Severe Intravascular Hemolysis After TranscatheterClosure of a Large Patent Ductus Arteriosus Using the

Amplatzer Duct Occluder: Successful Resolution byIntradevice Coil Deployment

George Joseph,* MD, Asishkumar Mandalay, MD, T.U. Zacharias, MD, and Biju George, MD

A 21-year-old female developed severe unremitting intravascular hemolysis followingclosure of a large patent ductus arteriosus using an Amplatzer duct occluder. Percuta-neous deployment of fibered platinum coils within the nitinol wire cage of the Amplatzerduct occluder abolished the residual shunt through the device and resulted in cessationof intravascular hemolysis and reversal of its adverse sequelae. Cathet CardiovascIntervent 2002;55:245–249. © 2002 Wiley-Liss, Inc.

Key words: congenital heart disease; interventional cardiac catheterization

INTRODUCTION

Intravascular hemolysis is a well-known though un-common complication of transcatheter closure of patentductus arteriosus (PDA) and has been reported after ductocclusion using the Rashkind occluder [1,2], emboliza-tion coils [3–6], and, more recently, the Amplatzer ductoccluder (ADO; AGA Medical, Golden Valley, MN)[7,8]. Hemolysis after percutaneous closure of PDAs isalways associated with the presence of residual ductalflow [9], and the likely mechanism of hemolysis is high-velocity turbulent blood flow past the ductal device pro-ducing mechanical fragmentation of erythrocytes. Reme-dial efforts in this situation have therefore been directedtoward achieving complete closure of the PDA, either bydeploying additional devices alongside the original de-vice in the PDA or by surgery [10]. Hemolysis related toresidual shunting following ADO deployment is a diffi-cult therapeutic problem, because the appropriately sizedand correctly deployed ADO completely occupies thelumen of the ductus, and residual shunting occursthrough the device and not between the device and theductal wall. In the only case reported thus far of severehemolysis following deployment of the ADO, residualshunting in an 11-month-old boy was abolished by tem-porary balloon occlusion of the aortic ampulla [7]. Othertechniques are required to address residual shunting afterADO deployment in adult patients in whom the aorta isdilated because of large ductal flow and/or concomitantaortic valve disease. We present the case of an adultpatient with a large PDA and dilated aorta who devel-oped severe intravascular hemolysis after ADO deploy-

ment related to residual shunting through the device.Successful resolution of the hemolysis and residualshunting was obtained by intradevice coil deployment.

CASE REPORT

A 21-year-old female known to have a heart murmurfrom childhood presented with exertional palpitations.Physical examination revealed a wide pulse pressure, leftventricular third heart sound, and a continuous murmurin the pulmonary area with eddy sounds. The chest X-raydisclosed cardiomegaly with left ventricular configura-tion, prominent pulmonary conus, and pulmonary pleth-ora. Echocardiography showed a large PDA, mild aorticvalve stenosis and regurgitation, and intact atrial andventricular septae. Cardiac catheterization (Table I) andangiography revealed a large-flow, short-length, type B2ductus [11] of minimum diameter 6.8 mm and confirmedthe echocardiographic findings. Transcatheter duct occlu-sion was performed in the same sitting by deploying a14-12 mm ADO (Fig. 1) using mild sedation, antibioticprophylaxis, and standard antegrade venous approach[8,12]. A device that was at least 4 mm larger than the

Departments of Cardiology and Clinical Hematology, ChristianMedical College Hospital, Vellore, India

*Correspondence to: Dr. George Joseph, Department of Cardiology,Christian Medical College Hospital, Vellore 632 004, India.E-mail: cardio1@cmcvellore.ac.in

Received 3 April 2001; Revision accepted 23 August 2001

Catheterization and Cardiovascular Interventions 55:245–249 (2002)

© 2002 Wiley-Liss, Inc.DOI 10.1002/ccd.10046

minimal ductal diameter was selected because devicestability was a concern in this large ductus, and overlapbetween the large aorta and pulmonary artery made siz-ing of the ductus by angiography imprecise. Loading theADO and delivering it to the ductal location via an 8 Frlong sheath was smooth. Optimal device seating wasobtained the first time it was deployed, and recapture andrepositioning of the device were not required. The reten-tion skirt at the aortic end of the ADO was correctlypositioned at the mouth of the ductus and was not pulledinto the ductus. Even so, significant residual flow (visu-alized in the lateral aortogram as a broad stream ofcontrast going through the device) was present at theconclusion of the procedure (Fig. 2A).

During the night following the procedure, the patientexperienced abdominal pain and noticed red coloredurine. Urinalysis revealed hemoglobinuria without hema-

turia. Over the next few days the patient’s hematocrit fellrapidly, necessitating two blood transfusions, while theserum creatinine, lactate dehydrogenase, and bilirubin(total and indirect) showed progressive elevation (Fig. 3).The patient was kept well hydrated and the urine wasalkalinized to minimize renal damage due to hemoglo-binuria. Doppler echocardiography on the fourth post-procedure day showed persistent shunting through thedevice, and in view of this and the continuing hemoglo-binuria, a decision was made to attempt abolition of theresidual shunt percutaneously, failing which to refer thepatient for surgical removal of the ADO and ductalrepair.

After informed consent and mild sedation, femoralarterial and venous access was obtained. Using an 8 FrAmplatz right coronary guiding catheter directed at theaortic aspect of the ADO, a 0.014� stiff hydrophilicsteerable guidewire (Shinobi Plus, Cordis, Miami Lakes,FL) was passed through the ADO and was caught as itemerged in the pulmonary artery with an Amplatz goose-neck snare (Microvena, White Bear Lake, MN). A 3 Frmonorail catheter with a 0.018�-guidewire-compatiblecentral lumen ending near the catheter tip (Multifunc-tional Probing Catheter, Schneider, Boston Scientific,Watertown, MA) was advanced into the ADO in mono-rail fashion on the 0.014� wire (Fig. 4). Traction on bothends of the 0.014� wire helped to steady the ADO and toadvance the tip of the 3 Fr catheter into the nitinol wirecage of the ADO. Four 0.018� VortX fibered platinumcoils of size 6 mm � 2 mm (Target, Boston Scientific)were delivered via the central lumen of the 3 Fr catheterinto the nitinol wire cage, on the aortic side of thepolyester fabric within the ADO and close to the centralaxis of the device. Angiography after 10 min showedsignificant reduction in shunting through the device (Fig.2B) that was associated with fall in pulmonary arterypressure and reduction in right heart oxygen step-up(Table I). Hemoglobinuria stopped within 24 hr, and thebiochemical abnormalities seen earlier disappeared over

Fig. 1. Schematic representation of a 14-12 mm Amplatzerduct occluder. The four dark lines within the device indicate thelocation and extent of Dacron polyester patches sewn to thenitinol wire frame.

TABLE I. Hemodynamic Data

Variable BaselinePostdevicedeployment

Postintradevicecoil deployment

Pulmonary artery systolic/diastolic (mean) pressures, mm Hg 34/14 (23) 29/9 (18) 18/7 (10)Aorta systolic/diastolic (mean) pressures, mm Hg 140/60 (86) 160/76 (100) 145/76 (95)Aorta oxygen saturation 99.0% 98.0% 98.0%Distal pulmonary artery oxygen saturation 79.8% 74.0% 75.5%Right atrium oxygen saturation 60.0% 65.4% 72.6%Right heart oxygen step-up 19.8% 8.6% 2.9%Pulmonary blood flow 4.61 L/min 3.7 L/min 4.28 L/minSystemic blood flow 2.27 L/min 2.42 L/min 3.79 L/minPulmonary/systemic blood flow ratio. 2.03 1.36 1.13Left-to-right shunt 2.34 L/min 0.98 L/min 0.49 L/minDescending aortogram Large ductal flow Moderate ductal flow Small ductal flow

246 Joseph et al.

the next few days (Fig. 3). Doppler echocardiographyafter 2 weeks showed no evidence of flow through thedevice.

DISCUSSION

The Amplatzer duct occluder has transformed percu-taneous closure of large PDAs, having several desirablecharacteristics such as simplicity of use, ability to recap-ture and reposition the device, high (� 95%) completeclosure rates, and requirement of relatively small intro-ducer sheaths [8]. The ADO is a self-expandable, highlyelastic, mushroom-shaped device made from a 0.004�thick nitinol (nickel-titanium alloy) wire mesh. The de-vice contains four Dacron polyester patches sewn to thewire frame with polyester threads, two patches at eachend, and two near the middle (Fig. 1). The polyesterpatches serve to close the PDA by induction of throm-bosis [8], besides mechanically impeding flow of bloodthrough the device. To occlude the PDA effectively, theADO must completely fill (stent) the ductus (oversizingby at least 2 mm is recommended), and the polyesterpatches must cover the cross-sectional area within thedevice. It is possible that in the present case one or moreof the polyester patches within the ADO may not have

been securely anchored to the nitinol mesh of the deviceor may have lacked continuity, leaving a part of thecross-sectional area within the device uncovered. Ashort-length, large-diameter PDA like the one seen in ourpatient is a situation where residual shunting could occurif a critically located polyester patch is out of place orincomplete. Most of the 8 mm axial length of an ADOused to occlude a very short ductus will lie within thepulmonary artery, and if the polyester patch correspond-ing to the location of the ductus is out of place ordiscontinuous, residual shunting could occur. High-ve-locity blood flow (due to the wide aorto-pulmonary pres-sure difference) going through the fine wire mesh of theADO can potentially produce mechanical fragmentationof erythrocytes and intravascular hemolysis. Residualshunting through the ADO usually disappears on fol-low-up and very rarely results in clinically significantintravascular hemolysis [8]. Therefore, if a residual shuntexists after optimal deployment of a correctly sizedADO, it is reasonable not to do anything further imme-diately, regardless of the degree of shunt.

In our patient, the ADO was oversized by 5.2 mm withrespect to the minimum ductal diameter and appearedoptimally indented at the point corresponding to thenarrowest part of the ductus, both on the day of implan-

Fig. 2. A: Lateral aortogram showing significant residual shunting after occlusion of a largepatent ductus arteriosus using the Amplatzer duct occluder. B: Reduced shunting after intrade-vice deployment of fibered platinum coils.

Hemolysis After PDA Device Closure 247

tation and 4 days later (Figs. 2A and 4). This indicatedappropriate oversizing of the device and ruled out under-estimation of the ductal diameter due to ductal spasm oroverlap between the large aorta and pulmonary artery.Intravascular ultrasound imaging with 3.8 Fr, 30 MHzcatheters can be used to measure ductal diameter accu-rately [13] and may be advantageous in situations whereangiographic measurements are potentially imprecise. Ifintravascular ultrasound is not available, it is sensible tooversize the ADO by at least 4 mm for large PDAs.

Intravascular hemolysis following ADO deploymentis rare. In a registry of 976 cases (K. Amplatz, per-sonal communication), only one case of hemolysis wasreported, which was self-limiting [8]. Conservativemanagement in anticipation of this occurrence is there-fore prudent. However, if the hemolysis is massive andunremitting, steps have to be taken to abolish theresidual shunt. In our patient, clinical and laboratoryparameters even at 4 days post-ADO deploymentshowed no evidence of the hemolysis abating, whilethe rising serum creatinine concentration indicated

progressive renal failure related to pigment nephrop-athy. Abolishing the residual shunt by another percu-taneous procedure or by surgery was therefore imper-ative. Packing fibered platinum coils within the wirecage of the ADO proved effective in abolishing boththe residual shunt and the intravascular hemolysis.Temporary balloon occlusion of the aortic ampulla toinduce thrombosis within the ADO [7] would not havebeen feasible in our case because of the large diameterof the descending aorta. Deploying additional devices(such as coils) alongside the ADO would have beenboth difficult and ineffective because the ADO com-pletely filled the ductal lumen and the residual shuntwent through it and not between the ADO and theductal wall.

In conclusion, our technique of deploying coilswithin the ADO to abolish residual shunting throughthe device is an effective therapeutic alternative tosurgery and should be considered whenever unremit-ting hemolysis occurs after PDA occlusion using theADO.

Fig. 3. Temporal changes in hematological and biochemical parameters noted after Amplatzerdevice closure of patent ductus arteriosus.

248 Joseph et al.

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Fig. 4. A multifunctional probing catheter has been advanced into the Amplatzer duct occluder(ADO) over a 0.014� wire. Vertical arrow indicates radiopaque catheter tip marker. The tip of thewire has been caught and pulled into the right ventricle with a snare.

Hemolysis After PDA Device Closure 249