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occlusion of viable distal coronary artery branches. Most interventionalists advocate clear angiographic evidence of the absence of any coronary branches or preferably balloon occlusion at the
site and observation for any ECG ST-segment evidence of ischemia. Most often, stable distal
occlusion is best achieved by creating a wire rail from the aorta through the coronary artery and
then through the drainage site that can be snared from a venous approach. The AV rail will then
allow placement of a delivery sheath from the venous side and deliver an obstructive plug in the
most distal portion of the communicating coronary artery.
Fig. 27.6 illustrates coil embolization of the coronary artery fistulae to a mediastinal tumor.
Multiple microcoils were placed in the distal coronary communications with a microcatheter
delivery system. Standard Gianturco coils (Cook-Bloomington, IN), specialized Interlock detachable fibered 0.0350 or 0.0180 platinum coils (Boston Scientific – Marlborough, MA), or Azur
detachable hydrocoils (Terumo-Somerset, NJ) are options for coil embolization through the
A
C D
Fig. 27.5 (A) Right coronary artery (RCA) injection showing the very tortuous dilated RCA communicating to
the coronary sinus (CS), as illustrated in the computed tomography (CT) images of Fig 27.3. (B) Passage of
a microcatheter and whisper coronary wire through the fistula into the right atrium, where the wire was snared
from the right internal jugular vein. (C) Passage of a 7F delivery sheath over the wire rail into the distal RCA.
(D) Initial position of an 18-mm AVP-II plug in the distal fistula, which was too large and obstructed a small
distal coronary branch.
B
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E F
G
Fig. 27.5 cont’d
nection of the RCA to the CS. (G) Postoperative CT images of the coronary arteries, which confirm the correct
distal position of the AVP-II at the connection to the CS with patency of the distal coronary artery branches.
(E and F) Correct placement of a 14-mm AVP-II plug in the distal narrowing at the con-
A B
Fig. 27.6 (A) Two fistulous connections from the left coronary artery into a large mediastinal tumor. (B) Occlusion of the fistulae with multiple 3- and 4-mm Cook micro (tornado) coils. They completely occluded the
coronary fistulae.
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microcatheter systems (Figs. 27.7 to 27.9). Similar precautions must be made to avoid excessive
coil size or length, which may result in proximal displacement of some of the coil material. There
are similar concerns for proximal clot progress and potential for myocardial infarction.
Procedural Steps
The procedure is typically performed under conscious sedation unless the anatomy is complex
and TEE guidance is needed. Here we describe an example procedure using an AV rail to close
a giant RCA-to-CS fistula.
Femoral arterial and venous access should be established with 7 to 10F sheaths, depending on
vascular plug size. Heparin should be administered to maintain activated clotting time (ACT)
300 s, typically starting with a bolus of 100 units/kg. If right heart catheterization has not been
recently performed, this is the first step and will confirm the degree of shunt.
The coronary artery should be selectively engaged with an appropriate catheter (e.g., JR4 for
RCA, JL4 for left system). Selective angiography may be performed by hand injection in the case
of smaller arteries. For giant coronary arteries, a pigtail catheter introduced into the coronary
artery may be used with a pump injector to enable sufficient opacification. Selective angiography
is used to confirm the number and location of drainage sites and the location of any proximal
branches of the coronary artery.
Fig. 27.7 The Terumo Azur Hydro coil system for embolization.
Fig. 27.8 The Terumo Azur Hydro coil system for embolization.
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Fig. 27.9 The Boston Scientific Interlock fibered platinum coils.
Potential Complications of Coronary Fistula Closure
Overall, when performed in an experienced center, the rates of complications for percutaneous
closure of coronary fistulae is low. Risks include loss of side branches or main branches leading
to myocardial ischemia, vessel dissection or perforation, and device embolization. Although data
are limited, the rates of these complications are likely in the region of 1%. Late myocardial
ischemia due to migration of coils or thrombus is possible and has been described.
The major complication is recanalization (20% at follow-up angiography at 1 to 2 years).
These rates are similar to those after surgical ligation.
Outcomes and Potential Complications
Clinical response to defect closure reflects the clinical impact of the lesion going into the procedure. The greater the attributable symptoms, the greater the improvement. Patients who were
symptomatic at rest or with little exertion will usually notice some improvement within a day of
closure. As the heart remodels after closure, there is often some additional improvement by
1 month. Patients who initially note improvement followed by return of their symptoms should
be evaluated for defect recurrence.
For coronary fistulae, there are specific concerns for perioperative or postoperative myocardial
infarction.
usually with Coumadin and aspirin. The major concern relates to persistent dilation of the feeding
coronary artery with sluggish flow prone to thrombosis, as illustrated in Fig. 27.6. There are no
specific guidelines for the duration of anticoagulation but likely as long as the coronary remains
dilated with sluggish flow. However, despite these efforts, thrombosis of nearby coronary vessels
and infarction may occur. Fistulae originating proximal to the main segment of the coronary artery
may be less prone to this complication since the dilated segment may regress as in Fig. 27.2.
We typically repeat a TTE at 1 month and 1 year.
3
Therefore many interventionalists have advised postoperative anticoagulant treatment,
Recommended follow-up after uncomplicated closure is 1 month, 6 months, and then 12 months.
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Conclusions
n
Abnormal Coronary communications should be evaluated for signs and symptoms of heart
failure or shunting.
n
Careful patient selection, preprocedural evaluation, and procedural planning with multi-
modality imaging will increase the likelihood of technical success and optimize clinical
outcomes.
n
Precautions and potential complications for coronary artery fistula embolization must be
considered when planning these embolizations.
References
1. Heifetz SA, Robinowitz M, Mueller KH, Virmani R. Total anomalous origin of the coronary arteries from
the pulmonary artery. Pediatr Cardiol. 1986;7(1):11-18.
2. Herlong JR. Congenital coronary artery anomalies. In A Garson, JT Bricker, DJ Fisher, SR Neish, eds.
Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, MD: Williams and Wilkins; 1998:1647-1666.
3. Said SM, Burkhart HM, Schaff HV, et al. Late outcome of repair of congenital coronary artery fistulas—
a word of caution. JTCVS. 2013;145(2):455-460.
e1
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Abstract: Congenital fistulae covers a wide spectrum of vascular disorder including arteriovenous malformations and venovenous communications. This chapter primarily deals with the
diagnosis and management of coronary arterial malformations and specifically fistulae. However
the catheter intervention techniques described can also be applied to extra-cardiac malformations as well. We detail both antegrade and retrograde approaches to interventions techniques.
We discussed the use of various coils and plugs for occlusion of fistulae. We noted concerns for
the long term observation of such fistulae. Some may require anticoagulation and antiplatelet
therapy to prevent coronary artery thrombosis due to sluggish flow and clotting in markedly
dilated coronary arteries.
Keywords: Coronary artery fistulae, veno-venous communications, percutaneous interventions.
vascular coils and plugs
CHAPTER 28
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Pulmonary Valve Interventions:
Valvuloplasty and Transcatheter
Pulmonary Valve Replacement
Allison K. Cabalka
Pulmonary Valvuloplasty
BACKGROUND
Pulmonary valve stenosis (PS) is a common form of congenital heart disease that may present at
any age, but often in infancy or early childhood in its more severe forms. The adult patient with
PS tends to have more clinical symptoms than a child with a similar degree of PS; gradients can
be progressive, often due to acquired right ventricular outflow tract (RVOT) infundibular muscle
hypertrophy. The patient with an interatrial communication (patent foramen ovale/atrial septal
defect [PFO/ASD]) may experience a right-to-left shunt as right ventricular hypertrophy progresses and compliance decreases. Percutaneous pulmonary balloon angioplasty (PBA) has been
performed safely and effectively since 1982 with low morbidity and mortality. Acute success rates
for PBA are reported to be between 80% and 90%, with potential for ongoing reduction in gradient due to spontaneous resolution of infundibular hypertrophy. However, the patient may require
beta-blocker therapy as RVOT obstruction resolves.
Indications for Intervention
Indications for intervention typically include the following: (1) pulmonary valve mean gradient
by Doppler
includes a normal pulmonary valve annulus with thin, doming pulmonary valve leaflets, and poststenotic main pulmonary artery dilation. A thickened, dysplastic pulmonary valve with hypoplastic annulus and/or no evidence of main pulmonary artery dilation is much less likely to respond
favorably to PBA, although some degree of reduction in valve gradient may provide symptomatic
relief in the adult.
35 to 40 mmHg and (2) symptomatic functional limitation. Favorable anatomy
AHA Recommendations
In adults with moderate or severe valvular pulmonary stenosis
and otherwise unexplained symptoms of heart failure, cyanosis
from interatrial right-to-left communication, and/or exercise
intolerance, balloon valvuloplasty is recommended
In asymptomatic adults with severe valvular pulmonary stenosis,
intervention is reasonable
I B-NR
IIa C-EO
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Preprocedural Imaging
Transthoracic echo is usually sufficient for diagnosis and can be reliable in determining the pulmonary valve gradient (mean Doppler gradient typically correlates best with peak-to-peak systolic
gradient measured at cardiac catheterization). The nature of the pulmonary valve leaflets should
be determined (i.e., thickened, dysplastic). The pulmonary valve annulus should be evaluated carefully and measured to provide guidance in balloon selection. In the adult patient with challenging
acoustic windows, transesophageal echo or computed tomography can be considered (particularly
to evaluate infundibular anatomy, anatomy of the main pulmonary artery, or pulmonary branch
arteries if other stenotic lesions are suspected).
Basic Procedure
Standard right heart catheterization is performed to document the pulmonary valve gradient.
The use of two femoral venous sheaths allows simultaneous right ventricular (RV) and pulmonary artery (PA) pressure measurement, in addition to providing immediate postvalvuloplasty
RV systolic pressure assessment if a single-balloon dilation is planned. Initial RV angiogram
confirms the anatomy of the RVOT, and a carefully calibrated measurement of the pulmonary
annulus should be performed at the hinge point of the valve leaflets (also correlating with the
annulus as measured by echocardiography). Straight lateral plane imaging is most accurate for
annulus measurement (Fig. 28.1). A stiff 0.0350 exchange wire is positioned deep within the
PA over which the balloon can be stabilized in the annulus. For the single-balloon technique,
a balloon approximately 120% of the pulmonary annulus is chosen (Fig. 28.2A); for a doubleballoon angioplasty, the combined diameters of the two balloons should be 1.5 to 1.7 times the
annulus diameter (see Fig. 28.2B). Alternatively, for the adult patient, the Inoue balloon may
be used for pulmonary valve dilation, again with a diameter 120% of the annulus. Controlled
balloon inflation should allow positioning within the leaflets; once stable, full inflation can be
performed. Double balloons may be more difficult to stabilize, and rarely is pacing needed for
stabilization. A pressure-monitored inflation device should be used—high-pressure balloons
are not needed. Careful pullback with an end-hole catheter after PBA may demonstrate the
residual gradient below the valve due to the RVOT muscle. A postdilation RV angiogram
should be performed and may reveal the typical appearance of subvalvular obstruction after
successful balloon valvuloplasty (Fig. 28.3).
Tips:
1. Care to achieve a stable, deep wire position will be beneficial for balloon stabilization
during valvuloplasty.
2. If there is significant resistance when advancing the valvuloplasty balloon through the
tricuspid valve, one should consider potential wire entrapment within the tricuspid chordal
apparatus. In this situation the existing guidewire should be removed and the tricuspid
valve recrossed, preferably with a balloon-tipped catheter (balloon inflated) to establish a
different wire position.
3. Balloon stability during inflation may be challenging. Adequate forward pressure on both
the balloon and wire is necessary, but if the balloon starts to “milk” forward through the
valve, additional forward pressure on the wire can be used to stabilize balloon position.
Longer balloons are recommended (4.0 cm).
Postprocedure
Initial noninvasive echocardiography should be performed to evaluate the pulmonary valve
gradient, RVOT infundibular gradient, and degree of pulmonary regurgitation. As noted,
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A
C
Fig. 28.1 (A) Anteroposterior (AP) projection of right ventricular (RV) angiogram demonstrating pulmonary
valve annulus (asterisk) with thin, doming pulmonary valve leaflets. (B) Lateral projection of same RV angiogram demonstrating thin, doming pulmonary valve leaflets (arrows) in this patient with moderate pulmonary
valve stenosis (PS); the annulus can be measured from this projection (asterisk). Moderate post-stenotic dilation of the main pulmonary artery (MPA) is also seen. (C) AP projection RV angiogram in a different patient
with severe PS; severe RV hypertrophy is present, in addition to more significant post-stenotic dilation of the
MPA. (D) Lateral projection in same patient shows the narrow jet of contrast passing through the stenotic
valve and evidence of infundibular hypertrophy (arrow).
B
D
RVOT infundibular gradients typically subside over time, and patients may be treated with
beta-blocker therapy. Approximately 10% of patients require additional treatment if the initial
valvuloplasty is successful. Pulmonary regurgitation is usually well tolerated. The long-term
outcome is very good.
Transcatheter Pulmonary Valve Replacement
BACKGROUND
RVOT intervention is common in the surgical treatment of patients with congenital heart disease
(CHD), such as those with tetralogy of Fallot (TOF), pulmonary valve atresia/ventricular septal
defect, truncus arteriosus, and transposition of the great arteries. In addition, patients who have
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A B
Fig. 28.2 (A) Single-balloon angioplasty with waist at pulmonary valve annulus (arrows) seen at maximal infla-
tion. Guidewire is positioned deep into the left pulmonary artery. Separate pressure monitoring catheter is
present in apex of right ventricle (asterisk). (B). Double-balloon angioplasty with waist at pulmonary valve annulus (arrows) present at maximal inflation. Guidewires are both positioned deep into the left pulmonary artery.
undergone RVOT conduit placement, such as the Ross procedure, often require later RVOT
conduit revision. Inevitably, conduit failure, valve deterioration (stenosis and/or insufficiency), or
progressive pulmonary insufficiency in the setting of transannular patch therapy will drive the
need for pulmonary valve insertion. Transcatheter pulmonary valve replacement (TPVR) is appealing in patients with CHD, as many have been subjected to more than one median sternotomy
procedure, and the overall goal in approaching patients with complex CHD is to avoid multiple
redo median sternotomies. TPVR therapy is now an important part of intervention for patients
with both congenital and acquired pulmonary valve dysfunction, and this type of intervention
should be performed by a pediatric interventional specialist or as part of a multidisciplinary
structural heart team that includes an experienced pediatric congenital interventionalist.
Fig. 28.3 Postdilation right ventricular (RV) angiogram in
a patient with long-standing severe pulmonary valve stenosis, demonstrating severe infundibular hypertrophy and
dynamic outflow obstruction (arrows). Significant poststenotic main pulmonary artery (MPA) dilation is seen.
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