Ductal Stent and Cavo-Atrial Sac Occlusion in an Adult with Profound Cyanosis after Palliated Cyanotic Congenital Heart Disease

Interventional catheterizations are an increasingly important component in the management of the growing population of adults with congenital heart disease (CHD). The experience of interventions on adults with CHD from some centers has been reported and the types of procedures resemble those that are common in the pediatric population.^1,2
We report a unique case of a 35-year-old female with palliated complex who underwent two unusual interventions during the same catheterization procedure. The first, patent ductus arteriosus (PDA) stent placement, is an uncommon procedure that is performed almost exclusively in newborn infants as a short-term palliation. A Gianturco- Grifka vascular occlusion device (GGVOD) was also used to occlude a residual superior vena caval-to-right atrial shunt in a classic Glenn anastomosis, an intervention that has not been previously described in the literature.

Case Report. A 35-year-old female was referred to Cincinnati Children’s Hospital Medical Center with marked cyanosis and erythrocytosis with progressive, severe activity intolerance (NYHC III). As an infant, she was diagnosed with pulmonary atresia with an intact ventricular septum and at 2 years of age, she underwent a classic Glenn procedure. This procedure entailed an end-to-side anastomosis of the superior vena cava (SVC) to the right pulmonary artery (RPA). The native SVC right atrial (RA) junction was ligated, but not divided. She was lost to follow up until her presentation to our institution 33 years later.
Upon her initial evaluation with us, she was noted to be intensely cyanotic with marked clubbing of her fingers and toes. Her oxygen saturation on room air was 73%. Her hematocrit was 73%. An echocardiogram confirmed her diagnosis of pulmonary atresia with an intact ventricular septum. Her superior cavo-pulmonary anastomosis appeared widely patent. Her left pulmonary artery could not be visualized. Although the patient adamantly refused the option of further palliative surgery, she was amenable to undergoing cardiac catheterization for diagnostic and possible therapeutic intervention. She was scheduled for a diagnostic cardiac catheterization to determine the source and extent of the pulmonary blood flow to her left lung and to assess possible pulmonary arteriovenous malformations of the right lung.

Angiography demonstrated a 2 mm PDA that perfused a hypoplastic left pulmonary artery (LPA) (Figure 3). Angiography of the cavo-pulmonary anastomosis demonstrated recanalization of the RA-SVC ligation (Figure 1). This allowed additional desaturated SVC blood to return through the recanalized RA-SVC connection to the heart, and also was a cause of “steal” of blood flow to the RA. The additional desaturated blood in the RA crossed the patent foramen ovale (PFO) and mixed with the pulmonary venous blood in the left atrium (LA) and left ventricle (LV). The combination of increased desaturated blood return to the heart and underperfusion of the lungs resulted in profound systemic hypoxemia, beyond what would be expected from the surgical palliation.
In order to improve the flow of desaturated SVC blood to the RPA, a 9 mm GGVOD was loaded onto an 87.5 cm 7.5 Fr delivery catheter and was advanced into the portion of the SVC inferior to the take off of the right pulmonary artery. The Grifka Sac was opened in the inferior portion of the superior vena cava and the coils were advanced into the sac. Prior to release of the device from the delivery catheter, SVC angiograms were performed to assure that the device completely occluded the SVC-RA anastomosis without obstructing blood flow to the RPA. The sac was released from the delivery catheter, occluding the SVC-to-RA junction (Figure 2).
The ascending aortogram was studied and demonstrated the ductus arteriosus supplying the left pulmonary artery (LPA) (Figure 3). In order to increase flow to the LPA, a Palmaz® (P154) stent (Cordis Endovascular, Miami Lakes, Florida) was loaded onto a Tyshak^® Z-Med 8-2 mm balloon (Nu Med, Inc., Encino, California). The stent was expanded in the PDA until the balloon was inflated to the graded burst pressure. An angiogram was performed of the stented ductus (Figure 4), showing a widely patent stented ductus measuring 8.0 mm in diameter, without extravasation of contrast. The interventions were performed without any complications and the patient was admitted overnight for observation.
The patient is now 7 years post intervention. She has maintained oxygen saturations in the 90% range, has returned to work and maintained improved exercise capabilities.

Discussion. Due to the significant advances in the surgical and medical care of children with CHD, more patients are surviving to adulthood.³ In order to continue to manage these patients, more diagnostic and therapeutic catheterizations need to be performed in adults with CHD. Adults with CHD who undergo cardiac catheterization are likely to have complex disease with clinically relevant residual structural defects.² Several types of catheter interventions have been performed in this population. Among the most common of these interventions are aortic and pulmonary valvuloplasty, PDA coil embolization and device occlusion of atrial septal defects. The patient described in this report is unique because of the rarity of adult patients whose only palliation has been a classic Glenn shunt with residual underperfusion of the LPA from ductal stenosis and underperfusion of the RPA from a residual “steal”, and because the therapeutic interventions performed have not been described in combination in pediatric or adult patients with CHD.
The GGVOD was first described in canine models and was found to be effective in occluding vessels of varying calibers.⁴ Grifka et al later described successful use of the GGVOD in transcatheter occlusion of PDA and of aortopulmonary collateral vessels.^5,6 Subsequently, the GGVOD has been noted by other interventionalists to be of particular use in occluding short, large-caliber vessels that may otherwise be a challenge. The GGVOD has been used both alone⁷ and in combination with a stent⁸ to occlude left superior vena cavae draining to the left atrium. There have been previous reports of use of the GGVOD to eliminate residual postoperative shunts, such as occlusion of residual blood flow through Blalock-Taussig shunts.^9,10 However, we have described a novel use of the device and the first description of the use of any transcatheter technique to occlude a residual right superior vena cava-to-RA shunt in a cyanotic patient with a classic Glenn palliation. The residual shunt between the SVC-RPA anastomosis and the RA contributed significantly to this patient’s systemic hypoxemia by both a residual right-to-left shunt, as well as by “stealing” perfusion from the RPA. It posed an unusual and challenging problem in terms of how to occlude a short-segment, large-diameter vessel without obstructing flow to the nearby RPA. The GGVOD was an excellent option for this problem. Since the time of the intervention, other occlusion devices have become commercially available that would likely have been adequate for the intervention described, such as the Amplatzer Vascular Occlusion device or the Amplatzer Ductal Occluder Device (AGA Medical, Plymouth, Minnesota).
The use of intravascular stents for maintaining ductus arteriosus patency was first described in animal models, but initially suggested a high rate of acute and late complications.^11–14 Palmaz stents have been placed in PDAs of human neonates in order to maintain patency in ductaldependent circulations.^15,16 Serial evaluations of the stent patency revealed a significant restenosis rate.¹⁶ However, these cases were all performed in young infants with small-caliber PDAs. The adult patient described in our report had blood flow to her left pulmonary artery that depended on the presence of her ductal patency. Although her ductus arteriosus had remained patent for 35 years without any intervention, her left pulmonary artery flow was limited by the small size of her PDA. The stent was placed to improve her cyanosis by increasing the degree of her total pulmonary perfusion, rather than to establish a securely patent ductus without the use of prostaglandins. This has not been previously described as an indication for ductus arteriosus stenting, nor has ductal stenting in adult patients.
In summary, this case report describes two uncommonly implemented techniques for what we feel are unique indications. The fact that both procedures were performed during the same catheterization and in an adult patient with CHD adds to the unusual nature of the case.

References

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