Bronchial Obstruction Due to Pulmonary Artery Anomalies. I. Vascular Sling

PEDIATRICS ◽  
1958 ◽  
Vol 22 (1) ◽  
pp. 48-48

The authors report three cases of respiratory embarrassment in infants from compression of the right bronchus and trachea by a "vascular sling" formed by an aberrant left pulmonary artery coursing anterior to the right main-stem bronchus and posterior to the trachea. Five similar cases have been described in earlier literature. The embryologic origin of this anomaly appears to be a disturbed time-sequence in the growth and union of the left pulmonary artery and the left lung bud. The clinical symptoms are those of respiratory distress in the neonatal period produced by tracheobronchial compression, associated with obstructive emphysema of the right lung. Bronchoscopic examination reveals extrinsic pressure on the right bronchus and posterior wall of the trachea. The esophagram does not reveal any posterior indentation and is thereby helpful in distinguishing this entity from the more common "vascular ring" which is a systemic arterial malformation causing constriction of the trachea and esophagus. Early recognition of an anomalous left pulmonary artery is particularly important in view of the fact that the condition can be corrected surgically.

2018 ◽  
Vol 28 (8) ◽  
pp. 1074-1076 ◽  
Author(s):  
Juan D. Cano Sierra ◽  
Camilo F. Mestra ◽  
Miguel A. Ronderos Dumit

AbstractMowat–Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat–Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat–Wilson syndrome than in the general population.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


1981 ◽  
Vol 50 (1) ◽  
pp. 102-106 ◽  
Author(s):  
P. S. Barie ◽  
T. S. Hakim ◽  
A. B. Malik

We determined the effect of pulmonary hypoperfusion on extravascular water accumulation in anesthetized dogs by occluding the left pulmonary artery for 3 h and then reperfusing it for 24 h. The lung was reperfused either at normal left atrial pressure (Pla) or during increased Pla induced by a left atrial balloon. In each case the extravascular water content-to-bloodless dry weight ratio (W/D) of the left lung was compared with that of the right lung. The W/D of the left lung of 3.26 +/- 0.49 ml/g was not significantly different from the value of 2.87 +/- 0.37 for the right lung after the reperfusion at normal Pla. However, the W/D of the left lung of 5.10 +/- 0.38 ml/g was greater (P less than 0.05) than the value of 4.42 +/- 0.34 for the right lung after reperfusion at Pla of 25 Torr. This difference could not be prevented by pretreatment with heparin, suggesting that the increase in lung water content was not due to activation of intravascular coagulation secondary to stasis occurring during the occlusion. Because the left lung was more edematous than the right one, even though both lungs had been subjected to the same increase in Pla, the results suggest that a period of pulmonary hypoperfusion causes an increase in the interstitial protein concentration.


1970 ◽  
Vol 5 (3) ◽  
pp. 68-79
Author(s):  
Ana Carolina Melo ◽  
Barbara Costa Barros ◽  
Helena Sant’Anna Grilo

RESUMOIntrodução: Sling da artéria pulmonar, ou alça da artéria pulmonar é o nome que se dá a uma doença congênita, onde se forma um anel vascular incompleto que comprime estruturas importantes da região do mediastino. O quadro clínico pode ser desde assintomático podendo chegar ao óbito. Dos sintomáticos, mais da metade é diagnosticada logo nos primeiros meses de vida, porém nesses casos o quadro é geralmente leve e evolui de maneira lenta, porém progressiva. Estridor respiratório, tanto na inspiração quanto na expiração e cianose são sintomas comuns desses pacientes. Casuística: Esse projeto visa relatar um caso de Sling de artéria pulmonar, diagnosticado em um Hospital Escola do sul de Minas Gerais que se manifestou logo após o nascimento. Discussão: A criança em questão apresentou os sintomas clássicos da doença, porém devido sua raridade e variedade de diagnósticos diferenciais houve dificuldade em afirmar que se tratava de um Sling. Conclusão: Conclui-se que a doença foi diagnosticada e tratada a tempo e de maneira eficaz, embora fosse severa a gravidade do quadro apresentado pelo recém-nascido.Palavras-chave: Anel vascular, Sling de artéria pulmonar, Estridor respiratório. ABSTRACTIntroduction: Pulmonary artery sling or pulmonary artery handle is the name given to a congenital disease which forms an incomplete vascular ring that compresses important structures of the mediastinal region. The clinical picture may be from asymptomatic, experiencing mild cases turning to severe and even to death. More than half of symptomatic is diagnosed early in life, but in these cases the picture is generally mild and evolves slowly but progressively. Stridor, both in inspiration and in expiration and cyanosis are common symptoms of these patients. Case report: This project aims to report a case of pulmonary artery sling, diagnosed in a hospital school in southern Minas Gerais expressed just after birth. Discussion: The child presented classical symptoms of the disease, due to its rarity and another possible diagnosis was certain difficulty to be sure that it was a sling. Conclusion: The disease was diagnosed and treated on time and on the right way despite the severe gravity of the case presented by the newborn.Keywords: Vascular ring, Pulmonary artery sling, Stridor


2013 ◽  
Vol 12 (4) ◽  
pp. 462-466
Author(s):  
A Santra ◽  
R Padhi ◽  
P Dutta ◽  
R Manjhi ◽  
S Pothal ◽  
...  

Proximal interruption of the unilateral pulmonary artery is a rare congenital anomaly, which is often associated with other cardiovascular abnormalities. It is usually diagnosed in children but rarely discovered in adulthood as an isolated phenomenon, occurring more frequently on the right side and is often associated with a contralateral aortic arch. We are presenting a rare case of a sixty year old male who was diagnosed with left lung hypoplasia due to proximal interruption of left pulmonary artery with left sided aortic arch without any associated cardiovascular anomalies. DOI: http://dx.doi.org/10.3329/bjms.v12i4.13689 Bangladesh Journal of Medical Science Vol. 12 No. 04 October ’13 Page 462-466


1989 ◽  
Vol 67 (2) ◽  
pp. 528-533 ◽  
Author(s):  
P. T. Overand ◽  
M. J. Bishop ◽  
B. L. Eisenstein ◽  
E. Y. Chi ◽  
M. Su ◽  
...  

We previously reported that pulmonary arterial occlusion for 48 h followed by 4 h of reperfusion in awake dogs results in marked edema and inflammatory infiltrates in both reperfused and contralateral lungs (Am. Rev. Respir. Dis. 134: 752–756, 1986; J. Appl. Physiol. 63: 942–950, 1987). In this experiment we study the effects of alveolar hypoxia on this injury. Anesthetized dogs underwent thoracotomy and occlusion of the left pulmonary artery. Twenty-four hours later the dogs were reanesthetized, and a double-lumen endotracheal tube was placed. The right lung was continuously ventilated with an inspiratory O2 fraction (FIO2) of 0.35. In seven study animals the left lung was ventilated with an FIO2 of 0 for 3 h after the left pulmonary artery occluder was removed. In six control animals the left lung was ventilated with an FIO2 of 0.35 during the same reperfusion period. Postmortem bloodless wet-to-dry weight ratios were 5.87 +/- 0.20 for the left lower lobe and 5.32 +/- 0.12 for the right lower lobe in the dogs with hypoxic ventilation (P less than 0.05 for right vs. left lobes). These values were not significantly different from the control dog lung values of 5.94 +/- 0.22 for the left lower lobe and 5.11 +/- 0.07 for the right lower lobe (P less than 0.05 for right vs. left lobes). All values were significantly higher than our laboratory normal of 4.71 +/- 0.06. We conclude that reperfusion injury is unaffected by alveolar hypoxia during the reperfusion phase.


2019 ◽  
Vol 10 (5) ◽  
pp. 654-656
Author(s):  
Philip B. Dydynski ◽  
John S. Austin ◽  
Deborah Kozik ◽  
Bahaaldin Alsoufi

We present the case of a neonate born with an unusual complex vascular ring formed by a left-sided aortic arch that had retroesophageal course to join a right-sided descending aorta and a very large right-sided arterial ductus from the right pulmonary artery to the descending aorta. Associated finings included aortic arch hypoplasia, aberrant right subclavian artery, and aberrant origin of the left pulmonary artery from the aorta. We focus on the role of computed tomography angiography in the preoperative and postoperative assessment of this complex anomaly.


Author(s):  
Nguyen Chi Lang ◽  
Nguyen Thi Ngoan ◽  
Nguyen Duc Khoi

We present the case of a 4-year-old girl, bronchofiberscopy (scope diameter 2.8mm): one third below oftrachea, which was divided into two orifices. The orifice on the right side was larger and divided into twosuborifices. The left orifice was stenosis and could not pass the bronchoscope. Whole trachea mucosa was redand edema, and could not observe the cartilage ring of trachea. Chest X-ray: infiltration of right lower lobe.Chest CT multiple probes with contrast injection confirmed that: At the sixth to seventh thoracic vertebral level,the left pulmonary artery was arisen from the right pulmonary artery then passed in between the behindtrachea and anterior oesophagus to reach the left lung hilar. And at the fourth to fifth thoracic vertebral level,the right upper bronchus was arisen from trachea and then the trachea was shrunk diameter to reach the leftlung hilar, at the end, the trachea divided 2 bronchi: left main bronchus and “bridging bronchus”. Thatbronchus from left lung hilar returned to the right lung.This patient was diagnosed birth defect: Left pulmonary artery sling with trachea stenosis, type IIA.


1976 ◽  
Vol 40 (6) ◽  
pp. 883-888 ◽  
Author(s):  
J. C. Newell ◽  
M. G. Levitzky ◽  
J. A. Krasney ◽  
R. E. Dutton

In 16 dogs ventilated with 100% O2, control blood flow to the left lung was 35 +/- 2% of aortic flow. When left lung atelectasis was induced, left pulmonary artery flow fell to 19 +/- 2% of aortic flow. A large retrograde component of flow developed in this pulmonary artery, suggesting that blood flows into the pulmonary arteries of both lungs during systole, but flows back out of the collapsed lung and into the uncollapsed lung during diastole. Systemic PaO2 remained above 78 mmHg. Subsequently, when the ventilation of the right lung was changed from oxygen to room air, systemic PaO2 fell to 64 +/- 3 mmHg and atelectatic left lung flow rose from 19 +/- 2% to 28 +/- 2% f aortic flow. This was associated with a reduction in reflux from the atelectatic lung. These results suggest that the attenuation of flow to an atelectatic lung is more pronounced if systemic normoxemia is maintained by adequate oxygenation of the normal lung.


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