Ostium Secundum Atrial Septal Defects.pdf

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9/9/2014 Ostium Secundum Atrial Septal Defects

http://emedicine.medscape.com/article/890991-overview

Ostium Secundum Atrial Septal Defects

Author: Ira H Gessner, MD; Chief Editor: Steven R Neish, MD, SM more...

Updated: Jun 17, 2013

Background

An ostium secundum atrial septal defect is an abnormally large opening in the atrial septum at the site of the foramenovale and the ostium secundum.

Embryology

In the early embryo heart, the atria comprise a common chamber. As the atria enlarge, the septum primum forms

and grows toward the developing atrioventricular canal area, which is later divided by the superior and inferiorendocardial cushions. Thesecushions fuse and bend with their convexity toward the atria, thereby approaching the

down-growing septum primum. This process continually narrows the passageway between the atria, which is then

defined as the ostium primum.[1]

The ostium primum completely closes; however, before this occurs, a central perforation appears in septum primum,

allowing continuous unrestricted flow from the right atrium to the left atrium. This perforation, the second opening inthe septum primum, is called ostium secundum. As the atria expand to either side of the truncus arteriosus, a fold is

produced within the atria just to the right of septum primum. This passively formed fold is septum secundum. Theleading edge of septum secundum is concave in shape and is called the foramen ovale. It comes to overlay the

ostium secundum but does not interfere with blood flow from right to left through ostium secundum. After birth, withonset of pulmonary blood flow and elevation of left atrial pressure, the septum primum is pushed against the septumsecundum, effectively closing the ostium secundum.

Fusion of the septum primum and the septum secundum closes the foramen ovale. Complete closure occurs in most

individuals. In 25-30% of normal adult hearts, however, a probe can be passed from the right atrium to the left atriumvia the foramen ovale and ostium secundum. This patent foramen ovale allows a tiny left-to-right shunt that can be

detected by sensitive techniques, such as color Doppler echocardiography, in 15-20% of adults.

Anatomy

A secundum atrial septal defect is usually bordered by the edge of the fossa ovalis and the exposed circumference

of ostium secundum. The shape of the defect varies from circular to oval. Less often, strands of tissue cross thedefect creating a fenestrated appearance that suggests multiple defects. Rarely, a defect can extend posteriorly and

inferiorly, approaching the site of inferior vena cava entrance into the right atrium.[2]

Pathophysiology

A secundum atrial septal defect can result from inadequate formation of the septum secundum so that it does notcompletely cover the ostium secundum. More often, the ostium secundum is excessively large because of increased

resorption so that septum secundum cannot cover it.

Natural history

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Although heart failure from secundum atrial septal defect rarely occurs in children, this complication can often occur

in adults. Adults also demonstrate a propensity for atrial dysrhythmias, including atrial flutter and fibrillation,presumably caused by chronic right atrial dilation. These complications may not be reversible if closure is delayed.

Data indicate that closure in persons older than 40 years does not reduce the risk of atrial dysrhythmia. Spontaneousclosure of a small secundum atrial septal defect does occur and is usually documented when the initial diagnosis is

made early in infancy.[3, 4]A moderate atrial septal defect may also decrease significantly in size or even close when

the defect is diagnosed early in life.[5] However, defects diagnosed later in childhood seldom decrease in size and

many significantly increase in size.[6]

The foramen ovale mechanism remains patent in at least 15% of adults (echocardiography can identify clinicallyinsignificant shunts with this frequency). Some of these individuals could be classified as having a small secundum

atrial septal defect. The presence of this phenomenon has been identified as a potential risk factor for stroke due to

embolization into the systemic arterial circulation.[7]

Although unusual, obstructive pulmonary vascular disease may occur in adults with an atrial septal defect.[8]

Epidemiology

Frequency

International

Isolated secundum atrial septal defects account for approximately 7% of congenital cardiac defects. Congenital heart

defects of significance occur in approximately 8 per 1000 live births. Therefore, 5-6 cases of secundum atrial septaldefect occur per 10,000 live births. This number refers only to defects that are large enough to come to clinical

attention. Many small defects that remain undetected occur in addition to numerous cases of patent foramen ovale,as mentioned above.

Mortality/Morbidity

An isolated secundum atrial septal defect very seldom causes significant symptoms in pediatric patients, regardless

of defect size.

Rarely, an infant may develop congestive heart failure in the presence of a secundum atrial septal defect.

Whether the defect alone is responsible for causing heart failure is not well established, although it certainlyadds to the patient's hemodynamic difficulties. Failure to thrivecaused only by a secundum atrial septal

defect is, similarly, a rare occurrence.Bacterial endocarditisis not a risk with this lesion and theAmerican Heart Association does not advise use of

antibiotic prophylaxis.No data indicate that an uncomplicated secundum atrial septal defect can cause pulmonary vascular disease

in pediatric patients.A patient with primary pulmonary hypertension may demonstrate a secundum atrial septal defect, but no

cause-and-effect relationship can be established.Patients with a significant secundum atrial septal defect who live at an altitude higher than 4000 feet may

incur a small risk of developing pulmonary vascular disease.Death from an isolated secundum atrial septal defect does not occur in pediatric patients.

Race

No significant racial influences have been identified.

Sex

Secundum atrial septal defect occurs more commonly in females than males, with a female-to-male ratio of 2:1.

Age

Secundum atrial septal defect is a congenital abnormality and, therefore, is present at birth. It may be diagnosed at

any age, including early infancy, although the characteristic findings on physical examination often escape detection
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until the patient reaches a few years of age. An acquired defect occurs only iatrogenically.

Contributor Information and DisclosuresAuthorIra H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies:American Academy of Pediatrics,American

College of Cardiology,American Heart Association,American Pediatric Society, and Society for PediatricResearch

Disclosure: Nothing to disclose.

Specialty Editor BoardPaul 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, ArkansasChildren's Hospital

Paul M Seib, MD is a member of the following medical societies:American Academy of Pediatrics,AmericanCollege of Cardiology,American Heart Association,Arkansas Medical Society, International Society for Heart

and Lung Transplantation, and Society for Cardiac Angiography and Interventions


Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of

Pharmacy; Editor-in-Chief, Medscape Drug Reference


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 ofScience,American Heart Association, and Society for Developmental Biology


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


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, andAmerican Heart Association


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