scholarly journals Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report 

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Hyun-Hwa Cha ◽  
Hae Min Kim ◽  
Won Joon Seong

Abstract Background Unilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity. Case presentation A 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation. Conclusion There is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

Author(s):  
Jorge Cervantes-Salazar ◽  
Jose García-Montes ◽  
Henry Peralta-Santos ◽  
Diego Ortega-Zhindón ◽  
Juan Calderón-Colmenero

We present two patients with history of recurrent respiratory infections, fatigue and sweating. They were diagnosed with absence of connection between the main pulmonary artery (MPA) and right pulmonary artery (RPA) and bilateral ductus arteriosus, with the RPA originating from the ductus arteriosus. Treatment was approached with a hybrid strategy: percutaneous intraluminal angioplasty with a right intraductal stent and device closure of the left ductus arteriosus and followed by surgical reconstruction with interposition of a graft from RPA to MPA. Both patients had a favorable outcome.


2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom

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.


2002 ◽  
Vol 10 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Ümrah Aydoğan

Transcatheter closure of patent ductus arteriosus using controlled-release coils was performed in 16 patients weighing < 10 kg. No embolization occurred. Procedure-related complications occurred in 3 patients (18.8%): massive femoral hemorrhage in 1 and femoral artery thrombosis in 2. The ductus recanalized in 1 of them because of mechanical hemolysis caused by streptokinase treatment. This was the only patient who underwent another occlusion procedure. Complete occlusion was achieved in 7 patients (43.8%) immediately, in 13 (81.2%) the following day, and in all 15 patients who had completed the 6-month follow-up. During follow-up, flow velocities between the left and the main pulmonary arteries and between the descending and the ascending aortae did not differ significantly. Flow velocity was > 2 m·sec−1 in 3 patients in the left pulmonary artery and in 1 in the descending aorta. Protrusion of the coil was seen in 3 of these patients. Flow velocity was also high in the main pulmonary artery in the 4th patient. In conclusion, coil occlusion of ductus arteriosus is feasible in the small child, but no more than half a loop of the coil should be left at the pulmonary site. High flow velocity does not always mean obstruction.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  

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.


2020 ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  

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.


Author(s):  
Bin Li ◽  
Aijun Liu ◽  
Ming Yang ◽  
Junwu Su

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.


2020 ◽  
Author(s):  
Mohammadreza Edraki ◽  
Bahram Ghasemzadeh ◽  
Kambiz Keshavarz ◽  
Ahmadali Amirghofran ◽  
Hamid Mohammadi ◽  
...  

Abstract Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs on its own or with some congenital cardiac disorders, particularly tetralogy of Fallot (TOF), while the hidden pulmonary artery might originate from a closed ductus arteriosus (DA) that can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of our nine TOF patients who had the absence of the left pulmonary artery before the operation. The patients were stratified in three groups: group one, whose closed DA were found and stented successfully to the hidden pulmonary artery; 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 the other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgically correction, while the pulmonary vein angiography of the group two patients showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of the group three patients, aged 12, 38, 60, and 63 months. Moreover, evaluation of the other six previously corrected patients who were operated with a right pulmonary artery revealed pulmonary artery hypertension of the entire 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.


2007 ◽  
Vol 15 (4) ◽  
pp. 335-338 ◽  
Author(s):  
Hani K Najm ◽  
Neerod K Jha ◽  
Michael Godman ◽  
Mansour Al Mutairi ◽  
Ahmed I Rezk ◽  
...  

Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.


2015 ◽  
Vol 12 (2) ◽  
pp. 65-68
Author(s):  
Urmila Shakya ◽  
Poonam Sharma ◽  
Manish Shrestha

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68


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