Published online February 1, 2007
PEDIATRICS Vol. 119 No. 2 February 2007, pp. 398-400 (doi:10.1542/peds.2006-2329)
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EXPERIENCE & REASON

Percutaneous Device Closure of Atrial Septal Defect in a Premature Infant With Rapid Improvement in Pulmonary Status

D. Scott Lim, MD and G. Paul Matherne, MD

Department of Pediatrics, University of Virginia, Charlottesville, Virginia

ABSTRACT

Atrial septal defects are a common congenital heart defect and may complicate the course of a premature infant by imposing volume overload to the lungs. Surgical closure requires cardiopulmonary bypass and, frequently, a midline sternotomy. Recently, percutaneous transcatheter devices were approved for atrial septal defect closure but have been limited to use in larger children. Here we present the first known report of a transcatheter device closure of an atrial septal defect in a premature infant, which resulted in rapid improvement of the patient's respiratory status.


Key Words: atrial septal defect • premature infant • cardiac catheterization

Abbreviations: ASD, atrial septal defect

Left-to-right shunting from an atrial septal defect (ASD) imposes a volume load on the right side of the heart and the pulmonary bed, which in premature infants may contribute to a more difficult pulmonary course, including prolonged ventilatory support. Here we present the case of an ASD complicating the course of an extremely low birth weight infant and subsequent percutaneous device closure of the ASD, with resultant clinical improvement.

CASE REPORTS

A 112-day-old infant, born at 23 weeks' gestation with a birth weight of 700 g, was referred to our institution for inability to wean from the ventilator and complicating congenital heart disease. The infant was a twin gestation, and the twin sibling subsequently died as a result of complications of extreme prematurity. A patent ductus arteriosus had been diagnosed earlier in the infant's course, as was an ASD. After failing medical management, the ductus arteriosus was ligated surgically. The infant had a prolonged period on high-frequency oscillator ventilator support and had failed to wean from the ventilator before transfer. At the time of transfer, the infant remained on moderate ventilator settings with significant hypercarbia and oxygen requirements, and a diagnosis of chronic lung disease was made (Fig 1A). It is notable that the infant had a previous history of intraventricular hemorrhage.


Figure 1
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FIGURE 1 A, Chest radiograph from the infant at presentation that demonstrates pulmonary vascular congestion with cardiomegaly. B, Chest radiograph 24 hours after the ASD device (black arrow) had been placed. There is significant clearing of the pulmonary vascular congestion and resolution of the cardiomegaly.

 
After discussion at a combined cardiac-surgical conference, it was recommended that the ASD be closed. Because of the complicating factors of severe lung disease and the previous intraventricular hemorrhage, the decision was made to take the infant to the catheterization laboratory for percutaneous ASD device occlusion, and informed consent was obtained from the infant's parents.

At the time of the procedure, the infant's weight was 2.3 kg. With the infant sedated and paralyzed, breathing 35% oxygen, femoral vascular access was obtained, and a right and antegrade left heart cardiac catheterization was performed. By oximetry, significant left-to-right shunting was seen, with a pulmonary/systemic flow ratio of >3, pulmonary hypertension, and pulmonary venous desaturation. In addition, there was elevated right ventricular diastolic pressure consistent with right heart failure from the volume overload. The infant received heparin (100 U/kg) and cefazolin (25 mg/kg) during the procedure. A 6F 22-cm Arrowflex sheath (Arrow International, Reading, MA) was shaped to approach the atrial septum in a perpendicular fashion and advanced from the femoral vein. Simultaneous transthoracic echocardiography demonstrated an unstretched diameter of the defect that measured 5.5 mm and left-to-right shunting (Fig 2A). A 6-mm Amplatzer septal occluder (AGA Medical, Golden Valley, MN) was chosen and deployed across the defect. With the delivery cable still attached to the device, significant caudal displacement of the atrial septum was noted. However, echocardiography demonstrated that the device was appropriately seated with atrial septal tissue captured between the left and right atrial parts of the device (Fig 2B). After release of the device from the delivery cable, the device and cardiac structures returned to a more normal orientation.


Figure 2
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FIGURE 2 A, Transthoracic echocardiogram with Doppler color flow before ASD device closure. Note the significant shunt (red jet) from the left to the right atria. B, The ASD device is shown in appropriate position on the atrial septum, occluding the ASD. It is important to note that the ASD device captured the atrial septal tissue between the 2 device disks.

 
Within 24 hours a chest radiograph demonstrated significant improvement in pulmonary congestion and normalization of the cardiomegaly (Fig 1B). The infant was able to be weaned rapidly from the ventilator, and he was extubated by postprocedure day 3.

DISCUSSION

To our knowledge, this is the first report of a percutaneous transcatheter device closure of an ASD in an extremely low birth weight infant with severe bronchopulmonary dysplasia. We reviewed the literature by a Medline search and found that the smallest previously reported patient undergoing such a procedure was a 7.8-kg 1.4-year-old infant.1 A single case of surgical closure in a premature infant with similar ventilator dependence has been reported2; however, given our patient's severe lung disease and previous history of intraventricular hemorrhage, we were reluctant to place the infant on cardiopulmonary bypass.

Transcatheter device closure of an ASD in such a small patient imposes certain technical challenges. Although our standard approach is to guide the device implantation with transesophageal echocardiography,3 the pediatric multiplane transesophageal probe (Siemens Medical, Malvern, PA) was felt to be too large to pass down the esophagus in this infant. Therefore, we performed simultaneous transthoracic echocardiographic imaging from the subcostal imaging plane using the ultrasound transducer in a sterile sleeve to guide the device delivery.

Approaching device deployment from an angle other than parallel with the atrial septum may lead to difficulties in seating the device because the left atrial component may prolapse through the defect. Although shaping the sheath may have assisted this, the straight, stiff delivery cable clearly pulled the atrial septum caudal and deformed the cardiac orientation in our patient. Although this was hemodynamically tolerated in our patient, it may not be so for future such patients. An alternate approach is to perform transhepatic access, which would allow a perpendicular sheath approach and a device delivery more parallel to the atrial septum.4

In children without concomitant lung disease, it is unusual to require closure of an ASD in infancy.5,6 However, it is possible that in a child with significant lung disease, the added burden of a left-to-right shunt may worsen the clinical status, as was the case with our patient. Although we do not recommend the routine closure of an ASD before 2 to 3 years of age, we feel that there may be certain patients with significant lung disease who would benefit from closure of their left-to-right shunts. However, these patients must be selected carefully, because the risks of the procedure, including perforation of the heart during device implantation, device erosion in the aorta, and interference with mitral and pulmonary venous flow, must be balanced against the benefits.

FOOTNOTES

Accepted Oct 3, 2006.

Address correspondence to D. Scott Lim, MD, Division of Pediatric Cardiology, University of Virginia, PO Box 800386, Charlottesville, VA 22901. E-mail: sl9pc{at}virginia.edu

The authors have indicated they have no financial relationships relevant to this article to disclose.

REFERENCES

  1. Bjornstad PG, Holmstrom H, Smevik B, Tonnessen TI, Fosse E. Transcatheter closure of atrial septal defects in the oval fossa: is the method applicable in small children? Cardiol Young. 2002;12 :352 –356[Web of Science][Medline]
  2. Motz R, Grassl G, Trawoger R. Dependence on a respiratory ventilator due to an atrial septal defect. Cardiol Young. 2000;10 :150 –152[Web of Science][Medline]
  3. Kleinman CS. Echocardiographic guidance of catheter-based treatments of atrial septal defect: transesophageal echocardiog-raphy remains the gold standard. Pediatr Cardiol. 2005;26 :128 –134[CrossRef][Web of Science][Medline]
  4. Shim D, Lloyd TR, Beekman RH 3rd. Transhepatic therapeutic cardiac catheterization: a new option for the pediatric interventionalist. Catheter Cardiovasc Interv. 1999;47 :41 –45[CrossRef][Web of Science][Medline]
  5. Mahoney LT, Truesdell SC, Krzmarzick TR, Lauer RM. Atrial septal defects that present in infancy. Am J Dis Child. 1986;140 :1115 –1118[Abstract/Free Full Text]
  6. Spangler JG, Feldt RH, Danielson GK. Secundum atrial septal defect encountered in infancy. J Thorac Cardiovasc Surg. 1976;71 :398 –401[Abstract]

PEDIATRICS (ISSN 1098-4275). ©2007 by the American Academy of Pediatrics

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This Article
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