Right aortic arch aortopulmonary window associated with left
pulmonary artery originate from patent ductus arteriosus
Qiang Fan* MD, Yunfei Ling* PhD, Yue Wang, Qi An# MD
Department of Cardiovascular surgery, West China Hospital, Sichuan
University
* These authors contributed equally to this work and should be
considered co-first authors
# Corresponding author information: Tel: +86 28 85422897, Fax: +86 28
85422897, E-mail: anqi@scu.edu.cn, Add: No. 37 Guo Xue Xiang, Chengdu,
Sichuan 610041, People’s Republic of China.
Keywords : aortopulmoanry window, patent ducts arteriosus,
anomalous origin of left pulmonary artery
Funding : none.
Abstract Aortopulmonary window (APW) is a rare but serious
congenital cardiac malformation, most patients with APW will die from
congestive heart failure a few months after birth. However, in this case
we presented is an extremely rare condition that consist of a type III
APW and a ductus arteriosus originated left pulmonary artery.
Preoperative diagnosis included echocardiography and chest computerized
tomography revealed anatomical structure of the heart and great vessel
clearly, cardiac catheterization indicated that the pulmonary
resistances indices were 2.92 wood \(\frac{U}{m^{2}}\) in LPA and 3.35
wood \(\frac{U}{m^{2}}\) in RPA, \(Q_{p}:Q_{s}\) was 3.26. This patient
underwent surgical correction at the age of 9 and successfully survived.
A 9-year-old girl was referred to our department with significant
fatigue and shortness of breath after exercise but without cyanosis and
clubbed fingers, the body weight and height was lower than those of the
same age. Physical examination showed the heart border was expanded and
heat murmur was noted over the left side of the chest. Electrocardiogram
recorded a heart rate of 88 beats per minutes and indicated a sinus
arrhythmia and high left ventricular voltage. Chest x-ray showed mild
pulmonary congestion, severe cardiomegaly and right hilum convergence
sign (panel A). The echocardiography revealed a right aortic arch and
suggested a type III aortopulmonary window (APW), the right pulmonary
artery (RPA) had a proximal origin from posterior part of the ascending
aorta (panel B), a patent ductus arteriosus (PDA) originated from
brachiocephalic artery (panel C), but the left pulmonary artery (LPA)
was not noted. Computerize tomography (CT) scan of chest was performed,
type III APW and anomalous origin of RPA was confirmed (panel D) while
the LPA was found to originated from PDA with a stenosed orifice (panel
E). The three-dimensional reconstruction of the CT scan revealed the
relationship between aorta and RPA and LAP clearly (panel G and H).
Cardiac catheterization was performed preoperatively revealed that the
small connection between PDA and LPA (panel F), the measured pulmonary
resistances indices were 2.92 wood \(\frac{U}{m^{2}}\) in LPA and 3.35
wood \(\frac{U}{m^{2}}\) in RPA, \(Q_{p}:Q_{s}\) was 3.26. The patient
then underwent open-heart surgery under cardiopulmonary bypass,
intraoperative observation was coincident with the preoperative
diagnosis (panel I). This patient stayed in ICU for 8 days and
discharged unevenfully 13 days after surgery.
Consent The study were approved by the relevant ethics
committees, and oral informed consent was obtained for the participant.
Figure legend : (A) Chest X-ray showed pulmonary congestion,
severe cardiomegaly and right hilum convergence sign. (B)
Echocardiography showed APW and the RPA. (C) Echocardiography showed PDA
(red arrow). (D) Chest CT showed APW and RPA. (E) Chest CT showed LPA
originated from PDA (red arrow). (F) Cardiac catheterization revealed
the small connection between PDA (red arrow) and LPA. (G) and (H) showed
the relationship between Ao and RPA and LAP. (I) Intraoperative
observation was coincident with the preoperative diagnosis. Ao,ascending aorta; PA, pulmonary artery; RPA, right
pulmonary artery; LPA, left pulmonary artery; PDA, patent
ductus arteriosus.