Hemoptysis In Cystic Fibrosis Research Articles

Pulmonary hemorrhage in cystic fibrosis: a review of current data

Pulmonary hemorrhage is a serious complication of cystic fibrosis (CF) with a prevalence up to 9.1%. According to the Registry of Patients with Cystic Fibrosis of the Russian Federation for 2020, the prevalence of pulmonary hemorrhage was 1.5% in the general population, and 6.5% in patients over 18 years of age. Despite the importance of this problem, there is no systematic information on pulmonary hemorrhage in CF.Aim. The purpose of this study was to review current data on the pathogenesis, risk factors, and approaches to the treatment of pulmonary hemorrhage in patients with CF. Information was searched in еLibrary and PubMed databases.Results. During the literature review, we summarized and systematized the collected scientific data on pulmonary hemorrhage in CF. The pathogenesis of pulmonary hemorrhage is chronic inflammation in the bronchial wall accompanied by increased angiogenesis factors that promote neovascularization with development of many tortuous, thin-walled vessels. Exacerbation of bronchial inflammation and mechanical strain contribute to erosion and damage of fragile vessels and cause the bleeding. The main risk factors for pulmonary hemorrhage were colonization with Pseudomonas aeruginosa, Staphylococcus aureus, diabetes mellitus, and FEV1 < 70%. Complex therapy for pulmonary hemorrhage includes the use of antibiotics and antifibrinolytic agents. Some of the published regimens use β-blockers and blood coagulation factor VII. Data on the efficacy of vitamin K are unclear, but it is also used in the treatment of pulmonary hemorrhage. In cases of massive bleeding, temporarily discontinuation of inhalations of hypertonic solution and kinesiotherapy is recommended. In case of recurrent massive bleedings, bronchial artery embolization is performed and lung resection can be done in extreme cases.Conclusion. Hemoptysis in CF is a complex clinical problem that requires a multidisciplinary approach. Currently, important aspects of the pathogenesis of pulmonary hemorrhage remain unclear. Given the increasing life expectancy of CF patients and the age-related risk of pulmonary hemorrhage, it is important to investigate the pathogenetic aspects of this complication for the subsequent development of effective and justified treatment algorithms.

Bronchial artery diameter in massive hemoptysis in cystic fibrosis

BackgroundMassive hemoptysis is a rare but potentially life-threatening condition of patients with cystic fibrosis (CF) and advanced pulmonary disease. Hypertrophied bronchial arteries are understood to cause massive hemoptysis when rupturing. Risk factors to predict massive hemoptysis are scarce and bronchial artery diameters are not part of any scoring system in follow-up of patients with CF. Aim of this study was to correlate bronchial artery diameter with massive hemoptysis in CF.MethodsBronchial artery and non-bronchial systemic artery diameters were measured in contrast enhanced computed tomography (CT) scans in patients with massive hemoptysis and compared to patients with end-stage CF and no history of hemoptysis. Demographic and clinical data and side of bronchial artery/non-bronchial systemic artery hypertrophy and coil embolization were documented.ResultsIn this retrospective multicenter study 33 patients with massive hemoptysis were included for bronchial artery/non-bronchial systemic artery diameter measurements, (13 female, 20 male, median age 30 years (18–55)). Bronchial artery diameters were significantly larger in the case group than in the control group with median 4 mm (2.2–8.2 mm), and median 3 mm (1–7 mm), respectively (p = 0.002). Sensitivity of bronchial arteries ≥ 3.5 mm to be associated with hemoptysis was 0.76 and specificity 0.71 with ROC creating an area under the curve of 0.719. If non-bronchial systemic arteries were present, they were considered culprit and embolized in 92% of cases.ConclusionBronchial arteries ≥ 3.5 mm and presence of hypertrophied non-bronchial systemic arteries correlate with massive hemoptysis in patients with CF and might serve as risk predictor for massive hemoptysis. Therefore, in patients with advanced CF we propose CT scans to be carried out as CT angiography to search for bronchial arteries ≥ 3.5 mm and for hypertrophied non-bronchial systemic arteries as possible risk factors for massive hemoptysis.

Angiographic and histopathological study on bronchial-to-pulmonary vascular anastomoses on explants from patients with cystic fibrosis after bronchial artery embolisation

Labelled backgroundHaemoptysis is a life-threatening complication of cystic fibrosis (CF). One treatment is bronchial artery embolisation (BAE) using embolic-microspheres (EMs). During BAE, pulmonary arteries can be seen on digital subtracted angiography while iodine containing contrast material injection is performed in the bronchial artery. This suggests that EMs could go from bronchial to nontarget pulmonary arteries. The aim was to evaluate if EMs could be found inside pulmonary arteries on lung explants after BAE in transplanted CF patients. MethodsRetrospective observational study including patients with CF who underwent lung transplantation and had previously needed BAE. Clinical, chest CT angiography, and angiographic data were reviewed from medical records. Pathology examination of lung explants was performed to analyze the EMs anatomical localisation. ResultsEight patients were included between 2013 and 2015, four males with a mean age of 29 (19-45) years. All patients had bronchial artery hypertrophy on CT and bronchial-to-pulmonary artery shunting during BAE. On pathology examination, EM ≤800 µm were found in the pulmonary arteries in all patients and were responsible for distal branch occlusions. Two pulmonary infarcts were observed on CT angiography after BAE and confirmed histopathologically. ConclusionsEM migration from the bronchial to pulmonary arteries is a common occurrence after BAE in patients with advanced stage CF. Although BAE is a highly effective means of controlling haemoptysis in CF, studies on the optimal particle size are needed to preserve pulmonary artery circulation, because these results suggest that low size EMs could lead to nontarget embolisation.

Coil embolisation for massive haemoptysis in cystic fibrosis

IntroductionMassive haemoptysis is a life-threatening event in advanced cystic fibrosis (CF) lung disease with bronchial artery embolisation (BAE) as standard of care treatment. The aim of our study was to...

Abstract No. 664 Elective bronchial artery embolization for hemoptysis in cystic fibrosis: is there a benefit over emergent embolization?

Hemoptysis is a well known complication of late stage cystic fibrosis, and can be acutely life threatening if not appropriately treated. Bronchial artery embolization (BAE) has become a first line treatment for patients with life threatening hemoptysis or those refractory to conservative management. However its use for non-critical management of hemoptysis remains unclear. This study compares outcomes between emergent procedures performed for life threatening hemoptysis and elective procedures performed for non life threatening hemoptysis in cystic fibrosis patients. A retrospective chart review of 34 bronchial artery embolization in 18 patients was performed. Procedures were subdivided into two groups – those requiring emergent procedures for life threatening hemoptysis and those who received elective procedures for nonlife threatening hemoptysis. Emergent was defined as massive hemoptysis (>250cc/24h) or hemodynamic instability. Pre-procedure variables, procedure variables, and outcomes were analyzed. There was no significant correlation between disease severity and amount of hemoptysis or outcomes. All but one patient had positive sputum cultures. BAE was performed emergently in 8 cases and electively in 26. Seven patients required re-embolization on 16 different instances. The average time between re-embolization was 249 days in the elective group and 93 days in the emergent group, although results did not show statistical significance (p=0.6). A lower rate of ICU admission was seen in the elective group compared to the emergent group (p=0.001). The average length of stay after procedure was 9.8 days in the elective group compared to 23.4 days for the emergent group, although results were not significant (p=0.15). There was a trend towards significance with decreased number of patients requiring intubation in the elective group (p=0.09). Bronchial artery embolization is a viable and effective technique for treatment of non–life-threatening hemoptysis in cystic fibrosis patients. Benefits of early intervention before hemoptysis becomes life threatening may offer advantages, and further research is needed.

Outcomes Following Bronchial Artery Embolisation for Haemoptysis in Cystic Fibrosis.

Bronchial artery embolisation (BAE) is recommended for the treatment of massive haemoptysis in cystic fibrosis (CF), but there are no randomised controlled trials of this therapy and its role in sub-massive haemoptysis is unclear. This study aimed to determine the outcomes and safety of BAE in adults with CF. All patients with CF undergoing BAE at our centre between March 2011 and January 2015 were identified at the time of the procedure. Patient records were reviewed at hospital discharge, death or one month post-procedure (whichever was soonest). Follow-up continued to January 2016. Severity of haemoptysis was classified as: massive (>240ml/24h or >100ml/day for ≥2days), moderate-severe (>20ml/24h) or mild (<20ml/24h). Twenty-seven patients underwent 51 BAE procedures over a median follow-up period of 26months (range 1-54). Ten patients (37%) required more than one BAE during the study. BAE was performed for massive haemoptysis in 18 cases (35%). Haemoptysis recurred after 31 (61%) of BAE procedures with no difference in recurrence rates between massive and sub-massive haemoptysis. Side effects were reported after 61% of procedures with chest pain the most common adverse event . Mortality after first BAE in the study was 3.9% at 30days and 14.8% at 12months. No significant predictors of mortality were identified. BAE is often effective in controlling haemoptysis but is associated with considerable morbidity and high recurrence rates.

Treatment and prognosis of massive hemoptysis in cystic fibrosis.

Massive hemoptysis (volume of expectorated blood ≥300 ml in 24 hours) occurred in 38 (5 per cent) of 728 patients with cystic fibrosis. Twenty-two of the patients were in the midst of a relatively long-lasting flare-up of pulmonary infection. There was no other consistent precipitating event. All but 7 of the patients were admitted after the initial episode and were treated with parenteral antimicrobial drugs. Postural drainage was resumed as rapidly as possible after cessation of acute bleeding. Vitamin K was administered to all patients. Volumes of expectorated blood were as large as 2,500 ml with the initial episode. Blood pressure changes occurred in 4 patients. Five patients received blood transfusions. Bleeding stopped without surgical intervention in all patients, usually within 4 days. The 38 patients had a total of 85 episodes of bleeding. No surgical treatment was undertaken at this center. One patient underwent a lobectomy at another hospital but continued to bleed postoperatively. Ten patients...

The CFTR and EGFR relationship in airway vascular growth, and its importance in cystic fibrosis

In this issue of the European Respiratory Journal , Martin et al. [1] focus on the life-threatening complication of haemoptysis in cystic fibrosis (CF). The authors examine the role of vascular endothelial growth factor (VEGF)-A on angiogenesis in the normal state and in CF. They report that CF airways at transplantation show increased vascularity and increased VEGF-A expression. In normal human airway epithelial cells they report that VEGF-A synthesis is produced via activation of the epidermal growth factor receptor (EGFR) and is suppressed by a selective EGFR inhibitor. Most novel and exciting are the findings that in airway epithelial cells containing cystic fibrosis transmembrane conductance regulator (CFTR), inhibition of CFTR increases EGFR activation and that an inhibitor of EGFR activation prevents the VEGF-A synthesis induced by suppression of CFTR, suggesting that there is a reciprocal relationship between CFTR and EGFR. Both CFTR and EGFR coexist on the airway epithelial surface. EGFR activation results in “pro-inflammatory responses”; in addition to VEGF-A, this includes other products such as interleukin-8 (a potent neutrophil chemoattractant) [2] and mucins [3]. The fact that inhibition of CFTR results in exaggerated production of VEGF-A suggests that CFTR normally suppresses the EGFR “inflammatory” responses. VEGF-A production occurs in response to EGFR activation. The study by Martin et al. [1] found that suppression of CFTR increased EGFR activation, but only after …

Beta-blocker management of refractory hemoptysis in cystic fibrosis: a novel treatment approach

/objective: Recurrent hemoptysis is a debilitating complication of cystic fibrosis (CF) and likely results from mucosal erosions into abnormal bronchial blood vessels due to chronic respiratory infection. We hypothesize that the use of beta-blockade will decrease mean arterial pressure resulting in lower bronchial artery blood flow and, subsequently, decrease the frequency and severity of hemoptysis, rate of hospitalizations, and usage of intravenous antibiotics. Retrospective chart review was performed on 12 CF patients with recurrent hemoptysis, aged 13-40 years old, along with a follow-up telephone survey to assess the effectiveness of beta-blockade for hemoptysis, tolerance of inhaled respiratory medications, activity tolerance, and potential adverse effects. A beta-blocker, specifically atenolol, was initiated in all subjects within 24 hours after experiencing recurrent hemoptysis episodes. A majority of patients (72.7%) had complete cessation of hemoptysis. There were significant decreases in the frequency of hemoptysis (p = 0.02) and the amount of hemoptysis (p = 0.004). The rate of hospitalizations significantly decreased from 1.33 to 0.67 (p = 0.05) after initiation of atenolol. There was a trend toward statistical significance in the reduction of intravenous antibiotics use (p = 0.08). No statistical difference was found when comparing the pre- and post-treatment means of forced expiratory volume in 1-second (p = 0.59). Very minimal adverse effects were observed with only one patient reporting intermittent facial flushing. Beta-blockade, particularly with atenolol, appears to successfully treat, if not resolve, recurrent hemoptysis refractory to conservative therapy in CF. Beta-blocker therapy appears to maintain an effective safety profile in CF.

Treatment massive haemoptysis in cystic fibrosis with tranexamic acid

We present the case of a young man with cystic fibrosis (CF) who presented with a massive haemoptysis. All management options were considered and treatment with tranexamic acid was commenced. We discuss the treatment options for patients with CF presenting with massive haemoptysis and the underlying evidence base.

Restless leg syndrome manifested by iron deficiency from chronic hemoptysis in cystic fibrosis

Restless leg syndrome (RLS) and periodic limb movement disorder (PLMD) are considered to be a continuum of a neurological sleep disorder associated with abnormal iron metabolism or deficiency. I describe a case of RLS and PLMD in a cystic fibrosis patient with iron deficiency from chronic hemoptysis. This is the first case that reports RLS and PLMD manifesting from iron deficiency caused by chronic hemoptysis in advanced cystic fibrosis lung disease.

Massive Hemoptysis in Cystic Fibrosis

Massive Hemoptysis in Cystic Fibrosis

Massive Hemoptysis in Cystic Fibrosis

Massive hemoptysis is a complication commonly reported in patients with cystic fibrosis (CF). An understanding of the pathophysiology of this complication and its consequences is important for the management of patients with CF. To identify risk factors associated with massive hemoptysis, and to determine the prognosis of patients following an episode of massive hemoptysis. A retrospective, observational cohort study of the National CF Patient Registry between the years 1990 to 1999. The Registry contained data on 28,858 patients with CF observed over 10 years at CF centers across the United States. Massive hemoptysis occurred with an average annual incidence of 0.87% and in 4.1% of patients overall. There was no increased occurrence by sex, but it was more prevalent in older patients (mean age, 24.2 +/- 8.7 years [+/- SD]) with more severe pulmonary impairment (nearly 60% of patients who had an episode of massive hemoptysis had FEV1 < 40% predicted). The principal risks associated with an increased occurrence of massive hemoptysis included the presence of Staphylococcus aureus in sputum cultures (odds ratio [OR], 1.3) and diabetes (OR, 1.1). There was an increased morbidity (eg, increased hospitalizations and hospital days) and an increased 2-year mortality following massive hemoptysis. Massive hemoptysis is a serious complication in CF patients, occurring more commonly in older patients with more advanced lung disease. Nearly 1 in 100 patients will have this complication each year. There is an attributable mortality to the complication and considerable morbidity, resulting in increased health-care utilization and a measurable decline in lung function.

Bronchial to subclavian shunt in a CF patient. A potential pitfall for embolization

Bronchial artery embolization is a well accepted and widely used technique in the management of massive haemoptysis in cystic fibrosis (CF). It can be a complex procedure requiring a deep knowledge of the bronchial artery anatomy including the possible bronchial anastomoses. We report a case of complex vascular anatomy of the left bronchial artery with multiple anastomoses with the ipsilateral subclavian artery as cause of non-attempted embolization.

Bronchial Artery Embolization for the Management of Nonmassive Hemoptysis in Cystic Fibrosis

Hemoptysis is a common complication in patients with cystic fibrosis (CF). Current approaches to patients with hemoptysis include conservative medical therapy, bronchial artery embolization (BAE), and surgery. We investigated the effectiveness of early BAE on the outcome in patients with minor bleeding. We reviewed the clinical records from the Cystic Fibrosis Service for eight consecutive patients treated with medical therapy who had undergone early BAE and eight matched patients treated with conservative medical therapy alone. We assessed the mean number of bleeding episodes, pulmonary exacerbations, lung function (FEV(1)), Shwachman score, and Nottingham Health Profile (NHP) scores, the year before BAE and for the 3 ensuing years. During a 3-year follow-up, patients who underwent embolization had significantly fewer bleeding episodes (p < 0.001) and pulmonary exacerbations (p < 0.006). Lung function declined significantly in both groups (p < 0.001). The modified Shwachman score declined significantly during the follow-up only in patients who did not undergo embolization (p < 0.001). Patients treated by early embolization had significantly better NHP scores, indicating a better quality of life (p < 0.05). None of the patients who underwent BAE had adverse reactions. Early BAE in patients with CF who have nonmassive hemoptysis is an effective, safe therapeutic approach offering better long-term control of recurrent bleeding and quality of life than medical therapy alone.

Extrapleural bronchial artery ligation for life-threatening hemoptysis in cystic fibrosis--a case report.

Hemoptyses are common in cystic fibrosis (CF) patients. They range from massive life-threatening (> 240 mL/24 hours) to recurrent minor streaking. Limited pulmonary reserve, potential concurrent chest infection, and the progressive nature of CF pose a high risk to this subgroup. Conservative management and selective bronchial artery embolization (BAE) control most acute episodes, but the recurrence rate is high. The possible need for lung transplantation in future makes an extrapleural approach for bronchial artery ligation desirable. The aim of this study was to assess the role of extrapleural bronchial artery ligation in the treatment of recurrent hemoptysis in CF patients. This is a retrospective analysis of four patients between 1986 and 1999 treated by extrapleural thoracotomy and ligation of bronchial arteries. Indications, surgical experience, and outcome are presented. Three patients underwent unilateral, and one patient bilateral extrapleural thoracotomy (in two separate sessions) for bronchial artery ligation. There were three men and one woman, with a mean age of 26.6 years (range 19-32 years). Indications were failure to stabilize the bronchial arterial catheter for BAE (three cases), recurrence after BAE previously controlled bleeding (one case), and communication with the right costocervical trunk signifying risk to the spinal circulation (one case). The mean follow-up was 68 months (range 3-144 months). There was one death in this series, a patient who was asphyxiated with hemoptysis, requiring ventilation preoperatively. He underwent successful extrapleural thoracotomy for bronchial artery ligation, with no further bleeding but succumbed to severe chest infection and multiorgan failure a few days later. Two patients had recurrent bleeding 12 and 36 months after surgery. Selective bronchial angiography proved the contralateral bronchial arteries to be the culprit. Extrapleural bronchial artery ligation is an effective method of controlling hemoptysis in CF, when BAE has failed. This approach minimizes pleural adhesions and is, therefore, desirable in the future consideration for lung transplantation. In this experience, muscle-sparing thoracotomy and postoperative epidural analgesia significantly improved the postoperative recovery.

Bronchial artery embolization in the management of hemoptysis in cystic fibrosis

Massive hemoptysis and/or recurrent expectoration of measurable amounts of blood are common complications of chronic bronchopulmonary infections in cystic fibrosis (CF). When conservative treatment fails to control bleeding, surgery or bronchial artery embolization (BAE) is frequently considered. We present our experience and long-term follow up of BAE in 14 CF patients (age range 15-39 years) with massive (6 subjects) and/or recurrent (8 subjects) hemoptysis not responsive to medical treatment. Seven had chronic hypercapnic respiratory failure. After angiographic evaluation, polyvinyl alcohol particles (Ivalon) were injected to embolize obviously enlarged bronchial arteries. Seventeen procedures were performed in 14 patients and 36 bronchial arteries were embolized. All the patients stopped bleeding immediately upon BAE. Most of the patients had postembolization fever, dysphagia, and transient chest pain which were managed symptomatically. After a median follow-up period of 10.5 months (range 0.5-38 months), no recurrence of hemoptysis was observed in 8 patients who are still alive. In 3 patients hemoptysis recurred and they underwent reembolization after 3, 22, and 25 months, respectively. Three subjects died of respiratory failure within 5 months from BAE. Presently, 50% of patients studied had a > or = 1 year interval free of major hemoptysis after the first BAE. Our experience indicates that massive and/or recurrent hemoptysis in CF patients can be safety and effectively managed by BAE if the procedure is performed by a skilled practitioner. The procedure was well tolerated and resulted in prolonged and satisfactory bleeding control in most patients.

Life threatening haemoptysis in cystic fibrosis: an alternative therapeutic approach.

Life threatening haemoptysis in cystic fibrosis: an alternative therapeutic approach.

Life threatening haemoptysis in cystic fibrosis: an alternative therapeutic approach.

Desmopressin and vasopressin were used to control massive haemoptysis in a patient with cystic fibrosis. After bolus doses a continuous infusion of vasopressin was maintained for 36 hours and haemoptysis stopped.

Bronchial Artery Embolization for Severe Hemoptysis in Cystic Fibrosis

We studied the long-term outcome after BAE for life-threatening hemoptysis in patients with CF. Data from pulmonary function tests were available for 18 of the 25 patients followed. A case-control comparison revealed that these 18 patients died sooner than hemoptysis-free patients with CF matched for age, sex, and pulmonary function (p less than 0.02), with the excess mortality occurring within the first three months after BAE. Of all 25 patients followed, six died of cardiorespiratory failure within three months of BAE; in two of them, hemoptysis was a contributing cause of death. The 19 patients who lived more than three months after BAE had a mean survival after embolization of 3.5 years (five were still alive at the end of the study). Most patients experienced long intervals (greater than 1 year) free of major hemoptysis. Extended follow-up (mean, 35 months) revealed a higher incidence of recurrent severe bleeding than previously reported for 13 of these patients followed a mean of 11 months. Repeat BAE for severe recurrence was performed successfully in eight of nine patients, without complication.