The role of angiogenesis in the development of pulmonary arteriovenous malformations in children after cavopulmonary anastomosis

1997 ◽  
Vol 7 (4) ◽  
pp. 370-374 ◽  
Author(s):  
Blair Marshall ◽  
Brian W. Duncan ◽  
Richard A. Jonas

AbstractPulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.

2002 ◽  
Vol 124 (3) ◽  
pp. 479-485 ◽  
Author(s):  
Sunil P. Malhotra ◽  
V.Mohan Reddy ◽  
Stephan Thelitz ◽  
You-Ping He ◽  
D.Michael McMullan ◽  
...  

2002 ◽  
Vol 283 (5) ◽  
pp. H2151-H2156 ◽  
Author(s):  
Sandra L. Starnes ◽  
Brian W. Duncan ◽  
Charles H. Fraga ◽  
Shailesh Y. Desai ◽  
Thomas K. Jones ◽  
...  

We developed a rat model of pulmonary arteriovenous malformations after cavopulmonary anastomosis. We sought to determine whether this model reproduces the angiographic and histologic features seen in the human condition. Eight Sprague-Dawley rats underwent a right superior cavopulmonary anastomosis with the use of microsurgical techniques. Between 2 and 13 mo, pulmonary angiography was performed, the animals were euthanized, and the lungs were removed. Microscopic sections of the lung were stained with an endothelial-specific antibody (von Willebrand factor). Microvessel density was determined by counting vessels staining positively for von Willebrand factor, and the shunted and nonshunted (control) lungs were compared for each animal. Pulmonary angiography revealed time-dependent development of arteriovenous malformations. Microvessel density demonstrated a time-dependent increase in the shunted lung compared with the control lung (simple linear regression of the ratio of the microvessel density of the shunted lung divided by the microvessel density of the control lung on time; R 2 = 0.79, P = 0.003). This animal model reproduces the same angiographic and microscopic features of pulmonary arteriovenous malformations that develop in humans after cavopulmonary anastomosis. This appears to be a valid model that may be used to further study etiologic mechanisms for this phenomenon.


2005 ◽  
Vol 80 (1) ◽  
pp. 338-340 ◽  
Author(s):  
Gonzalo E. Urcelay ◽  
Arturo J. Borzutzky ◽  
Pedro A. Becker ◽  
Maria E. Castillo

2018 ◽  
Vol 41 (12) ◽  
pp. 1849-1856
Author(s):  
Miyuki Maruno ◽  
Hiro Kiyosue ◽  
Norio Hongo ◽  
Shunro Matsumoto ◽  
Hiromu Mori

Abstract Purpose Reperfusion via pulmonary-to-pulmonary arterial anastomoses is known as one type of recurrence of pulmonary arteriovenous malformations (PAVMs) after embolization. It is important to occlude the fistulous portion beyond the origin of the last normal branch from feeding artery of PAVMs to prevent recurrence. In this study, we evaluate the origin of the last normal branch by CT as well as its visibility on pulmonary arteriography (PAG). Materials and Methods We reviewed forty patients with 77 PAVMs who underwent coil embolization between October 2007 and December 2017. All patients underwent MDCT before embolization. Axial and MPR CT lung images were reviewed with special interests in the origin of the last normal branch from feeding artery of PAVMs. The origin was classified into three portions, including sac, junction (portion just proximal to the sac) and proximal feeder (more than 5 mm proximal to the sac). We also evaluated whether PAG can depict the normal branches detected by MDCT. Results MDCT showed that the last normal branch originated from sac in 30 PAVMs (39.0%), junction in 39 (50.6%), and proximal feeder in 8 (10.4%).On selective PAG, the last normal branch could be visualized in 30 PAVMs (39.0%), although it could not be visualized due to high-flow shunt in the other 47 PAVMs. Conclusions Selective PAG frequently fails to demonstrate the last normal branch from feeding artery of PAVMs, which often originates from the sac. Pretherapeutic evaluation of CT images of the last normal branch is important to prevent reperfusion of PAVMs. Level of Evidence Level 3, local non-random sample.


2017 ◽  
Vol 37 (suppl_1) ◽  
Author(s):  
Yasutaka Kawamura ◽  
Motoji Fukazawa ◽  
Shinya Hasegawa ◽  
Yukio Chiba

Purpose: We report cases with pulmonary arteriovenous malformations (PAVM) treated with transcatheter arterial embolization. The clinical features of PAVM and the indication, techniques and longterm outcome of the embolization therapy were assessed. Patients: Six patients (age ranges from 44 to 67, five women and a man) had diagnosed as PAVM. Two patients diagnosed as definite HHT: Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome) with epistaxis and family history and one as possible HHT associating hemorrhagic skin telangiectasias and abnormally enlarged the internal thoracic artery. Indication of the pulmonary arterial embolization therapy: Four patients had the episodes of cerebral infarction, one developed cerebellar ataxia, and another patient was proved the decreased PaO 2 . Two patients revealed the enlargement of PAVM. CT and 3-dimensional CT angiography (CTA) was obtained on 64-multi-detector row unit (Toshiba, Aquilion64, Japan) with bolus injection of contrast agent. The transcatheter pulmonary arterial embolization was performed with microcatheter system (TERUMO, Japan) and 0.035 coils of 6, 4, 3mm in diameter (Boston Scientific, USA). Results: After the selective catheterization for PAVM, all feeding arteries >3 mm in diameter was embolized. Since coil size selection was crucial for the safe and productive procedure, the size of feeding arteries had been carefully estimated on CTA date in advance. All patients were successfully treated without any problems during and after the procedure. One patient with HHT, three years after the treatment of 4.3cm PAVM, showed the recurrent PAVM of 0.5mm in diamete. The other patient showed a small vessel emerged distal to the embolized PAVM draining to the pulmonary vein. These lesions were too small to consider the clinical management and had been followed up with no change. Conclusion: The pulmonary arterial embolization therapy for PAVM was discussed. The treatments were successful, however, some recurrences were noted. We did not thoroughly embolized the distal side of PAVM because to minimize the normal lung damage and to avoid the migration of coils. Deliberate clinical follow-up should be required and should apply the additional treatment, if necessary.


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