Pulmonary hypertension in the eyes of a clinician and a patient

This week (19th-27th November) in the UK is Pulmonary Hypertension awareness week. The theme for this year is “Let’s talk about PH” so we thought we’d do just that by inviting Dr. Stylianos E. Orfanos, Professor of Critical Care and Medicine at National and Kapodistrian University of Athens, to give us a comprehensive overview of the disease as well the current treatments available and the barriers that can prevent patients form being effectively treated.

What is pulmonary hypertension?

Pulmonary hypertension is a pathologic condition related to increased pressures in the pulmonary circulation.  Every five years the World Symposia on Pulmonary Hypertension takes place to provide state-of-the art knowledge on the disease. The 5th such Symposium took place in Nice France in early 2013. In 2015 the joined European Society of Cardiology / European Respiratory Society Guidelines for the diagnosis and treatment of Pulmonary Hypertension by Galiè N, Humbert M et al were published in the European Heart Journal and the European Respiratory Journal, providing a comprehensive tool on how to handle the disease.

Patients suffering from pulmonary hypertension are divided into 5 groups: in Group 1 are patients suffering from pulmonary arterial hypertension (PAH); Group 2 patients develop pulmonary hypertension due to left heart and valvular disease; in Group 3 are patients suffering from chronic lung diseases; in Group 4 patients with chronic thromboembolic pulmonary disease; Group 5 patients develop pulmonary hypertension related to multi-factorial reasons.

PAH is a rare but very severe disease; it may be idiopathic or related to several pathologies like connective tissues diseases, congenital heart diseases, etc. PAH may also be heritable or related to several drugs and toxins.

What are the causes?

This process will compromise the right heart of the patient, and if not controlled, will dramatically decrease patients’ survival.

Pulmonary circulation possesses a vast surface area necessary for the oxygenation of the living organism but also for its important metabolic properties. In PAH the pathologic process starts at the pulmonary vessels: their metabolic activity gets compromised, and vessel wall hypertrophy occurs; as the disease progresses further, more and more vessels are occluded: like a tree, the branches of which are pruned. Pulmonary vessel surface area will decrease, compromising oxygenation, and increasing pulmonary arterial pressure and pulmonary vascular resistance. This process will compromise the right heart of the patient, and if not controlled, will dramatically decrease patients’ survival.

A few decades ago, most patients suffering from PAH would live less than 3 years. Fortunately we now possess an armamentarium of specific medications that control the pathologic process. Patients now have significantly improved survival and better quality of life.

It is of paramount importance for the patient to be referred to a specialized referral center as early as possible for diagnosis, follow up, and appropriate treatment. Good collaboration between the treating referring physician and the specialized referral center is of great importance.

Early signs and treatment

PAH may be present for quite some time before the first symptoms develop. Unexplained shortness of breath from an everyday effort, such as climbing up stairs, may be the first symptom and should alarm both the treating physician and the patient. What usually follows is that patients undergo a trans-thoracic cardiac echocardiogram that will provide, among others, evidence of high pulmonary arterial pressure.

It is very important that patients belonging to high risk groups for PAH undergo regular testing in specialized centers, even in the absence of symptoms. It should be noted that the diagnosis of pulmonary hypertension and of PAH is made by right heart catheterization only, while several tests are needed to determine in which group the patient belongs.

Specific therapy for the treatment of PAH is directed to three biological pathways related to nitric oxide, a prostanoid known as prostacyclin, and endothelin. The two first “good” compounds help the lung vessels stay open, while the third “bad” one does the opposite. In lungs affected by PAH, prostacyclin and NO levels are low, while endothelin levels are high. Current PAH specific treatments aim to correct this imbalance. It should be noted however that although these specific treatments allow patients to live longer and have a better life, there is still no cure for this devastating disease.

Surgery, i.e. pulmonary thrombo-endarterectomy, when possible, in Group 4 patients may offer an impressive amelioration, while specific medication is also available when needed. Balloon angioplasty is also performed but full evaluation of this technique (when, how and to whom) is still under way.

Invisible patient

A disease that evolves, with no cure at present despite the stabilization that specific treatment may offer, is expected to affect patients both physically and psychologically, as well as their families. Mrs. Ioanna Alissandratou, President of Hellenic Pulmonary Hypertension (HPH; i.e. the Greek PH patients’ Association) provides the patients’ view:

We often call the PH patient an invisible patient who is not socially recognizable because the illness is not always obvious.

We often call the PH patient an invisible patient who is not socially recognizable because the illness is not always obvious, and sometimes people think that when you don’t look sick you are not sick. If someone does not see the patient having an oxygen mask on, he/she does not understand the problem and its severity. We on HPH try to aware people in every way that we can, so given this opportunity patients are gathered along with their caregivers and participate in events aiming to bring to life the “invisible patient”. The photos are taken at two such events.

Patients and their caregivers at the ‘authentic’ Athens marathon outside the kallimarmaron stadium.

Tasks that were once easy, such as climbing stairs, walking around the block, cleaning the house may become much more difficult and leave patients breathless; thinking and brain function will seem slower, more labored. In general, patients get tired earlier and easier. All these make necessary the need of a caregiver for the pulmonary hypertension patient, who will guide and assist patients on their every-day activities

Obstructions to treatment

The PAH patient is usually under many drugs daily that are very expensive (orphan drugs), and, unfortunately, many countries around the world cannot provide all the required specific medicines; this means that severely-ill patients may lose their life. Fortunately in Greece, we get all the required medical help and medicines, but bureaucracy-related delays that worry patients and physicians alike are sometimes present. Also crucial for patients in need of a heart or lung transplantation is the fact that there is no specialized lung transplantation center in Greece. This results in patients having to go abroad, increasing the financial costs and delays.

For all the above-mentioned reasons it is extremely important to have, in every country, a patients’ organization to support, seek and demand the best for the patients and their families. HPH is a member of 1) PHA , that is the US-based Association founded by patients, which also joins physicians, researchers and nurses fighting the disease, and 2) PH Europe . All together we are trying to achieve the best we can for the pulmonary hypertension patient.

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