The use of a membrane oxygenator with extracorporeal circulation in bronchoalveolar lavage for alveolar proteinosis
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《血管的通路杂志》
Department of Cardiovascular Surgery Service, Ramon y Cajal Hospital, Madrid, Spain
Abstract
Alveolar proteinosis is a rare pulmonary disease, characterised by the accumulation of a proteinaceous material in the alveoli, which severely reduces gas exchange and causes progressive disability of the patient, usually moderate, but sometimes completely disabling. Whole-lung lavage (WLL) is the preferred technique for the treatment of this disease, and it is performed by introducing into the lungs and draining a fluid (saline), which removes the phospholipidic material present in the alveoli, offering the patient a marked improvement in a short period of time. Sometimes the whole-lung lavage technique is not well tolerated by the patient, requiring the support of a membrane oxygenator during the course of the procedure. Few successful cases have been published. We report the case of a 33-year-old man with severe hypoxaemia who suffered cardiopulmonary arrest after the first attempt of bronchoalveolar lavage (BAL) and required support by means of extracorporeal venovenous circulation with a membrane oxygenator (ECMO). An initial incomplete lavage was performed with ECMO support, but the patient was re-admitted 7 months later and we performed a second WLL of both lungs in the same session, again using ECMO. Also, the BAL technique and its association with ECMO when necessary are reviewed.
Key Words: Pulmonary alveolar proteinosis; Extracorporeal membrane oxygenation; Bronchoalveolar lavage
1. Case report
Pulmonary alveolar proteinosis (PAP) is a rare disease first described in 1958 by Rosen and colleagues [1], characterised by an accumulation of phospholipidic and proteinaceous material in the alveoli, causing respiratory failure of variable severity due to the alteration of gas exchange and therefore the alteration of the ventilation/perfusion ratio [2].
We report the case of a 33-year-old man with a history of drug addiction and previous upper left lobectomy because of a tumour of inflammatory origin. In January 2003 he suffered an episode of severe respiratory failure. Chest CT considered alveolar proteinosis as the first possibility (Fig. 1). In February 2003 the first BAL was attempted in his reference hospital, but it could not be completed because the patient suffered a cardiopulmonary arrest secondary to hypoxia. As the patient's evolution was progressively worsening, he was referred to our hospital in June 2003 to attempt a new BAL.
In our hospital, left lung lavage was attempted first, because of its lower volume due to the previous lobectomy. During this initial lavage the patient maintained O2 saturation of 70% and gasometry (with FiO2 of 1) of: pH 7.13; pO2 55 mmHg and pCO2 87.6 mmHg. As it was impossible to continue the right lung lavage, the procedure was stopped.
Seven days later, we attempted a new BAL with ECMO support. The patient was again moved to the intensive care unit (ICU) where, under anaesthesia, a left double-lumen endotracheal tube was placed. Radial artery, ECG and SaO2 were monitored. Percutaneous catheters were introduced to initiate venovenous ECMO, 21 Fr and 17.8 cm cannula in the right internal jugular vein for oxygenated blood return (Medtronic) and 29 Fr, 76.2 cm cannula in the right femoral vein for blood drainage to the oxygenator (Medtronic). A biocompatible OPTIMA OPEN XP (Cobe) oxygenator was used. After administration of a sodium heparin bolus at 1.5 mg/kg body mass, a 10 mg/h perfusion was initiated, aiming to maintain ACT between 180 and 220 s.
During right lung lavage the left lung was independently ventilated. ECMO was maintained at a rate of 1.5 to 2 l/min, and systolic blood pressure remained between 100 and 120 mmHg throughout. Flow was regulated by monitoring O2 arterial saturation, which was never below 89% during the procedure. ECMO duration was 86 min. Because the patient had a left lung lavage previously, it was decided to finish the procedure without lavage in the prone position, so flows were reduced and ECMO was finalized, removing the cannulae by direct compression for 10 min. The patient was discharged 7 days later after a marked improvement of his functional class.
Seven months later, the patient suffered another progressive respiratory impairment (Table 1), and was referred to our centre in order to consider a new BAL, this time a whole-lung one. Our cooperation was again required in order to carry out a WLL with ECMO assistance in both lungs and in the supine and prone positions. The monitoring technique was the same as the first time, as well as the use of the ECMO system. In this case, venovenous assistance was rendered for 3 h and 35 min. Flow was maintained between 1650 and 2700 ml/min for an arterial saturation between 90 and 95%. The patient remained stable throughout the procedure, which allowed whole-lung BAL to be carried out in both lungs, first in the supine position, then in the prone position. The patient was discharged from the hospital after 5 days, with a marked improvement of his functional class. At present (15 months after the last lavage) the patient leads a normal life with good tolerance of exercise (Table 1).
2. Discussion
The treatment of alveolar proteinosis is palliative. WLL is the safest and most effective treatment of acquired alveolar proteinosis, with an average clinical benefit time of some 15 months [3,4]. This technique is performed in order to mechanically sweep and remove the phospholipidic matter from the alveolar spaces. The procedure is repeated in both lungs in the supine position for lavage of the posterior lung areas and then, after turning the patient over, the lavage is repeated in the anterior lung areas in the prone position. This technique is not without any risks. The following complications having been described: pneumothorax, hydrothorax, haemodynamic instability, infection, and arrhythmias [5,6].
On the other hand, there are cases in which BAL may not be completed, for example in very hypoxaemic patients. In these cases, BAL has been performed with the support of ECMO. This procedure may be performed by venovenous or venoarterial bypass. Only few cases have been described in the literature in which WLL has been performed in a single session when using ECMO assistance [7,8]. In the other published cases, BAL was performed in several sessions [9,10]. That was the reason why we did not complete the lavage in the prone position with assistance. Nevertheless, due to the relapse progressively suffered by the patient during the initial six months, we decided to perform a second WLL in a single session with venovenous ECMO assistance.
Whereas ECMO assistance is not without any risks, these are lessened when venovenous cannulation is used. Also, the progressive development of biocompatible equipment (cannulae and oxygenators) has reduced haemorrhagic complications, as the doses of heparin used are much lower. In our case, we did not have infectious complications or thrombosis of the venous systems used. The use of ECMO in our patient allowed us to perform the WLL without any complications such as arrhythmias or haemodynamic alterations secondary to the hypoxaemia that the patient might have suffered during the procedure.
In summary, we believe that the use of ECMO may be beneficial as a support to BAL in the cases of seriously affected patients. We believe this assistance may be indicated in cases of PAP with severe hypoxaemia before BAL, not improving with mechanical ventilation, or in patients with previous partial or complete pulmonary resections. We favour WLL in a single session, as the risks attached to such assistance are thus diminished.
References
Rosen SH, Castleman B, Lebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123–1142.
Ben-Dov I, Kishinevski Y, Roznman J, Soliman A, Bishara H, Zelligson E, Grief J, Mazar A, Perelman M, Vishnizer R, Weiler-Ravel D. Pulmonary alveolar proteinosis in Israel: ethnic clustering. Isr Med Assoc J 1999;1:75–78.
Trapnell BC, Whitsett JA, Nakata, K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527–2539.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002;166:215–235.
Hudes ET, Bradley JW, Brebner J. Hydropneumothorax – an unusual complication of lung lavage. Can Anaesth Soc J 1986;33:662–665.
p.Alfery D, Benumof J, Spragg R. Anesthesia for Broncopulmonary Lavage. In: Kaplan J. editor Thoracic Anaesthesia 1983;New York: Churchill Livingstone 403–419. In:.
Cohen ES, Elpern E, Silver MR. Pulmonary alveolar proteinosis causing severe hypoxemic respiratory failure treated with sequential whole-lung lavage utilizing venovenous extracorporeal membrane oxygenation. A case report and review. Chest 2001;120:1024–1026.
Kyung-Hwan K, Jin Hyun K, Young Whan K. Use of extracorporeal membrane oxygenation (ECMO) during whole lung lavage in pulmonary alveolar proteinosis associated with lung cancer. Eur J Cardiothorac Surg 2004;26:1050–1051.
Cai H, Cui SY, Jin L, Huang YZ, Cao B, Wang ZY, Mu GH, Zhou XM. Pulmonary proteinosis treated with whole-lung lavage utilizing extracorporeal membrane oxygenation: a case report and review of literatures. Chin Med J 2004;117:1746–1749.
Sivitanidas E, Tosson R, Wiebalck A, Laczkovics A. Combination of extracorporeal membrana oxygenation (ECMO) and pulmonary lavage in a patient with pulmonary alveolar proteinosis. Eur J Cardiothorac Surg 1999;15:370–372.(Tomasa Centella, Enrique )
Abstract
Alveolar proteinosis is a rare pulmonary disease, characterised by the accumulation of a proteinaceous material in the alveoli, which severely reduces gas exchange and causes progressive disability of the patient, usually moderate, but sometimes completely disabling. Whole-lung lavage (WLL) is the preferred technique for the treatment of this disease, and it is performed by introducing into the lungs and draining a fluid (saline), which removes the phospholipidic material present in the alveoli, offering the patient a marked improvement in a short period of time. Sometimes the whole-lung lavage technique is not well tolerated by the patient, requiring the support of a membrane oxygenator during the course of the procedure. Few successful cases have been published. We report the case of a 33-year-old man with severe hypoxaemia who suffered cardiopulmonary arrest after the first attempt of bronchoalveolar lavage (BAL) and required support by means of extracorporeal venovenous circulation with a membrane oxygenator (ECMO). An initial incomplete lavage was performed with ECMO support, but the patient was re-admitted 7 months later and we performed a second WLL of both lungs in the same session, again using ECMO. Also, the BAL technique and its association with ECMO when necessary are reviewed.
Key Words: Pulmonary alveolar proteinosis; Extracorporeal membrane oxygenation; Bronchoalveolar lavage
1. Case report
Pulmonary alveolar proteinosis (PAP) is a rare disease first described in 1958 by Rosen and colleagues [1], characterised by an accumulation of phospholipidic and proteinaceous material in the alveoli, causing respiratory failure of variable severity due to the alteration of gas exchange and therefore the alteration of the ventilation/perfusion ratio [2].
We report the case of a 33-year-old man with a history of drug addiction and previous upper left lobectomy because of a tumour of inflammatory origin. In January 2003 he suffered an episode of severe respiratory failure. Chest CT considered alveolar proteinosis as the first possibility (Fig. 1). In February 2003 the first BAL was attempted in his reference hospital, but it could not be completed because the patient suffered a cardiopulmonary arrest secondary to hypoxia. As the patient's evolution was progressively worsening, he was referred to our hospital in June 2003 to attempt a new BAL.
In our hospital, left lung lavage was attempted first, because of its lower volume due to the previous lobectomy. During this initial lavage the patient maintained O2 saturation of 70% and gasometry (with FiO2 of 1) of: pH 7.13; pO2 55 mmHg and pCO2 87.6 mmHg. As it was impossible to continue the right lung lavage, the procedure was stopped.
Seven days later, we attempted a new BAL with ECMO support. The patient was again moved to the intensive care unit (ICU) where, under anaesthesia, a left double-lumen endotracheal tube was placed. Radial artery, ECG and SaO2 were monitored. Percutaneous catheters were introduced to initiate venovenous ECMO, 21 Fr and 17.8 cm cannula in the right internal jugular vein for oxygenated blood return (Medtronic) and 29 Fr, 76.2 cm cannula in the right femoral vein for blood drainage to the oxygenator (Medtronic). A biocompatible OPTIMA OPEN XP (Cobe) oxygenator was used. After administration of a sodium heparin bolus at 1.5 mg/kg body mass, a 10 mg/h perfusion was initiated, aiming to maintain ACT between 180 and 220 s.
During right lung lavage the left lung was independently ventilated. ECMO was maintained at a rate of 1.5 to 2 l/min, and systolic blood pressure remained between 100 and 120 mmHg throughout. Flow was regulated by monitoring O2 arterial saturation, which was never below 89% during the procedure. ECMO duration was 86 min. Because the patient had a left lung lavage previously, it was decided to finish the procedure without lavage in the prone position, so flows were reduced and ECMO was finalized, removing the cannulae by direct compression for 10 min. The patient was discharged 7 days later after a marked improvement of his functional class.
Seven months later, the patient suffered another progressive respiratory impairment (Table 1), and was referred to our centre in order to consider a new BAL, this time a whole-lung one. Our cooperation was again required in order to carry out a WLL with ECMO assistance in both lungs and in the supine and prone positions. The monitoring technique was the same as the first time, as well as the use of the ECMO system. In this case, venovenous assistance was rendered for 3 h and 35 min. Flow was maintained between 1650 and 2700 ml/min for an arterial saturation between 90 and 95%. The patient remained stable throughout the procedure, which allowed whole-lung BAL to be carried out in both lungs, first in the supine position, then in the prone position. The patient was discharged from the hospital after 5 days, with a marked improvement of his functional class. At present (15 months after the last lavage) the patient leads a normal life with good tolerance of exercise (Table 1).
2. Discussion
The treatment of alveolar proteinosis is palliative. WLL is the safest and most effective treatment of acquired alveolar proteinosis, with an average clinical benefit time of some 15 months [3,4]. This technique is performed in order to mechanically sweep and remove the phospholipidic matter from the alveolar spaces. The procedure is repeated in both lungs in the supine position for lavage of the posterior lung areas and then, after turning the patient over, the lavage is repeated in the anterior lung areas in the prone position. This technique is not without any risks. The following complications having been described: pneumothorax, hydrothorax, haemodynamic instability, infection, and arrhythmias [5,6].
On the other hand, there are cases in which BAL may not be completed, for example in very hypoxaemic patients. In these cases, BAL has been performed with the support of ECMO. This procedure may be performed by venovenous or venoarterial bypass. Only few cases have been described in the literature in which WLL has been performed in a single session when using ECMO assistance [7,8]. In the other published cases, BAL was performed in several sessions [9,10]. That was the reason why we did not complete the lavage in the prone position with assistance. Nevertheless, due to the relapse progressively suffered by the patient during the initial six months, we decided to perform a second WLL in a single session with venovenous ECMO assistance.
Whereas ECMO assistance is not without any risks, these are lessened when venovenous cannulation is used. Also, the progressive development of biocompatible equipment (cannulae and oxygenators) has reduced haemorrhagic complications, as the doses of heparin used are much lower. In our case, we did not have infectious complications or thrombosis of the venous systems used. The use of ECMO in our patient allowed us to perform the WLL without any complications such as arrhythmias or haemodynamic alterations secondary to the hypoxaemia that the patient might have suffered during the procedure.
In summary, we believe that the use of ECMO may be beneficial as a support to BAL in the cases of seriously affected patients. We believe this assistance may be indicated in cases of PAP with severe hypoxaemia before BAL, not improving with mechanical ventilation, or in patients with previous partial or complete pulmonary resections. We favour WLL in a single session, as the risks attached to such assistance are thus diminished.
References
Rosen SH, Castleman B, Lebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123–1142.
Ben-Dov I, Kishinevski Y, Roznman J, Soliman A, Bishara H, Zelligson E, Grief J, Mazar A, Perelman M, Vishnizer R, Weiler-Ravel D. Pulmonary alveolar proteinosis in Israel: ethnic clustering. Isr Med Assoc J 1999;1:75–78.
Trapnell BC, Whitsett JA, Nakata, K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527–2539.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002;166:215–235.
Hudes ET, Bradley JW, Brebner J. Hydropneumothorax – an unusual complication of lung lavage. Can Anaesth Soc J 1986;33:662–665.
p.Alfery D, Benumof J, Spragg R. Anesthesia for Broncopulmonary Lavage. In: Kaplan J. editor Thoracic Anaesthesia 1983;New York: Churchill Livingstone 403–419. In:.
Cohen ES, Elpern E, Silver MR. Pulmonary alveolar proteinosis causing severe hypoxemic respiratory failure treated with sequential whole-lung lavage utilizing venovenous extracorporeal membrane oxygenation. A case report and review. Chest 2001;120:1024–1026.
Kyung-Hwan K, Jin Hyun K, Young Whan K. Use of extracorporeal membrane oxygenation (ECMO) during whole lung lavage in pulmonary alveolar proteinosis associated with lung cancer. Eur J Cardiothorac Surg 2004;26:1050–1051.
Cai H, Cui SY, Jin L, Huang YZ, Cao B, Wang ZY, Mu GH, Zhou XM. Pulmonary proteinosis treated with whole-lung lavage utilizing extracorporeal membrane oxygenation: a case report and review of literatures. Chin Med J 2004;117:1746–1749.
Sivitanidas E, Tosson R, Wiebalck A, Laczkovics A. Combination of extracorporeal membrana oxygenation (ECMO) and pulmonary lavage in a patient with pulmonary alveolar proteinosis. Eur J Cardiothorac Surg 1999;15:370–372.(Tomasa Centella, Enrique )