European Journal of Cardio-thoracic Surgery 15 (1999) 370–372
Case report
Combination of extracorporeal membrane oxygenation (ECMO) and
pulmonary lavage in a patient with pulmonary alveolar proteinosis
Evangelos Sivitanidis a, Rifaat Tosson a, Albrecht Wiebalck a , b ,*, Axel Laczkovics a
a
Department of Cardiac and Thoracic Surgery, Ruhr-University-Hospital Bergmannsheil, Bürkle-de-La-Camp-Platz 1, D-44789 Bochum, Germany
b
Department of Anaesthesiology, Ruhr-University-Hospital Bergmannsheil, Bochum, Germany
Received 19 October 1998; received in revised form 17 December 1998; accepted 22 December 1998
Abstract
We describe a rare case of pulmonary alveolar proteinosis in a young woman with dyspnea and progressive hypoxaemia due to the
alveolar deposition of insoluble, surfactant-like material. Routine treatment includes whole-lung-lavage (WLL) using double-lumen-tubes
for selective lavaging of each lung. We performed three whole-lung-lavages and used veno–venous extracorporeal membrane oxygenation
(v–vECMO) to support oxygenation during these procedures.  1999 Elsevier Science B.V. All rights reserved.
Keywords: Pulmonary alveolar proteinosis; Whole-lung-lavage; Extracorporeal membrane oxygenation
1. Introduction
Pulmonary alveolar proteinosis (PAP) is a rare disease,
causing dyspnea and progressive hypoxaemia due to the
deposition of insoluble, surfactant-like material in the
alveoli of the lungs. Whole-lung-lavage (WLL) with isotonic saline seems to be the only consistently successful
treatment best using double-lumen-tubes for selective lavaging of each lung [1]. However, it may induce a temporary
hypoxaemia with fatal consequences. To support oxygenation during lavaging, we used veno–venous ECMO (v–
vECMO) in a young woman for 6 days and report this
case since, to our knowledge, only few authors used the
same approach [2].
2. Patient, diagnosis and treatment
A 34-year-old woman (BMH-N29674), was transferred
to our hospital to explain a progressively increasing dyspnea
during the last 2 months. She smoked 20 cigarettes a day for
* Corresponding author. Tel.: +49-234-302-6000; fax: +49-234-3026010.
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12 years. During the last 6 months, she underwent several
upper airway infections. There were no environmental risk
factors nor known allergies. At admission, she presented in
a reduced general condition (weight 48 kg, height 158 cm)
with oedemas of ankles and knees which had developed
during the previous week. On chest auscultation, there
were vesicular breathing sounds without rales. Blood-pressure was 110/80 mmHg, heart rate 100 bpm. Chest X-ray
showed a normal heart size, but the lungs stroke with patchy
confluencing infiltrates and increased interstitial markings.
The immuno-serological examination was negative for
both, allergic alveolitis and lung fibrosis. The cytology of
bronchial lavage showed masses of acellular oval corpuscles of strong PAS-positive material and cell detritus. The
lung biopsy showed a preserved alveolar structure filled
with homogeneous eosinophilous material, partially granular and including few lamellar bodies. The histological
examination of the bronchial lavage confirmed the diagnosis
of PAP.
Under treatment with corticoids and antibiotics, neither
clinical nor radiological findings improved. After the first
bronchial lavage, the patient developed both, a respiratory
insufficiency documented by increasing pulmonary infiltrates of the chest X-ray, and a low-cardiac-output-syndrome. Oxygenation deteriorated (PaO2 of 54 mmHg
 1999 Elsevier Science B.V. All rights reserved.
E. Sivitanidis et al. / European Journal of Cardio-thoracic Surgery 000 (1999) 370–372
under oxygen 12 l/min) and the patient was intubated. Two
hours later, the PaO2 was 118 mmHg with a FiO2 of 1.0.
To assure adequate oxygen supply during lavage, we
implanted a v–vECMO. The oxygenation and cardiovascular situation improved rapidly. WLL was performed starting
the day after cardiopulmonary stabilization (Figs. 1 and 2).
During the first WLL, we used saline as recommended
[1–3]. We encountered severe cardiac arrhythmias that
could not be explained by the moderate drop of oxygen
tension. However, we found a considerable shift of potassium, a decrease of its plasma concentration from 5.8 to 4.0
mmol/l. The following WLL, we used Ringer’s solution,
and still induced arrhythmias, but did not observe a change
in the potassium level yet the core temperature of the patient
dropped to 35.6°C. During the third WLL, and during
further bronchial lavages, we prewarmed Ringer’s solution
up to 37°C using a blood/infusion warmer (warmflowy)
and faced no arrhythmias.
After three WLLs, the patient was easily weaned
from ECMO, but developed septic temperature. Candida,
Pseudomonas and oxacillin-resistent Staphylococcus aureus were found in blood- and tracheal-fluid cultures. A
tracheotomy was performed 5 days after ECMO-explantation. The situation of the patient improved and initial signs
of right cardiac insufficiency disappeared. Two weeks
later, the patient was transferred to the ward. Transthoracic
echocardiography was performed before discharge and
revealed diffuse myocardial impairment with left ventricular dilatation. The myocardial biopsy showed unspecific
myocardial injury concordant with a cardiomyopathy
probably due to the infection. The patient was discharged
home 7 weeks after the first bronchial lavage without any
signs of adult respiratory distress syndrome (ARDS), nor
had she disturbances of lung compliance.
The patient was readmitted twice with intervals of 6 and 8
weeks. She presented again with dyspnea, but uncomplicated WLL relieved her from shortness of breath. After
short hospital stays, she was discharged home again.
371
3. Discussion
Hypoxaemia and infection are the most important factors
influencing the outcome in pulmonary alveolar proteinosis
[3–5]. WLL seems to be the only consistently successful
treatment removing deposited material, as well as pathogenic organisms, from bronchial and alveolar spaces [5].
The problem is that this treatment may induce temporary
hypoxaemia with fatal consequences. The risk of hypoxaemia is greatest during the draining of the lavage fluid from
the lungs, allowing shunting of pulmonary blood flow
through the non-ventilated lung. Using only WLL, a mortality rate of about 30% is reported [1,3,6].
The patient was in a disastrous situation after the first
diagnostic and therapeutic bronchial lavage. Oxygenation
had deteriorated rapidly, she fainted and presented a lowcardiac-output syndrome. Conventional ventilation did not
result in substantially better oxygenation, hence, extracorporeal membrane oxygenation was indicated.
Two modes of ECMO are known: the arterio-venous and
the veno-venous mode. The arterio-venous mode has been
implemented several times in patients with PAP, mostly
only for WLL with rapid weaning after the procedure
[2,3,6–10]. We chose for the veno-venous mode for two
reasons: first, the oxygen saturation of the venous blood is
much higher, this may be important because the impaired
organ – the lungs – are better supplied with oxygen, second,
cannulation is easier and causes less risk of vascular injury
and limb ischaemia.
WLL was recommended to be carried out with saline [1–
3]. Our patient responded to saline with severe arrhythmias,
which did not occur when prewarmed Ringer’s solution was
used. It may be that the patient reacted more to saline since
she had undergone a prolonged hypoxaemic period with
impairment of the heart. On the other hand, the reduction
of arrhythmias during the three WLL may also be due to the
general recuperation. However, the authors are convinced
that optimization of the lavage fluid contributes to an
Fig. 1. Blood gas-exchange data.
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E. Sivitanidis et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 370–372
Fig. 2. Chest X-rays at admission and before discharge from the hospital.
improvement of outcome, at least in critically ill patients:
lavage fluid should be Ringer’s solution warmed up to 37°C.
4. Conclusion
Our case-report shows that v–vECMO is a safe and easily
applied method for adequate oxygenation during WLL in
case of respiratory failure due to PAP. WLL can be
improved by using 37°C warm Ringer’s solution in stead
of saline.
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