Severe Intravascular Hemolysis after Transcatheter Coil Occlusion of Patent Ductus Arteriosus

Since the initial description in late 1960s by Porstmann and associates^1,2 of a patent ductus arteriosus (PDA) occluding device, a number of other devices have been studied, as reviewed elsewhere.^3,4 Transcatheter closure of PDA using various devices and coils^5–14 is now an established practice in most cardiac centers. These techniques have proven to be safe and cost-effective.^14–16 Intravascular hemolysis secondary to coil occlusion of PDA is a rare complication.^17–19 It is thought to be related to a high-velocity blood flow jet from the residual shunt of a partially closed PDA. However, since only a minority of patients with residual shunts develop intravascular hemolysis, there may be other factors that contribute to its occurrence. We present a case of severe hemolysis after coil occlusion of the PDA in which the Dacron material was inadvertently stripped of the coil during its deployment, and may have contributed to the development of the hemolysis. Case Report. A 14-month-old female child was referred for transcatheter closure of a PDA. She was asymptomatic, but had a grade II to III/VI continuous murmur in the left upper sternal border and left infraclavicular region. An echocardiogram confirmed the presence of a moderate-sized ductus. There was mild enlargement of the left atrium and ventricle and Doppler-estimated pulmonary artery pressure was normal. There were no other associated cardiac or extracardiac defects. Cardiac catheterization revealed normal right and left heart pressures, with the exception of wide pulse pressure (100/45 mmHg) in the aorta. There was a step-up in oxygen saturation in the main pulmonary artery (MPA) and branch pulmonary arteries, with a calculated pulmonary-to-systemic blood flow ratio (Qp:Qs) of 1.5:1. Systemic arterial oxygen saturations were normal. An aortogram (30° right anterior oblique and straight lateral projections) using a 4 French (Fr) marker pigtail catheter showed a PDA with good opacification of the main and branch pulmonary arteries, consistent with a moderate-sized shunt (Figure 1). The ductus, at its narrowest diameter, measured 2.5 mm, the ampulla was 9 mm wide and the length of the ductus was 7 mm. There was no evidence of aortic coarctation. Based on these values, a 0.052 inch, 5 mm loop, 8 cm long Gianturco coil (Cook Cardiology, Bloomington, Indiana) was selected for implantation using bioptome-assisted coil delivery.20 Using the retrograde transfemoral arterial approach, a 4 Fr right coronary artery (RCA) Judkins catheter was advanced from the descending thoracic aorta into the MPA across the PDA with the help of a 0.035 inch soft/straight-tipped guidewire. The wire was changed for a 0.025 inch J-shaped wire, and the RCA catheter and the short sheath were removed and replaced with a 4 Fr, long, blue Cook® sheath that was also placed across the PDA into the MPA. At this point, a 3 Fr bioptome was advanced through a coil loader (Cook®) and the bioptome tips were used to capture the previously separated ball of the 0.052 inch coil. The coil was then withdrawn into the loader and was inserted into the back end of the 4 Fr long sheath, and the coil was advanced toward the distal end of the sheath with the bioptome. The coil was successfully delivered across the ductus using standard technique, with one loop in the pulmonary artery and the remaining loops in the ductal ampulla. An aortogram 15 minutes following coil placement demonstrated good coil position and only a small residual shunt at the upper border of the coil. In an attempt to close this, the RCA catheter was repositioned cross the PDA, but in the process, the initial coil was dislodged and embolized into the right lung. This was retrieved successfully using a 15 mm gooseneck snare (Microneva®) from the femoral venous approach without complications. Using the previously described technique, another 0.052 inch coil was placed across the ductus. A postprocedure angiogram showed a small residual shunt at the superior border of the coil (Figure 2). The residual shunt was not addressed because of concern of dislodgement of the initially placed coil. At the conclusion of the procedure, a significant amount of Dacron material (presumably stripped off the coil during advancement into the sheath with the bioptome) was found in the hub of the sheath. The patient’s immediate postprocedure course was unremarkable. However, five weeks later, at a regular follow-up visit, the patient was found to have a hemoglobin of 5.8 g/dl, but there were no symptoms. Her clinical picture was typical for intravascular hemolysis, with a low haptoglobin ( Discussion. Transcatheter occlusion of PDA has a very low rate of morbidity and mortality.^9,10,21,22 Complications can result from device protrusion, causing obstruction of a branch pulmonary artery or thoracic aorta, as well as embolization of the device into the pulmonary or systemic circulation.¹⁰ Severe hemolysis is a rare complication, though it has been reported with both the Rashkind umbrella and with coils. The European registry for transcatheter occlusion of PDA reported an incidence of intravascular hemolysis of 0.5% (4 of 651 patients) with the Rashkind device.¹⁰ Hosking et al.⁹ reported significant hemolysis in 2 of 186 patients (1%), and Ali Khan et al.²³ also found a similar incidence (0.5%) among their 174 patients who underwent PDA closure with a Rashkind device. Transcatheter coil occlusion of PDA also has a similarly low incidence of hemolysis. The PDA coil registry collected information on 535 patients from 38 centers and reported acute and follow-up data.^6,22 This registry reported no cases of intravascular hemolysis. Tomita et al.¹⁹ reported 5 cases of significant hemolysis in their survey of 218 patients who had PDA coil occlusion (1 patient had both a coil and a Rashkind umbrella). There are also several case reports documenting hemolysis.^17,18,24,25 Most of the reported cases had early hemolysis, whereas our patient developed hemolysis several weeks after the procedure. The proposed mechanism for intravascular hemolysis invokes damage to RBC membranes from a high-velocity jet (the residual shunt) impacting the prosthetic device/coil, a mechanism similar to that seen with perivalvular leaks in patients with mechanical cardiac valves.^{26, 27} It has been documented in an experimental setting that such high-velocity jets can produce shear forces in the order of 300 dynes per cm2, which can damage RBCs.^28,29 In support of the above mechanism is the fact that severe intravascular hemolysis after PDA closure occurs almost exclusively in patients with a residual shunt (though rarely, mild subclinical hemolysis has been reported with a completely occluded PDA³⁰). The incidence of a residual shunt within 24 hours of the procedure ranges from 30–57%.^{9,10,22,23,31,32} However, since most instances of intravascular hemolysis present within 24 hours of the procedure,^{17,19,25,30,33} these represent only a very small fraction of patients with a residual PDA shunt. Thus, there must be other factors that increase the risk of manifest hemolysis in the setting of a residual leak. Tomita et al.¹⁹ noted that compared to patients without intravascular hemolysis, the index cases had larger residual shunts and a smaller coil-to-ductal diameter ratio (ratio of the sum of the loop diameters of the implanted coils to the minimal diameter of the ductus): 2.2 ± 0.4 versus 3.1 ± 1.1. The shape and size of the ductus were not significant risk factors in this study. The Gianturco coils feature a mesh of Dacron strands on their surface that not only promote thrombosis, but also may help decrease the residual shunt jet velocity by slowing/trapping the RBCs in its strand network and dispersing the stream. However in the case described here, we noted a significant amount of Dacron material at the hub of the sheath at the end of the procedure. It was likely stripped off the coil while the coil was being loaded into the sheath. This, we believe, decreased the chances of shunt closure and also exposed the RBCs in the residual jet directly to the metal framework of the coils, resulting in accentuated trauma to these cells. We were unable to find any similar cases of Dacron stripping off the coils in the literature, thus it is difficult to comment on the incidence of such an event and the magnitude of its importance in the causation of coil-associated hemolysis. Also, the technique of bioptome-assisted coil placement, though well described and popular, may contribute to coil damage during deployment. Management of intravascular hemolysis in these situations also calls for additional thought. Even though a good percentage of residual shunts resolve over a period of time, shunts significant enough to cause severe hemolysis only rarely close spontaneously.^23,34 Transcatheter techniques involving placement of another coil or device are almost always successful in closing off the residual shunt^17,19,25,35 with minimum morbidity. Thus, surgical closure should not routinely be the first strategy choice. Conventionally, surgical technique has involved removal of the previously placed device and then ligation of the ductus. This often necessitates cross-clamping of the aorta. Chisholm et al. used a modified technique whereby they ligated the residual ductus over the previously deployed Rashkind double umbrella without the need for aortic cross-clamping³⁶ or device removal, thus simplifying the procedure. Despite this, our recommendation is coil occlusion of the residual shunt. In the absence of hemolysis, the management of a residual shunt could be more conservative (unless it is hemodynamically significant) for two reasons. First, a large percentage of these shunts will close spontaneously. Second, there is a chance, albeit small, of dislodgement (as in our case) of the first coil during deployment of the second coil.¹⁷ Thus, waiting several months to see if the residual shunt spontaneously resolves should be considered. There remains a small risk of endarteritis which mandates antibiotic prophylaxis.

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