Solitary arterial trunk

1994 ◽  
Vol 4 (1) ◽  
pp. 71-74
Author(s):  
Daniel J. Penny ◽  
Rakesh Dua ◽  
James L. Wilkinson
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Trunk ◽  
Descending Aorta ◽  
Perimembranous Ventricular Septal Defect ◽  
Arterial Trunk ◽  
Pulmonary Arterial

AbstractA heart is described in which there was a solitary arterial trunk that exited from the heart astride a large perimembranous ventricular septal defect, with the pulmonary arterial supply originating entirely from collaterals from the descending aorta. There was no evidence of either hilar pulmonary arteries or an atretic pulmonary trunk. The morphology of the ventricular outlet component, with absence of the outlet septum supports the description of this heart as solitary arterial trunk, rather than tetralogy of Fallot with pulmonary atresia.

Velo-Cardio-Facial Syndrome Associated With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries

PEDIATRICS ◽  
1992 ◽  
Vol 89 (5) ◽  
pp. 915-919
Author(s):  
Kerry B. Jedele ◽  
Virginia V. Michels ◽  
Francisco J. Puga ◽  
Robert H. Feldt
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Severe Form ◽  
Arterial Tree ◽  
Complete Repair ◽  
Pulmonary Arterial ◽  
Ear Anomalies ◽  
Velo Cardio Facial Syndrome

We report on 15 patients with velo-cardio-facial syndrome who had a severe form of tetralogy of Fallot (pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries). Noncardiac anomalies in these patients included typical facial and ear anomalies in 15, nasal speech in 13, palate anomalies in 10, and developmental delay in 10. Seven patients had significant bronchospasm, which has not been reported in association with the velo-cardio-facial syndrome. All 15 patients had severe abnormalities of the arborization of the pulmonary arterial tree, which also has not been reported in velo-cardio-facial syndrome. All patients underwent staging operations to prepare the true pulmonary vascular tree for complete repair of the defect (five underwent complete repair and three survived). Of the remaining 10 patients, 6 are awaiting further operation, 3 are not candidates for complete repair, and 1 has died.

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.

Deposition of collagen in the alveolar wall of lungs from patients with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries—an ultrastructural study

1994 ◽  
Vol 4 (3) ◽  
pp. 277-284
Author(s):  
Hikaru Matsuda ◽  
Tohru Kuratani ◽  
Yasuhisa Shimazaki ◽  
Keishi Kadoba ◽  
Jyunjiro Kobayashi ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Ultrastructural Study ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Alveolar Wall ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

AbstractAbstract Lung biopsies were taken at surgery from five patients (age 2–13, average 7.6 years) with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. The biopsies were then processed for ultrastructural study, comparing paired samples taken, on the one hand, from a segment connected to central pulmonary arteries and, on the other hand, from a segment supplied directly by collateral arteries. Specimens from patients with isolated ventricular septal defect, and from those without cardiac disease, were used as controls. In the patients with tetralogy of Fallot and pulmonary atresia, all biopsies taken from segments supplied by the major collateral arteries showed marked deposition of collagen in the alveolar wall, with an increase in the thickness of the basement membrane (3.6±1.2 µm, mean±SD) greater than seen in those taken from segments connected to central pulmonary arteries (0.9±0.6, p<0.05). The proportional fibrosis of the alveolar interstitial space was also significantly greater in the biopsies from the segments supplied by collateral arteries (30±9%) compared to those from segments fed by central pulmonary arteries (15±8%, p<0.05). No significant differences were found in these indices between the biopsies from segments connected to the central pulmonary arteries in patients with tetralogy of Fallot and pulmonary atresia and those from patients with isolated ventricular septal defect or normal controls. There was no apparent relation to the pulmonary arterial pressure in these findings. The results suggest that the pulmonary segments fed directly by major aortopulmonary collateral arteries in patients with tetralogy of Fallot and pulmonary atresia seem likely to be afflicted by alveolar wall fibrosis, although the etiology and clinical implications of this finding remain unclear.

Use of pulmonary arterial unifocalization into cryopreserved homograft pulmonary arterial bifurcation grafts to facilitate complete correction of pulmonary atresia with ventricular septal defect, nonconfluent (absent) central pulmonary arteries and multiple major aortopulmonary collateral arteries

1995 ◽  
Vol 5 (3) ◽  
pp. 217-224 ◽  
Author(s):  
Richard A. Hopkins ◽  
Deborah A. Imperato ◽  
John T. Cockerham ◽  
Stephen R. Shapiro
Keyword(s):  
Ventricular Septal Defect ◽  
Vascular Disease ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Vascular Disease ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Pulmonary Arterial ◽  
Aortopulmonary Collateral

AbstractThree children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.

Morphologic and hemodynamic results of staged repair of pulmonary atresia with ventricular septal defect in the presence of hypoplastic pulmonary arteries and systemic-to-pulmonary collateral arteries

1993 ◽  
Vol 3 (2) ◽  
pp. 98-103
Author(s):  
James L. Wilkinson ◽  
Yin Ming Ng ◽  
Krishnan S. Iyer ◽  
Roger B. B. Mee
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Ventricular Pressure ◽  
Pulmonary Vascular Disease ◽  
Time Interval ◽  
Collateral Arteries ◽  
Pulmonary Arterial

AbstractFollow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.

scholarly journals Correction of pulmonary atresia with ventricular septal defect in the absence of the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV)

1987 ◽  
Vol 94 (6) ◽  
pp. 911-914 ◽  
Author(s):  
Miguel Barbero-Marcial ◽  
Arlindo Rizzo ◽  
Antonio A.B. Lopes ◽  
Delmont Bittencourt ◽  
José O.A. Junior ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Trunk ◽  
Type Iv

Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect

2001 ◽  
Vol 42 (1) ◽  
pp. 63-69 ◽  
Author(s):  
C. Holmqvist ◽  
P. Hochbergs ◽  
G. Björkhem ◽  
S. Brockstedt ◽  
S. Laurin
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Operative Evaluation
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