Introduction
The beauty of the mountains is ‘breathtaking’. They astound us not only
with their grandiosity but also by drowning us through uneven pulmonary
vasoconstriction. [1] High Altitude Pulmonary Edema (HAPE) does not
reveal itself immediately because the pathophysiological changes begin
early but progress through clinically silent phases. [2] It usually
takes 2 to 5 days and an altitude above 3000m to manifest into its
fullest form. [3, 4, 5, 6]. The mountain Gods consider haste as a
sign of disrespect. A careless attempt to compete against time makes it
difficult to escape their wrath of acute mountain sickness and HAPE.
Over-exertion and ascending with respiratory tract infection further
increase the chances of suffering. [7, 8]
The clinical features of HAPE share a close resemblance with pneumonia:
cough (mostly productive), shortness of breath, fatigue, tachypnea,
tachycardia, mild fever, and crepitation. Unfortunately, the locations
where HAPE is initially recognized are usually extremely
resource-limited: where clues from history and clinical examination form
the backbone for making an accurate diagnosis. HAPE and pneumonia are
comparable to monozygotic twins, where one takes the blame for another’s
mischief. Diagnosis becomes more difficult when they both co-exist or
are preceded by an upper respiratory tract infection. Ascent profile,
duration of altitude exposure, and a previous history of HAPE provide
major clues for a diagnosis favoring HAPE. However, in some rare cases
of delayed onset HAPE, where the patient develops symptoms after staying
at a particularly high altitude for more than 5 days, diagnosis of HAPE
becomes a formidable task as alternative diagnoses must also be
considered and ruled out.
Oxygenation is the cornerstone for the treatment of HAPE. This can be
achieved either through increasing the pressure of inspired air (descent
or Gamow bag) or through ventilation (preferably non-invasive). [8, 9,
10, 11] Temporary management with supplemental oxygen until the
patient is stable enough to bear the exertion of descent by foot has
been in practice in the Himalayan Rescue Association Aid Post. Over the
years, this approach has been successful in the aid post. Here we
present three unique cases of HAPE that do not show consistency with
their usual natural history. We made the diagnosis based on clinical and
ultrasonographic parameters using Point of Care Ultrasound (POCUS)
(Butterfly iQ)
Clinical Case 1 (Delayed onset HAPE):
A 41 years male with a history of HAPE 13 years back had ascended from
Lukla (2860m) to Everest Base Camp (5364 m) within a span of 4 days and
had been residing in the Everest Base Camp. On the
11th day at the base camp, he started having shortness
of breath, which lasted for 2 days before presenting to HRA Aid Post,
Pheriche. A day after the onset of shortness of breath; he started
having a productive cough with no fever or chest pain. He had no
hypertension or prior medical illness and was not under any medications.
The COVID-19 vaccine doses were uptodate as per recommendation but
previous vaccination history could not be recalled. On examination, the
patient was able to walk into the clinic and was well oriented to time,
place and person. There were no signs of respiratory distress. A faint
“gurgling” sound from the chest was audible without a stethoscope. His
peripheral oxygen saturation was 53%, heart rate was
108min-1 and axillary temperature was 98.4 degrees
Fahrenheit. His blood pressure was 125/80 mm Hg. There was no
parasternal heave, pedal edema, hepatomegaly or elevation of JVP. Vocal
fremitus was normal bilaterally. A dull percussion note was observed
from the 5th intercostal space onwards anteriorly on the right side and
from the 4th intercostal space onwards anteriorly on
the left side. Auscultation revealed equal air entry along with
inspiratory crackles and broncho-vesicular breath sounds in all the
chest segments bilaterally. Heart sounds were normal. Ultrasonography of
the lungs did not reveal any pleural collections, the lung sliding was
normal. However, multiple B-lines with absent A-lines were seen in the
anterior and lateral segments, most prominent in the anteroinferior and
inferolateral segments. (Figure 1) The patient was given Nifedipine SR
20mg along with oxygen from an oxygen concentrator at 10
Lmin-1 for 3 hours, following which he got evacuated
to a well-facilitated center. During his stay, his oxygen saturation
improved from 53% to 91%. The patient apparently improved with
treatment in a hospital for 4 days but could not be contacted following
descent.
Clinical Case 2 (Early onset HAPE):
A 29 years lowlander male had been working as a porter in Namche for
2months, shifting loads up and down from Namche(3400m) to
Pangboche(3800m). During a particular trip from
Namche, while he was carrying a heavy load from Pangboche (where he
stayed a night on the way) to Thukla; at around 4500m, he started having
persistent cough and shortness of breath that persisted on rest. He had
no fever. His cough was frequently productive with ”watery-bubbly
sputum”. As he was severely short of breath, he spent the night inside a
cave. He descended to the clinic after no improvement of symptoms the
next morning. Upon arrival, the patient was well oriented but was having
labored breathing and using accessory muscles. There was fatigue without
headache, nausea or dizziness. No symptoms suggestive of pneumonia or
upper respiratory tract infection were present prior to decompensation.
3years ago, he suffered from HAPE at Amolapcha base (5000 m) and was
treated in Lukla for a week. He had no hypertension or any other medical
conditions. The COVID-19 vaccine doses were uptodate as per
recommendation but previous vaccination history could not be recalled.
His heart rate was 105 min-1, blood pressure was
122/86 mm Hg, respiratory rate was 32 min-1, oxygen
saturation at room air was 67% and the oral temperature was 99.1
degrees Fahrenheit. There was no pedal edema, parasternal heave or
hepatomegaly. Vocal fremitus was normal. Auscultation revealed
inspiratory crepitations in the infra-axillary regions bilaterally. USG
revealed multiple comet tail signs on the inferior axillary regions
bilaterally, which were more prominent on the right side. (Figure 2) The
B-lines in other segments were not as prominent. There was no
consolidation. Oral Nifedipine SR 20mg three times a day was started
along with O2 from an oxygen concentrator at 10L
min-1 via facemask. The treatment improved
SpO2 to 98% within 15 minutes. His oxygen saturation
was maintained at 93% from oxygen at 3L min-1. Cough
remitted and shortness of breath resolved the next morning. His
saturation in room air was 91% at rest and 88% following a short walk.
He was advised to descend while continuing Nifedipine for 3 days.
However, his descent happened only after he somehow managed to deliver
the goods up to Thukla.
Clinical Case 3 (Unilateral HAPE):
A 32 years female presented to the clinic with a cough for 8 days. The
productive cough started at 2800m in Phakding and was associated with
fever. Initial symptoms got resolved after 7 days of Amoxicillin.
However, there was some remnant intermittent dry cough. At Lobuche
(4800m) she started having persistent coughs with “watery” sputum.
Persistent cough brought her to the high-altitude clinic at 4200m. She
had shortness of breath during descent but no shortness of breath at
rest or lying down. She had no fever, chest pain or palpitation. She had
a mild generalized headache at Lobuche, which resolved following
descent. Her appetite was normal; she had no nausea, vomiting or
dizziness. She had been taking acetazolamide along with
chlorpheniramine-bromhexine cough syrup prior to her arrival at the
clinic. She had no significant medical history. Vaccination status was
up-to-date. There was no history of altitude-related illness in her only
altitude trip to 4200m. The patient was able to walk comfortably to the
clinic. Her heart rate was 115, oxygen saturation was 77%, respiratory
rate was 16 min-1, blood pressure was 126/82 and oral
temperature was 98.8 degrees Fahrenheit. There were no signs of
respiratory distress or pedal edema. The apex beat was palpable on the
5th intercostal space in the midclavicular line. There
was no parasternal heave. Vocal fremitus was normal in all segments.
Heart sounds were normal. There was equal air entry with
broncho-vesicular sounds in all segments of both lungs except the right
inferior regions that had late coarse inspiratory crackles, which
persisted until the early expiratory phase. Chest ultrasonography
revealed no pericardial or pleural collection. Lung sliding was normal
bilaterally. Left-sided chest ultrasonography revealed multiple A-lines
with no signs of consolidation. However, Right-sided chest
ultrasonography revealed A-lines along with multiple moderately
interspaced B-lines (7-8) on the inferior segment, but no signs of
consolidation. The superior segments revealed A-lines with few B-lines
(5-6) (Figure 3). Dexamethasone 8mg was given immediately; followed by
Nifedipine SR 20 mg, three times a day and tablet Dexamethasone 4mg four
times a day. The oxygen concentrator provided oxygen at 6L
min-1 via a simple facemask. Within 30 minutes of
treatment, her oxygen saturation improved to 93% and her heart rate to
102 min-1. Her cough was relieved 2 hours after
starting the treatment. She received treatment in the clinic for 12
hours. Following overnight treatment, her cough remitted while
saturation improved to 85% on room air. However, there were persistent
late inspiratory crackles, albeit of lesser duration and intensity. On
repeat ultrasonography, there were 2 B-lines in the left inferior chest
fields. (Figure 4) There was no immediate rescue available, so she had
to stay for another day at the same altitude without oxygen. She was
then discharged on 2 more doses of Dexamethasone and Nifedipine 20 mg
three times per day until descent and was asked to stay very close to
the treatment facility. She descended the day after the discharge. Upon
further contact, she stated that had no cough or difficulty breathing
and had a sound sleep on the night after her discharge.