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26—COARCTATION AND PDA CLOSURE 319
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Fig. 26.25 Once the device is in situ, it should be carefully imaged to ensure there is no impingement
in either the aorta or pulmonary artery. If no impingement is seen, the device is deployed.
References
1. Forbes TJ, Moore P, Pedra CA, et al. Intermediate follow-up following intravascular stenting for
treatment of coarctation of the aorta. Catheter Cardiovasc Interv. 2007;70:569-577.
2. Forbes TJ, Kim DW, Du W, et al. Comparison of surgical, stent, and balloon angioplasty treatment of
native coarctation of the aorta. J Am Coll Cardiol. 2011;58(25):2665-2674.
3. Forbes TJ, Garekar S, Amin Z, et al. Procedural results and acute complications in stenting native and
recurrent coarctation of the aorta in patients over 4 years of age: a multi-institutional study. Catheter
Cardiovasc Interv. 2007;70:276-285.
4. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and
natural history. Circulation. 1971;43:323-332.
5. Clyman RI, Chan CY, Mauray F, et al. Permanent anatomic closure of the ductus arteriosus in newborn
baboons: the roles of postnatal constriction, hypoxia, and gestation. Pediatr Res. 1999;45:19-29.
6. Feltes TF, Bacha E, Beekman RH, et al. Indications for cardiac catheterization and intervention in
pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation.
2011;123:2607-2652.
7. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults
with congenital heart disease: executive summary: a report of the American College of Cardiology/
American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;
73(12):1494-1563.
8. Philip R, Waller III BR, Agrawal V, et al. Morphologic characterization of the patent ductus arteriosus
in the premature infant and the choice of transcatheter occlusion device. Catheter Cardiovasc Interv.
2016;87:310-317.
9. Wang-Giuffre EW, Breinholt JP. Novel use of the medtronic micro vascular plug for PDA closure in
preterm infants. Catheter Cardiovasc Interv. 2017;89:1059-1065.
10. Krichenko A, Benson LN, Burrows P, et al. Angiographic classification of the isolated, persistently
patent ductus arteriosus and implications for percutaneous catheter occlusion. Am J Cardiol. 1989:
63(12):877-880.
e1
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Abstract: This chapter addresses questions related to catheter interventional management of
coarctation of the aorta, patent ductus arteriosus, and congenital fistulae. Figures illustrate both
devices and techniques used for catheter intervention.
Keywords: Coarctation of the aorta, patent ductus arteriosus, coronary artery fistulae, stenting,
coil embolization, vascular plugs
CHAPTER 27
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Closure of Abnormal Coronary
Communications: Coronary, Fistulae,
Congenital, and Iatrogenic
Donald Hagler Peter Pollak
Introduction
Congenital fistulae cover a wide spectrum of vascular disorders including a variety of arteriovenous
(AV ) malformations, but also can include venovenous communications. Structural and congenital
cardiology interventionalists most often may be asked to evaluated those fistulae involving the heart,
great vessels, and pulmonary circulation. Because of the widespread nature of such vascular malformations, it is difficult to determine an exact epidemiologic incidence of such disorders, although they
may be found with significant frequency during routine computed tomography (CT) screening for
other disorders. Perhaps the most frequent clinically significant malformations involve the coronary
and pulmonary circulation. The basic hemodynamic effect observed with such malformations is an
increase in cardiac output resulting in both right and left ventricular volume overload.
Fundamentally, flow will occur whenever there is a pressure gradient between two interlinked
structures. Pressure differences between the heart’s chambers are dynamic and may vary with
volume state, pulmonary or systemic resistance, and throughout the cardiac cycle. Moreover, the
heart may change in relation to a shunt over time. Longitudinal assessment over time may be
necessary to realize the impact of a defect.
The most common fistula encountered often in routine coronary angiography for coronary
artery disease assessment is a tiny coronary fistula to the front of the main pulmonary artery.
These fistulae likely represent a failure of involution or persistence of coronary artery anlagen in
the pulmonary artery, similar to that observed with anomalous origin of the coronary artery from
the pulmonary artery.
2
fects.
The tiny fistula from the left or right coronary artery to the main pulmonary artery is
generally hemodynamically insignificant and often has multiple sources, such as from both coronary arteries or brachiocephalic branches, as seen in Fig. 27.1. These tiny fistulae are difficult to
embolize but technically can be coiled if necessary. In general, they are asymptomatic and do not
require intervention.
Coronary artery fistulae are rare abnormal communications between a coronary artery and a
coronary chamber (coronary-cameral fistula) or a major vessel (coronary-AV fistula). Most commonly these are congenital in nature; however, there is a gradual increase in acquired coronary
fistulae resulting from infections, trauma, or iatrogenic causes. They often are found with very
large dilated and tortuous proximal coronary arteries, as shown in Fig. 27.2. These patients
nevertheless often remain asymptomatic and are only recognized later in adulthood.
The architecture of the coronary artery is affected by the location of the fistula. If the fistula
is proximal with the coronary artery, it results in dilatation of the proximal artery with a relatively
normal distal artery. A distal fistula typically results in dilatation of the entire artery.
1
There is a reported incidence of 0.2% to 0.4% of congenital cardiac de-
320
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Fig. 27.1 (A) A small fistula from the right coronary artery to the anterior surface of the main pulmonary artery
in a 19-year-old woman. (B) A similar type coronary fistula from the left coronary artery to the same location
on the anterior surface of the main pulmonary artery. Such hemodynamically insignificant fistula may originate
from either the left or the right coronary artery or both.
Potential complications of coronary fistulae include massive coronary artery dilatation and
aneurysm formation due to the continuous high-flow, intracoronary thrombus, which may result
in coronary occlusion and myocardial infarction, coronary rupture, arrhythmia, and infective endocarditis
2
(due to AV shunting). Patients with a coronary-to-bronchial artery fistula may present
with hemoptysis or bronchiectasis.
Most commonly a coronary artery fistula will have a single origin and a single outflow; however, complex fistulae may have multiple feeding arteries or multiple drainage sites. The most
common drainage sites are the pulmonary trunk/pulmonary arteries, right ventricle, or right
atrium. Spontaneous closure is rare but has been reported.
This chapter will present a standardized approach to these defects in four sections: (1) clinical
assessment and decision to close; (2) preprocedure assessment, defining the defect, and procedural
planning; (3) procedural techniques; and (4) clinical outcomes and follow-up assessment.
Clinical Assessment
Most often, given the sensitivity and availability of modern imaging, the structural interventional
cardiologist is presented with a discovered defect and posed with two questions: (1) Can this
defect be closed? and (2) Should this defect be closed? In this section we will look critically at the
decision to intervene.
Because this group of defects is so diverse, the operator must individualize the balance of risk
and benefit for each unique situation. To accurately assess the risk and potential benefits of defect
closure, one must evaluate the impact of the defect on the patient’s heart function in the context
of adjacent cardiac disease and the natural history if left untreated. For example, a relatively small
shunt may contribute to substantial symptoms in a patient with diastolic disfunction, whereas the
same-size shunt in another patient may be well tolerated without the need for intervention.
In principle, patients with defects that cause exercise limitations, change in chamber function (or enlargement), or heart failure are likely to benefit from successful closure. The clinical
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Fig. 27.2 (A and B) Anteroposterior (AP) and lateral images of a right coronary artery (RCA) injection with a
large fistula originating near the orifice of the RCA in a 57-year-old man who presented with fever and positive
blood cultures. The tortuous fistula courses in an irregular pathway over to the left aspect of the superior vena
cava (SVC). (C) A wire rail acquired through the fistula from the coronary artery to the right femoral vein.
(D) A 8F delivery sheath advanced up over the wire rail and positioned in the distal fistula at its SVC connection. An 8/10 Amplatz duct occluder was positioned in the distal fistula and released. (E) Angiogram
illustrating a right coronary artery injection 1 year after device closure of the fistula. The original fistula had
dramatically reduced in size with no residual shunt. All peripheral RCA branches are patent, and the
patient was asymptomatic.
E
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assessment should focus on the presence of these symptoms and findings. Auscultation is less
reliable for these defects because even high-flow fistulas may be inaudible.
Although no single laboratory value will dictate the decision to intervene, the additional information is always valuable. Laboratory assessments should include assessment of changes in
renal function, liver function, hemoglobin, and heart failure biomarkers (e.g., B-type natriuretic
peptide). Anemia may suggest acquired von Willebrand disease attributable to high defects causing high shear stress on the blood, which can be confirmed by testing the distribution of von
Willebrand multimers.
In the asymptomatic patient without heart failure or change in heart function, an exercise
study is a useful tool to quantify exercise capacity. Simultaneous measurement of VO
increase the precision and value of exercise testing.
Invasive hemodynamic testing allows objective assessment of left and right heart filling pressures
and pulmonary and systemic resistance and to calculate shunt fraction. Although no single shunt
value demands repair or suggests deferment, in principle, the larger the shunt flow, the greater the
physiologic implication and likelihood of benefit with closure. Qp/Qs ratios greater than 1.5 to 2.0
have been used historically with decision-making in the setting of atrial septal defects and represent
a starting point when incorporating into decisions about whether to proceed with closure.
can
2
AHA Guidelines
The 2008 American Heart Association (AHA) guidelines for the management of adults with
congenital heart disease are provided in the following box. The 2018 guidelines were less detailed
and stated that the presence of coronary artery fistula(s) requires review by a knowledgeable team
that may include congenital or noncongenital cardiologists and surgeons to determine the role of
medical therapy and/or percutaneous or surgical closure.
A large coronary arteriovenous fistula (CAVF), regardless of symptom-
atology, should be closed via either a transcatheter or surgical route
after delineation of its course and its potential to fully obliterate the
fistula
A small-to-moderate CAVF in the presence of documented myocardial
ischemia, arrhythmia, otherwise unexplained ventricular systolic or
diastolic dysfunction or enlargement, or endarteritis should be closed
via either a transcatheter or surgical approach after delineation of its
course and the potential to fully obliterate the fistula
Clinical follow-up with echocardiography every 3 to 5 years can be useful
for patients with small, asymptomatic CAVF to exclude development
of symptoms or arrhythmias or progression of size or chamber enlargement that might alter management
Patients with small, asymptomatic CAVF should not undergo closure III: Harm C
I C
I C
IIa C
Procedural Planning
Clearly defining the size, location, and extent of a given defect is vital for procedural planning.
Modern imaging techniques have become so clear that if a defect cannot be clearly characterized
with a combination of CT and transesophageal echocardiography (TEE), then the likelihood of
successful closure is small. Imaging analysis should focus on (1) defining the defect anatomy, (2)
adjacent anatomy (e.g., myocardial coronary branches, valve apparatus, conduction system, etc.),
and (3) physiologic impact of the defect (i.e., chamber size).
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Before deciding on intervention, it is important to establish whether the fistula has a single/
multiple inflow and/or a single/multiple outflow. Imaging will assess the degree of tortuosity and
length of catheter likely to be needed if percutaneous closure is performed.
Transthoracic echocardiography (TTE) is widely available and relatively inexpensive. More
posterior or basal structures can be challenging to characterize, and acoustic shadowing (from
calcium or prosthetic material) may compromise some imaging. Most patients have had a TTE
before or at diagnosis; however, for those who have not, it is advised to include preprocedure TTE
as a baseline.
Transesophageal echocardiography (TEE) is invasive but particularly valuable at defining
intracardiac defects and is often useful for guiding procedural closure. TEE provides high-resolution images of posterior cardiac structures along with Doppler-based flow measurements. More
apical structures are in the far field and may be better imaged with TTE. Three-dimensional TEE
imaging can be particularly useful for identifying the relationship of a defect to adjacent anatomy
or prosthetic material. Moreover, integration of TEE color flow imaging with cross-sectional
imaging provides the operator with a clear sense of defect size and drainage location.
CT: The combination of spatial resolution with cross-sectional imaging provides three-
dimensional visualization of anatomy. With an electrocardiogram (ECG)-gated CT data set it is
possible to thoroughly assess the size, extent, and location of a defect, noting entry and exit sites
and surrounding structures. One can simulate the fluoroscopic angle to find the optimal view for
use during implant and the relation to adjacent fluoroscopic markers (i.e., adjacent prosthetic
valves or leads).
Multiple rotational views of the entire fistula, the associated coronary artery branches, and the
final communications can be obtained to allow a better understanding of the complexity and
course of the fistula. Fig. 27.3 and Video 27.1 illustrate a coronary CT angiogram in a complex
right coronary artery (RCA) fistula to the coronary sinus (CS). There are multiple twists and
turns in the dilated RCA, and several terminal right ventricular branches are present distally in
the coronary. There also is a complex entry of the RCA fistula into the CS.
Three-dimensional printed models of cardiac defects are a new tool in the armamentarium of the
structural interventionalist. This technology can provide a tactile sense of defect location, size, and
relation to adjacent anatomy not possible with other imaging modalities. Moreover, beyond holding
Fig. 27.3 (A and B) Computed tomography (CT) images in a 40-year-old woman who presented with right
heart failure. The segments of a rotational CT reconstruction illustrate the very dilated and tortuous right
coronary artery (RCA). The distal connection to the coronary sinus (CS) is narrow and would be an ideal
location for placement of an occluding plug. All of the peripheral branches of the RCA are proximal to this
connection to the CS.
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a model heart, seeing and feeling the defect, it is possible with a printed model to test-occlude a
defect with actual equipment before the procedure. The insights from working with a printed model
may confirm an approach, point the operator toward alternative approaches or equipment, or suggest against the approachability of a given defect. Expertise with printing models is growing quickly,
and the optimal integration into practice for the structural cardiologist continues to evolve.
Procedural Approaches and Equipment
CHOOSING THE BEST EQUIPMENT AND APPROACH
The equipment used for closing intracardiac defects overlaps substantially with that used for
closing perivalvular leaks and coronary fistulas, that is the Amplatzer family of woven mesh occluder devices (Abbott, Chicago, IL), as well as coil embolization devices. The optimal choice of
device or combination of devices will match the anatomy of the defect and incorporate the delivery platform and strategy. Outside the United States, additional devices are also available from
Occlutech (Occlutech International AB, Helsingborg, Sweden).
In general, it is best to match a device closely to the defect size and shape while bearing in
mind the potential impact of the device on surrounding anatomy. Discrete “holes” are approached
with occluder devices matched to their size and shape, where multiple smaller round devices may
be stacked together to fill ovoid defects. “Spaces” are better filled with embolization coils. Coils
are available in many sizes and shapes and have the advantage of requiring very low-profile delivery systems (i.e., delivered through 2 to 3F microcatheters). Additionally, detachable coils can
be retrieved after deployment if the shape or location is suboptimal.
Coil and occluder devices can be combined to “fill a space” with coils and then “close the door”
with an occluder device. Although glue can be used for embolization in noncardiac spaces, we
have avoided the use of glue in closing cardiac communications due to concern for stroke. Familiarity with each device before use is important for patient safety and procedural success.
The ideal procedural strategy will minimize patient risk while optimizing successful delivery and
deployment of the chosen device or devices. As a rule, minimalism favors the patient—that is, fewer
vascular access points, avoiding general anesthesia (therefore without TEE guidance), and smallercaliber sheaths. Venous access is generally safer than arterial access, incurring less risk of bleeding and
arterial embolism. Nevertheless, ensuring adequate support within the delivery system for procedural
success must be considered, and a long procedure may be worse for the patient than a shorter, easier
procedure with larger, more supportive equipment or better imaging guidance.
Procedural Steps
The steps of procedural access remain analogous to other structural procedures (Fig. 27.4): (1)
cross the defect with a wire, (2) bring a delivery catheter through the defect, and (3) deploy the
device or devices while preserving myocardial perfusion branches. Fundamental approaches include (1) direct deployment, (2) delivery over an anchor wire, and (3) delivery over an AV rail.
These approaches have been described in detail in previous chapters.
Generally, the direct deployment method is quickest but yields the least support for delivery
and allows only a single device placement. This is best for larger defects, which are quicker to wire
and where loss of placement will not create a substantial time penalty.
Placement of an anchor wire or a wire rail can preserve access to the defect and increase
support for device delivery through the system through a single access point. It does require a
larger delivery sheath or catheter to accommodate the device alongside the anchor wire itself. A
number of atraumatic self-coiling wires can be used for this method. The 0.0250 Torayguide wire
(Toray Industries, Tokyo, Japan) and ProTrack Pigtail wire (Baylis Medical, Montreal, Canada)
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Antegrade approach
B
C
Fig. 27.4 Transcatheter closure techniques. (A) Retrograde (arterial) approach. The coronary vessel is in-
tubated, and the fistula is wired from its origin. The delivery catheter can then be delivered to the fistula over
a wire for device deployment. (B) Antegrade (venous) approach. The fistula termination site is intubated with
the delivery catheter. The delivery catheter is advanced over a wire to the appropriate landing zone. The occlusion device is then deployed and released. (C) Arteriovenous (AV) loop approach. In large and tortuous
fistulas, the AV rail can be formed with the aid of a snare device to maximize support for catheter and device
delivery. (© Mayo Clinic.)
are designed for use in the atria. The 0.0350 Confida (Medtronic, Minneapolis, MN) and Safari
wires (Boston Scientific, Marlborough, MA) are designed for placement in the ventricle.
The initial wire of choice for crossing a defect is operator and situation dependent. The 0.0350
stiffened angled Glidewire (Terumo Corp., Tokyo, Japan) nicely balances maneuverability, an atraumatic tip, and support and is a workhorse of structural intervention. Some 0.0180 wires, such as the
V18 Control wire (Boston Scientific, Marlborough, MA), are helpful with smaller defects or to
allow the delivery of larger equipment alongside through delivery sheaths or catheters. In extremely
tortuous fistulae, a 0.014 wire may be necessary and can also be snared to create and AV rail.
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Choice of Delivery Sheath
Functionally, any sheath that allows controlled delivery of the optimal device to the defect will
work. In planning, it is valuable to consider net total support of the system. Generally, more angulations and bends will decrease support available, whereas larger-diameter catheters, stiffer
wires, and straighter lines increase support. Larger devices will require more support for delivery,
whereas smaller devices require less. Multiple smaller devices may be deliverable through a system
that does not support a single larger device. We have found the Shuttle family of sheaths (Cook
Medical, Bloomington, IN) in 4 to 8F diameters balances conformability with support well. A
number of shaped sheaths are available that are designed to work with the Amplatzer devices
(Abbott, Chicago, IL).
It is valuable to carefully consider length and width compatibility. Although sizes are listed on
the packaging, it may be necessary to dry-test devices before attempting delivery in the body.
When considering length, assess both the length needed to reach and cross the defect as well as
the length available for the device being delivered. Generally, 80 cm will reach either ventricle
from a femoral venous approach, whereas 1001 cm is necessary if approaching the left ventricle
retrograde across the aorta. If one is anticipating using an AV rail or anchor wire during delivery,
check compatibility of the device alongside the wire in the sheath.
Finally, it is worthwhile to consider room setup for these procedures during the planning
phases. Consider the ergonomics of access points while factoring in who else will be in the
room and where communication will need to happen. If it is a general anesthesia case and
internal jugular (IJ) access is being used, coordinating space ahead of time can avoid frustration during the case. If GA, TEE, and IJ access are used together, personnel locations may
need to be adjusted (e.g., anesthesia moves to the patient’s left side, TEE above the head,
and interventional on the patient’s right). It may be necessary to identify someone else to
operate fluoroscopy or to move the fluoroscopy screen so it is visible to all parties to facilitate
procedural awareness.
Use of Amplatzer Vascular Plugs
Delivery of Amplatzer Vascular Plugs (AVP; typically AVP-II , AVP IV ) uses similar equipment
and an approach to those described in the chapters on paravalvular leak and pseudoaneurysm
closure. An important consideration in coronary fistula closure is the length of the defect and
ensuring that equipment is able to deliver the device to the desired occlusion point.
Fig 27.5 and Videos 27.2 and 27.3 illustrate an approach to a more complicated RCA fistula
to the CS, as observed in the CT images in Fig 27.3. In this example the RCA is extremely
tortuous and required multiple wire passage attempts before successful passage of a microcatheter
and whisper coronary wire, which was snared from a superior vena cava (SVC) approach. A 7F
delivery sheath was advanced over the wire rail into the distal RCA fistula. Initially an 18-mm
AVP-II plug was positioned in the fistula-to-CS connection. This plug was too large and obstructed the distal right ventricular (RV) branch of the coronary. This plug was removed and
replaced with a 14-mm AVP-II plug at the point of communication with the CS. This plug fit
well into the stenosis at the communication and did not obstruct any peripheral coronary
branches. A postoperative CT imaging (see Fig. 27.5 and Videos 27.2 and 27.3) illustrates the
final position of the AVP-II plug and normal filling coronary arteries.
Coil Embolization
When embolization of a coronary fistula is attempted, we generally attempt to embolize the
coronary artery at the most distal point before entry into the final drainage site in order to avoid
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