Development of Aortic Coarctation Following Device Closure of Patent Ductus Arteriosus

Abstract: A patient who had transcatheter closure of a large patent ductus arteriosus in early infancy developed aortic coarctation during follow-up. Initially, balloon angioplasty and subsequent stent implantation successfully relieved the aortic obstruction. Avoidance of use of large devices in small babies and modification of current devices so that they do not cause aortic obstruction may result in better outcomes. Once aortic obstruction develops, transcatheter management is useful in relieving the obstruction.  

J INVASIVE CARDIOL 2013;25(9):464-467

Key words: patent ductus arteriosus, coarctation of aorta, Gianturco-Grifka vascular occlude device, coarctation stent therapy

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Percutaneous occlusion of patent ductus arteriosus (PDA) is well described in literature and is currently widely used in clinical practice. It has been found to be a safe and effective method to close PDA.^1,2 We report a patient who had percutaneous transcatheter closure of large PDA at 4 months of age at another institution using the Gianturco-Grifka Vascular Occlude Device (GGVOD; Cook, Inc). She progressively developed coarctation of the aorta. The patient underwent balloon angioplasty of coarctation at 6 years of age at our institution with improvement. The degree of aortic obstruction became worse by the age of 12 years and was relieved by placement of a Palmaz-Genesis stent (Johnson & Johnson) across the coarctation segment, with excellent results. This case illustrates risk for development of coarctation with transcatheter PDA closure, mainly with older devices such as the GGVOD. 

Case Report

A 5-year-old, 16.3 kg female with trisomy 21 was seen in the outpatient clinic at our institution. She had history of a large PDA, which was closed with a GGVOD at 4 months of age at another institution. Following closure of the PDA with GGVOD, she was reported to have very mild gradient in the isthmus region of the descending aorta, which was deemed clinically insignificant. Later, at 5 years of age, her care was transferred to our institution. 

During evaluation at 6 years of age (weight, 18.9 kg; 25th percentile), the patient was noted to have blood pressure gradient of 20 mm Hg between upper and lower extremities, a grade 2/6 ejection systolic murmur in the left interscapular area, and mild brachial-femoral pulse delay. Echocardiogram showed peak instantaneous gradient of 44 mm Hg across the descending aortic narrowing. The narrowest aortic segment measured 3.6 mm and there was dampened pulse trace in the abdominal aortic Doppler. Cardiac catheterization revealed a peak-to-peak systolic pressure gradient of 20 mm Hg with dampened descending aortic pressure tracings. Angiogram showed a narrowest aortic segment of 4 mm and the descending aorta diameter at the level of the diaphragm was 9 mm. In lateral cineangiographic view, there was clear-cut encroachment of the aortic lumen by the GGVOD coils. Based on these data, an 8-mm diameter, 2-cm long Tyshak II balloon catheter (NuMed) was used for angioplasty; three balloon inflations were performed. Repeat angiogram after balloon angioplasty showed mild angiographic improvement, with a residual gradient of 10 mm Hg across the site of the narrowing (Table 1). Doppler gradient across the coarctation was 36 mm Hg on the day following the procedure and 20 mm Hg at 1-month follow-up exam; there was no diastolic extension of the Doppler flow signal. The patient was followed in the outpatient clinic periodically (Table 1).      

At 12 years of age, the patient was noted to have peak instantaneous gradient of 60 mm Hg across the coarctation, with diastolic extension of Doppler flow. Review of Doppler data at prior clinic visits (Table 1) revealed rapid increase in Doppler gradients between 4 and 5 years after balloon angioplasty. At this time, cardiac catheterization was performed to evaluate the aortic obstruction and to consider the possibility of additional catheter intervention. During this study, there was a peak-to-peak systolic gradient of 30 mm Hg (Figure 1A) across the site of the GGVOD coils (Figure 2) with low pulse pressure in the descending aorta tracing (Figure 1A). The narrowest aortic segment at the level of the GGVOD measured 3.5 mm, the descending aorta distal to the left subclavian artery measured 10 mm, the descending aorta distal to the narrowing was 11.5 mm, and the descending aorta at the level of the diaphragm was 10.3 mm. The GGVOD device was again noted to protrude into the aorta (Figure 2), which was particularly evident in the lateral view (Figure 2B). There was also a small posterior shelf at the level of the device. Based on these data, a 27-mm long Palmaz-Genesis stent mounted over a 6 mm x 2 cm / 12 mm x 3 cm balloon-in-balloon (BIB) catheter (Figure 3) was implanted across the narrowed aortic segment and the isthmus, with resultant improvement (Figure 4). Care was taken to avoid stenting the origin of the left subclavian artery (Figure 4B). There was less than 5 mm Hg residual gradient across the stent at the conclusion of the procedure (Figure 1B). Follow-up exams at 1 day and at 1, 6, 24, and 36 months after the procedure revealed normal blood pressures and no arm-leg blood pressure gradient (108/64 mm Hg in the right arm and 106/62 mm Hg in the right leg) and no echocardiographic evidence of recoarctation (Table 1). 

Discussion

Transcatheter closure of PDA was first performed by Porstmann and his associates in 1967. A number of PDA closure devices were subsequently described, and are reviewed elsewhere.^2,3 However, only a few devices are approved by the United States Food and Drug Administration for transcatheter PDA closure; these include Gianturco coil, GGVOD, and the Amplatzer Duct Occluder (ADO). The device type and size are best individualized based on the size (minimal ductal diameter) and probably the shape of the ductus.^1,2 Although considered safe, there are potential complications associated with this procedure, such as device embolization, residual shunts, left pulmonary artery obstruction, development of aortic coarctation, and vascular injury. With the GGVOD, immediate residual shunts were seen in 9% of patients, which completely closed during follow-up.^4,5 In the GGVOD series reported,^4,5 no aortic obstruction was mentioned.

Our patient had closure of the PDA with GGVOD in early infancy. Although the patient was reported to have mild gradient after PDA closure, it was felt to be insignificant by the cardiologist performing the procedure. During follow-up with us, we noted the development of significant aortic coarctation. It is not clear whether what was considered insignificant gradient developed into full-fledged coarctation later or if encroachment of the aortic lumen by the GGVOD device components (Figure 2) caused this problem. Or, it may be a combination of the above. Given the significant protrusion of the device components into the aortic lumen (Figure 2), we may have to conclude that the bulky device is responsible for the development of coarctation in our patient.

Use of large devices in small babies may result in aortic obstruction. In a recent study,⁶ outcomes of transcatheter occlusion of PDA in infants weighing ≤6 kg were examined. The authors successfully implanted a PDA occlusion device in 58 of 62 patients (94%). During follow-up, 2 patients had Doppler flow acceleration in the descending aorta, suggestive of coarctation. One patient had the PDA occluded with a Gianturco coil and the other with an ADO. These authors suggest that ADO should be the primary therapy for symptomatic babies weighing less than 6 kg. However, other workers reported aortic obstruction associated with implantation of an ADO to close PDAs.^7,8 Further modifications of the ADO device (angulated nitinol plug, double-disc design), resulted in the ADO II device, which is undergoing active investigation to resolve the aortic obstruction issue. Modification of current devices so they might not cause aortic obstruction may result in better outcomes.

When we detected aortic coarctation at 6 years of age, we performed less invasive balloon angioplasty because of growth issues related to stents in younger children. Improvement with mild residual gradient (10 mm Hg) and lack of systemic hypertension led us to advise periodic clinical follow-up. When the patient developed significant obstruction at 12 years of age (weight, 40 kg), we went ahead and implanted a stent across the narrowed aortic segment, relieving the obstruction (Figure 4). During a 3-year follow-up exam after stent placement, no recurrence was detected. Longer-term follow-up may result in development of obstruction, when dilatation of the stent may be required.

In the presence of both PDA and aortic coarctation, it is generally recommended that both are managed through either surgery or catheter intervention that can address both the issues. However, in some cases, the coarctation gradient may be fictitious secondary to increased flow across the aortic isthmus.¹¹ If the coarctation is fictitious, the gradient will be abolished by test occlusion of the ductus and surgery will be avoided.¹²

Conclusion

We report a case of aortic coarctation that developed after GGVOD occlusion of the PDA that is believed to be due to a bulky device encroaching into the lumen of the aorta. The patient had complete resolution of the coarctation after transcatheter stent implantation. Modification of existing devices so that they might not cause aortic obstruction and avoidance of large device use in small babies may improve outcomes of PDA closure.

References

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