Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations

2001 ◽  
Vol 11 (5) ◽  
pp. 505-511 ◽  
Author(s):  
Eva Strömvall Larsson ◽  
Laszlo Solymar ◽  
Bengt O. Eriksson ◽  
Anne de Wahl Granelli ◽  
Mats Mellander
Keyword(s):  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Positive Contrast ◽  
Pulmonary Angiography ◽  
Arterial Oxygen ◽  
Colloid Solution ◽  
Pulmonary Arteriovenous Malformations ◽  
The Right

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.

Pitfalls in the diagnosis of pulmonary arteriovenous malformations

1994 ◽  
Vol 4 (4) ◽  
pp. 408-410
Author(s):  
Michael Schiemmer ◽  
Gerald Tulzer ◽  
Maria Wimmer
Keyword(s):  
Arteriovenous Malformation ◽  
Arteriovenous Malformations ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Angiography ◽  
Arterial Oxygen ◽  
Upper Respiratory Tract ◽  
Upper Respiratory ◽  
The Right ◽  
Enhanced Computed Tomography ◽  
Diagnostic Pitfalls

SummaryA homogeneous opacity of the right upper lobe was found radiographically in a 15-month-old male child during investigation of an upper respiratory tract infection. Based on computerized tomography, the diagnosis was made of a solid tumor of the lung. At thoracotomy, dilated serpentine vessels were found on the surface of the right upper lobe, and the diagnosis of a large arteriovenous malformation was considered. Use of enhanced computed tomography without contrast and failure to note a reduced arterial oxygen saturation were two diagnostic pitfalls. The diagnosis of an arteriovenous malformation was confirmed by pulmonary angiography, and a right upper lobectomy was successfully performed.

scholarly journals Massive haemorrhagic complications of ruptured pulmonary arteriovenous malformations: outcomes from a 12 years’ retrospective study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang
Keyword(s):  
Arteriovenous Malformations ◽  
Arterial Oxygen Saturation ◽  
Arterial Oxygen ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Pulmonary Arteriovenous Malformations ◽  
Imaging Data ◽  
Peripheral Lung ◽  
Life Threatening ◽  
The Mean

Abstract Background The life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs. Methods We retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021. Results Eighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P < 0.05). The mean follow-up time was 3.2 ± 2.5 years (range, 7 months to 10 years). Conclusions Life threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.

Balloon dilation of the right ventricular outflow tract in tetralogy of Fallot: a palliative procedure

1999 ◽  
Vol 9 (1) ◽  
pp. 11-16 ◽  
Author(s):  
Sameh M. Arab ◽  
Abdel-Fattah E. Kholeif ◽  
Salah R. Zaher ◽  
Aly M. Abdel-Mohsen ◽  
A. Samir Kassem ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Balloon Dilation ◽  
Right Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Arterial Oxygen ◽  
Palliative Procedure ◽  
The Mean ◽  
The Right

AbstractFifteen patients requiring palliation for tetralogy of Fallot were treated by balloon dilation because of hypercyanotic spells. The mean age at dilation was 1.9 ± 0.7 years (range 0.5 – 3), and the mean weight 9.8 ± 2.1kg (range 6.0 –13.5). Dilation of the outflow tract was combined with dilation of the left and/or right pulmonary arteries in 5 patients. Successful dilation was achieved in 12 patients (80%), but failed in 3 patients with hypoplastic pulmonary arteries. In one patient, the stenosis of the right pulmonary artery could not be dilated because of a very sharp angle at the site of the stenosis. Two of the 3 patients in whom the procedure failed died of severe cyanotic spells within 24 hours of the unsuccessful procedure. No major complications occurred during or after the procedure in the cases undergoing successful dilation. The arterial oxygen saturation increased significantly, from 71 ± 5.7% to 89 ± 3.9%, immediately after the procedure (p < 0.005). During a period of follow up of 6 ± 3.7 months (range 1 – 13), the procedure was repeated on 3 occasions, and successfully accomplished in 2 of these. In conclusion, balloon dilation is a satisfactory palliative procedure for tetralogy of Fallot in those units in which total correction is not performed under 2 to 3 years of age.

Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries

2019 ◽  
Vol 29 (8) ◽  
pp. 1036-1039
Author(s):  
Yoichi Kawahira ◽  
Kyoichi Nishigaki ◽  
Koji Kagisaki ◽  
Takuji Watanabe ◽  
Kazuki Tanimoto
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Systolic Pressure ◽  
Cardiac Repair ◽  
Long Term Results ◽  
Arterial Oxygen ◽  
The Right

AbstractBackground:In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch.Methods:Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock–Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years.Results:There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I.Conclusion:Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

scholarly journals Surgical lobectomy of pulmonary arteriovenous malformations in a patient with presentations regarded as sequela of tuberculosis: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Peng Teng ◽  
Weidong Li ◽  
Yiming Ni
Keyword(s):  
Oxygen Saturation ◽  
Arteriovenous Malformations ◽  
Pulmonary Arteries ◽  
Bone Marrow Aspiration ◽  
Pulmonary Angiography ◽  
Pulmonary Arteriovenous Malformations ◽  
Low Oxygen ◽  
Case Presentation ◽  
Digital Clubbing ◽  
Arteries And Veins

Abstract Background Pulmonary arteriovenous malformations are uncommon conditions of abnormal communications between pulmonary arteries and veins, which are most commonly congenital in nature. Although such condition is not extremely rare, it is a challenge to the differential diagnosis of pulmonary problems such as hypoxemia and pulmonary lesions. Case presentation We report a meaningful case of a 23-year-old male presented with elevated hemoglobin (23.0 g/dl) on admission. Physical examination revealed cyanosis, digital clubbing and low oxygen saturation on room air. The patient was initially diagnosed as polycythemia vera while the subsequent result of bone marrow aspiration was negative. During further assessment, pulmonary arteriovenous malformations were detected by CT pulmonary angiography. Lobectomy was successfully performed with significant increase in oxygen saturation from 86 to 98%. The hemoglobin decreased to almost normal level of 14.9 g/dl 3 months after surgery and the patient had been followed up for nearly 5 years. Conclusions Pulmonary arteriovenous malformations should be suspected in patients with central cyanosis, digital clubbing, polycythemia, pulmonary lesion and without cardiac malformations. Embolization or surgery is strongly recommended to reduce the risks caused by pulmonary arteriovenous malformations.

scholarly journals Applicability of Transthoracic Contrast Echocardiography for the Diagnosis and Treatment of Idiopathic Pulmonary Arteriovenous Malformations

2021 ◽  
Vol 8 ◽  
Author(s):  
Yujiao Deng ◽  
Xin Huang ◽  
Guangyi Wang ◽  
Jian Cao ◽  
Shengshu Wang ◽  
...  
Keyword(s):  
Blood Test ◽  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Pulmonary Angiography ◽  
Observer Agreement ◽  
Pulmonary Arteriovenous Malformations ◽  
Digital Subtraction ◽  
Pulmonary Shunt ◽  
Grade 3 ◽  
Transthoracic Contrast Echocardiography

Objective: To explore the preferred test to screen for pulmonary arteriovenous malformations (PAVMs) and to predict the probability of interventional embolization.Methods: We performed a retrospective observational study evaluating patients with idiopathic PAVMs from 2009 to 2019. After clinical evaluation, a total of 105 patients were studied, including 71 patients with positive digital subtraction pulmonary angiography (DSPA) findings and 34 with negative DSPA findings. The following patient data were assessed: blood test, chest radiograph, transthoracic contrast echocardiography (TTCE), and DSPA findings.Results: The majority of patients with idiopathic PAVMs were female (66.2% with positive DSPA findings). We found a good κ-coefficient of 0.77 with strong consistency for inter observer agreement concerning the pulmonary right-to-left shunt (RLS) grade on TTCE, which was superior to conventional chest radiographs. The positive predictive value (PPV) of the radiographic features for PAVMs on DSPA was 0.83 (95% CI 0.64–1.0) and 0.44 for the possibility of embolization (95% CI 0.19–0.70). The PPV of the shunt grade of PAVMs on DSPA was 0.14 (95% CI 0.01–0.29) for grade 1, 0.74 (95% CI 0.60–0.88) for grade 2, and 0.97 (95% CI 0.92–1.0) for grade 3. The PPVs of pulmonary shunt grades 2 and 3 on TTCE for the possibility of embolization for PAVMs were 0.21 (95% CI, 0.05–0.36) and 0.87 (95% CI, 0.79–0.99), respectively.Conclusion: TTCE is the preferred screening test for PAVMs. The pulmonary RLS grade on TTCE not only identifies the likelihood of PAVMs but also predicts the probability for embolization.

scholarly journals Surgical Lobectomy of Pulmonary Arteriovenous Malformations in a Patient with Presentations Regarded as Sequela of Tuberculosis: A Case Report

2020 ◽  
Author(s):  
Peng Teng ◽  
Weidong Li ◽  
Yiming Ni
Keyword(s):  
Oxygen Saturation ◽  
Arteriovenous Malformations ◽  
Pulmonary Arteries ◽  
Bone Marrow Aspiration ◽  
Pulmonary Angiography ◽  
Pulmonary Arteriovenous Malformations ◽  
Low Oxygen ◽  
Case Presentation ◽  
Digital Clubbing ◽  
Arteries And Veins

Abstract Background: Pulmonary arteriovenous malformations are uncommon conditions of abnormal communications between pulmonary arteries and veins, which are most commonly congenital in nature. Although such condition is not extremely rare, it is a challenge to the differential diagnosis of pulmonary problems such as hypoxemia and pulmonary lesions.Case presentation: We report a meaningful case of a 23-year-old male presented with elevated hemoglobin (23.0 g/dl) on admission. Physical examination revealed cyanosis, digital clubbing and low oxygen saturation on room air. The patient was initially diagnosed as polycythemia vera while the subsequent result of bone marrow aspiration was negative. During further assessment, pulmonary arteriovenous malformations were detected by CT pulmonary angiography. Lobectomy was successfully performed with significant increase in oxygen saturation from 86% to 98%. The hemoglobin decreased to almost normal level of 14.9 g/dl three months after surgery and the patient had been followed up for nearly 5 years.Conclusions: Pulmonary arteriovenous malformations should be suspected in patients with central cyanosis, digital clubbing, polycythemia, pulmonary lesion and without cardiac malformations. Embolization or surgery is strongly recommended to reduce the risks caused by pulmonary arteriovenous malformations.

scholarly journals Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878669 ◽  
Author(s):  
Katie L. Mowers ◽  
Lynn Sekarski ◽  
Andrew J. White ◽  
R. Mark Grady
Keyword(s):  
Longitudinal Study ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Clinical Symptoms ◽  
Contrast Echocardiography ◽  
Genetic Diagnosis ◽  
Pulmonary Arteriovenous Malformations ◽  
Initial Screening ◽  
And Gender ◽  
Transthoracic Contrast Echocardiography

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for >1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood.

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