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

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.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

scholarly journals Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Vein ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Surgical Correction ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

scholarly journals Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery

2020 ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Vein ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Surgical Correction ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

scholarly journals P720 Antenatal and postnatal echocardiographic diagnosis of Truncus Arteriosus III (TAIII) complicated by spontaneous PDA (patent ductus arteriosus) closure and LPA (left pulmonary artery) disconnection

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N Alabdulkarim ◽  
A T I F Asahari ◽  
A M Alotay ◽  
SHERIF Thabet ◽  
TURKI Alqahtani
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Fetal Echocardiography ◽  
Ductus Arteriosus ◽  
Pulmonary Veins ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Truncus Arteriosus ◽  
Arterial Trunk ◽  
Ductal Closure

Abstract Introduction TA III is a rare form of truncus arteriosus when the branch pulmonary arteries originate independently from the common arterial trunk or aortic arch, with origin of one pulmonary artery from the underside of the aortic arch from a ductus arteriosus. Accurate diagnosis and timing of surgery is essential for survival of neonates affected. Purpose Illustration of various imaging modalities utilized to diagnose and manage this lesion and the importance of spontaneous PDA closure. Results TAIII diagnosed at 24 weeks gestation by fetal echocardiography images 1,2,3. Baby was spontaneously delivered at term with 3 Kg weight and 8,9 Apgar score. Diagnosis was confirmed by transthoracic echocardiography images 4,5 , however on 7th day of life routine echocardiogram was performed to assess PDA , markedly decreased flow noted in LPA/left pulmonary veins and no PDA could be seen at that time images 5,6. Spontaneous ductal closure was confirmed by tomography image 7 then cardiac intervention performed to establish LPA patency image 8,9. Successful total repair was done within the neonatal period with excellent results at follow up ( image 10 at 3 years old). Conculsions 1- TAIII can can be diagnosed and assessed by echocardiography. 2- PDA patency to maintain LPA continuity is essential in TAIII management. 3- Successful total surgical repair with good outcomes can be achieved for neonates with this rare complex cyanotic CHD. Abstract P720 Figure. Images

scholarly journals Prenatal diagnosis of crossed pulmonary artery with tetralogy of Fallot and right aortic arch: A rare case report and literature review

2021 ◽  
Author(s):  
Jinfeng Cheng ◽  
Yixiu Zhang ◽  
Hua Meng ◽  
Xining Wu ◽  
Yunshu Ouyang ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Chromosomal Abnormalities ◽  
Ductus Arteriosus ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Right Aortic Arch ◽  
Diagnosis And Prognosis ◽  
Rare Case Report

Crossed pulmonary arteries (CPA) is an unusual malformation characterized by abnormal origination of the pulmonary arteries from the main pulmonary artery (MPA), which is usually associated with complex cardiac pathologies and chromosomal abnormalities. We report a case of crossed pulmonary artery (CPA) associated with tetralogy of Fallot (TOF), right aortic arch (RAA), and absence of ductus arteriosus. Sonographic findings, complicated malformations, genetic anomalies, differential diagnosis, and prognosis analysis are discussed. Although the isolated CPA is relatively asymptomatic, when it is accompanied by other cardiac anomalies, the prognosis needs to be reevaluated.

scholarly journals Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery

2020 ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Vein ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Surgical Correction ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Introduction:The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

scholarly journals Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery pressure in patients Operated with a Pulmonary Artery

2021 ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Vein ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Surgical Correction ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

Utility of Pre- and Postoperative Computed Tomography Angiography for the Evaluation of a Complex Vascular Ring

2019 ◽  
Vol 10 (5) ◽  
pp. 654-656
Author(s):  
Philip B. Dydynski ◽  
John S. Austin ◽  
Deborah Kozik ◽  
Bahaaldin Alsoufi
Keyword(s):  
Computed Tomography ◽  
Pulmonary Artery ◽  
Aortic Arch ◽  
Computed Tomography Angiography ◽  
Vascular Ring ◽  
Left Pulmonary Artery ◽  
Aberrant Right Subclavian Artery ◽  
Descending Aorta ◽  
Right Pulmonary Artery ◽  
The Right

We present the case of a neonate born with an unusual complex vascular ring formed by a left-sided aortic arch that had retroesophageal course to join a right-sided descending aorta and a very large right-sided arterial ductus from the right pulmonary artery to the descending aorta. Associated finings included aortic arch hypoplasia, aberrant right subclavian artery, and aberrant origin of the left pulmonary artery from the aorta. We focus on the role of computed tomography angiography in the preoperative and postoperative assessment of this complex anomaly.

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