Abstract
In this case, we reported a 24-day-old infant with a rare combination of
the anomalous origin of coronary artery and the type A interrupted
aortic arch, who presented with significant shortness of breath. Besides
these anomalies, further cardiac examination including transthoracic
echocardiography and CT angiogram revealed ventricular septum defect,
patent foramen ovale and patent ductus arteriosus at the same time.
Surgical procedures including repair of ventricular septum defect,
ligation of patent ductus arteriosus, unroofing of right coronary artery
and end-to-side aortic anastomosis were performed to cure the infant.
This patient stayed in ICU for few days without any major complications
and discharged from hospital at the tenth day after surgery.
Keywords : Congenital heart disease
A 24-day-old male infant with persistent shortness of breath since birth
was referred to hospital for further treatment. His mother did not have
regular antenatal care, while pregnancy complications were either not
found. Physical examination revealed the respiratory rate of 30 bpm and
blood pressure of 80/40 mmHg. On presentation transthoracic
echocardiography (TTE) revealed ventricular septum defect (VSD), patent
foramen ovale (PFO) and patent ductus arteriosus (PDA). The aorta was
discontinuous at the level of descending aortic arch, and the
significantly enlarged arterial duct connected the descending aorta and
the pulmonary artery. The right coronary artery originated from the left
coronary sinus with an intramural segment could be found on TTE images
(Figure 1, panel A ). Contrast computed tomography angiogram (CTA)
and three-dimensional reconstruction was carried out, which demonstrated
the interrupted aortic arch (IAA) combined with anomalous aortic origin
of a coronary artery (AAOCA). The
site of aortic interruption was at the distal side of the left
subclavian artery, and the blood flow to the descending aorta was via an
enlarged PDA which diameter measured about 5mm (Figure 1, panel
B ). CTA also clearly revealed the right coronary artery originated from
the left coronary sinus with a 3mm-length intramural segment (Figure 2,panel C ). Besides, a subarterial ventricular septal defect with
the diameter of approximate 8mm was found on imaging. Thus, the patient
underwent procedure including repair of VSD, ligation of PDA, unroofing
of right coronary artery and end-to-side aortic anastomosis which
combined the descending aorta to the aortic arch. Postoperative TTE
revealed no residual shunt at the level of the interventricular septum
and the aorta. The boy recovered successfully and discharged from
hospital at the tenth day after surgery.
AAOCA is the most commonly reported arterial malformation in cases of
sudden death, which was usually caused by an intramural segment of
coronary artery. AAOCA is traditionally defined as coronary artery
originates from abnormal sinus including right, left or no coronary
sinus. Due to the variable reported prevalence in different population,
the true incidence remains unknown1,2. IAA is a
relatively rare congenital anomaly with a incidence of 2/100000
approximately in live births, which is characterized by complete
anatomic discontinuity between the ascending and descending aorta, while
descending aorta usually receive flow form pulmonary artery via
PDA3. IAA has been classified into three types (A, B
and C) according to the site of aortic interruption. These two main
anomalies combined with VSD, PFO and PDA conspired to cause the clinical
presentation of the boy. Considering extremely severe symptoms and
anatomical anomalies, surgical procedure was essential for this patient.