scholarly journals Idiopathic Pulmonary Arteriovenous Malformation - A Rare Entity

2021 ◽  
Vol 10 (41) ◽  
pp. 3604-3606
Author(s):  
Sanyukta Hepat ◽  
Ruchita Kabra ◽  
Abhijit Wadekar ◽  
Sourya Acharya ◽  
Samarth Shukla ◽  
...  

Pulmonary arteriovenous malformation (PAVM) is one of the rare pulmonary vascular anomalies. Pulmonary arteriovenous malformation results in right to left shunt due to the abnormal communications between the pulmonary arteries and the pulmonary veins bypassing the normal capillary bed.1 This condition being rare could be easily missed, hence, it is essential for clinicians to suspect it based on the classical clinical features. This helps in early diagnosis and deciding further appropriate treatment option. Here we report the case of a patient affected by a large idiopathic pulmonary arteriovenous malformation in the right lung. Most patients with pulmonary arteriovenous malformation are asymptomatic. This is due to the chronic compensation and secondary erythrocytic response. Dyspnoea due to PAVMs are a result of right-to-left shunt. Initial diagnostic tools include chest radiography and contrast enhanced computed tomography but the gold standard is pulmonary angiography.2 Because AVM has substantial morbidity rates associated with it, all patients with PAVMs who can undergo embolization should be treated with transcatheter embolization. In rest of the patients, surgical excision should be considered. The main objective of this study was to highlight the early suspicion and diagnosis of pulmonary arteriovenous malformation as this is easily missed and leads to undue delay of treatment.

2017 ◽  
Vol 6 (9) ◽  
pp. 205846011773210 ◽  
Author(s):  
Kohei Hamamoto ◽  
Emiko Chiba ◽  
Katsuhiko Matsuura ◽  
Tomohisa Okochi ◽  
Keisuke Tanno ◽  
...  

A pulmonary arteriovenous malformation (PAVM) is a direct connection between the pulmonary arteries and veins for which metallic coil transcatheter embolization is the standard of care. Detecting recanalization after PAVM treatment is crucial, but direct visualization with computed tomography or magnetic resonance imaging (MRI) is generally difficult. Here, we report a case of a recanalized PAVM that was directly detected with ultra-short echo time MRI. The detection of these signals in the coils was confirmed in a phantom study.


2020 ◽  
Vol 99 (10) ◽  

Introduction: Pulmonary arteriovenous malformation (PAVM) is formed by abnormal connections between pulmonary arteries and veins that bypass the pulmonary capillaries and transport deoxygenated blood through pulmonary veins to the left heart. This causes insufficient oxygenation of blood in the lungs. This condition remains symptomless for a long period of time. The most common symptoms include shortness of breath on exertion, nosebleeds, increased fatigue and a gradual development of cyanosis. Paradoxical embolism in the brain is a serious complication; it can present with a stroke or a brain abscess. Treatment of the disease consists of embolization of the pathological vascular connections, surgical resection of the affected pulmonary parenchyma and management of concomitant manifestations of the disease. PAVM in most common cases arises as a result of an autosomal dominant hereditary disorder referred to as hereditary hemorrhagic telangiectasia. Case report: In our communication, we document the diagnostic and therapeutic management in a young patient diagnosed with PAVM after falling off his bicycle. Based on comprehensive assessments, AV malformations with a 40% shunt of the pulmonary circulation were detected. An angiographic procedure was not an appropriate option considering the type and extent of the condition. Therefore, video-assisted thoracic resection of the affected pulmonary lobe was indicated. Conclusion: PAVM is a rare finding. PAVM should be ruled out in all patients with hereditary hemorrhagic telangiectasia (HHT) signs in the oral cavity. Contrast sonography of the heart and contract CT of the chest are the methods of choice for the diagnosis. Conservative or pharmacological treatment fails to improve the patient’s status. The condition is usually managed by embolization. Cases where PAVM is rather extensive or diffuse, where endovascular management would be inappropriate, can be well managed using endoscopic resection adequate to the extent of the condition.


2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Jihoon Hong ◽  
Sang Yub Lee ◽  
Jung Guen Cha ◽  
Jae-Kwang Lim ◽  
Jongmin Park ◽  
...  

Abstract Background To assess pulmonary arteriovenous malformation (PAVM) recanalization after embolization based on PAVM diameter changes on computed tomography (CT), with pulmonary angiography used as a gold standard. Methods A retrospective review was done of patients from 2008 to 2019 with a PAVM treated with endovascular embolization. The treatment outcome was determined by conventional angiography. Follow-up pulmonary angiography was performed when recanalization was suspected on CT, or embolization of all lesions in multiple PAVM patients could not be completed in a single session. Patients who had no preprocedural or follow-up CT were excluded. Draining vein, feeding artery, and venous sac diameter were measured on CT, and diameter reduction rates were compared with the widely-used, binary 70 % criteria. Results Forty-one patients with 114 PAVMs were treated during the study period. Eight patients with 50 PAVMs met the inclusion criteria. Mean vein, artery, and venous sac diameter reduction rates were as follows: 59.2 ± 9.3 %, 47.5 ± 10.6 %, and 62.6 ± 13.2 %, respectively, in the occluded group and 5.4 ± 19.5 %, 11.3 ± 17.7 %, and 26.8 ± 14.2 %, respectively, in the recanalized group. The area under the receiver operating characteristic curves for PAVM recanalization for the draining vein was 1.00, showing a better result than the artery (0.97) and sac (0.99). Patients showed > 42 % draining vein diameter reduction in the occluded group and < 32 % in the recanalized group. The widely-used 70 % criteria showed low specificity for predicting recanalization (draining vein, 7.3 %; venous sac, 41.7 %) but 100 % sensitivity for both the draining vein and venous sac. Conclusions The widely-used 70 % binary criteria showed limited performance in predicting outcomes in this angiographically-confirmed case series. Further investigations are warranted to establish a strategy for detecting recanalization after PAVM embolization.


2020 ◽  
Vol 18 (1) ◽  
pp. 28-30
Author(s):  
Zanda Grīnberga ◽  
Pauls Sīlis ◽  
Elīna Ligere ◽  
Ingūna Lubaua ◽  
Inta Bergmane ◽  
...  

SummaryCor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.


1995 ◽  
Vol 269 (4) ◽  
pp. R943-R947
Author(s):  
Y. Kikuchi ◽  
H. Nakazawa ◽  
D. L. Traber

We developed a chronic lung fistula that drains only the left lung, allowing for evaluation of injury in a single lung. To remove lymph drainage from the right lung into the caudal mediastinal lymph node, the right lower pulmonary ligament was severed. Pneumatic occluders were placed on the left pulmonary arteries and veins. To ensure that lymph drained from only the left lung, we increased the right pulmonary arterial pressure (RPAP) from 21.2 +/- 0.5 to 36.5 +/- 0.6 mmHg. The left pulmonary arterial pressure (LPAP) was kept at wedge pressure level for 1 h by inflating pneumatic occluders. Lymph flow from the left lung fistula was stable during this occlusion. Six hours after recovery was increased the LPAP from a baseline level of 19.1 +/- 1.0 to 36.4 +/- 0.9 mmHg and the RPAP from 21.2 +/- 0.5 to 38.0 +/- 0.8 mmHg for 2 h by inflating the pneumatic occluders on the left and right pulmonary veins. Lymph flow increased from 5.3 +/- 1.0 to 28.0 +/- 2.9 ml/h. Reflection coefficient was calculated at 0.80 +/- 0.02.


2001 ◽  
Vol 11 (5) ◽  
pp. 505-511 ◽  
Author(s):  
Eva Strömvall Larsson ◽  
Laszlo Solymar ◽  
Bengt O. Eriksson ◽  
Anne de Wahl Granelli ◽  
Mats Mellander

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.


2019 ◽  
Vol 53 (6) ◽  
pp. 492-496
Author(s):  
Yuan Li ◽  
Guang-Chao Gu ◽  
Bao Liu ◽  
Jiang Shao ◽  
Yu Chen ◽  
...  

Bronchial artery aneurysm (BAA) is a rare entity. Ruptured BAA can cause life-threatening hemorrhage. It is recommended that treatment should be initiated immediately after diagnosis. We present the case of a 56-year-old female with multiple BAAs and interstitial lung disease. Aortic computed tomography angiography demonstrated that the largest aneurysm at the right hilum was fed by right subclavian artery and right bronchial artery. A fistula between the pulmonary trunk and the aneurysm was also revealed. The patient underwent transcatheter embolization. Coils were placed in the feeding vessels instead of the aneurysms to avoid nontarget embolization of the pulmonary arteries through the fistula. The procedure achieved reduction in aneurysmal blood flow. The patient’s cough resolved at 6-month follow-up.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Can Yilmaz Yozgat ◽  
Erkan Cakir ◽  
Hakan Yazan ◽  
Hafize Otcu Temur ◽  
Kahraman Yakut ◽  
...  

Cor triatriatum sinister is a rare congenital cardiac anomaly. The anomaly is caused by a fibromuscular membrane that divides the left atrium into two cavities. This membrane can lead to the obstruction of left atrial flow and also create pulmonary venous hypertension. Pulmonary arteriovenous malformation (PAVM) is notorious for its aberrant blood flow between the pulmonary arteries and veins. Herein, we report a case of a 4-year-old toddler who had a unique form of pulmonary hypertension presenting with cor triatriatum sinister and diffuse PAVM. After the surgical treatment of cor triatriatum sinister, both pulmonary arteriovenous malformation and pulmonary hypertension disappeared.


Sign in / Sign up

Export Citation Format

Share Document