scholarly journals Pulmonary atresia with hypoplastic right ventricle and large subaortic ventricular septal defect

2011 ◽  
Vol 23 (3) ◽  
pp. 151-153
Author(s):  
Muhammad Arif Khan ◽  
Mohammed Omar Galal
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Hypoplastic Right Ventricle

Pulmonary Atresia with Dextroposition of the Aorta and Ventricular Septal Defect in Three Arabian Foals

1982 ◽  
Vol 19 (2) ◽  
pp. 160-168 ◽  
Author(s):  
A. Vitums ◽  
W. M. Bayly
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Interventricular Septum ◽  
Pulmonary Atresia ◽  
Pulmonary Trunk ◽  
Exercise Intolerance ◽  
Large Defect ◽  
Persistent Ductus Arteriosus

Three Arabian male foals were presented with cyanosis, heart murmur, and exercise intolerance. Results of clinical evaluation suggested a tentative diagnosis of ventricular septal defect in conjunction with malformations of the great arteries. Each foal had a poor prognosis and was killed at the owners' requests. At necropsy, the malformed hearts of the three foals were virtually identical. Each heart had a large defect in the upper interventricular septum. The aorta originated from the hypertrophied right ventricle and partially overrode the ventricular septal defect. The aortic ostium was guarded by three semilunar valvules. The position of the valvules and the ostia of the coronary arteries was abnormal. The pulmonary arteries arose from the persistent ductus arteriosus. The pulmonary trunk was reduced to a thin, fibrous cord extending from a short and narrow persistent ductus arteriosus to the level of the semilunar valve, where it fused with the wall of the aorta. No communication of the atretic pulmonary trunk with the cavity of the right ventricle was seen.

Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

2017 ◽  
Vol 8 (5) ◽  
pp. 564-569 ◽  
Author(s):  
Edon J. Rabinowitz ◽  
Shilpi Epstein ◽  
Nina Kohn ◽  
David B. Meyer
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Arterial Tree ◽  
Collateral Arteries ◽  
Complete Repair ◽  
Pulmonary Arterial

Background: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. Methods: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Results: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Conclusion: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

Bilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect

2021 ◽  
Vol 31 (4) ◽  
pp. 644-645
Author(s):  
Atsuko Kato ◽  
Kimihiko Yoshii ◽  
Hiroshi Nishikawa ◽  
Hajime Sakurai
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Ventricular Dysfunction ◽  
Pulmonary Atresia ◽  
Multiple Organ ◽  
Cardiopulmonary Support ◽  
Coronary Ischemia ◽  
The Right ◽  
Double Orifice

AbstractThis is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. She required extracorporeal cardiopulmonary support because of ventricular dysfunction and mitral regurgitation. Although she was temporarily weaned off the support after mitral valvuloplasty, she died from multiple organ failure. To the best of our knowledge, bilateral coronary ostial atresia associated with pulmonary atresia with ventricular septal defect has not been reported previously.

Hybrid procedure of right ventricle outflow tract stenting in small infants with pulmonary atresia and ventricular septal defect: early and mid-term results from a single centre

2019 ◽  
Vol 29 (3) ◽  
pp. 375-379 ◽  
Author(s):  
Sara Bondanza ◽  
Maria Grazia Calevo ◽  
Maria Elena Derchi ◽  
Francesco Santoro ◽  
Maurizio Marasini
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Hybrid Procedure ◽  
Outflow Tract ◽  
Congenital Defects ◽  
Hybrid Technique ◽  
Collateral Arteries

AbstractIntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow.MethodsWe retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes.ResultsMedian age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12–103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months.ConclusionsWe reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.

Pulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation

2020 ◽  
Vol 30 (11) ◽  
pp. 1679-1687
Author(s):  
Marien Lenoir ◽  
Virginie Fouilloux ◽  
Beatrice Desnous ◽  
Bilal Rahmani ◽  
Nabila El Gueddari ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Hospital Mortality ◽  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Biventricular Repair ◽  
Palliative Procedure ◽  
Blalock Taussig Shunt

AbstractBackground:Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock–Taussig shunt. We compared the clinical outcomes of the two procedures.Methods:From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock–Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth.Results:After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock–Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock–Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63).Conclusions:The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock–Taussig shunt for rate of biventricular repair and time to biventricular repair.

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