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Pediatric Atrial Septal Defects Follow-up

  • Author: Michael R Carr, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Jan 17, 2014
 

Further Outpatient Care

Provide routine medical care with special attention to signs of congestive heart failure (CHF) or increased pulmonary vascular resistance (PVR). Most patients with an atrial septal defect are asymptomatic and require only routine well-child care until they undergo elective surgical repair or transcatheter device placement for their defects.

Most children with uncomplicated atrial septal defects are followed up by their primary care provider and receive follow-up with a pediatric cardiologist every year or every other year. Children who require medical intervention or who have other comorbidities are seen by a cardiologist more frequently than this.

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Further Inpatient Care

Please refer to the sections regarding the specific subtypes of atrial septal defect (ASD) for potential inpatient issues.

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Transfer

An isolated atrial septal defect almost never causes clinically significant problems in the neonatal period or in infancy. Refer a child who is to have elective atrial septal defect surgical repair or transcatheter intervention to a pediatric center with experience in performing cardiopulmonary bypass and surgical atrial septal defect closure or catheter based procedures in young children.

A patient with an ostium primum atrial septal defect may have associated clinically significant AV valve insufficiency and may require earlier surgical intervention. Refer this patient to a center with experience in the evaluation and repair of this problem.

Any attempt at closure with a transcatheter device should be performed at a center with experience in pediatric interventional cardiology with surgical support. Additionally, since some atrial septal defects are detected outside of childhood, transcatheter interventions in adults are often performed by adult interventionalists with the assistance of a pediatric interventionalist. Adult interventionalists may be very comfortable with transcatheter patent foramen ovale closure, but not as familiar with the nuances of true atrial septal defectdevice closure, especially larger defects.

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Complications

Atrial septal defect is usually an asymptomatic disease. However, children with atrial septal defects are at increased risk for several complications, such as endocarditis (if associated mitral valve insufficiency is present) and respiratory tract infections, which are less well tolerated in children with atrial septal defects than in children without atrial septal defects. Any individual with an atrial level shunt is at risk for a paradoxical embolus from a venous thrombus, but in children, this is exceedingly rare, unless there is an underlying hypercoagulable state.

Children with clinically significant and untreated atrial septal defects are at risk for various cardiac complications, including CHF, pulmonary hypertension, and arrhythmias. However, these cardiac complications generally manifest in adulthood.

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Prognosis

The prognosis for a child with an atrial septal defect is good; the rate of surgical mortality is less than 1%. Many children are candidates for catheter-based device implantation, which also carries a very low procedural morbidity and mortality and avoids the risks associated with a median sternotomy and cardiopulmonary bypass.

Ostium secundum defects may spontaneously close. A wide range of spontaneous closure rates have been reported among different studies, ranging from 4-87%. The likelihood of spontaneous closure appears to be closely related to the initial size of the defect. One study demonstrated a 56% spontaneous closure rate and 30% regression to a diameter of less than 3 mm for defects 4-5 mm in diameter. Conversely, none of the defects more than 10 mm in diameter closed spontaneously, and 77% of those required intervention. The general thought is that spontaneous closure does not occur with ostium primum, sinus venosus, or coronary sinus defects.[19, 20]

Certain patients with ostium primum atrial septal defects and an abnormal mitral valves may require a second operation for mitral valve dysfunction later in their lives.

The repair of sinus venosus atrial septal defects can be more complex and often involves baffling of the right upper pulmonary vein to the left atrium and anastomosis of the superior vena cava to the right atrial appendage. Stenosis of the right upper pulmonary vein baffle or superior vena cava/atrial appendage anastomotic site may require further catheter-based or surgical intervention.[21]

Endocarditis of catheter-placed devices has been reported and may necessitate removal of the hardware and surgical repair.

Surgical or catheter-based intervention of atrial septal defects in individuals outside of childhood is generally feasible, even in the face of pulmonary vascular changes and evidence of pulmonary hypertension. This is distinctly different from similar aged individuals with moderate-to-large post-tricuspid shunts (ventricular septal defect, aortopulmonary window, patent ductus arteriosus, or major aortopulmonary collateral vessels), who often have markedly elevated pulmonary vascular resistance and Eisenmenger physiology. However, when compared with earlier timing of intervention, some evidence suggests that patients repaired later in life have higher longer-term morbidity.[22]

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Patient Education

Focus patient education on ensuring that the family and caregivers understand potentially serious symptoms so that they seek prompt medical attention when necessary. However, parents also require consistent education regarding long-term prognosis, which is generally quite good, as well as the expected signs and symptoms that can be seen with the defect, which are usually minimal.

Reassurance is often needed due to the stigmata associated with the diagnosis of congenital heart disease (CHD). Some children may be unnecessarily restricted from activity by well-meaning medical personnel or by over-cautious parents.

Education regarding care of an atrial septal defect and its complications should also include input from the cardiologist and cardiac surgeon.

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Contributor Information and Disclosures
Author

Michael R Carr, MD Pediatric Cardiologist, Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine

Michael R Carr, 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

Disclosure: Nothing to disclose.

Coauthor(s)

Brent R King, MD, MMM Clive, Nancy, and Pierce Runnells Distinguished Professor of Emergency Medicine, Professor of Pediatrics, University of Texas Health Science Center at Houston; Chair, Department of Emergency Medicine, Chief of Emergency Services, Memorial Hermann Hospital and LBJ Hospital

Brent R King, MD, MMM is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, American College of Emergency Physicians, American Association for Physician Leadership, Society for Academic Emergency Medicine

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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Subcostal echocardiographic view of a child with a secundum atrial septal defect (ASD). Note the position of the defect in the atrial septum. RA = Right atrium; LA = Left atrium; SVC = Superior vena cava.
Subcostal long-axis view of the same child as in the previous image with a secundum atrial septal defect (ASD). RA = Right atrium; LA = Left atrium; RUPV = Right upper pulmonary vein.
Parasternal short axis view of a child with a secundum atrial septal defect (ASD). RA = Right atrium; LA = Left atrium; AO = Aorta.
Apical echocardiographic view of a primum atrial septal defect (ASD). Note the position of the defect when compared with a secundum ASD. RA = Right atrium; LA = Left atrium; RV = Right ventricle; LV = Left ventricle.
Apical echocardiographic view of a primum atrial septal defect (ASD). Note that the atrioventricular valves are at the same level (instead of mild apical displacement of the tricuspid valve), which is seen in the spectrum of atrioventricular canal defects. RA = Right atrium; LA = Left atrium; RV = Right ventricle; LV = Left ventricle.
Apical color Doppler echocardiographic view of a primum atrial septal defect (ASD). Note the flow across the defect from the left atrium to the right atrium (RA), and note the mitral regurgitation (MR) through a cleft in the anterior leaflet of the mitral valve. MV = Mitral valve; LV = Left ventricle.
Subcostal short-axis view of a child with a sinus venosus atrial septal defect (ASD). Note the position of the defect compared with that of a secundum or primum ASD. Also note the anomalous position of the right upper pulmonary vein (RUPV). RA = Right atrium; LA = Left atrium.
ECGs from a child with a secundum atrial septal defect (ASD). Note the right-axis deviation and rSR' pattern in lead V1.
ECG from a child with a primum atrial septal defect (ASD). Note the left-axis deviation with a counterclockwise vector of depolarization (small q waves in leads I and aVL) and right ventricular hypertrophy and/or volume overload (rSR' pattern and upright T wave in lead V1).
 
 
 
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