Brief overview
- Symptoms: Limited exercise tolerance and increasing shortness of breath, water retention (edema), bluish discoloration of mucous membranes (cyanosis)
- Course of disease and prognosis: Dependent on early and consistent treatment; without therapy, progressive changes in heart and lungs, progressive shortness of breath and shortened life expectancy
- Causes and risk factors: underlying diseases of the lung (especially COPD, sometimes pulmonary fibrosis due to sarcoidosis or tuberculosis); in acute cor pulmonale usually pulmonary embolism
- Examinations and diagnosis: medical history, physical examination, imaging (chest x-ray, cardiac ultrasound), electrocardiogram (ECG), cardiac catheter examination
- Treatment: smoking cessation, oxygen therapy, relieving medications (prostacyclins, broncho-spasmolytics), treatment of heart failure (with dietary changes and medications)
What is cor pulmonale?
The second part of the disease’s name refers to the cause of cardiac dilation – it lies in the lungs: increased flow resistance in the pulmonary arteries causes the right ventricle of the heart to work harder and harder to pump the deoxygenated blood flowing in from the body into the pulmonary circulation.
In response, the muscle wall of the ventricle initially thickens. If resistance continues to increase, the chamber enlarges as blood backs up and the structure of the muscle fibers is disrupted. The ventricle literally leaks. A so-called right heart weakness develops and eventually cor pulmonale – a condition that cannot be reversed.
Acute cor pulmonale
Chronic Cor pulmonale
Chronic cor pulmonale develops gradually. Possible triggers are various lung diseases that have one thing in common: They cause the pressure in the pulmonary circulation to rise. As a result, blood that is pumped from the right heart into the lungs partially flows back again. This leads to additional strain on the right ventricle.
The heart muscle is forced to work harder and harder to overcome the increased resistance and continues to enlarge. The tight structure of the muscle cells is disrupted, and connective tissue is deposited. As a result of these changes, the pumping force of the right ventricle continues to decrease. This results in what is known as right heart insufficiency.
Cor pulmonale: What are the symptoms?
Since chronic cor pulmonale develops over years, symptoms are mild at the beginning of the disease. However, with increasing weakness of the right ventricle (right heart failure), typical symptoms appear.
For example, blood backs up into the right atrium of the heart and into the veins of the large systemic circulation. The pressure created here causes fluid to leak from the blood vessels into the tissues. Water deposits develop between the cells and in the connective tissue (edema), especially in the area of the lower legs and ankles and on the back of the foot.
Oxygen deficiency throughout the body
Due to the reduced pumping capacity of the right heart into the pulmonary circulation, less oxygen-rich blood also reaches the left ventricle – and thus the body. This may result in an oxygen deficiency that eventually turns the skin and mucous membranes bluish (cyanosis).
In addition, patients with cor pulmonale suffer from shortness of breath, which is initially only felt during heavy physical exertion, and later even at rest. The neck veins become visibly prominent in some sufferers due to congestion at the heart. Other symptoms include hoarseness, coughing, sometimes with bloody sputum, and a feeling of pressure in the chest.
Patients with cor pulmonale are physically less resilient and more quickly exhausted. In advanced stages, physical overload occasionally leads to collapse and unconsciousness.
Acute cor pulmonale means danger to life
Cor pulmonale: What is the life expectancy?
Chronic cor pulmonale is a progressive disease that, if left untreated, leads to death after only a few years. Sufferers experience an increasing reduction in their quality of life, as they are less and less able to perform physically. In addition, there is shortness of breath, chronic coughing, and persistent exhaustion and fatigue.
If the disease has already led to organ changes in the lungs and heart, these are no longer reversible. However, the quality of life and life expectancy can be significantly improved by the use of various drugs combined with long-term oxygen therapy. With consistent therapy, the progression of the disease is delayed or comes to a halt.
In the case of cor pulmonale, it is important for smokers to stop smoking immediately. This is the only way to stop the progressive strain on the heart and lungs.
Acute cor pulmonale is a medical emergency. The prognosis depends on how quickly the trigger – for example, a pulmonary embolism – is recognized and treated.
Cor pulmonale: causes and risk factors
Chronic cor pulmonale develops due to an increase in pressure in the lungs, or pulmonary arteries. This is also referred to as pulmonary arterial hypertension. Damage to the lung tissue and pulmonary arteries causes the lungs to take in less oxygen-depleted blood from the right heart, causing it to back up.
This puts a lot of stress on the right ventricle, which is upstream from the lungs. It thickens its muscle wall, expands and, in the case of clinically manifest cor pulmonale, is only able to perform to a reduced degree. The cause is usually chronic lung disease, first and foremost COPD.
As COPD progresses, the smallest bronchi and alveoli become damaged: The partition walls between the alveoli are destroyed and large bubbles form from the tiny alveoli. This results in overinflation of the lungs, called pulmonary emphysema, which is another possible cause of cor pulmonale.
Diseases with increased formation of connective tissue in the lungs, called pulmonary fibrosis, are also possible triggers for cor pulmonale. This causes the lung tissue to lose elasticity, which in turn results in more difficult gas exchange. Examples of diseases associated with pulmonary fibrosis are sarcoidosis, tuberculosis, silicosis or asbestosis.
External respiratory obstruction also leads to cor pulmonale in some cases. An example is a spinal curvature (kyphoscoliosis), which constricts the lungs and increases the pressure in their blood vessels.
Causes of acute cor pulmonale
Due to the occlusion, the pressure in the still functioning pulmonary arteries rises sharply. The blood backs up to the right ventricle. At the same time, the flow of oxygen-rich blood to the left ventricle is so low that it no longer pumps enough blood into the systemic circulation. In a large (fulminant) pulmonary embolism, the circulation then often collapses completely. In the worst case, the heart stops working.
Other causes of acute cor pulmonale are a particularly severe asthma attack (status asthmaticus) or a so-called tension pneumothorax, i.e. collapse of a lung lobe when air enters the gap between the lung and the chest. Signs of this are severe shortness of breath, rapid heartbeat, sweating and agitation, and even fear of death.
Cor pulmonale: examinations and diagnosis
He will probably ask about cigarette use, cough and sputum condition, shortness of breath, recurrent respiratory infections, and exercise tolerance. He will also want to know if there is any known heart or lung disease.
Inspection and physical examination
Even an external examination of the patient (inspection) often provides the physician with initial indications of possible cor pulmonale. For example, those affected often show a bluish discoloration of the lips and fingertips. The end links of the fingers are sometimes distended into so-called drumstick fingers, and the fingernails are curved into “watch glass nails.” All of these are signs of a lack of oxygen in the body.
Water retention (edema) on the dorsum of the foot, the ankle joints and above the tibia bone are also possible indications of cor pulmonale.
By palpating the liver, the physician determines whether the organ is enlarged due to congestion. Venous congestion is often visible in the area of the upper body and neck. An important sign of water accumulation in the tissues is the formation of dents in response to external pressure. For example, the lower legs often show constrictions caused by the cuff of stockings or socks. After pressure with the finger, a visible dent remains for several minutes.
Indications from the laboratory
Laboratory blood tests also provide some typical clues to cor pulmonale. For example, the number of oxygen-transporting red blood cells (erythrocytes) is increased because the body is trying to compensate in this way for the poorer gas exchange in the lungs. Nevertheless, the oxygen content in the arterial blood is often lower than normal.
X-ray and other instrumental examinations
Diagnostic imaging provides further indications of cor pulmonale. For example, an X-ray of the chest often shows a widened right heart shadow as a result of right heart strain. By ultrasound examination of the heart (echocardiography), the physician precisely measures the enlargement of the right heart. Increased pressure in the pulmonary artery and leaking heart valves can also be detected here. An enlarged liver also becomes visible with the help of an ultrasound examination (sonography).
A further, fixed examination component for suspected cor pulmonale is the electrocardiogram (ECG). It shows the electrical excitation of the heart – the prerequisite for the heart muscle to contract in a coordinated manner. In cor pulmonale, the excessively dilated right ventricle causes typical changes in the conduction of excitation.
A cardiac catheter examination is more complex, but very precise. This allows the physician to precisely determine the pressures in the right heart and the large vessels and to compare them with each other. Typically, he advances the catheter from the inguinal vein to the heart. Via the great vena cava, the catheter reaches the right atrium and then via the right ventricle to the pulmonary artery.
If a pulmonary embolism (the most common cause of acute cor pulmonale) is suspected, the physician uses the cardiac catheter to inject a contrast medium into the pulmonary artery for special X-ray imaging. If the diagnosis is confirmed, in many cases the clot can be dissolved or disrupted (recanalization of the pulmonary artery) using special drugs or mechanically via the catheter.
Cor pulmonale: Treatment
Oxygen therapy, usually as a long-term treatment, significantly improves patients’ exercise tolerance and quality of life. Physical rest and draining drugs relieve the overstretched right ventricle.
Certain drugs also directly or indirectly reduce the pressure in the pulmonary arteries. For example, prostacyclins or endothelin receptor antagonists directly dilate the pulmonary arteries, while broncho-spasmolytics and expectorant drugs reduce hyperinflation of the lungs. This otherwise also increases resistance in the blood vessels.
Cortisone-type drugs (corticosteroids) are also used for severe inflammation of the airways. Severe bacterial infections usually require the use of an antibiotic.
Bloodletting is also used as a therapy in certain cases. The controlled loss of blood leads to a “thinning” of the blood in the body. This improves its flow properties and relieves the heart.
If the above-mentioned therapies are not sufficient to maintain the quality of life of the affected person, doctors may also consider lung or heart-lung transplantation.
Emergency therapy for acute cor pulmonale
Acute cor pulmonale is a medical emergency that requires rapid diagnosis and treatment. In addition to the absolutely necessary oxygen supply and sedative as well as pain-relieving medications, the doctors try to relieve the heart in the short term by means of fast-acting medications.
