The acute form is related to a temporary restriction of the pulmonary vessels and occurs in hospital resuscitation rooms and operating rooms. It is life threatening for patients.
Chronic PAH is caused by a proliferation of cells in the walls of small pulmonary arterioles. This progressive re-profiling of the vessels leads to their constriction and a consequent rise in pulmonary blood pressure. It leads to reduced heart and lung function to the point of fatality.
A disease with many causes1
Acute PAH presents:
In newborns suffering from a failure of the normal extra-uterine circulatory transition, leading to severe respiratory distress (Persistent Pulmonary Hypertension of the Newborn)
In newborns or children following surgery for congenital heart disease
In adults during or after cardiac surgery (valve replacement, transplant, etc.)
Between 1 and 2 births per 1,000 present with persistent pulmonary hypertension2.
PAH in its chronic form may be:
Idiopathic (without identifiable cause)
Familial or hereditary
Secondary as a result of medication (e.g. anorectics)
Associated with a chronic disease (HIV infection, parasitic infection, chronic hemolytic anemia, etc.).
Globally, the most frequent forms of PAH are those that are associated with a clearly identified cause, such as left heart failure, chronic obstructive and chronic post-embolic respiratory disease.
If left untreated, acute PAH leads to reduced oxygen supply and cardiac failure.
In its chronic form, it presents with clinical signs such as shortness of breath, reduced ability to exercise, chest pain and fainting, which gradually reduce patient quality of life and limit activity.
Diagnosis requires additional examination using specialized facilities
These non-specific symptoms can also present in a broad range of cardiac and pulmonary diseases. Diagnosis requires additional examination using specialized facilities, which can delay commencement of treatment. Delays of two years or more before diagnosis of PAH and the start of treatment are frequent3.
The hope of new treatments4, 5 and 6
Although inhaled and other therapies are used to dilate the pulmonary vessels in the acute phase of PAH, there is currently no curative treatment available for chronic PAH.
To relieve the symptoms, treatment generally involves the use of non-specific medications, such as anticoagulants, diuretics and oxygen, if necessary. Lung transplantation is offered only for those patients where medication has delivered no improvement.
Committed to PAH care
Over the last 20 years or so, inhaled therapies delivered in-hospital in combination with other active substances have reduced hospital mortality rates following heart and lung surgery.
Air Liquide Healthcare’s continued commitment to working alongside healthcare professionals in providing PAH care and treatment allows us to offer solutions that are adapted to each patient, ensure continuity of care and anticipate future needs.
As soon as the chronic disease is diagnosed and following prescriptions by a physician, our teams make an active contribution to delivering a sustainable improvement in patient quality of life through the provision of specific treatments, including nebulizers, infusion and oxygen therapy.
For PAH presenting in hospital resuscitation rooms and operating rooms, the healthcare service provider delivers comprehensive support, including the supply of medical gases, appropriate administration and monitoring equipment for completely safe product delivery, training for medical teams, and all the necessary technical support.
For chronic PAH, the healthcare service provider works with healthcare professionals to support each patient throughout his/her treatment.
Healthcare service provider teams work in patients' home to put in place the prescribed care protocols by delivering the services, products and equipment required, supporting and training patients and their families to make them as self-sufficient as possible, and monitoring their treatment.
Simoneau G, Robbins IM, Beghetti M et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 25 (Suppl): D34-41
Walsh-Sukys MC, Tyson JE, Wright LL, Bauer CR, Korones SB, Stevenson DK, Verter J, Stoll BJ, Lemons JA, Papile LA, Shankaran S, Donovan EF, Oh W, Ehrenkranz RA, Fanaroff AA. Persistent pulmonary hypertension of the newborn in the era before nitric oxide: practice variation and outcomes. Pediatrics 2000; 105: 14–20.
Humbert M, Sitbon O, Simoneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425-1436.
Miller OI, Tang SF, Keech A, Pigott NB, Beller E, Celermajer DS. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. Lancet 2000 Oct 28; 356 (9240): 1464-9.