Diagnosis and Transvascular Embolization for Ruptured Pulmonary Arteriovenous Malformations: Outcomes from a 12 Years’ Retrospective Study

Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang

Abstract Background Pulmonary arteriovenous malformations (PAVMs) are rare, and the life-threatening hemorrhagic complications, including hemoptysis and hemothorax, are extremely uncommon. However, the management of large series of ruptured PAVMs has not been described in recent years. Methods We retrospectively reviewed patients who developed ruptured PAVMs between January 2008 and December 2019. We assessed the clinical and imaging data to summarize the incidence and characteristics of ruptured PAVMs, and outcomes following embolization with coils or plugs. A paired-sample t-test analysis, Kaplan–Meier method and the simple linear regression were accessed, as appropriate.Results Eighteen (5.49%) of 328 patients diagnosed with PAVMs developed hemorrhagic complications. Twelve of these 18 patients were clinically diagnosed with hereditary hemorrhagic telangiectasia (HHT) (incidence, 66.67%). Eleven of 18 patients were female. Eight of the 11 females were diagnosed with HHT. Twenty-eight lesions were detected, and the simple type was more common (82.14%) than the complex type. 89.29% of lesions were located in the peripheral lung. Computed tomography angiography (CTA) showed indirect signs to predict the ruptured PAVMs in cases of hemothorax. Technical success of embolotherapy was achieved in all cases. Two days after treatment, pulmonary function was significantly improved and bleeding was controlled (P<0.05), and no patients showed recanalization of PAVMs. Two patients in the hemoptysis group died of other reasons during follow-up and the mean follow-up time was 3.22±2.52 years (range, 7 months to 10 years). The hemorrhage volume was linearly associated with the diameter of the afferent arteries in the ruptured lesions.Conclusions CTA was able to diagnose ruptured PAVMs, and embolotherapy led to successful resolution of symptoms in all 18 patients who had ruptured PAVMs. Ruptured PAVMs must be considered when managing patients with hemothorax or hemoptysis, especially female patients with HHT.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang

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.


2021 ◽  
pp. 1-10
Author(s):  
Ann Mansur ◽  
Alex Kostynskyy ◽  
Timo Krings ◽  
Ronit Agid ◽  
Ivan Radovanovic ◽  
...  

OBJECTIVE The aim of this study was to 1) compare the safety and efficacy of acute targeted embolization of angiographic weak points in ruptured brain arteriovenous malformations (bAVMs) versus delayed treatment, and 2) explore the angioarchitectural changes that follow this intervention. METHODS The authors conducted a retrospective analysis of a prospectively acquired database of ruptured bAVMs. Three hundred sixteen patients with ruptured bAVMs who presented to the hospital within 48 hours of ictus were included in the analysis. The first analysis compared clinical and functional outcomes of acutely embolized patients to those with delayed management paradigms. The second analysis compared these outcomes of patients with acute embolization to those with angiographic targets who did not undergo acute embolization. Finally, a subset of 20 patients with immediate postembolization angiograms and follow-up angiograms within 6 weeks of treatment were studied to determine the angioarchitectural changes after acute targeted embolization. Kaplan-Meier curves for survival between the groups were devised. Multivariate logistical regression analysis was conducted. RESULTS There were three deaths (0.9%) and an overall rerupture rate of 4.8% per year. There was no statistical difference in demographic variables, mortality, and rerupture rate between patients with acute embolization and those with delayed management. Patients with acute embolization were more likely to present functionally worse (46.9% vs 69.8%, modified Rankin Scale score 0–2, p = 0.018) and to require an adjuvant therapy (71.9% vs 26.4%, p < 0.001). When comparing acutely embolized patients to those nonacutely embolized angiographic targets, there was a significant protective effect of acute targeted therapy on rerupture rate (annual risk 1.2% vs 4.3%, p = 0.025) and no difference in treatment complications. Differences in the survival curves for rerupture were statistically significant. Multivariate analyses significantly predicted lower rerupture in acute targeted treatment and higher rerupture in those with associated aneurysms, deep venous anatomy, and higher Spetzler-Martin grade. All patients with acute embolization experienced complete obliteration of the angiographic weak point with various degrees of resolution of the nidus; however, some had spontaneous recurrence of their bAVM, while others had spontaneous resolution over time. No patients developed new angiographic weak points. CONCLUSIONS This study demonstrates that acute targeted embolization of angiographic weak points, particularly aneurysms, is technically safe and protective in the early phase of recovery from ruptured bAVMs. Serial follow-up imaging is necessary to monitor the evolution of the nidus after targeted and definitive treatments. Larger prospective studies are needed to validate these findings.


2013 ◽  
Vol 47 (1) ◽  
pp. 50-56 ◽  
Author(s):  
Sławomir Blamek ◽  
Dawid Larysz ◽  
Leszek Miszczyk ◽  
Adam Idasiak ◽  
Adam Rudnik ◽  
...  

Abstract Background. The treatment of large arteriovenous malformations (AVMs) or AVMs involving eloquent regions of the brain remains a challenge. For inoperable lesions, observation, volume-staged radiosurgery or hypofractionated stereotactic radiotherapy (HFSRT) are proposed. The aim of our study was to assess the safety and efficiency of HFSRT for large AVMs located in eloquent areas of the brain. Materials and methods. An analysis of records of 49 patients irradiated for cerebral AVMs with a mean dose of 19.9 Gy (12-28 Gy) delivered in 2-4 fractions with planned gap (at least one week) between fractions. Actuarial obliteration rates and annual bleeding hazard were calculated using Kaplan-Meier survival analysis and life tables. Results. Annual bleeding hazard rates were 4.5% and 1.6% after one and two years of the follow-up, respectively. Actuarial total obliteration rates were 7%, 11%, and 21% and total response rate (total and partial obliterations) 22%, 41%, and 55% after one, two and three years of the follow-up, respectively. There was a trend towards larger total obliteration rate in patients irradiated with fraction dose ≥ 8 Gy and total dose > 21 Gy for lesions of volume ≤ 8.18 cm3 which was not observed in case of partial obliterations. Conclusions. HFSRT results with relatively low obliteration rate but is not associated with a significant risk of permanent neurological deficits if both total and fraction doses are adjusted to size and location of the lesion. Predictive factors for total and partial obliterations can be different; this observation, however, is not firmly supported and requires further studies.


2018 ◽  
Vol 73 (11) ◽  
pp. 951-957 ◽  
Author(s):  
P.E. Andersen ◽  
P.M. Tørring ◽  
S. Duvnjak ◽  
O. Gerke ◽  
H. Nissen ◽  
...  

Thorax ◽  
1995 ◽  
Vol 50 (7) ◽  
pp. 719-723 ◽  
Author(s):  
T J Haitjema ◽  
T T Overtoom ◽  
C J Westermann ◽  
J W Lammers

2020 ◽  
Author(s):  
Pil Gyu Park ◽  
Byung-Woo Yoo ◽  
Jason Jungsik Song ◽  
Yong-Beom Park ◽  
Sang-Won Lee

Abstract Background: We assessed the rate of and the predictor for all-cause mortality in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients receiving plasma exchange (PLEX) and evaluated the survival-benefit of PLEX for diffuse alveolar haemorrhage (DAH) between AAV patients receiving PLEX and those not receiving. Methods: We retrospectively reviewed the medical records of 212 AAV patients. Demographic, clinical and laboratory data at the time of PLEX was collected from both 9 patients receiving PLEX and 10 AAV patients with DAH. The follow-up duration was defined as the period from the time of PLEX or DAH occurrence to death for the deceased patients and as that to the last visit for the survived patients. Results: The median age of 9 AAV patients receiving PLEX was 71.0 years and 5 patients were men. Four of 9 patients receiving PLEX died at a median follow-up duration of 92.0 days. Three died of sepsis and one died of no response to PLEX. When patients with DAH receiving PLEX and those not receiving were compared, there were no significant differences in variables between the two groups. The cumulative patients’ survival rate between patients with DAH receiving PLEX and those not receiving were also compared using the Kaplan-Meier survival analysis but no survival-benefit of PLEX for DAH was observed. Conclusion: The rate of all-cause mortality in 9 AAV patients receiving PLEX was assessed as 44.4% and it was controversial that PLEX is beneficial for the improvement of prognosis of AAV-related DAH.


EP Europace ◽  
2020 ◽  
Vol 22 (2) ◽  
pp. 281-287
Author(s):  
Roy Beinart ◽  
Valentina Kutyifa ◽  
Scott McNitt ◽  
David Huang ◽  
Mehmet Aktas ◽  
...  

Abstract Aims To explore the association between resting heart rate (RHR) and ventricular tachyarrhythmias (VTA) events among patients who were enrolled in MADIT-RIT. Methods and results Multivariate Cox proportional hazards regression modelling was employed to evaluate the association between baseline RHR [dichotomized at the lower quartile (≤63 b.p.m.) and further assessed as a continuous measure] and the risk for any VTA, fast VTA (&gt;200 b.p.m.), and appropriate implantable cardioverter-defibrillator (ICD) therapy, among 1500 patients who were enrolled in MADIT-RIT. Kaplan–Meier survival analysis showed that at 2 years of follow-up the rate of any VTA was significantly lower among patients with low baseline RHR (≤63 b.p.m.) as compared with faster RHR (11% vs. 19%, respectively; P = 0.001 for the overall difference during follow-up). Similar results were shown for the association with the rate of fast VTA (8% vs. 14%, respectively; P = 0.016), and appropriate ICD therapy (10% vs. 18%, respectively; P = 0.004). Multivariate analysis, after adjustment for medical therapy, showed that low baseline RHR was associated with a significant 45% (P = 0.002) reduction in the VTA risk as compared with faster baseline RHRs. When assessed as a continuous measure, each 10 b.p.m. decrement in RHR was associated with a corresponding 13% (P = 0.014) reduction in the VTA risk. Conclusion In MADIT-RIT, low RHR was independently associated with a lower risk for life-threatening arrhythmic events. These findings suggest a possible role for RHR for improved selection of candidates for ICD therapy.


2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Alaa Nabawi ◽  
Nader Abraham ◽  
Ayman Nabawi

Abstract Arteriovenous malformations (AVMs) are congenital vascular anomalies resulting from defects in angiogenesis. Approximately 40% of AVMs go undetected after birth and only experience the delayed clinical onset of symptoms in adulthood. AVMs are rare, representing only 1.5% of all vascular anomalies. The most common sites for the aberrant vascular nidus are the oral cavity and maxillofacial region, which represent 50% of the cases. AVMs are the most challenging and life-threatening form of vascular malformation. Exsanguination, thrombus detachment and embolization are the most hazardous operative risks. Small case series revealed a 75% recurrence rate during a 5-year follow-up, which adds another layer of complexity to their management. Large lesions in the head and neck cause deformation to the patient and present a challenge to the surgeon during their excision among vital structures and reconstruction of the 3D complex defects.


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