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Coronary Sinus Atrial Septal Defects Clinical Presentation

  • Author: Louis I Bezold, MD; Chief Editor: P Syamasundar Rao, MD  more...
Updated: Mar 30, 2016


Patients with atrial septal defects (ASDs) may be recognized during infancy; however, ASDs are often not diagnosed until later in childhood because of lack of symptoms and subtlety of physical signs. Most patients undergo their initial evaluation because a cardiac murmur is detected. On the contrary, frequent use of echocardiography has resulted in identification of some of these ASDs.

  • Small and even moderate-to-large coronary sinus ASDs usually result in no clinically significant symptoms in childhood. However, on occasion, even infants develop clinically important symptoms of congestive heart failure, generally in conjunction with additional contributing factors.
  • Failure to thrive because of an ASD alone rarely, if ever, occurs.
  • Mild exercise intolerance, frequent respiratory infections, or reactive airway disease may be observed in some patients.
  • Patients with other associated complex congenital cardiac abnormalities generally present earlier because of their other hemodynamically significant associated defects.
  • In a surgical series of 25 patients with partial coronary sinus fenestrations, 7 had signs or symptoms at least partially attributable to these defects, including cerebral abscess, transient ischemic attacks, and cyanosis. [2]

Physical Examination

Relatively normal precordial impulse may be present with small defects. A precordial bulge, main pulmonary artery impulse, or hyperdynamic right ventricular impulse (heave) occurs with large shunts, especially in thin patients. A right ventricular tap or particularly prominent main pulmonary artery impulse in the second left intercostal space suggests pulmonary hypertension. Cyanosis may occur with pulmonary vascular obstructive disease.

The first heart sound is normal or split with an accentuated pulmonary component. The loud second component is caused by a larger-than-normal excursion of tricuspid valve leaflets during contraction of the volume-loaded right ventricle. The second heart sound is characteristically widely split and fixed in regard to respiration (except in small left-to-right shunts), with the aortic and pulmonic components widely and constantly separated. This separation changes little with inspiration or with Valsalva maneuver. Narrow splitting and increased intensity of the pulmonic component of S2 suggests the onset of elevated pulmonary vascular resistance.

Normal splitting of the second heart sound is due to an inspiratory increase in the interval between the descending limbs of the pulmonary arterial and right ventricular pressure curves reflecting increased pulmonary vascular bed capacitance. In the patient with ASD, the overall pulmonary vascular bed capacitance is already increased, with no additional increase during inspiration. Furthermore, the inspiratory increase in systemic venous return approximately compensates for the diminution of left-to-right shunting. The net result is that little respiratory variation occurs in right and left ventricular filling.

A pulmonary ejection systolic murmur is noted in moderate-to-large shunts. The presence of a thrill is unusual in ASDs and may suggest pulmonary valve stenosis. A mid diastolic low-frequency tricuspid valve inflow murmur may be heard with Qp/Qs ratios of greater than approximately 2 to 1, and a low-pitched pulmonary regurgitation murmur may be present. A higher-pitched pulmonary regurgitation murmur suggests pulmonary hypertension.

Contributor Information and Disclosures

Louis I Bezold, MD Professor, Department of Pediatrics, Ohio State University College of Medicine; Director, Cardiology Consultation Service, Nationwide Children's Hospital

Louis I Bezold, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

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