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Mustard Procedure Br Heart J: First Published As 10.1136/Hrt.72.1.85 on 1 July 1994

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Mustard Procedure Br Heart J: First Published As 10.1136/Hrt.72.1.85 on 1 July 1994 Br Heart J 1994;72:85-88 85 Transcatheter stent implantation for recurrent pulmonary venous pathway obstruction after the Mustard procedure Br Heart J: first published as 10.1136/hrt.72.1.85 on 1 July 1994. Downloaded from Martin C K Hosking, Kenneth A Murdison, Walter J Duncan Abstract pathway obstruction reaching a maximum A 5 year old boy presented with obstruc- flow velocity of 2'0 m/s associated with a tion of the pulmonary venous pathway stenosis measuring 2-3 mm in diameter. four years after the Mustard procedure. Right ventricular dysfunction was a persistent A successful balloon dilatation ofthe pul- echocardiographic finding associated with an monary venous pathway was performed increase in left ventricle dimension in but the benefit was transient. Placement response to the rise of pulmonary arterial of a 10 mm balloon expandable intravas- pressure secondary to the pulmonary venous cular stent across the recurrent stenosis inflow obstruction. A significant systemic to resulted in complete relief ofthe obstruc- pulmonary venous chamber baffle leak mea- tion with prompt resolution ofthe clinical suring 5 mm x 4 mm with right to left shunting signs. The delivery system was modified was seen by Doppler colour flow mapping and to facilitate stent delivery. contributed to central cyanosis. Serial chest x ray films showed persistence of cardiomegaly (Br Heart 1994;72:85-88) with increasing evidence of pulmonary venous congestion. To avoid the risks of surgical repair in a Paediatric experience with transcatheter patient with impaired right (systemic) ventric- placement of endovascular stents for residual ular function, the initial therapeutic inter- stenosis has expanded from the initial studies vention included balloon dilatation of the of systemic vein and pulmonary artery dilata- pulmonary venous pathway obstruction with tion to include recent reports of stenting of a 12 mm x 2 cm balloon (Meditech, the ductus arteriosus, aortopulmonary collat- Watertown, MA, USA) along with catheter erals, and postoperative coarctation of the occlusion of the baffle leak with a 17 mm aorta.' 2 After the Mustard operation for trans- PDA Rashkind occluder device.5 After this, position of the great arteries, pulmonary transoesophageal Doppler assessment with venous pathway obstruction is a less common simultaneous haemodynamic correlation http://heart.bmj.com/ but more severe complication than systemic showed a reduction of the pulmonary venous venous pathway obstruction.3 In an attempt to outlet gradient to 6-8 mm Hg with an avoid surgery with its attendant high operative increase in diameter from 3 mm to 7 mm. risks balloon dilatation has shown some Clinically there was a considerable improve- benefit, although restenosis often occurs.4 ment with reduction in oxygen requirement. We report the successful percutaneous Diuretic treatment was no longer required as intravascular stenting of recurrent pulmonary the pulmonary oedema seen previously venous pathway obstruction after a previously cleared. on September 23, 2021 by guest. Protected copyright. successful balloon dilatation. We discuss the The benefit was transient and seven technical modifications found useful in facili- months after catheterisation, his clinical state tating stent placement in locations with diffi- had once again deteriorated with increased cult access. oxygen and diuretic requirements as before. Careful imaging and Doppler colour flow mapping again showed a velocity of 2 m/s Case report across the pulmonary venous baffle with a The 5 year old patient was diagnosed in the recurrence of the stenosis measuring 3-4 mm immediate newborn period with atrioventric- in diameter. As the previous catheterisation ular concordance, ventricular arterial discor- had shown the distensibility of this baffle dance (d-transposition of the great arteries), stenosis, to avoid further restenosis and main- and intact ventricular septum. A balloon atrial tain an optimal passage for pulmonary venous Division of septostomy was on the first day of flow we decided to implant an intravascular Cardiology, Childrens performed Hospital ofEastern life, then a Mustard procedure at three stent across this area of narrowing. Ontario, Ottawa, months. Canada During the next five years his clinical CATHETERISATION PROCEDURE M C K Hosking K A Murdison course was characterised by persistent deteri- Informed consent was obtained and the pro- W J Duncan oration in oxygen saturation associated with cedure was performed under general anaes- Correspondence to: an increasing requirement for diuretics and thesia. A transoesophageal biplane 5 MHz Dr Martin C K Hosking, to maintain echocardiographic probe was placed before Division of Cardiology, supplemental oxygen adequate Childrens Hospital of oxygenation. Serial precordial Doppler and the start of the catheterisation and left in situ Eastern Ontario, 401 Smyth showed An arterial catheter was Road, Ottawa, Ontario, cross sectional echocardiography throughout. placed by Canada K1H 8L1. evidence of progressive pulmonary venous a standard percutaneous approach and the 86 Hosking, Murdison, Duncan Figure 1 (A) Angiogram (anterior to posterior projection) ofthe proximal pulmonary venous atrium showing position and severity ofthe baffle neck obstruction (arrows). (B) Anterior to posterior view Br Heart J: first published as 10.1136/hrt.72.1.85 on 1 July 1994. Downloaded from showing the non-inflated stent (arrows) in position across the baffle obstruction. Note the tortuous catheter course with the guide wire positioned in the left lower pulmonary vein. The delivery sheath has been retracted before inflation. The 17 mm Rashkind occuder device is in position across a previous systemic to pulmonary venous baffle defect (open arrow) and was used to help position the stent optimaly before inflation. (C) Similar view as before showing the inflated stent in position. Note how the delivery technique was used to avoid the stent has attained the curve potential for kinks to develop in the 7 French ofthe superior baffle aspect delivery sheath once the dilator was removed. (arrows). ruPV, right upperpulmonary vein; After placement of the sheath and dilator sys- d PVA, distalpulmonary tem within the abdominal aorta, the dilator venous atrium; p PVA, was replaced with the stent/balloon dilating proximalpulmonary venous atrium. catheter. Ensuring that only the tip of this catheter extruded from the sheath, thus acting as a smooth guiding tip for the sheath while still protecting the stent, the entire system was manoeuvred over the guide wire and posi- tioned across the area of stenosis. Even with the use of a stiff 0-032 inch Amplatz guide wire careful alternating traction and advance- ment of the wire was requiured for the catheter to traverse the trabeculated right ventricle and make the final curves across the baffle steno- http://heart.bmj.com/ sis. Once in an optimal position, the sheath was withdrawn and the stent deployed in the patient was heparinised with 100 U/kg. The standard method. No complications occurred diagnosis ofpulmonary venous outlet obstruc- during the stenting procedure. tion was confirmed by haemodynamic mea- Before stenting, both angiography and surement by pullback tracing over the stenotic transoesophageal echocardiography showed a area and angiographic assessment (right ante- discrete wedge of tissue projecting into the rior oblique and lateral projections). venous channel from the superior atrial wall on September 23, 2021 by guest. Protected copyright. A retrograde arterial approach was used to resulting in a minimal pulmonary channel of gain access to the pulmonary venous atrium. 4 mm (fig 2A). Haemodynamic measure- An end hole balloon catheter (Arrow ments showed a mean gradient of 12 mm Hg, International, Reading, PA, USA) was with a maximal Doppler velocity of 1-9 m/s deflected from the morphological right ventri- and non-laminar colour flow Doppler. After cle across the right atrioventricular valve and stent placement indwelling transoesophageal positioned within the distal pulmonary venous echocardiography clearly showed the stent in atrium. With the aid of a steerable guide wire an optimal position with a widely patent (Hi-Torque Floppy, Advanced Cardio- lumen of 10 mm diameter and laminar unob- vascular systems, Temecula, CA, USA) the structed colour flow Doppler across the open area of stenosis was crossed and measured stent (maximal velocity of 0-8 m/s). To avoid haemodynamically and with angiograms to potentially dislodging the stent a pressure delineate it (fig 1(A)). A stiff 0-032 inch catheter was not placed across the area and Amplatz guide wire was positioned in the left the wire and catheters were removed without lower pulmonary vein and the stent delivery complication. The patient was maintained on a catheter was manoeuvred over this. A 2 cm heparin infusion (20 U/kglh) for 24 hours Palmaz stent (Johnson and Johnson then subsequently placed on aspirin (8 Interventional Systems) carefully secured on a mg/kg/day). 10 mm x 3 cm balloon dilating catheter Since the procedure a dramatic clinical (Optiplast, Mississauga, Ont) was successfully improvement has been seen. His chest x ray placed across the area of baffle stenosis. The film has cleared, there is no further need for final stent diameter was 10 mm (fig 1 (A-C)). diuretic treatment, and oxygen saturations Due to the tortuous route leading to the are above 95% in room air. His activity level baffle stenosis, a modification in the standard and general demeanour have dramatically
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