CASE CONFERENCES The Proceduralist Section Editor: George Eapen, M.D.
Endobronchial Valve Placement for a Giant Bulla in a Patient with Hypercapnic Respiratory Failure Jennifer Bierach1, James D. Maloney1,2, and J. Scott Ferguson1 Departments of 1Medicine and 2Surgery, School of Medicine and Public Health, University of Wisconsin–Madison, Madison, Wisconsin
Keywords: bronchoscopy; lung volume reduction; humanitarian device exemption
A 64-year-old woman with a giant bulla causing respiratory failure was referred for treatment to facilitate weaning from mechanical ventilation. The left upper lobe bulla was reduced by bronchoscopic placement of one-way endobronchial valves within the segmental airways leading to the left upper lobe. After the uneventful procedure, the patient was liberated from mechanical ventilation and discharged home. A 64-year-old woman, with a long history of respiratory functional impairment attributed to bullous lung disease, was admitted to the hospital with respiratory distress for the sixth time in 5 months. She had a history of a surgical bullectomy
at age 23 years, complicated by a ruptured cerebral aneurysm. In 1991, she was evaluated for bullectomy or lung transplantation, but was declined, because of poor functional status and the presence of pulmonary hypertension. Since that time, she was treated with supplemental oxygen, inhaled bronchodilators, and a diuretic agent. In recent years, she experienced progressive dyspnea on exertion, and serial chest imaging studies have shown a gradual increase in the size of a left upper lobe bulla. Before this admission, spirometry and lung plethysmography revealed evidence of very severe expiratory airflow obstruction (FEV1 = 0.65 L, 33% of predicted; residual
volume = 3.70 L, 216% of predicted; total lung capacity = 4.91 L, 111% of predicted). The diffusing capacity for carbon monoxide was severely reduced (20.36 mm/min/mm Hg, 18% of predicted). On a 6-minute-walk test, she traveled 278 ft (85 m) while breathing pulsed supplemental oxygen via nasal cannula at a rate of 3–4 L/min. In the emergency department, the woman was found to be in severe respiratory failure (initial pH, 7.21; PCO2 . 130 mm Hg). After intubation and initiation of mechanical ventilation, she was admitted to the intensive care unit. Subsequent attempts at weaning from mechanical ventilation were not productive. A computed
Figure 1. Preprocedural chest computed tomography (CT). Shown are representative slices from a chest CT obtained at the time of admission.
(Received in original form June 9, 2013; accepted in final form June 21, 2013 ) Correspondence and requests for reprints should be addressed to J. Scott Ferguson, M.D., Suite 5233, 1685 Highland Avenue, Madison, WI 53705. E-mail: [email protected] Ann Am Thorac Soc Vol 10, No 5, pp 521–524, Oct 2013 Copyright © 2013 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201306-147OT Internet address: www.atsjournals.org
Case Conferences: The Proceduralist
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CASE CONFERENCES
Figure 2. Shown is an endobronchial valve placed in a left upper lobe airway.
tomography (CT) scan of the chest demonstrated a bulla in the left upper lobe (Figure 1). This dominant bulla appeared to cause an intrathoracic mass effect, and was thought to be a major cause of her recurrent respiratory failure. Because of
her compromised overall condition, a consulting thoracic surgeon advised that she might not survive lung volume reduction surgery (LVRS) or bullectomy. Similarly, she was considered not to be a candidate for lung transplantation.
Given that the patient had no other apparent options for liberation from mechanical ventilation, we considered placement of one-way endobronchial valves to reduce the size of her giant bulla. Because endobronchial valves are approved in the United States only for treatment of persistent postoperative air leaks under a Food and Drug Administration Humanitarian Device Exemption, we sought and obtained internal review board approval (UW IRB protocol 2012-0143) for emergency off-label use of endobronchial valves (IBV Valve System; Spiration, Inc., Redmond, WA). The patient was taken to the operating room for implantation of endobronchial valves on Hospital Day 6. The left upper lobe airways were targeted for treatment. Each segment of the left upper lobe was sized using a balloon occlusion technique. A valve insertion catheter was then used to place valves introduced via a flexible bronchoscope in left bronchus (LB) 1, LB2, LB3, LB4, and LB5 (Figure 2), taking care to occlude each segmental airway. Serial chest
Figure 3. Representative axial, sagittal, and coronal CT slices before and after treatment.
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AnnalsATS Volume 10 Number 5 | October 2013
CASE CONFERENCES radiographs demonstrated a progressive decrease in the size of the left upper lobe bulla. At 4 days after the procedure, she was successfully weaned from mechanical ventilation. A CT scan of the chest confirmed a reduction in the size of the bulla and return of the mediastinal structures to a midline position (Figure 3). Postcapture three-dimensional
processing of the CT images (VIDA Diagnostics, Iowa City, IA) demonstrated a marked decrease in the volume of the left upper lobe and improved aeration in radiographically apparent atelectasis throughout the remaining lungs (Figure 4 and Table 1). The patient was discharged to home from the hospital 5 days after extubation.
Discussion Our patient had severe hypercapnic respiratory failure associated with a giant left upper lobe bulla in the setting of lifelong bullous lung disease. Placement of one-way endobronchial valves in the left upper lobe segmental bronchi allowed the bulla to partially collapse, thereby improving her respiratory mechanics, anatomy, and
Figure 4. Lobar lung volume before and after treatment. Shown are representative slices from CT scans before and after endobronchial valve placement. Volumetric analysis was performed with color overlay to identify the lobes. Red, right upper lobe; orange, right lower lobe; green, left upper lobe; blue, left lower lobe. The volumes resulting from the analysis are given in Table 1.
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CASE CONFERENCES Table 1. Lung volumes measured by three-dimensional volumetric computed tomographic scanning before and after valve placement Right Lung 3
Volume (cm ) Total Upper lobe Middle lobe* Lower lobe
Left Lung
Before
After
Before
1,617.40 735.69 285.34 596.37
2,001.87 1081.85 39.95 879.85
2,884.16 2643.03 NA 241.13
After 1,415.37 817.92 NA 597.66
Definition of abbreviation: NA = not applicable. *Postprocedure atelectasis from mucous plugging is present.
physiology. The bronchoscopic procedure enabled successful liberation from mechanical ventilation. Pulmonary function testing, functional exercise testing, and volumetric chest CT imaging demonstrated pre- and post-procedure anatomic and functional improvement. Pulmonary bullae are airspaces that measure greater than 1 cm in greatest diameter. Giant bullae are defined as filling greater than 30% of a hemithorax. Giant bullae compromise gas exchange and respiratory mechanics as the less affected lung is compressed. Emphysematous bullae tend to arise from paraseptal emphysema. Bullous lung disease is a separate clinical entity that arises in otherwise normal-appearing lung tissue through poorly understood mechanisms. LVRS and surgical bullectomy have been used to treat giant bullae resulting from
both emphysema and bullous lung disease. However, many patients who have symptoms attributable to bullae are not surgical candidates, and the morbidity and mortality of LVRS remains high, even in carefully selected patients. Endobronchial valves may be an alternative treatment for severe bullous disease. In patients with severe emphysema, bronchoscopic placement of endobronchial valves appears to have lower morbidity and mortality than LVRS, but clear evidence for clinical utility in this population has been elusive. Current clinical trials are underway to examine the efficacy of endobronchial valves in the treatment of emphysema. However, no trial published to date has tested the utility of endobronchial valves in the treatment of bullous lung disease. Giant bulla can cause severe, lifethreatening respiratory failure. There are no
Recommended Reading Greenberg JA, Singhal S, Kaiser LR. Giant bullous lung disease: evaluation, selection, techniques, and outcomes. Chest Surg Clin N Am 2003;13: 631–649. Heard BE. A pathological study of emphysema of the lungs with chronic bronchitis. Thorax 1958;13:136–149. Koebe HG, Kugler C, Dienemann H. Evidence-based medicine: lung volume reduction surgery (LVRS). Thorac Cardiovasc Surg 2002;50: 315–322. Sciurba FC, Ernst A, Herth FJF, Strange C, Criner GJ, Marquette CH, Kovitz KL, Chiacchierini RP, Goldin J, McLennan G; VENT Study Research Group. A randomized study of endobronchial
524
clear recommendations for treatment when patients are not surgical candidates. Optimal treatment depends on knowledge of the patient’s anatomy and physiology, and the local expertise available. If investigational devices are considered for treatment of a patient outside of a clinical trial, physicians must carefully consider the implications of treatment in the absence of specific data, and discuss these issues with the local internal review board.
Follow Up At the time of an outpatient follow-up visit 2 months after hospital discharge, the patient reported an improvement in exercise tolerance and the quality of her life. Compared with the results obtained on the last study before endobronchial valve placement, her 6-minute walk test distance improved to 352 ft (107 m) while she again breathed pulsed supplemental oxygen at a rate of 3–4 L/min. Compared with her last preprocedure study, the residual volume decreased from 3.70 L (216%) to 2.69 L (156%), and the total lung capacity decreased from 4.91 L (111%) to 4.04 L (91%). There was no significant change in spirometry results or her diffusing capacity. n Author disclosures are available with the text of this article at www.atsjournals.org.
valves for advanced emphysema. N Engl J Med 2010;363: 1233–1244. Santini M, Fiorelli A, Vicidomini G, Di Crescenzo VG, Messina G, Laperuta P. Endobronchial treatment of giant emphysematous bullae with oneway valves: a new approach for surgically unfit patients. Eur J Cardiothorac Surg 2011;40:1425–1431. Coxson HO, Nasute Fauerbach PV, Storness-Bliss C, Muller ¨ NL, Cogswell S, Dillard DH, Finger CL, Springmeyer SC. Computed tomography assessment of lung volume changes after bronchial valve treatment. Eur Respir J 2008;32:1443–1450. Berger RL, Wood KA, Cabral HJ, Goodnight-White S, Ingenito EP, Gray A, Miller J, Springmeyer SC. Lung volume reduction surgery: a metaanalysis of randomized clinical trials. Treat Respir Med 2005;4:201–209.
AnnalsATS Volume 10 Number 5 | October 2013
Endobronchial Valve Placement for a Giant Bulla in a Patient with Hypercapnic Respiratory Failure Jennifer Bierach1, James D. Maloney1,2, and J. Scott Ferguson1 Departments of 1Medicine and 2Surgery, School of Medicine and Public Health, University of Wisconsin–Madison, Madison, Wisconsin
Keywords: bronchoscopy; lung volume reduction; humanitarian device exemption
A 64-year-old woman with a giant bulla causing respiratory failure was referred for treatment to facilitate weaning from mechanical ventilation. The left upper lobe bulla was reduced by bronchoscopic placement of one-way endobronchial valves within the segmental airways leading to the left upper lobe. After the uneventful procedure, the patient was liberated from mechanical ventilation and discharged home. A 64-year-old woman, with a long history of respiratory functional impairment attributed to bullous lung disease, was admitted to the hospital with respiratory distress for the sixth time in 5 months. She had a history of a surgical bullectomy
at age 23 years, complicated by a ruptured cerebral aneurysm. In 1991, she was evaluated for bullectomy or lung transplantation, but was declined, because of poor functional status and the presence of pulmonary hypertension. Since that time, she was treated with supplemental oxygen, inhaled bronchodilators, and a diuretic agent. In recent years, she experienced progressive dyspnea on exertion, and serial chest imaging studies have shown a gradual increase in the size of a left upper lobe bulla. Before this admission, spirometry and lung plethysmography revealed evidence of very severe expiratory airflow obstruction (FEV1 = 0.65 L, 33% of predicted; residual
volume = 3.70 L, 216% of predicted; total lung capacity = 4.91 L, 111% of predicted). The diffusing capacity for carbon monoxide was severely reduced (20.36 mm/min/mm Hg, 18% of predicted). On a 6-minute-walk test, she traveled 278 ft (85 m) while breathing pulsed supplemental oxygen via nasal cannula at a rate of 3–4 L/min. In the emergency department, the woman was found to be in severe respiratory failure (initial pH, 7.21; PCO2 . 130 mm Hg). After intubation and initiation of mechanical ventilation, she was admitted to the intensive care unit. Subsequent attempts at weaning from mechanical ventilation were not productive. A computed
Figure 1. Preprocedural chest computed tomography (CT). Shown are representative slices from a chest CT obtained at the time of admission.
(Received in original form June 9, 2013; accepted in final form June 21, 2013 ) Correspondence and requests for reprints should be addressed to J. Scott Ferguson, M.D., Suite 5233, 1685 Highland Avenue, Madison, WI 53705. E-mail: [email protected] Ann Am Thorac Soc Vol 10, No 5, pp 521–524, Oct 2013 Copyright © 2013 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201306-147OT Internet address: www.atsjournals.org
Case Conferences: The Proceduralist
521
CASE CONFERENCES
Figure 2. Shown is an endobronchial valve placed in a left upper lobe airway.
tomography (CT) scan of the chest demonstrated a bulla in the left upper lobe (Figure 1). This dominant bulla appeared to cause an intrathoracic mass effect, and was thought to be a major cause of her recurrent respiratory failure. Because of
her compromised overall condition, a consulting thoracic surgeon advised that she might not survive lung volume reduction surgery (LVRS) or bullectomy. Similarly, she was considered not to be a candidate for lung transplantation.
Given that the patient had no other apparent options for liberation from mechanical ventilation, we considered placement of one-way endobronchial valves to reduce the size of her giant bulla. Because endobronchial valves are approved in the United States only for treatment of persistent postoperative air leaks under a Food and Drug Administration Humanitarian Device Exemption, we sought and obtained internal review board approval (UW IRB protocol 2012-0143) for emergency off-label use of endobronchial valves (IBV Valve System; Spiration, Inc., Redmond, WA). The patient was taken to the operating room for implantation of endobronchial valves on Hospital Day 6. The left upper lobe airways were targeted for treatment. Each segment of the left upper lobe was sized using a balloon occlusion technique. A valve insertion catheter was then used to place valves introduced via a flexible bronchoscope in left bronchus (LB) 1, LB2, LB3, LB4, and LB5 (Figure 2), taking care to occlude each segmental airway. Serial chest
Figure 3. Representative axial, sagittal, and coronal CT slices before and after treatment.
522
AnnalsATS Volume 10 Number 5 | October 2013
CASE CONFERENCES radiographs demonstrated a progressive decrease in the size of the left upper lobe bulla. At 4 days after the procedure, she was successfully weaned from mechanical ventilation. A CT scan of the chest confirmed a reduction in the size of the bulla and return of the mediastinal structures to a midline position (Figure 3). Postcapture three-dimensional
processing of the CT images (VIDA Diagnostics, Iowa City, IA) demonstrated a marked decrease in the volume of the left upper lobe and improved aeration in radiographically apparent atelectasis throughout the remaining lungs (Figure 4 and Table 1). The patient was discharged to home from the hospital 5 days after extubation.
Discussion Our patient had severe hypercapnic respiratory failure associated with a giant left upper lobe bulla in the setting of lifelong bullous lung disease. Placement of one-way endobronchial valves in the left upper lobe segmental bronchi allowed the bulla to partially collapse, thereby improving her respiratory mechanics, anatomy, and
Figure 4. Lobar lung volume before and after treatment. Shown are representative slices from CT scans before and after endobronchial valve placement. Volumetric analysis was performed with color overlay to identify the lobes. Red, right upper lobe; orange, right lower lobe; green, left upper lobe; blue, left lower lobe. The volumes resulting from the analysis are given in Table 1.
Case Conferences: The Proceduralist
523
CASE CONFERENCES Table 1. Lung volumes measured by three-dimensional volumetric computed tomographic scanning before and after valve placement Right Lung 3
Volume (cm ) Total Upper lobe Middle lobe* Lower lobe
Left Lung
Before
After
Before
1,617.40 735.69 285.34 596.37
2,001.87 1081.85 39.95 879.85
2,884.16 2643.03 NA 241.13
After 1,415.37 817.92 NA 597.66
Definition of abbreviation: NA = not applicable. *Postprocedure atelectasis from mucous plugging is present.
physiology. The bronchoscopic procedure enabled successful liberation from mechanical ventilation. Pulmonary function testing, functional exercise testing, and volumetric chest CT imaging demonstrated pre- and post-procedure anatomic and functional improvement. Pulmonary bullae are airspaces that measure greater than 1 cm in greatest diameter. Giant bullae are defined as filling greater than 30% of a hemithorax. Giant bullae compromise gas exchange and respiratory mechanics as the less affected lung is compressed. Emphysematous bullae tend to arise from paraseptal emphysema. Bullous lung disease is a separate clinical entity that arises in otherwise normal-appearing lung tissue through poorly understood mechanisms. LVRS and surgical bullectomy have been used to treat giant bullae resulting from
both emphysema and bullous lung disease. However, many patients who have symptoms attributable to bullae are not surgical candidates, and the morbidity and mortality of LVRS remains high, even in carefully selected patients. Endobronchial valves may be an alternative treatment for severe bullous disease. In patients with severe emphysema, bronchoscopic placement of endobronchial valves appears to have lower morbidity and mortality than LVRS, but clear evidence for clinical utility in this population has been elusive. Current clinical trials are underway to examine the efficacy of endobronchial valves in the treatment of emphysema. However, no trial published to date has tested the utility of endobronchial valves in the treatment of bullous lung disease. Giant bulla can cause severe, lifethreatening respiratory failure. There are no
Recommended Reading Greenberg JA, Singhal S, Kaiser LR. Giant bullous lung disease: evaluation, selection, techniques, and outcomes. Chest Surg Clin N Am 2003;13: 631–649. Heard BE. A pathological study of emphysema of the lungs with chronic bronchitis. Thorax 1958;13:136–149. Koebe HG, Kugler C, Dienemann H. Evidence-based medicine: lung volume reduction surgery (LVRS). Thorac Cardiovasc Surg 2002;50: 315–322. Sciurba FC, Ernst A, Herth FJF, Strange C, Criner GJ, Marquette CH, Kovitz KL, Chiacchierini RP, Goldin J, McLennan G; VENT Study Research Group. A randomized study of endobronchial
524
clear recommendations for treatment when patients are not surgical candidates. Optimal treatment depends on knowledge of the patient’s anatomy and physiology, and the local expertise available. If investigational devices are considered for treatment of a patient outside of a clinical trial, physicians must carefully consider the implications of treatment in the absence of specific data, and discuss these issues with the local internal review board.
Follow Up At the time of an outpatient follow-up visit 2 months after hospital discharge, the patient reported an improvement in exercise tolerance and the quality of her life. Compared with the results obtained on the last study before endobronchial valve placement, her 6-minute walk test distance improved to 352 ft (107 m) while she again breathed pulsed supplemental oxygen at a rate of 3–4 L/min. Compared with her last preprocedure study, the residual volume decreased from 3.70 L (216%) to 2.69 L (156%), and the total lung capacity decreased from 4.91 L (111%) to 4.04 L (91%). There was no significant change in spirometry results or her diffusing capacity. n Author disclosures are available with the text of this article at www.atsjournals.org.
valves for advanced emphysema. N Engl J Med 2010;363: 1233–1244. Santini M, Fiorelli A, Vicidomini G, Di Crescenzo VG, Messina G, Laperuta P. Endobronchial treatment of giant emphysematous bullae with oneway valves: a new approach for surgically unfit patients. Eur J Cardiothorac Surg 2011;40:1425–1431. Coxson HO, Nasute Fauerbach PV, Storness-Bliss C, Muller ¨ NL, Cogswell S, Dillard DH, Finger CL, Springmeyer SC. Computed tomography assessment of lung volume changes after bronchial valve treatment. Eur Respir J 2008;32:1443–1450. Berger RL, Wood KA, Cabral HJ, Goodnight-White S, Ingenito EP, Gray A, Miller J, Springmeyer SC. Lung volume reduction surgery: a metaanalysis of randomized clinical trials. Treat Respir Med 2005;4:201–209.
AnnalsATS Volume 10 Number 5 | October 2013
