymptomatic but may develop symptoms as they age.

Excellent surgical results with a mortality rate near 0% can be expected. This is particularly true in patients who undergo repair when younger than 15 years. An atrial septal defect was the first lesion repaired using cardiopulmonary bypass in 1954 by John Gibbon, MD, at the Mayo Clinic.

The more common sinus venosus type defect (often referred to as the “usual type”) occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC). The lesion is rostral and posterior to the fossa ovalis (where secundum type defects occur) and is separate from it. It is almost always associated with anomalous pulmonary venous drainage of the right upper pulmonary vein into the SVC. See the image below.

Less commonly, the defect may occur at the junction of the right atrium and inferior vena cava (IVC) and be associated with anomalous connection of the right lower pulmonary vein to the IVC. Rarely, sinus venosus defects occur posterior to the fossa ovalis without bordering the SVC or IVC. The predominant hemodynamic consequence is a left-to-right shunt through the defect. See the image below. 

During normal embryonic development, the right horn of the sinus venosus encompasses the right superior vena cava (SVC) and inferior vena cava (IVC). If abnormal resorption of the sinus venosus occurs, an atrial septal defect results near the orifice of either the SVC or IVC.

Atrial septal defects occur as associated anomalies in many major complex congenital lesions but sinus venosus atrial septal defects occur more often as an isolated abnormality.

Other abnormalities may exacerbate an atrial septal defect. For example, systemic hypertension in an adult with a sinus venosus atrial septal defect may result in left ventricular hypertrophy and reduce left ventricular compliance, which, in turn, exacerbates the atrial level left-to-right shunt. Mitral stenosis, which is either congenital or acquired, may also exacerbate the atrial level left-to-right shunt.

Sinus venosus atrial septal defects represent approximately 1% of congenital cardiac lesions.

No racial predilection is known; however, atrial septal defects affect females more often than males. The female-to-male ratio is 2:1. No difference in outcome is associated with sex.

Sinus venosus atrial septal defects are congenital lesions present at birth. The age at presentation depends on the size of the left-to-right shunt (atrial and partial anomalous pulmonary venous return). Atrial septal defects in infancy are usually asymptomatic. They are usually detected by echocardiography while undergoing a cardiac evaluation.

The prognosis is excellent for young patients who undergo repair of uncomplicated defects. Repair delayed until the third decade of life is associated with a decrease in life expectancy. [9]

Surgical repair in the first 2 decades of life is associated with a mortality rate near zero. Life expectancy approaches that of the general population if the defect is repaired during this time. Right heart dilation rapidly regresses after surgery, and the functional result is excellent. In cases of repair during adulthood, life expectancy may be decreased despite successful repair. Surgical morbidity rates are related to early postoperative pericardial effusion, early postoperative pulmonary venous or systemic venous obstruction, and supraventricular arrhythmias. If the baffle directing pulmonary venous blood to the left atrium is not placed correctly, it may obstruct pulmonary venous drainage. If the baffle bulges into the SVC, it may obstruct SVC inflow, necessitating the placement of an augmentation patch on the anterior surface of the SVC and right atrial junction.

Untreated atrial septal defects are associated with a significantly shortened life expectancy. After age 20 years, the mortality rate is approximately 5% per decade with 90% of patients dead by age 60 years. These patients present with an increase in left-to-right shunting and occasionally with congestive heart failure with pulmonary hypertension in the fourth to sixth decades of life. Guidelines for the diagnosis and treatment of pulmonary artery hypertension have been established. [2]  Late problems in untreated patients also include the risk of paradoxical embolus as well as atrial fibrillation, pulmonary hypertension, and right heart failure.

Complications include the following:

Sinus node dysfunction

Pulmonary venous obstruction

Atrial fibrillation, atrial flutter, or supraventricular tachycardia (SVT)

Pulmonary hypertension

Atrial baffle leak

Pericardial effusion or postpericardiotomy syndrome

SVC syndrome

Patient education mainly focuses on preoperative and postoperative care and recovery, which are especially important in young children undergoing surgery. Centers with experienced child life personnel are invaluable in preparing children for open-heart surgery.

Alpendurada F, Wage R, Mohiaddin R. Evaluation of a sinus venosus atrial septal defect by magnetic resonance: a case report. Rev Port Cardiol. 2008 Oct. 27(10):1317-21. [Medline].

[Guideline] Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004 Dec. 25(24):2243-78. [Medline]. [Full Text].

Chen CA, Wang JK, Hsu JY, Hsu HH, Chen SJ, Wu MH. Diagnosis of inferior sinus venosus atrial septal defects using transthoracic three-dimensional echocardiography. J Am Soc Echocardiogr. 2010 Apr. 23(4):457.e4-6. [Medline].

Crystal MA, Al Najashi K, Williams WG, Redington AN, Anderson RH. Inferior sinus venosus defect: echocardiographic diagnosis and surgical approach. J Thorac Cardiovasc Surg. 2009 Jun. 137(6):1349-55. [Medline].

Gajjar TP, Hiremath CS, Desai NB. Surgical closure of sinus venosus atrial septal defect using a single patch–transcaval repair technique. J Card Surg. 2011 Jul. 26(4):429-34. [Medline].

Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. 1984 Dec. 38(6):601-5. [Medline].

Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. 1995 Dec. 60(6 Suppl):S614-7. [Medline].

Shahriari A, Rodefeld MD, Turrentine MW, Brown JW. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg. 2006 Jan. 81(1):224-9; discussion 229-30. [Medline].

Banka P, Bacha E, Powell AJ, Benavidez OJ, Geva T. Outcomes of inferior sinus venosus defect repair. J Thorac Cardiovasc Surg. 2011 Sep. 142(3):517-22. [Medline].

Black MD, Pike N, Tede N, Popper R. Video-enhanced repair of sinus venosus atrial defects: with/without anomalous pulmonary venous drainage. Heart Surg Forum. 2003. 6 S1:S28. [Medline].

Campbell M. Natural history of atrial septal defect. Br Heart J. 1970 Nov. 32(6):820-6. [Medline].

Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am. 1999 Apr. 46(2):355-68, x. [Medline].

Freed MD, Nadas AS, Norwood WI, Castaneda AR. Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?. J Am Coll Cardiol. 1984 Aug. 4(2):333-6. [Medline].

Fukazawa M, Fukushige J, Ueda K. Atrial septal defects in neonates with reference to spontaneous closure. Am Heart J. 1988 Jul. 116(1 Pt 1):123-7. [Medline].

Kyger ER 3rd, Frazier OH, Cooley DA, et al. Sinus venosus atrial septal defect: early and late results following closure in 109 patients. Ann Thorac Surg. 1978 Jan. 25(1):44-50. [Medline].

Li J, Al Zaghal AM, Anderson RH. The nature of the superior sinus venosus defect. Clin Anat. 1998. 11(5):349-52. [Medline].

Mas MS, Bricker JT. Clinical Physiology of Left-to-Right Shunts. Garson A, Bricker JT, McNamara DG, eds. The Science and Practice of Pediatric Cardiology. Lippincott Williams & Wilkins; 1990. Vol 2: 999-1001.

Murphy JG, Gersh BJ, McGoon MD, 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 Dec 13. 323(24):1645-50. [Medline].

Radzik D, Davignon A, van Doesburg N, et al. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol. 1993 Sep. 22(3):851-3. [Medline].

Sachweh JS, Daebritz SH, Hermanns B, et al. Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect. Ann Thorac Surg. 2006 Jan. 81(1):207-13. [Medline].

Walker RE, Mayer JE, Alexander ME, et al. Paucity of sinus node dysfunction following repair of sinus venosus defects in children. Am J Cardiol. 2001 May 15. 87(10):1223-6; A8. [Medline].

Gary M Satou, MD, FASE Director, Pediatric Echocardiography, Co-Director, Fetal Cardiology Program, Mattel Children’s Hospital; Associate Clinical Professor, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine

Gary M Satou, MD, FASE is a member of the following medical societies: American Academy of Pediatrics, Society of Pediatric Echocardiography, American College of Cardiology, American Heart Association, American Society of Echocardiography

Disclosure: Nothing to disclose.

Brian L Reemtsen, MD Assistant Professor of Cardiothoracic Surgery, Keck School of Medicine, University of Southern California

Brian L Reemtsen, MD is a member of the following medical societies: American Medical Association, Society of Thoracic Surgeons, Western Thoracic Surgical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Disclosure: Nothing to disclose.

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children’s Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: Abbott Medical .

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children’s National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, Society for Pediatric Research

Disclosure: Received research grant from: Medtronic.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Jeff L Myers, MD, PhD, and James Jaggers, MD, to the writing and development of this article.

Sinus Venosus Atrial Septal Defects

Research & References of Sinus Venosus Atrial Septal Defects|A&C Accounting And Tax Services