Prenatal diagnosis of left‐sided aortic arch, right descending aorta and right‐sided ductus arteriosus associated with anomalous origin of left pulmonary artery from ascending aorta

Abstract Extensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.

Download Full-text

Anomalous Origin of Left Pulmonary Artery From Ascending Aorta: Embryological Model

10.22541/au.163439267.79149923/v1 ◽

2021 ◽

Author(s):

Bin Li ◽

Aijun Liu ◽

Ming Yang ◽

Junwu Su

Keyword(s):

Pulmonary Artery ◽

Congenital Heart ◽

Left Subclavian Artery ◽

Ductus Arteriosus ◽

Main Pulmonary Artery ◽

Left Pulmonary Artery ◽

Ascending Aorta ◽

Anomalous Origin ◽

Left Wall ◽

Heart Malformation

Background Anomalous origin of the left pulmonary artery (ALPA) from the ascending aorta (AA) is a rare congenital heart malformation. Aim To give some of our embryological considerations of ALPA from the AA. Methods We present a patient with ALPA from the AA, right-sided aortic arch, right-sided ductus arteriosus (DA), and aberrant left subclavian artery (LSCA). Results The distal end of ALPA was cut off, the proximal end was sutured, and the distal end was directly anastomosed to the left wall of the main pulmonary artery (MPA). Conclusion The failure of migration and differentiation of cardiac neural crest cells at the fourth and sixth archs result in unilateral arch agenesis or failure of detachment of the left sixth arch from the aortic sac, which form ALPA the AA.

Download Full-text

Right aortic arch, bilateral ductus arteriosus, and anomalous origin of left pulmonary artery from innominate artery in a fetus with normal intracardiac anatomy

Echocardiography ◽

10.1111/echo.14668 ◽

2020 ◽

Vol 37 (5) ◽

pp. 796-798 ◽

Cited By ~ 1

Author(s):

Balaganesh Karmegaraj ◽

Balu Vaidyanathan

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Ductus Arteriosus ◽

Innominate Artery ◽

Left Pulmonary Artery ◽

Right Aortic Arch ◽

Anomalous Origin ◽

Bilateral Ductus Arteriosus

Download Full-text

Pathogenesis of solitary right aortic arch: a mass effect hypothesis based on observations of serial human embryonic sections

Cardiology in the Young ◽

10.1017/s1047951115002152 ◽

2015 ◽

Vol 27 (2) ◽

pp. 359-368

Author(s):

Zhe W. Jin ◽

Tomonori Yamada ◽

Ji H. Kim ◽

José F. Rodríguez-Vázquez ◽

Gen Murakami ◽

...

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Ductus Arteriosus ◽

Pulmonary Arteries ◽

Mass Effect ◽

Left Pulmonary Artery ◽

Sympathetic Ganglia ◽

Right Aortic Arch ◽

Descending Aorta ◽

The Right

AbstractIn general, solitary right aortic arch carries the left-sided ductus arteriosus communicating between the left subclavian and pulmonary arteries or the right-sided ductus connecting the descending aorta to the left pulmonary artery. Serial sections of fifteen 5- to 6-week-old embryos and ten 8- to 9-week-old fetuses suggested that the pathogenesis was unrelated to inversion due to dysfunction in gene cascades that control the systemic left/right axis. With inversion, conversely, the ductus or the sixth pharyngeal arch artery should connect to the right pulmonary artery. The disappearance of the right aortic arch started before the caudal migration of the aortic attachment of the ductus. Sympathetic nerve ganglia developed immediately posterior to both aortae, with a single embryonic specimen showing a large ganglion at the midline close to the union of the aortic arches. These ganglia may interfere with blood flow through the distal left arch, resulting in the ductus ending at the descending aorta behind the oesophagus. In another fetus examined, a midline shift of the ductus course resulted in the trachea curving posteriorly. Therefore, solitary right arch is likely to accompany abnormalities of the surrounding structures. The timing and site of the obstruction should be different between types: an almost midline obstruction near the aortic union needed for the development of the left-sided ductus and a distal obstruction near the left subclavian arterial origin needed for the development of the right-sided ductus. A mass effect of the sympathetic ganglia may explain the pathogenesis of any type of anomalous ductus arteriosus shown in previous reports of the solitary right arch.

Download Full-text

Dilatation of the entire thoracic aorta in patients with bicuspid aortic valve: a magnetic resonance angiography study

VASA ◽

10.1024/0301-1526.34.3.181 ◽

2005 ◽

Vol 34 (3) ◽

pp. 181-185 ◽

Cited By ~ 12

Author(s):

Westhoff-Bleck ◽

Meyer ◽

Lotz ◽

Tutarel ◽

Weiss ◽

...

Keyword(s):

Magnetic Resonance ◽

Magnetic Resonance Angiography ◽

Aortic Valve ◽

Pulmonary Artery ◽

Aortic Arch ◽

Thoracic Aorta ◽

Aortic Root ◽

Ascending Aorta ◽

Descending Aorta ◽

Severe Aortic Regurgitation

Background: The presence of a bicuspid aortic valve (BAV) might be associated with a progressive dilatation of the aortic root and ascending aorta. However, involvement of the aortic arch and descending aorta has not yet been elucidated. Patients and methods: Magnetic resonance angiography (MRA) was used to assess the diameter of the ascending aorta, aortic arch, and descending aorta in 28 patients with bicuspid aortic valves (mean age 30 ± 9 years). Results: Patients with BAV, but without significant aortic stenosis or regurgitation (n = 10, mean age 27 ± 8 years, n.s. versus control) were compared with controls (n = 13, mean age 33 ± 10 years). In the BAV-patients, aortic root diameter was 35.1 ± 4.9 mm versus 28.9 ± 4.8 mm in the control group (p < 0.01). The diameter of the ascending aorta was also significantly increased at the level of the pulmonary artery (35.5 ± 5.6 mm versus 27.0 ± 4.8 mm, p < 0.001). BAV-patients with moderate or severe aortic regurgitation (n = 18, mean age 32 ± 9 years, n.s. versus control) had a significant dilatation of the aortic root, ascending aorta at the level of the pulmonary artery (41.7 ± 4.8 mm versus 27.0 ± 4.8 mm in control patients, p < 0.001) and, furthermore, significantly increased diameters of the aortic arch (27.1 ± 5.6 mm versus 21.5 ± 1.8 mm, p < 0.01) and descending aorta (21.8 ± 5.6 mm versus 17.0 ± 5.6 mm, p < 0.01). Conclusions: The whole thoracic aorta is abnormally dilated in patients with BAV, particularly in patients with moderate/severe aortic regurgitation. The maximum dilatation occurs in the ascending aorta at the level of the pulmonary artery. Thus, we suggest evaluation of the entire thoracic aorta in patients with BAV.

Download Full-text

Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta

Cardiology in the Young ◽

10.1017/s1047951102000392 ◽

2002 ◽

Vol 12 (2) ◽

pp. 186-188 ◽

Cited By ~ 6

Author(s):

Mi-Jin Jung ◽

Shi-Joon Yoo

Keyword(s):

Prenatal Diagnosis ◽

Pulmonary Artery ◽

Ascending Aorta ◽

Anomalous Origin ◽

Fetal Ultrasound ◽

Cardiac Screening ◽

Rare Anomaly ◽

Right Pulmonary Artery ◽

The Right

We report a case of anomalous origin of the right pulmonary artery from the ascending aorta that was diagnosed by fetal ultrasound at 21 weeks of gestation. The clue to the diagnosis was present in the three-vessel view, this being one of the views that we use for fetal cardiac screening. The anomaly was corrected surgically at 11 days of age. We discuss the importance of prenatal diagnosis in the management of this rare anomaly.

Download Full-text

Anomalous origin of a pulmonary artery in a teenager with tetralogy of Fallot with associated double aortic arch and coronary arteriovenous fistula

Cardiology in the Young ◽

10.1017/s1047951119000933 ◽

2019 ◽

Vol 29 (5) ◽

pp. 727-729

Author(s):

Vishal Agrawal ◽

Parth Solanki ◽

Ritesh Shah ◽

Divyakant Parmar ◽

Amit Mishra

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Tetralogy Of Fallot ◽

Vascular Ring ◽

Right Ventricular Outflow Tract ◽

Left Pulmonary Artery ◽

Ventricular Outflow Tract ◽

Double Aortic Arch ◽

Anomalous Origin ◽

Tract Fistula

AbstractWe report the case of a 14-year-old female who had tetralogy of Fallot along with anomalous origin of the left pulmonary artery from the ascending aorta with co-dominant double aortic arch forming a complete vascular ring compressing the oesophagus along with a left main coronary artery to right ventricular outflow tract fistula. She underwent surgical correction without conduit placement.

Download Full-text

Two Rare Vascular Rings With Ductal Origin of the Left Pulmonary Artery: A Previously Unrecognized Syndrome?

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135116675166 ◽

2016 ◽

Vol 9 (3) ◽

pp. 352-356

Author(s):

Poonam P. Thankavel ◽

Kristine J. Guleserian ◽

Robert H. Anderson

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Clinical Course ◽

Left Pulmonary Artery ◽

Descending Aorta ◽

Vascular Rings ◽

Arterial Duct

We report two neonates with distal ductal origin of the left pulmonary artery who also had rare vascular rings comprised of a left aortic arch and right arterial duct with a midline/rightward descending aorta. To the best of our knowledge, this association has not previously been described, although other abnormalities of the left pulmonary artery in the setting of vascular rings have been reported. We review the embryology, utility of imaging, and clinical course.

Download Full-text