Double-Outlet Right Ventricle, Pulmonary Atresia, and Discontinuous Branch Pulmonary Arteries Supplied by Bilateral Ducti

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.

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Persistent 5th Aortic Arch with Coexisting Discontinuous Pulmonary Arteries, Double-Outlet Right Ventricle, and Complete Atrioventricular Septal Defect

Texas Heart Institute Journal ◽

10.14503/thij-13-3817 ◽

2015 ◽

Vol 42 (1) ◽

pp. 92-93 ◽

Cited By ~ 1

Author(s):

George T. Nicholson ◽

Christopher J. Petit ◽

Tracy Pasquarella ◽

M. Eric Ferguson

Keyword(s):

Right Ventricle ◽

Aortic Arch ◽

Septal Defect ◽

Pulmonary Arteries ◽

Atrioventricular Septal Defect ◽

Double Outlet Right Ventricle ◽

Complete Atrioventricular Septal Defect ◽

Complete Atrioventricular

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The Use of Internal and External Vascular Conduits for Correction of D-Transposition of the Great Arteries and Double-Outlet Right Ventricle with Pulmonary Atresia

The Annals of Thoracic Surgery ◽

10.1016/s0003-4975(10)63372-3 ◽

1979 ◽

Vol 27 (6) ◽

pp. 567-568 ◽

Cited By ~ 2

Author(s):

Taro Yokoyama ◽

Guillermo Young ◽

Jerome Harold Kay

Keyword(s):

Right Ventricle ◽

Pulmonary Atresia ◽

Double Outlet Right Ventricle

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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

Cardiology in the Young ◽

10.1017/s1047951118002482 ◽

2019 ◽

Vol 29 (3) ◽

pp. 375-379 ◽

Cited By ~ 3

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.

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Echocardiographic findings in children with Tetralogy of Fallot

Nepalese Heart Journal ◽

10.3126/njh.v12i2.13383 ◽

2015 ◽

Vol 12 (2) ◽

pp. 65-68

Author(s):

Urmila Shakya ◽

Poonam Sharma ◽

Manish Shrestha

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Ductus Arteriosus ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Anatomical Variations ◽

Left Pulmonary Artery ◽

Double Outlet Right Ventricle ◽

Echocardiographic Findings

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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Creation of an intraatrial tunnel to produce a total cavo-pulmonary connection in a patient with dominant right ventricle, totally anomalous pulmonary venous connection, and pulmonary atresia with non-confluent pulmonary arteries

Cardiology in the Young ◽

10.1017/s1047951108003156 ◽

2008 ◽

Vol 18 (6) ◽

pp. 641-643

Author(s):

Koichi Sughimoto ◽

Mitsuru Aoki ◽

Tadashi Fujiwara

Keyword(s):

Pulmonary Artery ◽

Right Ventricle ◽

Single Ventricle ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Surgical Strategy ◽

Anomalous Pulmonary Venous Connection ◽

Goretex Patch

AbstractThe surgical strategy for patients having a functionally single ventricle associated with totally anomalous pulmonary venous connection and pulmonary atresia with non-confluent pulmonary artery has yet to be agreed. We created an intraatrial tunnel to produce a total cavo-pulmonary connection in such a patient, also creating a confluence for the pulmonary arteries. By minimizing the use of the GoreTex patch, the patient was able to discontinue the use of warfarin.

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