Abstract
We reported a case of a 3-month-old infant presented with supravalvular
aortic stenosis with congenital right coronary artery deficiency.
According to cardiovascular CT results, Doty technique was adopted to
restore the aortic root geometry under cardiopulmonary bypass. An
angioplasty was performed to establish right coronary blood flow at the
same time. The patient had no abnormal cardiac symptoms after surgery.
Keywords: Congennital heart disease
A 3-month-old infant was referred for further intervention due to
systolic murmurs found at birth. Echocardiography demonstrated a
supravalvular aortic stenosis (SVAS) at the sinutubular junction level,
bicuspid aortic valves (BAV) with the raphe on the left cusp, mild
aortic regurgitation and secundum atrial septal defect (SASD) (Fig. A,
B). Computed tomography (CT) and three-dimensional reconstruction
suggested the absence of right coronary artery (Fig. C, D). Left
coronary was demonstrated features of stenosis at the ostium (Fig. E).
The patient then received surgical intervention
under cardiopulmonary bypass.
Exploration during surgery revealed that main branch of the right
coronary artery was present but not connected to the aorta, so an
angioplasty was performed to recover right coronary blood flow. The SASD
was repaired using autologous pericardium and left coronary artery
received patch augmentation. Doty technique was adopted to restore the
aortic root geometry. This technique of extended aortoplasty for
symmetric reconstruction of the aorta should provide more predictable
relief of aortic obstruction and improved function of the aortic
valve1.
SVAS is characterized by the stricture of the lumen of the ascending
aorta, especially in the supravalvular aorta at the sinutubular
junction. SVAS may occur as an isolated defect or combined with other
angiostenosis, especially stenosis of pulmonary artery, and usually with
arrhythmia at the same time2. SVAS can be classified
into two categories based on the degree of involvement of the ascending
aorta described by echocardiogram reports: discrete SVAS and diffuse
SVAS3. Discrete type is characterized by a localized
ring-like thickening at the sinutubular junction that results in an
hourglass-shaped aortic root while diffuse SVAS is characterized by
tubular hypoplasia and thickening of the entire ascending
aorta4.
Some successful cases reported angioplasty of coronary artery performing
SVAS repair at the same time, but these studies primarily described
cases in the left coronary artery. Congenital absence of RCA is probably
related to dysplasia or congenital occlusion of the RCA during the
development of embryo. This kind of may lead to the deficiency of blood
supply in sinoatrial and atrioventricular node, eventually causing their
dysfunction. Until now, there are few reports on congenital absence of
RCA. This may because RCA has non-specific clinical features. We adopted
angioplasty to establish right coronary blood flow for the patient.
Angioplasty can improve blood supply of the heart without increasing the
risk of major complications.
This case image also suggested that compared to echocardiography or
catheter angiography, cardiovascular CT can provide excellent
visualization of complex congenital anatomies. Anyway, we treated this
case with Doty technique and angioplasty procedures mentioned above.
Short-term prognosis of the patient revealed good.