Supplemental Material
Patient Case Reports
Patient 1
11 year-old male with cyanosis, agitation and severe respiratory distress,
which worsened in supine position. Chest radiograph revealed a large mediastinal
mass with partial tracheal obstruction at the thoracic inlet, and initial blood gas had
a pH of 6.99 and pCO2 of 100mmHg on 2 liters oxygen. Heliox 80/20 improved his
symptoms, but a chest CT was deferred due to his inability to lie supine.
His level of consciousness sharply deteriorated and he was placed on VA
ECMO using ketamine, midazolam and local anesthesia with lidocaine, breathing
spontaneously and assisted with BVM. A 21Fr Biomedicus cannula was placed in the
right femoral vein and a 17Fr Biomedicus cannula was placed in the left femoral
artery. Additional venous cannulas were placed in the distal parts of both vessels to
facilitate drainage and distal limb perfusion, respectively.
Ketamine and midazolam infusions were titrated and he maintained
spontaneous ventilation with HHFNC. He was awake, comfortable and talkative.
Treatment of his T-cell acute lymphoblastic leukemia with vincristine, doxorubicin,
and prednisone commenced on ECMO. In the ensuing days, his respiratory distress
improved and mass effect was assessed by serial CXRs. By HD#6, tracheal
narrowing improved, and the patient was on RA, so chest CT was performed. Airway
patency was confirmed and the patient was decannulated in the operating room
under uncomplicated general anesthesia. He was discharged home 4 days after
decannulation.
Patient 2
Five year-old male with severe respiratory distress, wheezing, agitation and
inability to lie supine. CXR showed a mediastinal mass occupying >90% of the
thorax. Despite initiation of heliox 80/20, his respiratory status worsened and he
began to fatigue.
Using ketamine and midazolam for sedation and lidocaine for local
anesthesia, the patient was emergently cannulated for VA ECMO using a 12Fr
Biomedicus cannula in the right femoral artery and two 15Fr Biomedicus cannulas
in the right and left femoral veins.
In the PICU, he was supported on CPAP and sedated with ketamine and
midazolam infusions. Chemotherapy with vincristine, doxorubicin, and prednisone
for presumed T-cell NHL was begun. Airway compression was assessed by serial
CXR and degree of respiratory distress. On HD#4 tracheal narrowing had improved
and he was decannulated in the operating room under uncomplicated general
anesthesia. Biopsy of the mediastinal mass at the same time confirmed the diagnosis
of T cell NHL. He was weaned to RA and discharged home 4 days after
decannulation.
Patient 3
4 year-old female with respiratory distress, hypoxia and fever originally
admitted to floor on HFNC and antibiotics. By HD#8 her respiratory status
worsened and a CXR revealed a large pneumomediastinum. Following transfer to
the ICU, her respiratory status continued to decline, necessitating, intubation, HFOV
and iNO.
Worsening air leak and oxygenation prompted initiation of VV ECMO on
HD#19. Two Biomedicus venous cannulas (15Fr and 17Fr) were placed in the right
and left femoral veins. Lung biopsy performed at the time of cannulation showed
nonspecific interstitial pneumonitis. Cyclophosphamide, hydroxychloroquine and
methylprednisolone were initiated.
Several bronchoscopies with surfactant and N-acetylcysteine instillation
were performed to help with airway clearance. Her oxygenation improved sharply
after an additional venous drainage cannula was placed, and she was extubated on
HD#27 to let her air leak heal. Serial chest CTs demonstrated marked improvement
of the pneumomediastinum, though lung consolidation persisted. Sedation was
discontinued and po feeds were initiated on HD#37.
She was reintubated on HD#49 for interventional procedures but decision
was made to maintain her intubated to recruit her lungs with positive pressure. On
HD#73 she was transferred to another hospital for evaluation of lung transplant on
VV ECMO and minimal ventilator settings. Ultimately, she was decannulated at the
referral center after 84 days on ECMO, but underwent lung transplant a year later
for worsening lung disease.
Patient 4
17 year-old female acute promyelocytic leukemia in remission (diagnosed 5
months before, on maintenance chemotherapy) with shortness of breath and shock.
She was intubated, resuscitated with fluids and inotropes, and received empiric
antibiotics. Echocardiogram revealed severe contractile dysfunction with a
shortening fraction <10%, likely related to anthracycline chemotherapy.
Her condition deteriorated during the next few hours and she was placed on
VA ECMO on HD#2. A 20Fr Avalon cannula and a 21Fr Biomedicus cannula were
placed in the left femoral vein and left femoral artery, respectively. An 18Fr Avalon
catheter was placed in her LA to decompress her left heart, and all inotropes, except
milrinone, were stopped.
On HD#5, feeds were started and advanced to full volume after 2 days. All
cultures returned negative, and antibiotics were discontinued. On HD# 10, she was
extubated to BiPAP and was transitioned to RA 2 days later. Sedation was tapered
off, and the patient remained awake began eating and drinking normally.
Several trials weaning off ECMO support failed due to a poor contractile
function. She underwent evaluation for VAD placement; as she was not a candidate
for transplant this soon after treatment for malignancy. On HD#22, she was
discharged to an adult hospital for VAD placement, on ECMO support, extubated and
breathing spontaneously.
Patient 5
12 year-old male with symptoms of small bowel obstruction, hypotension
and obtundation. During intubation in the ED, he vomited and aspirated stomach
contents. Bowel obstruction was surgically relieved, but patient transferred to ICU
for worsening respiratory status. Bedside bronchoscopy yielded copious secretions,
food particles, and severe airway inflammation, consistent with aspiration. His
clinical condition continued to decline, requiring HFOV, iNO and high doses of
pressors.
Because of worsening oxygenation in the face of aggressive ventilation, on
HD#2 he was cannulated with a 23Fr Avalon double lumen cannula in the RIJV
placed on VV ECMO. Inotropes and iNO were discontinued and he was converted to
BiVent to attempt to maintain recruitment of aerated segments.
Serial bronchoscopies and aggressive pulmonary toilet cleared his airways
and disclosed improvement in airway inflammation but had no discernible impact
on airspace disease. Initial chest CT demonstrated persistent opacification
consistent with ARDS, and marked airway distention resembling bronchiectasis. A
second venous catheter was placed to improve oxygenation, and he was extubated
on HD#15 to afford complete lung rest. He required 2 brief re-intubations for
interventional procedures and a third chest CT which showed improving aeration
and resolving parenchymal disease. Sedatives were discontinued and he started po
feedings with clear liquids and solid food.
Oxygenation, dyspnea and CXRs all continued to improve, such that on
HD#26, an LMA was placed, decannulation performed, and following reversal of
anesthesia, he was placed on HHFNC. He was discharged home 2 weeks later off
supplemental O2.
Patient 6
3 year-old former 32-week premature male with acute respiratory distress,
cough, congestion and fever for 2 days. Initial CXR showed pneumomediastinum and
DFA positive for RSV. Admitted to PICU on HFNC.
On HD#2 his respiratory status deteriorated, so he was intubated and placed
on conventional ventilation and iNO. With positive pressure ventilation, his airleak
and oxygenation worsened, so he was cannulated with a 16Fr Avalon double lumen
cannula in the RIJV and placed on VV ECMO. He underwent bronchoscopy for
diagnostics and airway clearance.
To allow airleak to seal and to minimize ventilator induced lung injury, he
was extubated to HHFNC on HD#3. Inhaled DNAse and chest physiotherapy were
started. CXR showed improved aeration and his respiratory distress decreased. He
was electively reintubated on HD#6 for bronchoscopy, which showed clear airways,
and promptly extubated to HHFNC. ECMO was discontinued on HD#7. He was
discharged from the PICU 2 days, breathing RA.
Patient 7
35-week preterm male born with meconium aspiration and PAH. Admitted to
NICU on DOL#2 for hypoxemia, but despite HFJV, iNO and inotropes his oxygenation
remained poor. On HD#8, he underwent cannulation with a 12Fr Origen double
lumen catheter in the RIJV and was placed on VV ECMO.
Conventional ventilation on low settings was restarted, and inotropes and
sedatives were decreased. He underwent 3 bronchoscopies on HD#10, 13 and 16
with removal of obstructing mucous plugs. Treatments with intrapulmonary
percussive ventilation (IPV) and DNAase were added. Antibiotics were started after
isolation of coagulase-negative staphylococci in the lines and ECMO circuit.
He was extubated to RA on HD#18 to provide complete lung rest and avoid
ventilator-associated lung injury. He exhibited little initial respiratory effort, and his
CXRs showed complete opacification of both lungs. Though he was switched to nasal
cannula to stimulate breathing, his respiratory effort appeared to depend more on
his PCO2 than on the nasal cannula flow rate. He required brief reintubation on
HD#21 for ECMO circuit change. His respiratory effort increased the following days,
with subsequent increase in lung aeration.
The patient developed signs of fulminant liver failure. CVVHD for bilirubin
clearance was started due to a progressive rise in its blood levels despite
phenobarbital and ursodeoxicholic acid. Increased values of iron (264 mcg/dl) and
ferritin (6861 ng/dl) worrisome for neonatal hemochromatosis prompted the
initiation of plasmapheresis to counter alloimmune mediated liver injury.
On HD#24 he suffered a hypocalcemic cardiac arrest during plasmapheresis
and remained hypotensive despite aggressive resuscitation. He was reintubated for
planned conversion to VA ECMO which failed due to inability to gain access to his
RICA. Given his acute deterioration and overall poor prognosis due to his fulminant
liver failure, the decision was made to withdraw care on HD#26.
Patient 8
Two-day term infant with prenatally diagnosed right-sided CDH containing
colon, small bowel, liver and right kidney was admitted for severe hypoxemia
despite HFOV, iNO and inotropes. He was cannulated for VA ECMO with an 8Fr
Biomedicus arterial cannula in the RCCA and a 10Fr Biomedicus venous cannula in
the RIJV. Conventional ventilation resumed on rest settings and iNo and inotropes
were discontinued
On HD# 3, he was extubated to RA to provide lung rest and decrease
ventilator-induce lung injury. The following night the ECMO circuit clotted and the
patient had a cardiorespiratory arrest, requiring reintubation and CPR for several
minutes until a new circuit was spliced in. His respiratory status, end-organ
laboratory values and CXR remained stable after the episode, so he was extubated to
RA several hours after the incident. He exhibited good respiratory effort, with
minimally increased work of breathing, but the CXR showed persistent
opacification.
On HD#6 he underwent reintubation for bronchoscopy and recruitment
maneuvers. He underwent CDH repair on HD#17 and was successfully decannulated
on HD#20.
On HD#33 he was transferred to the NICU on conventional ventilation. He
remained ventilator –dependent and with severe feeding intolerance. On HD#81 a
MRI of the brain showed extensive encephalomalacia and the patient developed
seizure-like activity. On HD#90, due to persistent feeding intolerance, he
underwent an exploratory laparotomy to identify the cause of his feeding
intolerance. His bowels were completely frozen. Care was withdrawn soon
thereafter.
Patient 9
Three year-old boy with abdominal rhabdomyosarcoma admitted for fever,
neutropenia and respiratory distress, transferred to the ICU on HD#5 and intubated
for persistent hypoxemia and bilateral pulmonary consolidation on chest CT.
Severe hypoxemia persisted despite trials of different ventilation modes, so
he was cannulated for VV ECMO on HD#8 with a 19-Fr Avalon double lumen
catheter and placed on conventional ventilation at lung rest settings. That evening,
he developed hemopericardium and pericardial tamponade, suffered cardiac arrest,
despite drainage of the pericardium, his myocardial performance remained poor
and he was converted toVA ECMO with placement of a 14-Fr Biomedicus cannula in
the RCCA.
His lungs remained consolidated on CXR and a second chest CT showed a
hemothorax with leftward mediastinal shift. Chest tube placement, recruitment
maneuvers (IPV, DNAase, lung conditioning) and bronchoscopies failed to reinflate
his lungs. He received 10 days of IV azithromycin for Bordetella pertussis isolated
from a BAL. Sedatives were tapered, CVVH was initiated for renal insufficiency and
fluid overload, and by HD #21 had achieved full-volume enteral feeding.
On HD#28 he was extubated and CXR showed persistent diffuse
consolidation and gasless chest. Subsequent CXRs showed improving aeration, so he
was reintubated and given positive pressure ventilation for alveolar recruitment on
HD#38. With lung rest, mucous plug removal, positive pressure recruitment, and
time both lungs showed improved aeration.
A week later, however, his respiratory status deteriorated and a third chest
CT demonstrated contrast extravasation from a bronchial artery. On HD#46
embolization was attempted, but ultimately the patient underwent thoracotomy
with removal of a necrotic right lower lobe on HD#56 while still on ECMO. Trials of
left side intubation and surfactant instillation failed to recruit the left lung. On HD#
78 air entrained in the ECMO circuit, although cannulas had not moved and the
patient arrested and died despite resuscitation while trying to evacuate air from the
circuit.
Patient 10
Six year-old previously healthy male transferred from another hospital to the
PICU with worsening respiratory distress, pneumonia, and pleural effusion. Despite
antibiotics and VATS drainage of right-sided effusion, hypoxemia worsened, and
attempts to stabilize with HFOV, iNO and inotropes failed.
On PICU day #2, the patient was cannulated for VV ECMO with an Avalon 23Fr double lumen cannula in the RIJV. Chest CT on ECLS showed bilateral pulmonary
consolidation. Conventional ventilation was restarted, iNO and inotropes were
discontinued, enteral feeding was initiated, and broad-spectrum antibiotics were
continued. Serial bronchoscopies revealed gradual improvement in secretions and
airway appearance and Mycoplasma pneumoniae was isolated from multiple BALs.
To provide complete lung rest, decrease sedative requirements and avoid
ventilator-associated lung injury, he was extubated to room air on HD# 7. Five days
later he was switched to HHFNC for comfort, given clinical dyspnea, despite normal
blood gases. On HD# 27 he was briefly reintubated for chest tube placement to drain
a large pleural effusion. Extubation to HFNC occurred the following day. Pulmonary
toilet maneuvers and physiotherapy to avoid deconditioning were performed
during this time.
His respiratory status continued to improve, and he was decannulated on
HD#21. Five days later, chest CT revealed improved aeration. He was transferred to
the floor on HD# 32 and weaned to RA the following week. He was discharged home
on HD# 48 on RA, requiring bronchodilators and rehabilitation. See Figure 1 in the
main article for his serial radiographic studies.
Patient 11
Term infant male with meconium aspiration and severe asphyxia admitted
for ECMO evaluation on DOL #5. Despite progressive escalation with HFOV, iNO and
inotropes severe hypoxemia persisted VV ECMO was initiated on PICU day #1, using
a 12-Fr Origen double lumen cannula. He was placed conventional ventilation on
low settings, inotropes and sedatives were reduced, CVVH was initiated for postasphyxial renal insufficiency.
The day after cannulation, the endotracheal tube was dislodged during
bronchoscopy, and multiple attempts to reintubate resulted in severe supraglottic
edema, so the patient remained extubated on RA and systemic steroids for 4 days,
with improved work of breathing and decreased distress. On HD#6 the supraglottic
edema improved, and the patient was reintubated and recruitment with positive
pressure initiated. Because of hemodynamic instability later that same day, the
patient was converted to VA ECMO with an 8Fr Biomedicus cannula in the RCCA.
Bilateral pleural effusions seen on CXR required drainage on HD#8. Aeration of both
lungs progressively improved and he was decannulated on HD# 16.
In the weeks following decannulation, the patient progressively worsened,
with recurrent pneumothoraces, severe hypoxemia and hemodynamic instability.
Life-sustaining care was withdrawn on HD#53.
Patient 12
Five month-old female admitted for surgical correction of aortic recoarctation that was complicated by left main bronchus disruption. Lung collapse
cardiac arrest occurred 72 hours postoperatively (HD# 5), requiring emergent
central cannulation for VA ECMO with an 8-Fr Biomedicus arterial cannula was in
the aorta and a 16-Fr DLP Right Angle venous cannula in the RA.
Bronchoscopy performed after cannulation showed no evidence of airway
perforation. Attempted re-expansion of atelectatic lungs caused pneumothoraces.
Several bronchoscopies encountered thick secretions and subglottic edema and
inflammation. On HD# 10 a bronchogram disclosed contrast leak from the proximal
left main bronchus with distal occlusion. Given the fistula and persistent atelectasis,
ventilator support was reduced to a CPAP of 5 on HD# 11.
Since the arch repair was satisfactory and cardiac function and
hemodynamics were stable, the patient was converted to VV ECMO with a 12-Fr
Origen double lumen cannula followed by VA cannula removal and sternal closure
on HD# 14. Intraoperative bronchoscopy showed edematous subglottic airway, so
the patient was extubated to promote airway healing and decrease edema on HD#
15.
Despite improvement in her respiratory status and progressive spontaneous
aeration on CXR and airway healing on bronchoscopy, on HD# 19the patient became
acutely hypotensive and developed a ventricular dysrhythmia from which she could
not be resuscitated. . She was deemed not to be a candidate for repeat VA ECMO and
expired.
Patient 13
Term infant male with birth asphyxia, meconium aspiration and severe
hypoxemia admitted for ECMO evaluation DOL#1. Despite HFOV, iNO and inotropes,
his oxygenation remained poor and he was cannulated for VV ECMO using a 15-Fr
Origen double lumen cannula and a 8-Fr Biomedicus cephalad cannula were placed
in the RIJV. He was converted to conventional ventilation, and inotropes and
neuromuscular blockade were quickly stopped afterwards.
To reduce ventilator-induced lung injury, he was extubated to RA on HD# 4.
Initial respiratory distress improved with HHFNC. Pulmonary toilet and recruitment
maneuvers were performed during this period
Lung aeration progressively improved. On HD#10 he was reintubated for
ECMO decannulation. He was definitively extubated to HHFNC on HD# 15,
transferred to the floor five days later and discharged home on HD# 53, on regular
diet and normal activity, without any complications.
Patient 14
19-day female term infant with prenatal diagnosis of left-sided CDH repaired
without complications at 4 days of life was admitted for pulmonary hypertension
from sepsis. Prior to surgical repair the baby had elevated RV pressures but
subclinical PAH. Despite HFOV, iNO, inotropes and sildenafil infusion, she was
cannulated for VV ECMO with a 15Fr Origen double lumen cannula in the RIJV.
Conventional ventilation resumed on rest settings.
On HD# 4 she was extubated for lung rest and remained on HFNC for 2 days
without complications. Due to worsening pulmonary atelectasis and oxygen
saturations decision was made to re-intubate the patient to perform a
bronchoscopy. An ultrasound showed a large pleural effusion compressing the left
ventricle, and a chest tube was placed with subsequent improvement in
oxygenation.
However, and despite daily pulmonary recruitment attempts, maximum
treatment for pulmonary hypertension and 7 days of lung rest on ECMO, no
improvement was seen in her respiratory status, and decision was made with the
family to withdraw life-sustaining care.
Patient 15
Two-year old (ex 29 week premature) previously healthy female with
Influenza A was admitted for pneumonia with worsening respiratory distress.
Intubation was performed on HD#1 and high pressures were required on
conventional ventilation to ensure oxygenation.
On HD#7 worsening hypoxia occurred and CXR showed an extensive
pneumomediastinum. She was then cannulated for VV ECMO with an Origen 18-Fr
double lumen cannula in the RIJV. Complete opacification of the lungs was seen and
a bronchoscopy was performed to clean her airways from secretions. An additional
RFV cannula was added on HD# 10 for venous drainage with oxygenation
improvement.
Course became complicated by Pseudomonas superinfection in the
respiratory tract (BAL cultures) and sedation-induced delirium. To provide
complete lung rest and decrease sedative requirements, she was extubated to NC on
HD# 20. She remained off the ventilator for 5 days with increased cough, less
oversedation and overall better aeration. She got enteral feeding during the ECMO
run and was able to play video games during the extubation time.
On HD#25 propofol infusion was given to help weaning sedatives and the
patient became apneic requiring reintubation. The same day, during acute episode
of agitation the neck cannula migrated. She was sedated and the catheter was
secured without incident.
Her respiratory status continued to improve, sedation requirements
decreased, and she was finally decannulated on HD#28. Three days later, she was
transferred to the floor and weaned to RA two weeks later. She was discharged
home on HD# 47 on RA, requiring bronchodilators and rehabilitation.
Patient 16
5-year-old male who presented with a 3 week history of URI symptoms
showed leukocytosis with abnormal cells on peripheral smear, thrombocytopenia
and metabolic acidosis. He was diagnosed with T cell ALL via bone marrow aspirate
and was started on induction chemotherapy. RSV pneumonitis was diagnosed by
lung biopsy. Due to concern for worsening hypoxia he was transferred to PICU and
subsequently intubated and switched to HFOV on HD# 40.
Respiratory deterioration continued. Because the patient was likely to go on
ECMO and the course was expected to extend at least 2 weeks in duration, a CSF
reservoir was placed 24 hours before cannulation for intrathecal chemotherapy. On
HD#43 and he was cannulated for VV ECMO with a double lumen 19Fr Avalon
cannula 14Fr cephalad cannula in the RIJV. He received intrathecal methotrexate
ECLS.
On HD# 44 to avoid further ventilator-induced lung injury, the patient was
extubated to HHFNC without complications. Two days later, CXRs showed improved
bilateral aeration. Sedatives were discontinued leading to vigorous cough,
interaction with his surrounding (talking with parents, watching TV, playing video
games). BiPAP was added overnight for respiratory comfort.
On HD# 53, his mental status acutely changed. Head CT showed massive
acute right parietal hemorrhage with herniation so he was reintubated with hopes
of stopping extracorporeal support and reversing anticoagulation for possible
hematoma evacuation. However, patient became bradycardic when the pump was
stopped and CPR was not initiated per family request.