Data availability statement:
Funding statement: None.
Conflict of interest disclosure: None declared.
Institutional Review Board approval or waiver: This study is
exempt under the Institutional Review Board at our institution.
Patient consent statement: Consent was obtained from her
parents.
Abstract
Sinus of Valsalva aneurysm is a rare disease characterized by the
partial elevation of the aortic root. Few reports are available on the
surgical treatment for infants. We report the repair of an extremely
rare case of a sinus of Valsalva defect with a ventricular septal defect
and right ventricular outflow tract stenosis in an infant. It was not a
sinus of Valsalva aneurysm, but it exhibited abnormal partial bulging of
the aortic root and forming an aneurysm-like cavity within the right
ventricular myocardium. We performed direct closure of the sinus of
Valsalva aneurysm-like cavities and intracardiac repair in two stages.
Three years after total repair, the patient remained healthy and
asymptomatic.
Introduction
Sinus of Valsalva aneurysm (SVA) is a rare condition characterized by
partial bulging of the aortic root 1,2. We report an
extremely rare case of a sinus of Valsalva defect repaired in infancy
and a deformity that exhibited abnormal partial bulging of the aortic
root and formed SVA-like cavities within the right ventricular
myocardium. This study is exempt under the Institutional Review Board at
our institution. Consent was obtained from her parents.
Case Report
A one-day-old infant girl was transferred to our institution because of
dyspnea and a heart murmur. She was neonatally diagnosed with a
ventricular septal defect (VSD), an atrial septal defect (ASD), patent
ductus arteriosus (PDA), persistent left superior vena cava, right
ventricular outflow tract (RVOT) stenosis (RVOTS), and a suspected SVA
on echocardiography. She had no family history of connective tissue
disease.
Preoperative echocardiography revealed SVA-like cavities protruding
toward the RVOT (Figure 1A, B), as well as RVOTS (2.5 m/s), and a large
VSD (14.6 mm). Preoperative cardiac catheterization revealed the
SVA-like cavities to be large. Preoperative contrast computed tomography
revealed large SVA-like cavities protruding from the right coronary
sinus (RCS) toward the RVOT and small SVA-like cavities below the
non-coronary sinus (NCS).
At 2 months old (4.9 kg), the infant underwent pulmonary artery banding
(PAB) and PDA ligation. At 1 year old (7.7 kg), she underwent total
repair, detailed below. Under cardiopulmonary bypass, the ascending
aorta was transected above the sinotubular junction. The walls of the
right coronary and non-coronary sinuses of Valsalva were deficient,
forming aneurysm-like cavities with trabeculation (Figure 2A, B),
connected behind the commissure between the right and non-coronary cusps
(Figure 2C). Thus, we diagnosed the infant with a sinus of Valsalva
defect rather than an SVA. Anatomically, the transition between the
aortic media and annulus was unclear; we believed that suturing would
injure the leaflets and distort the aortic annulus, leading to aortic
valve deformity. Therefore, patch closure of the sinus of Valsalva
defect was abandoned. Direct closure of the aneurysm-like cavities was
performed using a pledgeted 5-0 polyvinylidene fluoride (PVDF) suture
(Figure 2D). A 0.4-mm expanded polytetrafluoroethylene patch was applied
to the VSD using pledgeted 5-0 PVDF sutures. The SVA-like cavities
protruding into the RVOT was closed with horizontal mattress suturing
using a pledgeted 5-0 PVDF suture. After debanding, reconstruction of
the pulmonary artery was performed using an autologous pericardial
patch.
The postoperative course was uneventful. Postoperative echocardiography
revealed normal aortic valvular function with trivial aortic
regurgitation and no residual VSD or RVOTS (Figure 3). Three years after
total repair, the patient remained healthy and asymptomatic, with grade
I aortic regurgitation.
Discussion
SVA is characterized by a lack of continuity between the aortic media
and annulus 3. Congenital SVA is often concomitant
with other congenital heart defects 4. An unruptured
SVA may develop pathologies such as RVOTS and aortic regurgitation5. The pathogenesis of this case was considered a
large deficiency in fetal sinus of Valsalva wall development, leading to
the exertion of aortic pressure on the right ventricular myocardium, and
bulging of the latter into the RVOT, forming SVA-like cavities in the
sinus of Valsalva. Our patient presented with heart failure symptoms due
to VSD and PDA. We performed PAB and PDA ligation when she was 2 months
old. We performed direct closure of the SVA-like cavities and
intracardiac repair in two stages, as the procedure is risky in the
early infancy owing to tissue fragility. We know of no reports of
similar sinus of Valsalva defects in an infant. Following satisfactory
surgical outcomes, long-term follow-up will be required.
Acknowledgements: We acknowledge Editage (www.editage.com) for
English language editing.
Legends
Figure 1. Pre-total-repair examination
A, Long- and B, short-axis views of transthoracic echocardiography
reveal a large sinus of Valsalva aneurysm-like cavities protruding from
the right coronary sinus toward the right ventricular outflow tract. Ao,
aorta; LA, left atrium; LV, left ventricle
Figure 2. Intraoperative image
A, B, The defects of the walls of the right coronary and partial
non-coronary sinuses of Valsalva, and a myocardial component and
trabeculation in the sinus of Valsalva. C, Intraoperative schema. D,
Post-direct closure of sinus of Valsalva aneurysm-like cavities.
Figure 3. Post-total-repair examination
A, Short-axis view of transthoracic echocardiography; the right
ventricular outflow tract stenosis was released. B, Long-axis view of
transthoracic echocardiography revealed trivial aortic insufficiency.
Ao, aorta
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