Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study.

Chronic interstitial lung abnormalities have been described in sickle cell disease (SCD) and attributed to repetitive episode of acute chest syndrome. We report a series of 22 cases of diffuse cystic lung disease in SCD with a case-control study to hunt for mechanism. On pathological analysis of a surgical lung biopsy of the index case, the bronchioles had the appearance of constrictive bronchiolitis. Pulmonary function test results revealed lower forced expiratory flow from 25% to 75% of vital capacity in cases versus controls. These findings suggest a bronchiolar mechanism that was not associated with more acute chest syndrome.

Pulmonary Arteriovenous Malformations: Safety and Efficacy of Microvascular Plugs.

OBJECTIVE: The purpose of this study is to assess the safety and efficacy of microvascular plugs for the treatment of pulmonary arteriovenous malformations (PAVMs). MATERIALS AND METHODS: From July 2014 to March 2017, 22 consecutive patients with hereditary hemorrhagic telangiectasia underwent treatment of PAVMs using microvascular plugs. The number, location, and type (simple or complex) of PAVM and the diameter of the feeding artery were assessed at angiography. Safety was evaluated by successful detachment and absence of migration of the microvascular plug after deployment. Efficacy was assessed by technical success, defined as immediate stasis in the feeding artery above the microvascular plug at the time of angiography, and by the persistence rate at 1-year follow-up CT. RESULTS: Thirty-nine PAVMs (36 simple and three complex) were treated with 52 microvascular plugs in 22 consecutive patients. Thirty-three PAVMs were undergoing initial treatment and six were undergoing retreatment after previous embolotherapy. All microvascular plugs were successfully detached. No microvascular plug migration was observed. The mean (± SD) feeding artery diameter was 2.3 ± 0.7 mm. Technical success was achieved for 51 of 52 (98%) microvascular plug deployments. Follow-up CT, which was available for 20 of 22 (91%) patients, with a mean delay of 12.6 ± 3.1 months, showed two persistent PAVMs (persistence rate, 6%), one due to recanalization through the microvascular plug and the other due to reperfusion from an untreated adjacent pulmonary feeding artery. CONCLUSION: Microvascular plugs are safe and effective for treatment of PAVMs, with a low persistence rate (6%) 1 year after treatment.

Severe Hemoptysis Associated with Bacterial Pulmonary Infection: Clinical Features, Significance of Parenchymal Necrosis, and Outcome.

PURPOSE: Severe hemoptysis (SH) associated with non-tuberculosis bacterial lower respiratory tract infection (LRTI) is poorly described, and the efficacy of the usual decision-making process is unknown. This study aimed at describing the clinical, radiological patterns, mechanism, and microbiological spectrum of SH related to bacterial LRTI, and assessing whether the severity of hemoptysis and the results of usual therapeutic strategy are influenced by the presence of parenchymal necrosis. METHODS: A single-center analysis of patients with SH related to bacterial LRTI from a prospective registry of consecutive patients with SH admitted to the intensive care unit of a tertiary referral center between November 1996 and May 2013. RESULTS: Of 1504 patients with SH during the study period, 65 (4.3%) had SH related to bacterial LRTI, including non-necrotizing infections (n = 31), necrotizing pneumonia (n = 23), pulmonary abscess (n = 10), and excavated nodule (n = 1). The presence of parenchymal necrosis (n = 34, 52%) was associated with a more abundant bleeding (volume: 200 ml [70-300] vs. 80 ml [30-170]; p = 0.01) and a more frequent need for endovascular procedure (26/34; 76% vs. 9/31; 29%; p < 0.001). Additionally, in case of parenchymal necrosis, the pulmonary artery vasculature was involved in 16 patients (47%), and the failure rate of endovascular treatment was up to 25% despite multiple procedures. CONCLUSIONS: Bacterial LRTI is a rare cause of SH. The presence of parenchymal necrosis is more likely associated with bleeding severity, pulmonary vasculature involvement, and endovascular treatment failure.

Chronic pulmonary aspergillosis complicating sarcoidosis.

Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA.This was a retrospective single-centre study on CPA-SA.In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroids or immunosuppressive drugs). Chronic cavitary pulmonary aspergillosis (CCPA) was the most frequent CPA pattern. Regarding treatment, 55 patients required long-term antifungals, 14 interventional radiology, 11 resection surgery and two transplantation. Nearly half of the patients (27; 41.5%) died (mean age 55.8 years); 73% of the patients achieved 5-year survival and 61% 10-year survival. Death most often resulted from advanced SA and rarely from haemoptysis. CPI, fibrosis extent and PH predicted survival. Comparison with paired healthy controls without CPA did not show any difference in survival, but a higher percentage of patients had high-risk mould exposure.CPA occurs in advanced pulmonary SA. CPA-SA is associated with high mortality due to the underlying advanced SA rather than to the CPA. CPI, fibrosis extent and PH best predict outcome.

Interaction Between ALK1 Signaling and Connexin40 in the Development of Arteriovenous Malformations.

OBJECTIVE: To determine the role of Gja5 that encodes for the gap junction protein connexin40 in the generation of arteriovenous malformations in the hereditary hemorrhagic telangiectasia type 2 (HHT2) mouse model. APPROACH AND RESULTS: We identified GJA5 as a target gene of the bone morphogenetic protein-9/activin receptor-like kinase 1 signaling pathway in human aortic endothelial cells and importantly found that connexin40 levels were particularly low in a small group of patients with HHT2. We next took advantage of the Acvrl1(+/-) mutant mice that develop lesions similar to those in patients with HHT2 and generated Acvrl1(+/-); Gja5(EGFP/+) mice. Gja5 haploinsufficiency led to vasodilation of the arteries and rarefaction of the capillary bed in Acvrl1(+/-) mice. At the molecular level, we found that reduced Gja5 in Acvrl1(+/-) mice stimulated the production of reactive oxygen species, an important mediator of vessel remodeling. To normalize the altered hemodynamic forces in Acvrl1(+/-); Gja5(EGFP/+) mice, capillaries formed transient arteriovenous shunts that could develop into large malformations when exposed to environmental insults. CONCLUSIONS: We identified GJA5 as a potential modifier gene for HHT2. Our findings demonstrate that Acvrl1 haploinsufficiency combined with the effects of modifier genes that regulate vessel caliber is responsible for the heterogeneity and severity of the disease. The mouse models of HHT have led to the proposal that 3 events-heterozygosity, loss of heterozygosity, and angiogenic stimulation-are necessary for arteriovenous malformation formation. Here, we present a novel 3-step model in which pathological vessel caliber and consequent altered blood flow are necessary events for arteriovenous malformation development.

Early Chest Computed Tomography Scan to Assist Diagnosis and Guide Treatment Decision for Suspected Community-acquired Pneumonia.

RATIONALE: Clinical decision making relative to community-acquired pneumonia (CAP) diagnosis is difficult. Chest radiograph is key in establishing parenchymal lung involvement. However, radiologic performance may lead to misdiagnosis, rendering questionable the use of chest computed tomography (CT) scan in patients with clinically suspected CAP. OBJECTIVES: To assess whether early multidetector chest CT scan affects diagnosis and management of patients visiting the emergency department with suspected CAP. METHODS: A total of 319 prospectively enrolled patients with clinically suspected CAP underwent multidetector chest CT scan within 4 hours. CAP diagnosis probability (definite, probable, possible, or excluded) and therapeutic plans (antibiotic initiation/discontinuation, hospitalization/discharge) were established by emergency physicians before and after CT scan results. The adjudication committee established the final CAP classification on Day 28. MEASUREMENTS AND MAIN RESULTS: Chest radiograph revealed a parenchymal infiltrate in 188 patients. CAP was initially classified as definite in 143 patients (44.8%), probable or possible in 172 (53.8%), and excluded in 4 (1.2%). CT scan revealed a parenchymal infiltrate in 40 (33%) of the patients without infiltrate on chest radiograph and excluded CAP in 56 (29.8%) of the 188 with parenchymal infiltrate on radiograph. CT scan modified classification in 187 (58.6%; 95% confidence interval, 53.2-64.0), leading to 50.8% definite CAP and 28.8% excluded CAP, and 80% of modifications were in accordance with adjudication committee classification. Because of CT scan, antibiotics were initiated in 51 (16%) and discontinued in 29 (9%), and hospitalization was decided in 22 and discharge in 23. CONCLUSIONS: In CAP-suspected patients visiting the emergency unit, early CT scan findings complementary to chest radiograph markedly affect both diagnosis and clinical management. Clinical trial registered with www.clinicaltrials.gov (NCT 01574066).

Severe haemoptysis in patients with nonsmall cell lung carcinoma.

Severe haemoptysis due to nonsmall cell lung cancer (NSCLC) is considered a grim condition, and there is still scarce data on its characteristics and outcome, despite new imaging and treatment modalities. This retrospective study sought to describe the clinical characteristics, pathophysiology and outcome of NSCLC-related haemoptysis. We included 125 consecutive patients with severe haemoptysis (>100 mL) at admission, 65 (52%) exhibiting squamous cell carcinoma. Tumour cavitation/necrosis was reported in 26 (21%) patients. 52 patients had received anticancer treatment, but none had received anti-angiogenic agents. Severe haemoptysis was related mainly to the bronchial artery (82%), and major pulmonary artery involvement was rare (6.4%). Interventional radiology was performed in 102 patients. Bleeding cessation was achieved in 108 (87%) out of 125 patients. The overall in-hospital and 1-year survival rates were 69% and 30%, respectively. Performance status (PS) ≥ 2 (OR 3.6, 95% CI 1.3-9.6), advanced stage (OR 8.6, 95% CI 2-37) and mechanical ventilation (OR 13, 95% CI 4.5-36) were independent predictors of in-hospital mortality. Performance status ≥ 2 (hazard ratio (HR) 2.4, 95% CI 1.5-3.7), advanced stage (HR 4, 95% CI 2.1-7.7), cancer progression (HR 2, 95% CI 1.01-2.7) and cavitation/necrosis (HR 1.7, 95% CI 1.21-3.2) were independently associated with 1-year mortality. Management of severe haemoptysis related to NSCLC should be improved, given our observed survival rates after hospital discharge.

How to differentiate benign from malignant myometrial tumours using MR imaging.

PURPOSE: To retrospectively evaluate the ability of magnetic resonance imaging (MRI) to differentiate malignant from benign myometrial tumours. METHODS: Fifty-one women underwent MRI before surgery for evaluation of a solitary myometrial tumour. At histopathology, there were 25 uncertain or malignant mesenchymal tumours and 26 benign leiomyomas. Conventional morphological MRI criteria were recorded in addition to b 1,000 signal intensity and apparent diffusion coefficient (ADC). Odds ratios (OR) were calculated for each criterion. A multivariate analysis was performed to construct an interpretation model. RESULTS: The significant criteria for prediction of malignancy were high b 1,000 signal intensity (OR = +∞), intermediate T2-weighted signal intensity (OR = +∞), mean ADC (OR = 25.1), patient age (OR = 20.1), intra-tumoral haemorrhage (OR = 21.35), endometrial thickening (OR = 11), T2-weighted signal heterogeneity (OR = 10.2), menopausal status (OR = 9.7), heterogeneous enhancement (OR = 8) and non-myometrial origin on MRI (OR = 4.9). In the recursive partitioning model, using b 1,000 signal intensity, T2 signal intensity, mean ADC, and patient age, the model correctly classified benign and malignant tumours in 47 of the 51 cases (92.4 %). CONCLUSION: We have developed an interpretation model usable in routine practice for myometrial tumours discovered at MRI including T2 signal, b 1,000 signal and ADC measurement. KEY POINTS: • MRI is widely used to differentiate benign from malignant myometrial tumours. • By combining T2-weighted, b 1,000 and ADC features, MRI is 92.4 % accurate. • DWI may limit misdiagnoses of uterine sarcoma as benign leiomyoma. • Patient age is important when considering a solitary myometrial tumour.

Pulmonary artery occlusion with ethylene vinyl alcohol copolymer in patients with hemoptysis: initial experience in 12 cases.

OBJECTIVE: The purpose of this study was to evaluate the feasibility and efficacy of pulmonary artery occlusion with ethylene vinyl alcohol copolymer in patients with hemoptysis. MATERIALS AND METHODS: We reviewed the cases of 12 consecutively registered patients (10 men, two women; age range, 21-83 years; mean, 54.5 years) who were treated for hemoptysis by pulmonary artery occlusion with ethylene vinyl alcohol copolymer. The indications, immediate hemoptysis control, and clinical tolerability were analyzed. RESULTS: The underlying diseases were necrotizing pneumonia in four patients, necrotizing aspergillosis in one patient, complex aspergilloma in two patients, active tuberculosis in two patients, lung cancer in two patients, and Behçet disease in one patient. Ethylene vinyl alcohol copolymer was used alone in nine patients and with steel coils in three patients. The main indications were a small-necked pulmonary artery pseudoaneurysm in five patients, necrotic process in four patients, presence of systemic reperfusion of a pulmonary artery pseudoaneurysm in one patient, absolute necessity for anticoagulation therapy in one patient, and need to complete pulmonary artery aneurysm sac occlusion in one patient. The injection procedure was well tolerated. Hemoptysis of pulmonary arterial origin was controlled in all but one patient, who had progression of the infectious disease and underwent surgery. In two patients, hemoptysis recurred from systemic arteries and was treated with embolization of the systemic arteries in one patient and surgery in the other patient. CONCLUSION: Ethylene vinyl alcohol copolymer embolization for hemoptysis of pulmonary arterial origin is feasible and efficacious. Use of this embolization agent is beneficial in patients with pulmonary artery injuries, especially those with small-necked lesions.

Early prediction of in-hospital mortality of patients with hemoptysis: an approach to defining severe hemoptysis.

BACKGROUND: The severity of hemoptysis is usually assessed on the amount of blood expectorated, although no threshold has been agreed upon. Respiratory or hemodynamic failures are additional severity criteria but occur in few cases. OBJECTIVES: Early identification of the in-hospital mortality determinants might be helpful to best characterize severe hemoptysis. METHODS: This is a retrospective cohort study of consecutive patients with hemoptysis admitted to the ICU of a teaching hospital during a 14-year period. The model for early prediction of in-hospital mortality was developed on a derivation sample (67% of patients) using multiple logistic regression. Calibration and discrimination of the model were tested using the remaining validation sample. A scoring system was developed for clinical use. RESULTS: The in-hospital mortality of the 1,087 patients (age 54 years, 71% male) was 6.5% (95% CI 5-8). Chronic alcoholism, cancer or aspergillosis, pulmonary artery involvement, infiltrates involving two quadrants or more on the admission radiograph, and mechanical ventilation at referral predicted independently mortality. The model showed good concordance between predicted and observed probabilities of death and good discrimination (receiver operating characteristic curve area 0.87; 95% CI 0.82-0.92). The model-based score (chronic alcoholism, pulmonary artery involvement, and radiographic patterns, 1 point each; cancer, aspergillosis, and mechanical ventilation, 2 points each) predicted the probability of death as follows: score 0, 1%; score 1, 2%; score 2, 6%; score 3, 16%; score 4, 34%; score 5, 58%; score 6, 79%, and score 7, 91%. CONCLUSIONS: Our results provide useful information about the short-term prognosis of patients with hemoptysis, which could help design therapeutic approaches and management plans according to the risk of in-hospital mortality.

Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output.

CONTEXT: The only treatment available to restore normal cardiac output in patients with hereditary hemorrhagic telangiectasia (HHT) and cardiac failure is liver transplant. Anti-vascular endothelial growth factor treatments such as bevacizumab may be an effective treatment. OBJECTIVES: To test the efficacy of bevacizumab in reducing high cardiac output in severe hepatic forms of HHT and to assess improvement in epistaxis duration and quality of life. DESIGN, SETTING, AND PATIENTS: Single-center, phase 2 trial with national recruitment from the French HHT Network. Patients were 18 to 70 years old and had confirmed HHT, severe liver involvement, and a high cardiac index related to HHT. INTERVENTION: Bevacizumab, 5 mg per kg, every 14 days for a total of 6 injections. The total duration of the treatment was 2.5 months; patients were followed up for 6 months after the beginning of the treatment. MAIN OUTCOME MEASURE: Decrease in cardiac output at 3 months after the first injection, evaluated by echocardiography. RESULTS: A total of 25 patients were included between March 2009 and November 2010. Of the 24 patients who had echocardiograms available for reread, there was a response in 20 of 24 patients with normalization of cardiac index (complete response [CR]) in 3 of 24, partial response (PR) in 17 of 24, and no response in 4 cases. Median cardiac index at beginning of the treatment was 5.05 L/min/m(2) (range, 4.1-6.2) and significantly decreased at 3 months after the beginning of the treatment with a median cardiac index of 4.2 L/min/m(2) (range, 2.9-5.2; P < .001). Median cardiac index at 6 months was significantly lower than before treatment (4.1 L/min/m(2); range, 3.0-5.1). Among 23 patients with available data at 6 months, we observed CR in 5 cases, PR in 15 cases, and no response in 3 cases. Mean duration of epistaxis, which was 221 minutes per month (range, 0-947) at inclusion, had significantly decreased at 3 months (134 minutes; range, 0-656) and 6 months (43 minutes; range, 0-310) (P = .008). Quality of life had significantly improved. The most severe adverse events were 2 cases of grade 3 systemic hypertension, which were successfully treated. CONCLUSION: In this preliminary study of patients with HHT associated with severe hepatic vascular malformations and high cardiac output, administration of bevacizumab was associated with a decrease in cardiac output and reduced duration and number of episodes of epistaxis. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00843440.

CT features of community-acquired pneumonia at the emergency department.

BACKGROUND: Chest computed tomography (CT) was reported to improve the diagnosis of community-acquired pneumonia (CAP) as compared to chest X-ray (CXR). The aim of this study is to describe the CT-patterns of CAP in a large population visiting the emergency department and to see if some of them are more frequently missed on CXR. MATERIALS AND METHODS: This is an ancillary analysis of the prospective multicenter ESCAPED study including 319 patients. We selected the 163 definite or probable CAP based on adjudication committee classification; 147 available chest CT scans were reinterpreted by 3 chest radiologists to identify CAP patterns. These CT-patterns were correlated to epidemiological, biological and microbiological data, and compared between false negative and true positive CXR CAP. RESULTS: Six patterns were identified: lobar pneumonia (51/147, 35%), including 35 with plurifocal involvement; lobular pneumonia (43/147, 29%); unilobar infra-segmental consolidation (24/147, 16%); bronchiolitis (16/147, 11%), including 4 unilobar bronchiolitis; atelectasis and bronchial abnormalities (8/147, 5.5%); interstitial pneumonia (5/147, 3.5%). Bacteria were isolated in 41% of patients with lobar pneumonia-pattern (mostly Streptococcus pneumoniae and Mycoplasma pneumonia) versus 19% in other patients (p = 0.01). Respiratory viruses were equally distributed within all patterns. CXR was falsely negative in 46/147 (31%) patients. Lobar pneumonia was significantly less missed on CXR than other patterns (p = 0.003), especially lobular pneumonia and unilobar infra-segmental consolidation, missed in 35% and 58% of cases, respectively. CONCLUSION: Lobar and lobular pneumonias are the most frequent CT-patterns. Lobar pneumonia is appropriately detected on CXR and mainly due to Streptococcus pneumoniae or Mycoplasma pneumoniae. Chest CT is very useful to identify CAP in other CT-patterns. Prior the COVID pandemic, CAP was rarely responsible for interstitial opacities on CT.

Diagnosis and prognosis of acute respiratory distress syndrome related to diffuse pneumonic-type adenocarcinoma: a single-center case series study.

Background: The absence of diagnosis of acute respiratory distress syndrome (ARDS) concerns 20% of cancer patients and is associated with poorer outcomes. Diffuse pneumonic-type adenocarcinoma (P-ADC) is part of these difficult-to-diagnose ARDS, but only limited data are available regarding critically ill patients with diffuse P-ADC. We sought to describe the diagnosis process and the prognosis of P-ADC related ARDS patients admitted to the intensive care unit (ICU). Methods: Single-center observational case series study. All consecutive patients admitted to the ICU over a two-decade period presenting with (I) histologically or cytologically proven adenocarcinoma of the lung and (II) ARDS according to Berlin definition were included. Clinical, biological, radiological and cytological features of P-ADC were collected to identify diagnostic clues. Multivariate logistic regression analyses were performed to assess factors associated with ICU and hospital mortality. Results: Among the 24 patients included [70 (61-75) years old, 17 (71%) males], the cancer diagnosis was performed during the ICU stay in 19 (79%), and 17 (71%) required mechanical ventilation. The time between the first symptoms and the diagnosis of P-ADC was 210 days (92-246 days). A non-resolving pneumonia after 2 (2 to 3) antibiotics lines observed in 23 (96%) patients with a 34 mg/L (19 to 75 mg/L) plasma C-reactive protein level at ICU admission. Progressive dyspnea, bronchorrhea, salty expectoration, fissural bulging and compressed bronchi and vessels were present in 100%, 83%, 69%, 57% and 43% of cases. Cytological examination of sputum or broncho-alveolar lavage provided a 75% diagnostic yield. The ICU and hospital mortality rates were 25% and 63%, respectively. The time (in days) between first symptoms and diagnosis [odds ratio (OR) 1.02, 95% confidence interval (95% CI): 1.00-1.03, P=0.046] and the Simplified Acute Physiology Score II (OR 1.16, 95% CI: 1.01-1.33, P=0.040) were independently associated with ICU mortality. Conclusions: Non-resolving pneumonia after several antibiotics lines without inflammatory syndrome, associated with progressive dyspnea, salty bronchorrhea, and lobar swelling (i.e., fissural bulging, compressed bronchi and vessels) were suggestive of P-ADC. Delayed diagnosis of diffuse P-ADC seemed an independent prognostic predictor and disease timely recognition may contribute to prognosis improvement.

Randomised trial of first-line bronchial artery embolisation for non-severe haemoptysis of mild abundance.

BACKGROUND: Whereas first-line bronchial artery embolisation (BAE) is considered standard of care for the management of severe haemoptysis, it is unknown whether this approach is warranted for non-severe haemoptysis. RESEARCH QUESTION: To assess the efficacy on bleeding control and the safety of first-line BAE in non-severe haemoptysis of mild abundance. STUDY DESIGN AND METHODS: This multicentre, randomised controlled open-label trial enrolled adult patients without major comorbid condition and having mild haemoptysis (onset <72 hours, 100-200 mL estimated bleeding amount), related to a systemic arterial mechanism. Patients were randomly assigned (1:1) to BAE associated with medical therapy or to medical therapy alone. RESULTS: Bleeding recurrence at day 30 after randomisation (primary outcome) occurred in 4 (11.8%) of 34 patients in the BAE strategy and 17 (44.7%) of 38 patients in the medical strategy (difference -33%; 95% CI -13.8% to -52.1%, p=0.002). The 90-day bleeding recurrence-free survival rates were 91.2% (95% CI 75.1% to 97.1%) and 60.2% (95% CI 42.9% to 73.8%), respectively (HR=0.19, 95% CI 0.05 to 0.67, p=0.01). No death occurred during follow-up and no bleeding recurrence needed surgery.Four adverse events (one major with systemic emboli) occurred during hospitalisation, all in the BAE strategy (11.8% vs 0%; difference 11.8%, 95% CI 0.9 to 22.6, p=0.045); all eventually resolved. CONCLUSION: In non-severe haemoptysis of mild abundance, BAE associated with medical therapy had a superior efficacy for preventing bleeding recurrences at 30 and 90 days, as compared with medical therapy alone. However, it was associated with a higher rate of adverse events. TRIAL REGISTRATION NUMBER: NCT01278199.

Impact of MDCT angiography on the management of patients with hemoptysis.

OBJECTIVE: The aim of this study was to evaluate the ability of MDCT angiography to modify early results in patients undergoing endovascular embolization for hemoptysis. MATERIALS AND METHODS: A comparative study was performed of two consecutive cohorts with 200 patients each who were treated by endovascular embolization for severe hemoptysis; one cohort underwent MDCT angiography and the other did not, but patients underwent further exploration with global aortography. We compared the two groups for patients' age and sex and for the volume, cause, and mechanisms of hemoptysis. Evaluation included the number of sessions needed to control the hemoptysis, the primary failure of bronchial artery embolization, the recurrence of hemoptysis, pulmonary artery vasoocclusion, urgent surgical resection, and death by massive hemoptysis during hospitalization. Failure was defined as the absence of embolization during the first session. Recurrence was defined as rebleeding despite technically successful bronchial artery embolization. RESULTS: Differences between the groups were statistically significant for patient age (p < 0.05), endovascular treatment failure among patients older than 70 years (p < 0.05), pulmonary artery vasoocclusion in comparison with diagnostic pulmonary artery angiography (p < 0.0001), and urgent surgical resection (p = 0.034). CONCLUSION: The impact of MDCT angiography was significant in reducing the rate of vascular catheterization failure in patients older than 70 years, increasing the number of pulmonary artery vasoocclusions, and reducing the number of urgent surgical resection.

Systemic arterial embolization in patients with hemoptysis: initial experience with ethylene vinyl alcohol copolymer in 15 cases.

OBJECTIVE: The purpose of this study was a preliminary evaluation of the use of ethylene vinyl alcohol copolymer to treat patients with hemoptysis of systemic arterial origin. MATERIALS AND METHODS: We reviewed the cases of 15 consecutively registered patients (10 men, five women; mean age, 53.7 years) who underwent endovascular administration of ethylene vinyl alcohol copolymer in the management of hemoptysis. The indications, immediate control of hemoptysis, and clinical tolerability were analyzed. RESULTS: The indications for embolization were mainly early recurrence of hemoptysis with reperfusion of systemic arteries in seven cases; unstable microcatheter in two cases; large ectopic bronchial artery in two cases; and, in one case each, bronchial arterial bleeding through a small anastomotic network, aneurysm of the left internal thoracic artery in a patient with invasive aspergillosis, a potentially risky connection between the bronchial and right coronary arteries, and occlusion of a systemic artery due to Pryce type 1 intralobar lung sequestration. Hemoptysis was controlled in all but one case and did not recur in the other 14 cases. The injection procedure was well tolerated. CONCLUSION: Ethylene vinyl alcohol copolymer embolization for hemoptysis of systemic arterial origin is feasible. The agent seems beneficial in the care of patients with recurrent hemoptysis after previous bronchial arterial embolization, patients who would be at high risk during embolization with microparticles, and patients who need embolization of large systemic arteries through small-caliber anastomoses.

Sequence variations of ACVRL1 play a critical role in hepatic vascular malformations in hereditary hemorrhagic telangiectasia.

BACKGROUND: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder characterized by multiple telangiectases and caused by germline disease-causing variants in the ENG (HHT1), ACVRL1 (HHT2) and, to a lesser extent MADH4 and GDF2, which encode proteins involved in the TGF-ß/BMP9 signaling pathway. Common visceral complications of HHT are caused by pulmonary, cerebral, or hepatic arteriovenous malformations (HAVMs). There is large intrafamilial variability in the severity of visceral involvement, suggesting a role for modifier genes. The objective of the present study was to investigate the potential role of ENG, ACVRL1, and of other candidate genes belonging to the same biological pathway in the development of HAVMs. METHODS: We selected 354 patients from the French HHT patient database who had one disease causing variant in either ENG or ACVRL1 and who underwent hepatic exploration. We first compared the distribution of the different types of variants with the occurrence of HAVMs. Then, we genotyped 51 Tag-SNPs from the Hap Map database located in 8 genes that encode proteins belonging to the TGF-ß/BMP9 pathway (ACVRL1, ENG, GDF2, MADH4, SMAD1, SMAD5, TGFB1, TGFBR1), as well as in two additional candidate genes (PTPN14 and ADAM17). We addressed the question of a possible genetic association with the occurrence of HAVMs. RESULTS: The proportion of patients with germline ACVRL1 variants and the proportion of women were significantly higher in HHT patients with HAVMs. In the HHT2 group, HAVMs were more frequent in patients with truncating variants. Six SNPs (3 in ACVRL1, 1 in ENG, 1 in SMAD5, and 1 in ADAM17) were significantly associated with HAVMs. After correction for multiple testing, only one remained significantly associated (rs2277383). CONCLUSIONS: In this large association study, we confirmed the strong relationship between ACVRL1 and the development of HAVMs. Common polymorphisms of ACVRL1 may also play a role in the development of HAVMs, as a modifying factor, independently of the disease-causing variants.

Severe hemoptysis of pulmonary arterial origin: signs and role of multidetector row CT angiography.

BACKGROUND: Hemoptysis of pulmonary arterial origin is a diagnostic challenge in patients admitted to a respiratory ICU (RICU) for treatment of hemoptysis. Its early accurate recognition and treatment reduce morbidity and prevent mortality. Multidetector row CT angiography (MDCTA) is an accurate method for imaging the systemic vascular network. Our aim was to assess the MDCTA signs and role in managing hemoptysis of pulmonary arterial origin. METHODS: We performed a retrospective clinical and radiologic analysis of all consecutive patients who were referred for severe hemoptysis to our RICU and were treated by endovascular means between January 2004 and December 2006. We reviewed all of those cases with hemoptysis of pulmonary arterial origin. RESULTS: Of 272 patients who were referred for severe hemoptysis to the RICU, 189 patients were treated by endovascular means. Thirteen patients (nine men, four women; mean age, 45 years) had hemoptysis of pulmonary arterial origin. Signs of pulmonary arterial hemoptysis seen on MDCTA were of the following three types: pseudoaneurysm (n = 5); aneurysm of the pulmonary artery (n = 3); or the presence of a pulmonary artery in the inner wall of a cavity (n = 5). Hypertrophy of the bronchial arteries seen on MDCTA associated with any of these signs predicted the necessity to treat both the bronchial and pulmonary arteries. Pulmonary artery vasoocclusion was performed as a first treatment in eight patients with such an association (n = 1) or without such an association (n = 7) along with bronchial artery embolization. The remaining five patients were treated with systemic artery embolization, followed by surgery (n = 1), pulmonary artery vasoocclusion (n = 3), and death from massive hemoptysis (n = 1). CONCLUSIONS: MDCTA performed prior to endovascular treatment allows the correct identification and early appropriate management of severe hemoptysis of pulmonary arterial origin.