Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation

2017 ◽  
Vol 27 (6) ◽  
pp. 1162-1166 ◽  
Author(s):  
Hironori Ebishima ◽  
Kenichi Kurosaki ◽  
Jun Yoshimatsu ◽  
Isao Shiraishi

AbstractObjectivesThis study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.MethodsThe Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio – the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation.ResultsWe included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, p<0.005). Besides, the flow of the ductus arteriosus was directed from the aorta to the pulmonary artery in the ductus arteriosus-dependent group during the fetal period.ConclusionsThe main pulmonary artery cross-section ratio was the most significant variable for predicting postnatal outcomes in fetuses with tetralogy of Fallot.

Author(s):  
Jinfeng Cheng ◽  
Yixiu Zhang ◽  
Hua Meng ◽  
Xining Wu ◽  
Yunshu Ouyang ◽  
...  

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.


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.


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.


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.


1996 ◽  
Vol 4 (3) ◽  
pp. 178-180
Author(s):  
Jacques AM van Son ◽  
Volkmar Falk ◽  
Friedrich W Mohr

A modified technique for the repair of tetralogy of Fallot with absent pulmonary valve syndrome and stenosis at the origin of the lobar or segmental pulmonary artery branches is described. This consists of generous reduction plasty of the redundant anterior and posterior walls of the aneurysmally dilated branches of the pulmonary artery, resection of the main pulmonary artery, pericardial patch augmentation of the stenotic origins of the lobar or segmental pulmonary artery branches, insertion of a valved pulmonary allograft, and closure of the ventricular septal defect. The advantages of this technique, in addition to relief of the left-to-right shunt, consist of complete decompression of the tracheobronchial structures, and restoration of the capacitance of the central pulmonary arteries. This may result in a reduced tendency for postoperative respiratory insufficiency as well as long-term preservation of right ventricular compliance. The technique was successfully performed on 2 critically ill neonates and a 3-month-old infant.


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


2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
A Tiwari ◽  
PARAG Barwad ◽  
U M A Dabi

Abstract Introduction Right ventricular outflow tract (RVOT) obstruction in some or other form is one of the major components of Tetralogy of Fallot. It can occur at any level, isolated or in combination, from Infundibulum (most common), Pulmonary valve, Main pulmonary artery, Right and Left pulmonary arteries and up to their segmental branches. Incidence of pulmonary artery anomalies are reported between 15-20%, though some reported as high as 40%.  Purpose  The objective of this retrospective  observational study is to determine the presence and degree of pulmonary artery  abnormalities and associated cardiac defects in patients with Tetralogy of Fallot and to  define the pulmonary arterial anatomy to guide the further surgical management. Method A total of 100 newly diagnosed or follow up cases of TOF irrespective of age (range 5 days- 32 years) and gender (32 female, 68 male) who were planned for surgical management and referred for evaluation of pulmonary artery anatomy. All patient underwent non-ECG gated CT pulmonary Angiography in Toshiba 64 slice CT scanner.  CT data was analyzed retrospectively to look for cardiac and pulmonary artery anatomy by one independent observer having experience in cardiac radiology. Results 92 patient had infundibular and valvular stenosis and 8 patients has atretic pulmonary valve with additional supravalvar pulmonary stenosis in 17% patient. Out of total 100 patients, 35% had pulmonary artery abnormalities. This included 19 patient (19%) with isolated main pulmonary artery (MPA) abnormality, 6 % had isolated left pulmonary artery (LPA) abnormality, 2 had isolated right pulmonary artery abnormality, 4% had  combined MPA and LPA  abnormality, 2% had combined MPA and RPA abnormalities and 1% patient had all 3 (MPA,LPA,RPA) involvement in form of stenosis or hypoplasia. As TOF is commonly associated with other cardiac structural anomalies, we encountered following associations in our study – aortopulmonary collaterals (37%), Patent ductus arteriosus (29%), right sided aortic arch (19%), bilateral superior vena cava (10%), bovine arch (3%), double aortic arch (2%), d malposition of great vessels (2%), l malposition of great vessels (1%), situs inversus (1%), dextrocardia (1%), annuloaortic ectasia (1%) aortopulmonary window (1%) and cardiac totally anomalous pulmonary venous connection (1%). Conclusions Anomalies of pulmonary arteries are important determinant of surgical outcome of TOF patients. CT angiography is now established modality to delineate pulmonary artery and other anatomical details in TOF patient. Pulmonary artery anomaly can range from isolated branch pulmonary stenosis to diffuse hypoplasia of pulmonary vasculature, based on which management can differ from complete correction to palliative or medical management only. Our study emphasize the importance of pulmonary artery evaluation and its variation among patients of TOF. Abstract P268 Figure.


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