Female patient, aged 57, retired, non-smoking, presents dyspnea on
exertion, at minimal effort, chronic severe fatigue, daytime sleepiness,
insomnia, broken sleep associated with snoring and apneas, nocturia (2-3 times/night),
morning headaches and xerostomia, periodic pain in the periumbilical region.
From
history: juvenile obesity (100 kg), hypertensive since the age of 50, presents
sleep disturbances and noisy snoring for over 10 years. The physical examination reveals
dry skin, mild inferior limbs edema, acrocyanosis (lips, legs), obesity with the
BMI = 47 kg/m2 (m=118 kg, h=154 cm), neck girth = 43 cm, abdominal circumference
= 140 cm, umbilical hernia 3x4 cm, free nasal breathing, diminished vesicular
sounds, stable hemodynamics (HR= 68/min; BP= 130/80 mmHg), Mallampati score III and17
points on the Epworth Sleepiness Scale.
Chest X-ray was normal, ECG revealed
signs of right ventricular strain. The EcoCG revealed slight dilation of both atria
(up to 56 mm) with the left ventricle of normal dimensions and preserved ejection
fraction (50%), severe right ventricle (RV) dilatation (46 mm) and severe
pulmonary hypertension (pulmonary artery systolic pressure 80 mm Hg). SpO2 at rest is 95%.
Spirometric and blood gazes values are as follows FEV1-81%; FVC-80%; FEV1/FVC-109%,
pH 7,43; PaO2 96 mm Hg; PaCO2 44 mm Hg.
Questions:
Considering
the anamnestic data and the physical examination, which are the probable causes
of pulmonary hypertension?
What
tests do you suggest for confirmation?
Which
treatment is the most suitable?
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