The rare cause of Severe Pulmonary Hypertension (free full text from 2017 Moldovan Journal of Health Sciences)

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?

  Free full text is HERE!

Continuous Positive Airway Pressure: Life-Saving Technology (full text from Current Respiratory Medicine Reviews)

Dear friends World Sleep Day 2016 is under corner (18 March 2016)!

World Sleep Day is an annual event, intended to be a celebration of sleep and a call to action on important issues related to sleep, including medicine, education, social aspects and driving. It is organized by the World Sleep Day Committee of the World Association of Sleep Medicine (WASM) and aims to lessen the burden of sleep problems on society through better prevention and management of sleep disorders.

The obstructive sleep apnea syndrome (OSAS) remains a common diagnosis made by pulmonary practitioners. OSAS is a common respiratory disorder affecting at least 2% to 4% of the adult population. The mainstay of treatment of moderate and severe OSAS is continuous positive airway pressure (CPAP). The development of this therapeutic intervention in 1981 by Sullivan, stimulated the rapid progression of sleep testing and therapy as a new field in medicine.

Full text from Current Respiratory Medicine Reviews:

http://benthamscience.com/journals/current-respiratory-medicine-reviews/volume/12/issue/1/page/2/

Happy Birthday to CPAP!!!

Almost exactly 33 years after the publication of his pioneering paper on positive airway pressure for sleep apnoea (PAP) in The Lancet, Colin Sullivan continues to extend the boundaries of sleep medicine. In those three decades, PAP has gone from an obscure treatment available to few to an effective, portable treatment that has improved the sleep, and thus lifestyles, of millions worldwide.

Sullivan and his team undertook experiments with dogs on airway obstruction, and created a mask for use on human patients. Air pressure was controlled with a circuit that raised pressure until passive obstruction of the airway was cleared. His first test patient was a 43-year-old construction worker who did not want a tracheostomy—back then the only treatment. At very low pressures, PAP cleared the man's airway and allowed him to sink into a deeper sleep, as well as being sleep-free the next day. An elated Sullivan tried the technique on four other patients, and these findings formed the basis for his 1981 Lancet paper: Reversal of obstructive sleep apnoea by continuous positive airway pressure applied through the nares.

“When we were writing the paper,” says Dr. Sullivan, “we had no idea how many people had sleep apnea”.

Abstract from this great article: Five patients with severe obstructive sleep apnoea were treated with continuous positive airway pressure (CPAP) applied via a comfortable nose mask through the nares. Low levels of pressure (range 4·5-10 cm H2O) completely prevented upper airway occlusion during sleep in each patient and allowed an entire night of uninterrupted sleep. Continuous positive airway pressure applied in this manner provides a pneumatic splint for the nasopharyngeal airway and is a safe, simple treatment for the obstructive sleep apnoea syndrome.