PIIS091450871930084X
PIIS091450871930084X
PIIS091450871930084X
Journal of Cardiology
journal homepage: www.elsevier.com/locate/jjcc
Original article
A R T I C L E I N F O A B S T R A C T
Article history: Background: We aimed to evaluate medium- and long-term outcomes of transcatheter closure (TC) of
Received 19 December 2018 ruptured sinus of Valsalva aneurysm (RSVA), which is a rare and mostly congenital heart disease.
Received in revised form 7 February 2019 Methods: Retrospective analysis included 23 patients (14 males) aged 15–79 years (y; 39.9 18.5)
Accepted 14 March 2019
selected for TC of RSVA between 2007 and 2017 in two tertiary centers in Poland and Ukraine. Fifteen
Available online 23 April 2019
patients were in New York Heart Association (NYHA) class III or IV before TC; 5 patients had acquired
RSVA after previous cardiac surgery. We applied 22 duct, 3 muscular, and 1 atrial septal Amplatzer or
Keywords:
Amplatzer-like occluders by the anterograde venous approach after arterio-venous loop creation in all
Ruptured sinus of Valsalva aneurysm
Congenital heart disease
but 1 patient. Mean follow-up conducted in outpatient clinic was 5.5 3.5 (1–11) y.
Percutaneous intervention Results: The procedure was successful in 19/23 patients (82.6%). Four procedures were abandoned and
Treatment outcome the device percutaneously retrieved due to coronary artery compression (1 patient), transient increase of
aortic regurgitation (AR; 1 patients) or embolization (2 patients). New onset of significant AR was noted
in one of the latter patients after device removal. NYHA class improved in all treated patients but 2, in
whom it remained stable (p < 0.05), with 10 patients in class I. Three patients needed percutaneous re-
intervention during follow-up because of significant residual shunt in 1 and late recurrent RSVA in
2 patients. The follow-up of the remaining patients was uneventful. Neither erosion, embolization, new
AR, nor death were observed.
Conclusions: The percutaneous closure of RSVA is a safe and effective method of treatment with good
clinical outcome. However, although not described previously, recurrent shunts after TC of RSVA are
possible and can be treated successfully with another transcatheter intervention.
© 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
https://doi.org/10.1016/j.jjcc.2019.03.012
0914-5087/© 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
382 M. Galeczka et al. / Journal of Cardiology 74 (2019) 381–387
myocardial ischemia, complete heart block, or right ventricle measured and pulmonary to systemic flow ratio (Qp/Qs) calculat-
outflow tract obstruction, or can rupture into one of the heart's ed, followed by aortic root angiography. RSVA was measured at its
chambers, which results in aortocardiac shunting and leads to aortic and rupture site in both TEE and angiography. The RSVA
congestive heart failure with dyspnea, chest pain, bounding pulse, aortic orifice to coronary artery ostium distance was evaluated
and ‘machinery-type’ murmur being the most frequent symptoms. exclusively in TEE in 11 patients and in both TEE and coronary
Open-heart surgery with patch closure at both ends of the defect angiography in the remaining 12 patients. The distance was less
remains the mainstay therapy for ruptured sinus of Valsalva than 5 mm only in one patient (No. 3). The defect was crossed with
aneurysm (RSVA). different types of catheters (mostly Judkins right coronary
However, since the first report in 1992 by Hourihan et al. [4], catheter) on a glide wire (Terumo Inc., Tokyo, Japan) and
successful RSVA transcatheter closures (TC) with different types of exchanged for a long 0.03500 standard guidewire. This wire was
devices have been described, predominantly as single case reports snared and exteriorized out of the femoral vein, which created an
and small series [5–13]. This method not only avoids sternotomy arteriovenous loop. Over such a loop, trans septal sheath was
and extracorporeal circulation, especially in hemodynamically introduced from vein access through RSVA to the ascending aorta
unstable patients, but allows also for shorter hospitalization with in order to enable the occluder deployment. However, retrograde
cost reduction [10]. In order to emphasize the percutaneous arterial approach was used in one patient with acquired left
approach, its long-term observation is highly expected. To date, coronary sinus to left atrium shunt (No. 20). Twenty one type I duct
only a few such reports have been published [11,13]. We present occluders were applied including nine Amplatzer (ADO I, AGA
our multicenter study with up to 11-year follow-up of TC of RSVA. Medical Corp., Plymouth, MN, USA), five Cardi-O-Fix (Starway
Medical Technology, Beijing, China), five HeartR (Lifetech Scientif-
Methods ic, Shenzen, China), two MemoPart (Lepu Medical Technology,
Shanghai, China) as well as one Nit-Occlud duct occluder (PFM
Patient characteristics Medical, Cologne, Germany). Moreover, two Amplatzer and one
Lifetech muscular VSD occluders and one Amplatzer atrial septal
Retrospective analysis of in-hospital data, medium- and long- occluder were applied. Overall 26 devices were used in 23 patients
term follow-up of 23 elective Caucasian patients selected for during a single primary session. Implants were selected according
percutaneous closure of RSVA between March 2007 and December to RSVA morphology and deployed in the aortic RSVA orifice except
2017 in two tertiary centers in Poland and Ukraine was performed. four patients, in whom the device was deployed in the distal end of
There were 9 females and 14 males with a mean age of RSVA because of either RSVA anatomy or device displacement (No.
39.9 18.5 years (range 15–79 years) (Table 1). 4, 15, 16, 23). Ductal occluders 1–7 mm larger than RVSA aortic
Four patients were in New York Heart Association (NYHA) class orifice diameter were generally chosen. The balloon calibration
IV, 11 patients in class III, and other patients in class II, all with was abandoned in all but two patients (No. 7, 15) in order to
continuous heart murmur in physical examination. Congenital prevent further damage of fragile tissue around the RSVA.
RSVA was diagnosed in 18 patients; among whom was a patient Angiography and TEE evaluation were repeated before and after
with BAV who had undergone primary RSVA surgery one year releasing the device. Patient No. 21 had native coarctation of aorta
before and was admitted with a defect recanalization (recurrent stented during the same session.
symptoms occurred after strenuous physical effort; No. 2). Five
remaining patients had probably acquired RSVA after previous Follow-up protocol
cardiac surgeries: tetralogy of Fallot complete repair (No. 3),
mechanical aortic valve implantation (No. 11), left ventricular Follow-up data were collected during previously scheduled
outflow tract obstruction relieve (LVOTO; No. 18), atrioventricular visits in out-patient clinic 1, 3, 6, 12 months after the procedure and
septal defect repair (No. 20), and coarctation of aorta repair with annually thereafter [clinical examination, electrocardiogram
VSD closure (No. 22). Causative factor of SVA rupture was identified (ECG), transthoracic echocardiography]. Aspirin 150 mg a day for
in five patients: strenuous physical effort in three, acute infection 6 months was administered. Mean follow-up was 5.5 3.5 years
with high temperature in one, and chest trauma in another patient. (range 1–11; in 10 patients >5 years).
The RSVA diagnosis was established after detailed clinical and
echocardiographic examination, moreover, 13 patients had com- Statistics
puted tomography performed before the procedure for better
RSVA visualization, which is essential. Rupture of right- or non- All continuous variables are expressed as mean values and
coronary sinus into the right atrium in 17 patients and to the right standard deviation or median with range as appropriate, and
ventricle in four patients as well as from the left coronary sinus to discrete variables are presented as percentages. Univariate analysis
pulmonary artery in one patient and to the left atrium in another was performed by the Student t-test. A p-value <0.05 was
(No. 3, 20, respectively; both with acquired defects) were considered statistically significant. The data were analyzed with
confirmed. None or trivial AR was present in all patients, but Statistica 13.3 software (StatSoft Inc.).
moderate in one patient (No. 18). All patients (or caregivers) were
informed about both surgical and transcatheter treatment options Results
and the latter method was preferred by all except patient 11, who
was disqualified from surgery. The informed consent was obtained. In-hospital observation
Procedure Mean pulmonary artery pressure was 24.0 7.9 mmHg (5–
38 mmHg; in 8 patients >25 mmHg) and Qp/Qs ranged from 1.4 to
The procedure was conducted under general anesthesia in 3.7 (median 2.2). The defect's mean aortic orifice diameter was
12 patients and under local anesthesia in 11 patients with two- or 9.5 3.3 mm (4–16 mm). Transcatheter closure of RSVA was
three-dimensional transesophageal echocardiography (TEE) and successfully completed in 19/23 patients (82.6%) (Figs. 1 and 2).
angiography guidance. After femoral vein and artery access In patient No. 3, with a 7 mm postsurgical left coronary sinus
completion (6–7 F sheath) intravenous heparin (50 IU/kg) and to pulmonary artery connection (after tetralogy of Fallot repair)
cefazolin were administered. Right and left heart pressures were and with the distance between left coronary artery orifice and
Table 1
Clinical in-hospital data, mid- and long-term outcome of patients in whom attempt of percutaneous closure of ruptured sinus of Valsalva aneurysm was performed.
Pt No. Age [y]/sex NYHA class Mean PA Qp/Qs RSVA type RSVA size [mm] Device, size [mm] Implantation Complications Fluoroscopy Follow-up Follow-up
before/at latest pressure aortic/rupture success duration [min] duration [y]
follow-up [mmHg] orifice
1 51/m III/I 19 2.2 RCS-RVOT 10/12 ADO 14/12 + Bigeminy, SVT 10 11 Uneventful
2 23/m III/I 17 2 RCS-RA 5/5 ADO 10/8 + - 16.4 10.9 Second RSVA closed with
ADO 10/8 after 6 months,
then 10 y observation
uneventful
3 18/f III/- 28 1.5 LCS-PA 7/3.8 ADO 8/6 Left coronary artery 34.9 - -
compression, occluder
withdrawn, sent for surgery
4 41/m II/I 22 2.2 RCS-RA 12/6 ADO 8/6 ! ASO 6a + - 19 10.3 After 8 y recurrent RSVA
closed with ADO 14/12, then
2 y observation uneventful
5 27/f II/I 14 1.7 NCS-RA 4/4 ADO 8/6 + - 17 10.6 After 4 y recurrent RSVA
closed with ADO 10/8, then
6 y observation uneventful
383
384 M. Galeczka et al. / Journal of Cardiology 74 (2019) 381–387
Fig. 1. Aortography. (A) Noncoronary sinus to right atrium ruptured sinus of Valsalva aneurysm (asterisk). (B) Defect closed with 14/12 HeartR duct occluder – no residual
shunt (No. 17).
Fig. 2. Transesophageal echocardiography. (A) Noncoronary sinus to right atrium ruptured sinus of Valsalva aneurysm (RSVA). (B) Three-dimensional reconstruction,
guidewire through RSVA. (C) RSVA closed with 14/12 Cardi-O-Fix duct occluder (No. 9).
RSVA opening of less than 5 mm in TEE, ST segment depression in RSVA enlargement, the non-coronary aortic cusp was sucked into
ECG occurred immediately after only 1 mm oversized Amplatzer the RSVA mouth and eventually immobilized (during following
8/6 duct occluder implantation (undoubtedly due to left coronary RSVA surgery the aortic valve proved to be intact) (Fig. 4). All four
artery compression) and the device was withdrawn with ECG patients with unsuccessful attempts at TC of RSVA were
change resolution. In patient No. 18 after previous LVOTO surgery scheduled for elective cardiac surgery, which was successful in
with moderate AR before the intervention, after HeartR 14/12 all of them.
duct occluder deployment, an increase of AR was observed and In patient No. 4 Amplatzer 8/6 duct occluder was initially
the procedure was abandoned with AR regression (Fig. 3). implanted in the distal RSVA orifice (6 mm), but it migrated
Occluders’ embolization occurred in 3 patients and in all of through RSVA over the delivery cable to the right atrium, another
them it was possible to retrieve the implant percutaneously. In attempt with Amplatzer 6 atrial septal occluder in the same
patient No. 8, an undersized Amplatzer 8/6 duct occluder which position was successful. In a 72-year-old woman with iatrogenic
embolized to the pulmonary artery immediately after its release RSVA (No. 11; after aortic valve replacement; with chronic aortic
was retrieved with 6F bioptome and a bigger Cardi-O-Fix 12/10 wall dissection and multiple comorbidities), Cardi-O-Fix 16/14
duct occluder device was successfully implanted. In patients No. duct occluder was deployed in the aortic RSVA orifice; however,
23 and 15 (in whom previous balloon calibration was performed) because of a significant residual leak on the edge of the device, the
MemoPart 14/12 duct occluder and NitOcclud 11.5 mm, respec- procedure was supplemented by Amplatzer 10 muscular VSD
tively, embolized to pulmonary artery and were removed with occluder implantation in the distal RSVA orifice, which finally
lasso. In both of these patients, the device was implanted in the resulted in the disappearance of the shunt. Muscular VSD occluder,
RSVA exit. Furthermore, in patient No. 23, the new onset of severe which has symmetrical discs, was chosen in order to prevent its
AR was observed after the device removal. Presumably due to the embolization.
Fig. 3. Aortography. Right coronary sinus to right atrium ruptured sinus of Valsalva aneurysm (asterisk), mild aortic regurgitation. (B) Severe aortic regurgitation after 14/12
HeartR duct occluder deployment, the procedure was abandoned (No. 18).
M. Galeczka et al. / Journal of Cardiology 74 (2019) 381–387 385
Fig. 4. Transesophageal echocardiography. Noncoronary sinus to right atrium ruptured sinus of Valsalva aneurysm (RSVA) (asterisk). Severe aortic regurgitation persisted
after withdrawal of 14/12 MemoPart duct occluder. Noncoronary cusp (NCC) sucked into the RSVA and immobilized presumably because of the defect enlargement (No. 23).
Mean fluoroscopy and procedure time were 18.0 11.0 (5–48) de novo 5 mm non-coronary sinus to the right atrium shunt
min and 103.0 44.6 (35–195) min, respectively. revealed itself in pregnancy during the fourth year of follow-up.
There was one patient with new onset of AR (unsuccessful case, After successful delivery by cesarean section (NYHA II class) it was
No. 23) and no new AR or AR progression among successfully electively closed during another session with Cardi-O-Fix 10/8 duct
closed RSVA. Trivial in 5 patients, mild in one patient (No. 22), and occluder without any problems (Fig. 6). During the following
significant in another one (No. 2), residual shunts were diagnosed 6 years she was pregnant once again, but it was uneventful at that
with echocardiography in the post-procedural period. In the latter time. Augmented cardiac output as well as hormonal changes
patient, a second RSVA was revealed after the primary procedure. during pregnancy are considered to be a risk factor for RSVA [13]. In
All patients with trivial residual leak had complete RSVA closure a man with arterial hypertension (No. 4), in whom the preceding
confirmed at the one-month observation. One major complication device had been deployed in distal RSVA orifice, recurrent 6 mm
occurred, femoral arteriovenous fistula, which required urgent right coronary sinus to right atrium leak (next to the previous one)
vascular surgery (No. 11). Ventricular bigeminy and supraventric- was revealed after 8 years and was electively closed with the
ular tachycardia were found in one patient (No. 1) during in- HeartR 14/12 duct occluder. Further observation of the other
hospital observations and after amiodarone administration they 15 patients was uneventful, mild residual shunt persisted in one
have yielded. patient (No. 22). Patient No. 7 was lost from observation. Neither
erosion, late embolization, endocarditis, stroke, new AR, complete
Follow-up heart block, nor death have been observed during follow-up.
NYHA class had improved in all but two patients at the latest Discussion
check-up, with most patients (10) in NYHA I class (p < 0.05) (Fig. 5).
During the medium- and long-term observation, three patients RSVA was first described by Hope in 1839 [15] and although it
needed percutaneous re-intervention because of another RSVA. In usually occurs in adulthood, it was even described in a neonate [
patient No. 2 (BAV, after previous RSVA surgery) a significant 16]. SVA is more prevalent in Asians, hence all series of
5.6 mm residual shunt (second RSVA) was closed with another percutaneous closure of RSVA come from the Far East [5,6,8–13],
Amplatzer 10/8 duct occluder after 6 months, then the 10-year despite our previous short-term observations [7]. To the best of our
observation was uneventful. In a young woman (No. 5), subsequent knowledge, this multicenter report is the only long-term follow-up
of TC of RSVA among the European population.
Five of our patients (5/23, 21.7%) probably had an acquired SVA
after previous cardiac surgeries. SVA rupture can occur spontane-
ously, after exertion, chest trauma, or even heart catheterization.
Severe infection with high temperature (1 patient), strenuous
effort (3 patients), chest trauma (1 patient), and pregnancy
(1 patient) are suspected to be the causes of SVA rupture in this
report.
Since 1957, urgent open-heart surgery under cardiopulmonary
bypass remains the first method of RSVA treatment [17]. It carries
low mortality, an up to 95% survival rate after 20 years and there
was no fistula recurrence in patients in whom patch closure at both
Fig. 5. New York Heart Association (NYHA) class: preprocedural and during last defects’ ends was performed [3,18]. The first transcatheter closure
follow-up. of an acquired RSVA was accomplished by Hourihan with a double
386 M. Galeczka et al. / Journal of Cardiology 74 (2019) 381–387
Fig. 6. Aortography. (A) Recurrent noncoronary sinus to right atrium ruptured sinus of Valsalva aneurysm (RSVA) (asterisk) 4 years after primary transcatheter closure with 8/
6 Amplatzer Duct Occluder I. (B) Defect closed with 10/8 Cardi-O-Fix duct occluder (No. 5). Recurrent RSVA occurred during pregnancy and was closed after delivery with
cesarean section.
umbrella device in 1992 [4]. Since then, more than 200 procedures Follow-up
of TC of RSVA have been reported in the literature, mostly with type
I duct occluders [19]. According to Kuriakose et al. [29] recurrent RSVAs were
described after surgical closure, but never after TC. However, data
Procedure on long-term outcomes of TC approach are limited. In our material
during up to 11-year observation we had two cases of late recurrent
Three-dimensional TEE guidance with color Doppler, which we RSVA – 4 and 8 years after first intervention (2/19 patients; 10.5%).
used during the 12 procedures, provides excellent RSVA morphol- The second percutaneous RSVA closure was effective in both
ogy and the assessment of AR progression or a residual shunt. patients.
However, computed tomography can be vital [20], providing Among the four patients in whom we closed the RSVA exit, one
operators with essential information about defect anatomy as well needed re-intervention because of a recurrent shunt, in another
as giving opportunity for novel three-dimensional image fusion two patients the device embolized, and only in one patient both
during the procedure. Two-dimensional TEE can effectively the intervention and long-term observation was uneventful. It
evaluate coronary arteries to RSVA opening proximity – we used confirms the thesis of Chang et al. [6] stating that the retention disc
this method exclusively in 11 patients. In one of them, with a rare should be deployed in the SVA aortic orifice and not in the
left coronary sinus to pulmonary artery rupture (after tetralogy of aneurysm itself to avoid progressive RSVA dilatation or defect
Fallot surgery), the procedure was abandoned because of ECG recurrence.
changes presumably due to the left coronary artery compression No new AR nor AR progressions after successful TC of RSVA
with only slightly oversized Amplatzer duct occluder. It suggests were observed in our group. This specific result is in common with
that even avoiding oversized duct occluders in patients with other reports [19] and underlines the superiority of RSVA TC over
proximity of coronary artery to RSVA opening, coronary compres- surgery.
sion is a possible and unpredictable risk. Although balloon RSVA Our study confirms the high utility of original ADO I and other
calibration is helpful in selecting the proper device [5], we avoided type I duct occluders in the percutaneous closure of RSVA. The
it in all but two patients. We believe that this can cause further incidence of RSVA in Central and Eastern European populations
damage to a fragile tissue around the defect. Such a mechanism remains unclear.
could have happened in one of our patients – even though we used
the balloon calibration, the NitOcclud duct occluder turned out to Study limitations
be undersized and embolized to pulmonary artery.
Different types of occluders were used in previous reports, with This is a multicenter, retrospective, but non-randomized study
duct and ventricular septal occluders being applied most and the device selection depended on the operator's choice.
commonly. Rashkind double umbrella [4,5,21], coils [6,22,23],
atrial septal [5,7,24], and patent foramen ovale [25] occluders as
well as vascular plugs [26,27] were also reported. We used Conclusions
26 devices in 23 patients overall during a single session: 22 duct,
3 muscular VSD, and 1 atrial septal occluder. The size of the duct Percutaneous closure of RSVA is a safe and effective method of
devices was chosen due to RSVA shape. The idea was to implant the treatment with good clinical outcome. However, recurrent shunts
device in the aortic RSVA orifice rather than in its tunnel in order to after TC of RSVA are possible and can be treated successfully with
prevent embolization. In cone-shaped defects, the duct occluders another transcatheter intervention. Cautious follow-up is manda-
were generally chosen to be more oversized than aortic RSVA tory. In appropriately selected patients, percutaneous approach
orifice in comparison to tunnel-shaped ones. In one patient with could replace surgery and become the method of choice.
16 mm RSVA, we successfully used a 22/20 duct occluder, which is
in contradiction to the study by Zhang et al. [28]; that such big
RSVA is not applicable for TC. Funding
Our TC of the RSVA success rate was 82.6% (19/23) but even a
100% success rate was described by Sivadasanpillai et al. (in None.
7 patients) [9]. From our point of view, the most important points
are to avoid the new onset of AR or AR progression and to carefully
select the device in order to avoid embolization. In our opinion Conflict of interest
patients with RSVA and moderate or severe AR should not be
treated percutaneously. None.
M. Galeczka et al. / Journal of Cardiology 74 (2019) 381–387 387
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[15] Magee R. A cardiac clinico-pathological conference in 1882 an historical
Supplementary data associated with this article can be found, in vignette. Heart Lung Circ 2004;13:322–5.
the online version, at doi:10.1016/j.jjcc.2019.03.012. [16] Steflik DJ, Churchill TL, Chowdhury SM. Sinus of Valsalva aneurysm rupture in
an infant. Cardiol Young 2018;28:338–40.
[17] Lillehei CW, Stanley P, Varco RL. Surgical treatment of ruptured aneurysms of
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