Pulmonary hypertension - management update

Pulmonary Hypertension-
Management Updates
MAJOR DR. MD. AMINUL HAQUE
M.D (Cardiology)
Classified Cardiologist
CMH, Dhaka

Pulmonary Hypertension (PH)
Pulmonary hypertension (PH) is a haemodynamic and
pathophysiological condition defined as an increase in
mean pulmonary arterial pressure (PASP) 25 mmHg at
rest as assessed by right heart catheterization.
Pulmonary arterial hypertension (PAH) is defined as PH
with normal left-sided filling pressure (PCWP<15 mm Hg)
and an increased PVR.
Pulmonary venous hypertension exists when pulmonary
venous or LA pressure (PCWP) rises above 15 mm Hg. To
maintain forward blood flow, pulmonary arterial
pressure increases; however, this does not necessarily
result in an increase in PVR.
PH can be mixed, defined as PH with a PCWP >15 mm Hg
and an increased PVR.

Recommendations for general measures
Statement Class of
recommendation
Level of
evidence
It is recommended to avoid pregnancy in patients with
PAH
I C
Immunization of PAH patients against influenza and
pneumococcal infection is recommended
I C
Physically deconditioned PAH patients should be
considered for supervised exercise rehabilitation
IIa B
Psychosocial support should be considered in patients
with PAH
IIa C
In-flight O2 administration should be considered for
patients in WHO-FC III and IV and those with arterial
blood O2 pressure consistently less than 8 kPa (60 mmHg)
IIa C
Epidural anaesthesia instead of general anaesthesia
should be utilised, if possible, for elective surgery
IIa C
Excessive physical activity that leads to distressing
symptoms is not recommended in patients with PAH
III C

Recommendations for supportive therapy
Statement Class of
recommendation
Level of
evidence
Diuretic treatment is indicated in PAH patients with signs
of RV failure and fluid retention
I C
Continuous long-term O2 therapy is indicated in PAH
patients when arterial blood O2 pressure is consistently
less than 8 kPa (60 mmHg)
I C
Oral anticoagulant treatment should be considered in
patients with IPAH, heritable PAH, and PAH due to use of
anorexigens
IIa C
Oral anticoagulant treatment may be considered in
patients with APAH
IIb C
Digoxin may be considered in patients with PAH who
develop atrial tachyarrhythmias to slow ventricular rate
IIb C

Evidence-based treatment algorithm for pulmonary arterial hypertension patients
(for group 1 patients only)

Route of administration, half-life, dose ranges, increments, and
duration of administration of the most commonly used agents
for pulmonary vasoreactivity tests
Drug Route
Half-
life
Dose range Increments Duration
Epoprostenol Intravenous 3 min 2–12 ng/kg/min 2 ng/kg/min 10 min
Adenosine Intravenous 5–10 s 50-350 µg/kg/min 50 µg/kg/min 2 min
Nitric oxide Inhaled 15–30 s 10–20 p.p.m – 5 mind

Suggested assessments and timing for
the follow-up of patients with PAH

Determinants of Prognosis
Determinants of Risk Lower Risk (Good
Prognosis)
Higher Risk (Poor
Prognosis)
Clinical evidence of RV failure No Yes
Progression of symptoms Gradual Rapid
WHO class II, III IV
6MW distance Longer (greater than 400 m) Shorter
(less than 300 m)
CPET Peak VO2 greater than 10.4
mL/kg/min
Peak VO2 less than 10.4
mL/kg/min
Echocardiography Minimal RV dysfunction Pericardial effusion, significant
RV enlargement/dysfunction,
right atrial enlargement
Hemodynamics RAP less than 10 mm Hg, CI
greater than 2.5 L/min/m2
RAP greater than 20 mm Hg, CI
less than 2.0 L/min/m2
BNP Minimally elevated Significantly elevated

Pulmonary hypertension - management update

Pulmonary hypertension - management update

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