Pulmonary atresia with intact septum versus severe pulmonary stenosis. 20 years of experience

2016 ◽  
Author(s):  
Javier Rodriguez-Fanjul
Keyword(s):  
Pulmonary Stenosis ◽  
Pulmonary Atresia ◽  
Years Of Experience

Chromosome 22q11 deletion in a patient with pulmonary atresia, intact ventricular septum, and confluent branch pulmonary arteries

2017 ◽  
Vol 28 (3) ◽  
pp. 467-470 ◽  
Author(s):  
Varun Aggarwal ◽  
Michaki Imamura ◽  
Carlos Acuna ◽  
Antonio G. Cabrera
Keyword(s):  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
22Q11.2 Deletion Syndrome ◽  
Ventricular Septum ◽  
Deletion Syndrome ◽  
Intact Ventricular Septum ◽  
Chromosome 22Q11 ◽  
Chromosome 22Q11.2 ◽  
22Q11.2 Microdeletion

AbstractIn this study, we report a patient with pulmonary atresia with intact ventricular septum (PA/IVS), confluent pulmonary arteries supplied by an arterial duct, and chromosome 22q11.2 microdeletion. The 22q11.2 deletion syndrome has been associated with anomalies of the outflow tracts, such as tetralogy of Fallot with either pulmonary stenosis or atresia, but we are aware of a solitary case described with pulmonary atresia when the ventricular septum is intact. The presence of genetic malformations can have long-term co-morbidities. By describing our patient, we aim to create awareness of this rare association.

More Than 25 Years of Experience in Managing Pulmonary Atresia With Intact Ventricular Septum

2014 ◽  
Vol 98 (5) ◽  
pp. 1680-1686 ◽  
Author(s):  
Adriaan W. Schneider ◽  
Nico A. Blom ◽  
Eline F. Bruggemans ◽  
Mark G. Hazekamp
Keyword(s):  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Years Of Experience ◽  
Intact Ventricular Septum

scholarly journals Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience

2011 ◽  
Vol 171 (3) ◽  
pp. 579-586 ◽  
Author(s):  
Ming-Tai Lin ◽  
Jou-Kou Wang ◽  
Yih-Sharng Chen ◽  
Wen-Jeng Lee ◽  
Hsin-Hui Chiu ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Years Of Experience ◽  
Pulmonary Arterial

Outcome of the construction of a Blalock–Taussig shunt in adolescents and adults

2005 ◽  
Vol 15 (4) ◽  
pp. 368-372 ◽  
Author(s):  
Aubrey Peries ◽  
Amira A. A. Al-Hay ◽  
Elliot A. Shinebourne
Keyword(s):  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Symptomatic Improvement ◽  
Cardiac Events ◽  
Volume Load ◽  
Adverse Cardiac Events ◽  
Adolescents And Adults ◽  
Blalock Taussig Shunt

Aims:The purpose of our study was to ascertain the outcome of the construction of a Blalock–Taussig shunt in patients aged 12 years and over.Patients and methods:We identified 21 patients in whom a Blalock–Taussig shunt had been constructed subsequent to the age of 12 years. Of the patients, 9 were female, and their median age was 18.5 years, with a range from 12 to 46 years. All had usual atrial arrangement, and the atrioventricular connections were concordant in 11, with univentricular atrioventricular connection in 10. Pulmonary atresia was present in 8 (38 per cent), and pulmonary stenosis, either valvar or subvalvar, in 13. An interposition graft had been placed between the subclavian and pulmonary arteries in 16 patients, and an end-to-side anastomosis between the arteries in 5.Results:One patient had died in hospital, while 4 patients had died during the period of follow-up after initial construction of the shunt. Long-term follow-up was available in 86 per cent of patients. In the 3 patients lost to follow-up, the shunt had been known to be functioning at periods of 4, 8, and 10 years, respectively. Actuarial freedom from death after a period of 17 years was 76 per cent. In 8 patients, a period of 10 years had elapsed with the shunt patent, and a further 10 had a patent shunt after 5 years follow-up. Symptomatic improvement was reported in 16 (76 per cent) patients, although adverse cardiac events had occurred during follow-up in 17, including congestive heart failure in 3, atrial fibrillation in 3, and endocarditis in 2. In 2 patients, it had been possible to proceed to biventricular repair, one with tetralogy of Fallot, and the other having a Rastelli procedure. Further in 3 patients, it had been possible to construct the Fontan circulation, or one of its variants. One patient has undergone cardiac transplantation, while 2 are awaiting transplantation.Conclusions:Symptomatic improvement can be achieved by construction of a Blalock–Taussig shunt in older subjects, and the risks of surgery are low. Later repair may be feasible in some patients, but adverse cardiac events may follow the increased volume load on the systemic ventricle.

Coil embolization of aortopulmonary collateral arteries as an emergency in infants and children

1993 ◽  
Vol 3 (2) ◽  
pp. 141-143 ◽  
Author(s):  
Ugo Vairo ◽  
Luigi Ballerini ◽  
Duccio di Carlo ◽  
Luciano Pasquini ◽  
Roberto Di Donato ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Transcatheter Embolization ◽  
Ventricular Outflow Tract ◽  
Pulmonary Congestion ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

SummaryEmbolization of major aortopulmonary collateral arteries by coils introduced via catheterization is a well-established procedure for the control of excessively high pulmonary blood flow in patients with tetralogy of Fallot and pulmonary stenosis or atresia. Under special circumstances, this procedure can be life-saving. Between October 1987 and February 1992, four patients with tetralogy of Fallot and pulmonary atresia and one patient with pulmonary stenosis rather than atresia underwent transcatheter embolization of major aortopulmonary collateral arteries as an emergency. Their ages ranged from 41 days to 13 years, with a mean of 45 months. Two patients had recently undergone surgical repair of tetralogy of Fallot while one had been palliated by enlarging the right ventricular outflow tract without closing the ventricular septal defect. All three had been dependent on mechanical ventilation in the postoperative period with signs of pulmonary congestion or intraparenchymal hemorrhage. Eight embolizations were attempted with 0.025 or 0.038 inch coils; seven collateral arteries were completely occluded and one was partially occluded. Thirteen coils were used in total. The systemic saturation rose in one postsurgical patient, with radiological evidence of decreased pulmonary congestion and regression of hemorrhage. In the other cases, the systemic saturation was unchanged. Peak pulmonary arterial pressures dropped in two patients but remained unchanged in three. Our experience suggests that embolization of major aortopulmonary collateral arteries can be beneficial in critical patients, thus avoiding the risks of surgical reexploration.

Arterial supply to the lungs in tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis

1992 ◽  
Vol 2 (1) ◽  
pp. 65-72 ◽  
Author(s):  
Siew Yen Ho ◽  
Gualtiero Catani ◽  
Jeong-Wook Seo
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Arterial Supply ◽  
Collateral Arteries ◽  
Pulmonary Parenchyma ◽  
Different Origins ◽  
Different Sources ◽  
Critical Pulmonary Stenosis

SummaryThe arterial supply to the lungs in cases with tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis is mostly via the arterial duct or else by collateral arteries arising directly or indirectly from the aorta. Nine anatomical specimens with collateral arteries were studied by blunt dissection to determine the arterial supply to each pulmonary segment. The precise supply varied from case to case but, overall, nearly two-thirds of the pulmonary segments were connected to the central pulmonary arteries. A quarter were supplied exclusively by systemic-to-pulmonary collateral arteries, with the remainder having a dual supply. Coexistence of ductal connections and systemic-to-pulmonary collateral arteries was seen in one case. Anastomoses between vessels of different origins were observed up to the segmental level in eight of the nine cases. In four cases, the anastomoses formed arterial rings. The extent of pulmonary parenchyma supplied by arteries from different sources and the presence of arterial anastomoses in these malformations have implications on the planning of surgical repair.

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