Is dying from pulmonary hypertension painful

Pulmonary hypertension is no longer a death sentence

It is well known that many people have high blood pressure. Pulmonary hypertension, on the other hand, is less known. A particularly rare form of this disease, pulmonary arterial hypertension, only affects around 50 out of a million people.

Patient Siegfried Hennecke keeps fit as much as possible

Siegfried Hennecke is one of them. Almost a year ago, the actually sporty pensioner suddenly had problems getting up the stairs. "There were 38 steps leading to my apartment. Up until just before Christmas I was still able to get up there well. And suddenly, just before the third to the last step, nothing worked," he recalls. His condition got worse and worse until his doctor admitted him to a local hospital.

The team of doctors there moved Hennecke to the university clinic in Bonn. There he was treated by the pulmonologist Dirk Skowasch - great luck for the patient, because Skowasch quickly recognized what Hennecke was suffering from: pulmonary hypertension.

This occurs when the pulmonary arteries - which lead into the lungs - are narrowed. The blood that comes from the right ventricle then builds up. This overburdens the heart.

Behind the bottleneck, however, not enough oxygen-rich blood flows from the lungs back to the left heart and into the body: the left half of the heart has much less to do than the right. In addition, muscles and organs are no longer adequately supplied with oxygen.

University professor Ghofrani, the pharmacologist Stasch and the physician Frey have brought the active ingredient to market maturity

Hennecke had noticed symptoms before but didn't know what was wrong with him. "The diagnosis is difficult," says his doctor Dirk Skowasch, "because the disease is really rare and the symptoms are unspecific".

A pulmonary hypertension patient usually complains of shortness of breath and fatigue. But that could have many causes, says Skowasch: "The heart specialist will say: It is not a relevant heart disease. The lung specialist will see an inconspicuous lung function, and the patient will be unsettled at first until the correct diagnosis is finally made, and also he first heard of the bad prognosis. "

As recently as the 1990s, such a "bad prognosis" meant in principle a death sentence. Patients were given about 2.5 years afterwards. Today the pulmonary hypertension has lost its horror. This is thanks, among others, to Johannes-Peter Stasch. The pharmacologist from Bayer Pharma AG in Wuppertal discovered a promising molecule in 1994.

For Dirk Skowasch, the modern sCG stimulators are a lifesaver

The treatment principle has been known since the 19th century

The mechanism is similar to an active principle that has been known for 130 years: Nitroglycerin, inhaled as a spray, for example, provides relief in the case of angina pectoris.

However, it wasn't until the 1990s that researchers found out exactly why this works: In the human body, nitroglycerin is converted into nitric oxide. "This is a gas that causes the coronary vessels to relax and widen," explains Stasch: "It causes the symptoms of angina pectoris to disappear."

However, it is not that straightforward, because there are still a few steps between the formation of nitric oxide - from nitroglycerine - and the expansion of the blood vessels. So the nitric oxide first has to find a place where it can go.

Extensive studies preceded the approval

"The substance it encounters is a protein molecule - an enzyme," explains the pharmacologist. "It is called soluble guanylate cyclase (sGC), which is present in every cell in the human body." In the next step, the SGC enzyme produces - like a catalyst, so to speak - a messenger substance called cyclic guanosine monophosphate (cGMP).

And that leads to the actual positive improvement, says Stasch: "It generally leads to an expansion of blood vessels and prevents the growth and proliferation of cells in these vessels so that the vessels are not constricted and cells that lead to it proliferate that the resistance increases - and with it the pressure in the vessels. "

New active ingredient stimulates sCG enzyme

The new active ingredient against pulmonary hypertension is called riociguat and the drug on the market is called Adempas. The class of drugs is known as sCG stimulators. It is now approved in over 50 countries. In principle, it works exactly like nitric oxide in nitroglycerin spray - it docks with the sCG enzyme and thus leads to the release of the same cGMP messenger substance.

  • The heart - a beating marvel

    Marvel of nature

    The heart is a marvel of nature: the fist-shaped hollow muscle contracts around seventy times a minute and pumps up to 10,000 liters through the body every day. And that for a lifetime. If necessary - when jogging, for example - the heart even transports five times as much blood through the body.

  • The heart - a beating marvel

    Double work

    Our heart is actually made up of two pumps. Because there is not just one blood circulation, but two. The right ventricle pumps blood to the lungs, where it is refueled with oxygen. At the same time, the left ventricle transports the same amount of blood into the body's circulation. Not easy. Because there is much higher pressure in the body's circulation than in the pulmonary circulation.

  • The heart - a beating marvel

    Boom, boom

    Each half of the heart consists of an atrium and a heart chamber. The blood can only flow in one direction, since heart valves (green) that work like check valves are located between the atria and the chambers and between the chambers and the adjoining vessels.

  • The heart - a beating marvel

    Real muscle work

    The heart is just a muscle - but a very special one. It is similar to those on the arm and leg, because it can contract just as quickly and powerfully. But he is particularly persistent and does not tire. In addition, all heart muscle cells are coupled to one another so that the entire heart muscle always contracts at the same time.

  • The heart - a beating marvel

    Natural pacemaker

    Try using willpower to keep your heart beating! This does not work because the heart is not controlled by nerves, but has its own clock: Special muscle cells in the sinus node regularly generate a small current that spreads over the whole heart at lightning speed and causes it to contract. If the sinus node is defective, the AV node takes over.

  • The heart - a beating marvel

    Out of step

    If the heart gets out of rhythm, for example with ventricular fibrillation, it no longer relaxes, but remains constantly tense. Then the organ can no longer pump blood. A shock generator, the defibrillator, interrupts the life-threatening constant excitation in the heart so that the natural clock can take over again. Even a layperson can use the device.

  • The heart - a beating marvel


    If a patient's heart beats too slowly, an artificial pacemaker can help. The device generates electrical impulses and transmits them to the heart muscle. Doctors first implanted a pacemaker in 1958. A modern cardiac pacemaker lasts between five and twelve years, with an average of eight years.

  • The heart - a beating marvel

    With an open heart

    To be able to operate on the heart, doctors have to stop it briefly and shut down the circulation - actually a death sentence. But in the 1950s, scientists solved the dilemma: They developed the heart-lung machine. The device takes over the function of the heart and lungs for a short time, enriches the blood with oxygen and pumps it through the body.

  • The heart - a beating marvel

    Through the groin into the heart

    Modern medicine allows the heart to be examined or operated on without cutting open the patient's chest. To do this, the doctor inserts a heart catheter - a kind of thin plastic tube - through the groin, the crook of the elbow or the wrist and pushes the tube over veins or arteries to the heart. The patient is only anesthetized locally beforehand.

  • The heart - a beating marvel

    Foldable heart valve

    If one of the heart valves is broken or worn out, a new one is needed. Doctors use either organic pig replacements or mechanical metal heart valves. There are now also artificial heart valves that can be folded up (see photo) and can therefore be inserted using a catheter in a minimally invasive manner. Open heart surgery is then no longer necessary.

  • The heart - a beating marvel

    Clogged vessels

    The coronary vessels supply the heart muscle with blood, i.e. nutrients and oxygen. If one of these vessels becomes blocked, the tissue no longer supplied with blood dies - heart attack! The cardiac surgeon bridges the narrowed area with a bypass (green in the picture). To do this, he takes a vein from the patient that is no longer needed or a vascular prosthesis made of plastic.

  • The heart - a beating marvel

    Metal lifeguards

    If a coronary artery is narrowed, the doctor can insert a catheter into the blood vessel and expand the constriction with a balloon. To prevent the vessel from contracting again afterwards, it is held open with a stent: These are fine metal sleeves that support the blood vessel wall from the inside. The tubes can also be coated with medication.

  • The heart - a beating marvel

    When your own heart doesn't want anymore

    The first heart transplant was performed by cardiac surgeons in 1967. A sensation back then. In the meantime, the operation is no longer uncommon: every year, doctors transplant several thousand donor hearts from deceased people around the world. The recipients, however, have to take medication for life to prevent their own body from rejecting the foreign organ.

  • The heart - a beating marvel

    Life on credit

    Donor hearts are rare. If your own heart is no longer working properly, for example if it is insufficient, it can be supported with an artificial heart. Your own, sick heart remains in the body, supported by an implanted pump. The drive and energy supply for the pump are located outside the body.

  • The heart - a beating marvel

    An artificial heart

    The researchers' dream is an artificial heart, which completely replaces the patient's sick heart. It should be able to be inserted into the body without connecting hoses to the outside world and be maintenance-free for many years. There are already prototypes.

    Author: Brigitte Osterath

For pulmonary hypertension patients, the discovery is a lifesaver, because with nitroglycerin spray, their chronic illness could not be brought under control in the past, because nitroglycerin only works against acute attacks in angina pectoris but not in pulmonary hypertension.

With the new drug, the pulmonary hypertension cannot be cured, but it can be brought under control. "The patients take one tablet three times a day. We selected this dosage in order to have the most even drug level in the blood and to avoid large peaks," says the doctor Reiner Frey. He was involved in the discovery at Bayer and promoted its further development into a drug. "So we have a constant level in the blood and in the tissue, in the cells. After all, it has to get into the cell in order to have an effect."

Patients must take the drug Adempas for their entire life

Lifelong use of medication

Patients may have to take the drug for life, but if they do, they have a good chance of survival. "The long-term data that we have from the study participants show very positive survival rates and efficacy data," says pulmonologist Ardeschir Ghofrani.

He led the clinical studies at Giessen University Hospital - from the very first laboratory tests to the drug's approval in the USA in 2013 and in Europe in 2014. or over 90 percent. That is a very positive result, if you had otherwise expected survival rates of 70 and 50 percent. "

In any case, the patient, Siegfried Hennecke, is optimistic that he can control the disease well. If he leaves the house for a long time, he takes an oxygen device with him to be on the safe side. Most importantly, he should take his pills regularly. "I notice when I don't take one. I hope it stays that way. I'll never get it away. But I've already ridden a bike!"

And Hennecke wants to continue to do so. The only thing that worries him now is the oxygen device, because it is too heavy to anchor it securely on the luggage rack.

Neurochemist Johannes-Peter Stasch and doctors Reiner Frey and Ardeschir Ghofrani received the German Future Prize for developing the active ingredient riociguat.