The case of successful stage surgical treatment of pulmonary atresia with septal defect with a double aortic arch and focomelia

2018 ◽  
Vol 15 (2) ◽  
pp. 106-108
Author(s):  
A.A. Chvamaniya ◽  
A.A. Svobodov ◽  
L.V. Kosheleva ◽  
S.A. Kotov ◽  
M.R. Tumanyan
Keyword(s):  
Surgical Treatment ◽  
Aortic Arch ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Double Aortic Arch

Double aortic arch: an elusive diagnosis in a child with CHD

2021 ◽  
pp. 1-3
Author(s):  
Mariana Lemos ◽  
Miguel Fogaça da Mata ◽  
Ana Coutinho Santos
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Aortic Arch ◽  
Ct Angiography ◽  
Septal Defect ◽  
Vascular Ring ◽  
Pulmonary Atresia ◽  
Double Aortic Arch ◽  
Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

Combination of double aortic arch and interruption of aortic arch in pulmonary atresia with ventricular septal defect

2015 ◽  
Vol 25 (5) ◽  
pp. 994-995
Author(s):  
Dai Asada ◽  
Toshiyuki Itoi ◽  
Kenji Hamaoka
Keyword(s):  
Ventricular Septal Defect ◽  
Aortic Arch ◽  
Carotid Arteries ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Double Aortic Arch ◽  
Left And Right

AbstractNo reports on pulmonary atresia with ventricular septal defect with the combination of double aortic arch and interruption between left and right carotid arteries have been published so far.

scholarly journals Double aortic arch associated with pulmonary atresia with ventricular septal defect

2016 ◽  
Vol 31 (1) ◽  
Author(s):  
Fernando Cesar Gimenes B. Santos ◽  
Ulisses Alexandre Croti ◽  
Carlos Henrique De Marchi ◽  
Sírio Hassem Sobrinho
Keyword(s):  
Ventricular Septal Defect ◽  
Aortic Arch ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Double Aortic Arch

scholarly journals The pulmonary vascular blood supply in the pulmonary atresia with ventricular septal defect and its implications in surgical treatment

2003 ◽  
Vol 18 (1) ◽  
pp. 23-31
Author(s):  
Ulisses Alexandre Croti ◽  
Miguel Lorenzo Barbero Marcial ◽  
Carla Tanamati ◽  
Marcelo Biscegli Jatene ◽  
Sergio Almeida de Oliveira
Keyword(s):  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Blood Supply ◽  
Septal Defect ◽  
Pulmonary Atresia

Truncus arteriosus communis in combination with cor triatriatum sinsitrum

2015 ◽  
Vol 26 (1) ◽  
pp. 194-196
Author(s):  
Elmar Klusmeier ◽  
Nikolaus A. Haas ◽  
Eugen Sandica
Keyword(s):  
Surgical Treatment ◽  
Aortic Arch ◽  
Septal Defect ◽  
Coarctation Of The Aorta ◽  
Venous Drainage ◽  
Truncus Arteriosus ◽  
Unique Case ◽  
Cardiac Defects ◽  
Hypoplastic Aortic Arch

AbstractTruncus arteriosus communis is a rare CHD, accounting for only 1% of all congenital cardiac abnormalities. It has been associated with other malformations of the heart, mainly truncal valve (bicuspid/quadricuspid) and aortic arch abnormalities such as right, interrupted, and hypoplastic aortic arch. Cor tratriatrum sinistrum is another rare CHD, and it has been associated with other cardiac defects such as anomalous pulmonary venous drainage, ventricular septal defect, coarctation of the aorta, and tetralogy of Fallot. The combination of truncus arteriosus communis and cor tratriatrum sinistrum has not been reported so far. This case study describes the diagnosis of a unique case, including these two very rare cardiac defects and the successful surgical treatment thereafter.

scholarly journals Diagnosis and surgical repair of congenital double aortic arch in infants

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Yiting Yang ◽  
Xin Jin ◽  
Zhengxia Pan ◽  
Yonggang Li ◽  
Chun Wu
Keyword(s):  
Aortic Arch ◽  
Septal Defect ◽  
Arterial Switch Operation ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Vascular Ring ◽  
Correct Diagnosis ◽  
Double Aortic Arch ◽  
Intracardiac Repair ◽  
Congenital Vascular Malformation

Abstract Objectives Double aortic arch (DAA) is a rare congenital vascular malformation. This study aims to summarize the experience of diagnosis and surgical treatment for congenital double aortic arch. Methods The clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively. Results A total of 24 cases, including 12 patients with isolated DAA and 12 patients with DAA and associated intracardiac defects were identified. There were 14 males and 10 females, with an average age of 11 months. The associated intracardiac malformations included ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), pulmonary stenosis (PS), and patent foramen ovale (PFO). Of the 12 patients with DAA and intracardiac malformations, 7 patients underwent intracardiac repair simultaneously, however, 3 patients underwent isolated double aortic arch correction. One patient with DAA and TGA underwent surgical correction of congenital vascular ring at the first stage, and the arterial switch operation was performed at the second stage. The clinical outcomes of 23 patients were promising, however, in one patient, parents decided not to do the surgery due to personal reasons. The average follow-up time was 35 months. Conclusions Tracheal and esophageal compression are commonly seen in patients with DAA, however could be relieved significantly after surgery. In particular cases, the simultaneous intracardiac defects repair could be performed. Misdiagnosis was easily established with isolated echocardiography. Fortunately, the correct diagnosis of DAA and associated intracardiac defects could be established with the use of combined chest computed tomography.

Abstract 12635: Nationwide Long-term Follow-up of Pulmonary Atresia With Ventricular Septal Defect

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Anu Kaskinen ◽  
Juha-Matti Happonen ◽  
Ilkka P Mattila ◽  
Olli M Pitkänen
Keyword(s):  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Palliative Surgery ◽  
Total Study Population ◽  
Collateral Arteries

Introduction: The naturally poor survival of pulmonary atresia with ventricular septal defect (PA+VSD) has improved due to evolved perioperative and surgical treatment. Studies including PA+VSD patients, both with and without major aortopulmonary collateral arteries (MAPCAs), with extensive follow-up are scarce. This nationwide study aimed to investigate survival and surgical treatment in PA+VSD patients with and without MAPCAs. Methods: Study comprised 109 PA+VSD patients born in Finland between 1970 and 2007. We reviewed retrospectively medical records and operative reports through December 2011, as well as first available angiograms and preoperative angiograms prior to repair attempt. Results: The median follow-up time for the total study population, including also patients who died during the follow-up, was 11.4 years (IQR 0.8 - 21.1). The incidence of PA+VSD, which could be determined reliably from 1995 to 2007, was 6.1 per 100 000 live births. Although the patients with (n = 43) or without MAPCAs (n = 66) showed no difference in survival (p = 0.74), the patients without MAPCAs had better probability to achieve repair (64% vs. 28%, p < 0.0001). The bigger size of true central pulmonary arteries assessed by McGoon index at first angiogram [HR 0.66 (CI95% 0.49 - 0.88) per 0.5 McGoon index units, p = 0.006] and achievement of repair [HR 0.07 (CI95% 0.03 - 0.17), p < 0.0001] improved the overall survival. After successful repair survival was 93% at 1 year and 91% from 2 years on. Palliated patients, instead, had survival at 1, 5, 10, and 20 years of age of 55%, 42%, 34%, and 20% respectively. However, patients with right ventricle - pulmonary artery connection and septal fenestration had better survival than rest of the palliated patients (p = 0.001). Palliation with a systemic-pulmonary artery shunt increased McGoon index by 41% (p < 0.0001). Conclusions: The patients with MAPCAs had higher risk to remain palliated than patients without, although their survival was similar. Survival of PA+VSD was influenced by the initial size of true central pulmonary arteries and whether repair was achieved. Although palliative procedures may not improve the final outcome of PA+VSD, palliative surgery may have a role in its treatment.

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