Coronary Sinus Atrial Septal Defects Clinical Presentation

  • Author: Louis I Bezold, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Nov 29, 2011
 

History

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.

  • 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 dyspnea, cerebral abscess, transient ischemic attacks, and cyanosis.[1]
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Physical

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 disease.

  • The first heart sound is normal or split with an accentuated second 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 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 systolic murmur is noted in moderate-to-large shunts. The presence of a thrill suggests 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.
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Causes

  • The inheritance pattern of isolated coronary sinus ASDs is not known.
    • Splenic or heterotaxy syndromes, often associated with coronary sinus defects or absent coronary sinus, may have autosomal recessive, autosomal dominant, or X-linked inheritance patterns.
    • The familial recurrence rate for ASDs in general is highest for affected siblings, followed by mothers, and then fathers.
  • No specific risk factors or known teratogens have been associated with coronary sinus ASDs.
  • Isolated coronary sinus ASDs are typically not associated with other noncardiac syndromes or organ-system anomalies.
    • Coronary sinus ASDs may be observed in association with complex forms of congenital heart disease, most often in association with abnormalities of atrial situs and heterotaxy syndromes with polysplenia or asplenia.
    • No other specific genetic syndromes are known to be associated with coronary sinus ASDs.
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Contributor Information and Disclosures
Author

Louis I Bezold, MD  Associate Professor and Vice Chair of Inpatient Services, Department of Pediatrics, University of Kentucky College of Medicine; Chief, Division of Pediatric Cardiology, Medical Director, Kentucky 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, and Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and Society for Cardiac Angiography and Interventions

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  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

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

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

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