What Is Pulmonary heart Disease

What Is Pulmonary Heart Disease: The Heart-Lung Interaction And Its Impact On Patient Phenotypes

Cor pulmonale is a heart disease that occurs due to a dysfunction of the lungs (or of the arteries that connect the heart to the lungs) and which causes an alteration of the anatomical structure of the right ventricle. The muscular wall of the latter, in fact, relaxes (therefore the ventricular cavity widens) and/or thickens (right ventricular hypertrophy), compromising its pumping action of deoxygenated blood towards the lungs.
The cause of cor pulmonale is pulmonary hypertension , or high pressure in the pulmonary arteries .
Typical symptoms and signs of cor pulmonale are: dyspnea , chest pain , cyanosis and leg edema .
The diagnosis must also focus on the causes of cor pulmonale, as their identification allows the therapy to be better planned.

Brief Reminder Of The Anatomy Of The Heart

Before describing the disease, it is useful to review some fundamental characteristics of the heart.
With the help of the image, readers are reminded that:

  • The heart is divided into two halves, right and left. The right heart is composed of the right atrium and the underlying right ventricle . The left heart is composed of the left atrium and the underlying left ventricle . Each atrium is connected to the ventricle below it by a valve .
  • The right atrium receives non-oxygenated blood , via the vena cava .
  • The right ventricle pumps blood into the pulmonary arteries , which lead to the lungs. In the lungs, the blood is loaded with oxygen.
  • The left atrium receives oxygenated blood returning from the lungs through the pulmonary veins .
  • The left ventricle pumps oxygenated blood to the organs and tissues of the human body , through the aorta .
  • Each ventricle communicates with its efferent vessel by means of a valve. Therefore, there are four valves in the heart in total.

Figure: anatomy and blood circulation within the heart. The right cavities of the heart (including the vessels that reach and depart from it) are colored blue, while the left cavities (also in this case, including vessels) are highlighted in red.

What Is Cor Pulmonale?

Cor pulmonale is a very serious cardiac pathology, which arises as a result of a dysfunction of the lungs or pulmonary arteries, characterized by an enlargement of the right ventricular cavity and/or a thickening of the myocardium which constitutes the right ventricle ( right ventricular hypertrophy ) .
In other words, cor pulmonale can be defined as an anatomical alteration of the right ventricle, due to a persistent problem in the lungs or in the arteries that conduct blood to oxygenate in the lungs.

Myocardium and meaning of right ventricular hypertrophy
The myocardium is the muscle of the heart. Therefore, the right ventricular hypertrophy that characterizes cor pulmonale is a form of muscle hypertrophy .
Muscle hypertrophy is generally defined as “the increase in muscle volume caused by the increase in volume of the elements that constitute the muscle (therefore fibres , myofibrils , connective tissue , sarcomeres , contractile proteins etc.)”.


Morphological alterations of the heart in response to pulmonary hypertension. From the site mbbsdost.com.

Since, following cor pulmonale, the oxygenation of the blood in the lungs is insufficient, all the tissues of the body undergo a state of hypoxia (i.e. lack of oxygen).


Doctors distinguish two forms of cor pulmonale: acute cor pulmonale and chronic cor pulmonale .
The acute form is usually characterized by an enlargement of the ventricular cavity, while the chronic form is mainly characterized by a thickening of the myocardium.
Sometimes, however, it may happen that cor pulmonale is first characterized by an enlargement of the right ventricle and then by its thickening.


Although the structural changes are the same, right ventricular hypertrophy that arises as a result of a problem in the left department of the heart (for example in the case of hypertrophic cardiomyopathy or an aortic valve defect ) is not considered cor pulmonale.


The cause of cor pulmonale is pulmonary hypertension , which is an increase in blood pressure within the pulmonary arteries and right chambers of the heart.


Generally, pulmonary hypertension occurs when the walls of the pulmonary arteries undergo an alteration, as a result of which they narrow and become completely occluded. This has two consequences: on the one hand, it increases the resistance that the blood encounters going from the heart to the lungs and, on the other, the pumping action of the right ventricle is hindered.

Figure: comparison section between the pulmonary arteries of a healthy subject and the pulmonary arteries of a subject with pulmonary hypertension. From www.cdc.gov

In other words, the origin of pulmonary hypertension is almost always a vasoconstriction of the arteries that conduct blood from the heart to the lungs; this vasoconstriction reduces blood flow to the lungs and prevents the right ventricle from functioning normally.
The conditions that can lead to the onset of pulmonary hypertension are numerous and mainly consist of:

Causes of pulmonary hypertension that can give acute cor pulmonale Causes of pulmonary hypertension that can give chronic cor pulmonale
  • Pulmonary embolism and, more generally, chronic venous thromboembolism
  • Respiratory distress syndrome
  • Chronic obstructive pulmonary disease (COPD)
  • Interstitial lung disease
  • Sleep apnea syndrome
  • Cystic fibrosis
  • Sarcoidosis
  • Chronic hemolytic anemia (NB: among the variants of this type of disease there is also sickle cell anemia )
  • Polycythemia
  • Pulmonary emphysema
  • Pulmonary fibrosis
  • Idiopathic pulmonary hypertension (NB: in medicine, a disease or condition is said to be idiopathic when it occurs without an identifiable reason)
  • Pulmonary veno-occlusive disease
  • Congenital malformations of the heart
  • Connective tissue diseases (e.g. scleroderma , systemic lupus erythematosus , or rheumatoid arthritis )
  • Persistent asthma
  • Pneumoconiosis ( silicosis )
  • Pierre Robin syndrome

Since cor pulmonale generally results from pulmonary hypertension, all the causes of the latter are also considered triggers of cor pulmonale.


Currently, there is no statistical data that reports the exact annual incidence of cor pulmonale. However, according to doctors and researchers, the acute form is mainly linked to pulmonary embolism, while the chronic form has a particular link with chronic obstructive pulmonary disease.

An interesting numerical fact

In the United States, cor pulmonale deaths due to pulmonary embolism are estimated to be between 20,000 and 25,000 per year.

For a reason still unknown, the majority of patients with cor pulmonale are female.
Furthermore, the following are certainly more at risk of developing this condition: heavy smokers , those who live in particularly polluted cities, workers in industrial plants where toxic dust and fumes are produced, etc. (in other words, all those who are exposed to harmful agents for the lungs).

Symptoms And Complications

For further information: Heart Pulmonary Symptoms

At the beginning, cor pulmonale could be completely asymptomatic (i.e. without obvious manifestations) or be characterized by mild and not always perceptible disorders.
In the more advanced stages, however, the disease is characterized by marked and very evident symptoms.
The main symptoms and signs of cor pulmonale are:

  • Dyspnea (or shortness of breath ), both under exertion ( exertional dyspnea ) and at rest ( rest dyspnea )
  • Sense of fatigue even when doing the simplest activities
  • Dizziness
  • Sense of faintness and syncope
  • Pain or feeling of pressure in the chest
  • Edema of the legs , hips and sometimes even the abdomen ( ascites )
  • Increased heart rate ( tachycardia )
  • Jugular vein distention (or jugular vein turgidity )
  • Murmurs and other abnormal heart “noises”.
  • Persistent cough


Feeling unusual dyspnea during even minimal effort should lead those who suffer from it to immediately contact their doctor for an in-depth analysis of the situation.
Furthermore, the appearance of chest pain, edema in the legs (or other parts of the body) and cyanosis also deserves further investigation.


The vasoconstriction of the pulmonary arteries could cause a reflux of the blood present in the right heart towards the venous system (from which it had previously arrived).
Among the various veins that can be affected by this reflux, there is also the hepatic vein , i.e. the blood vessel that normally collects the blood that has just oxygenated the liver cells ( hepatocytes ).
The permanence of deoxygenated blood in the hepatic vein (and in the small adjacent hepatic venules ) leads to the establishment of the so-called hepatic congestion (or congestion of the liver).
One of the consequences of liver congestion is the condition known as liver stasis .


Cor pulmonale is difficult to diagnose, basically for two reasons:

  • It may be asymptomatic and go unnoticed
  • The symptoms and signs that it can cause are very similar to those of other heart and lung diseases.

In light of this, in order to identify its presence, the doctor is required to subject the patient to various examinations and diagnostic tests.
Usually, after a thorough objective examination, the following are followed:

  • Blood tests
  • Echocardiogram
  • CT scan (or computed tomography )
  • Nuclear magnetic resonance
  • Right heart catheterization
  • Chest X-ray
  • Lung perfusion analysis
  • Spirometry
  • Lung biopsy

The considerable number of diagnostic tests carried out serve not only to establish the precise cardiac problem in progress, but also to understand its causes. Knowledge of the triggering reasons allows you to plan an adequate treatment.


Cor pulmonale therapy depends on the causes of pulmonary hypertension.


The first treatment that doctors resort to in case of pulmonary hypertension is pharmacological. Usually, in fact, a combination of different medicines is envisaged, which are chosen depending on the causes triggering the vasoconstriction of the pulmonary arteries.
In other words, the drug combination varies from case to case, based on the characteristics of pulmonary hypertension.
If the medicines provide poor results or the situation is particularly serious, then surgery will become necessary. In particular, the interventions that can be used are atrial septostomy , pulmonary embolectomy (in the presence of pulmonary embolism), phlebotomy (in the presence of polycythemia) and heart , lung or both transplants . Oxygen therapy
has excellent effects on some patients (for example COPD patients). Oxygen therapy consists of administering oxygen and allows the state of hypoxia to be resolved, at least in part.

Warning : To know, in detail, all the possible medicines for pulmonary hypertension, the reader is advised to consult Medicines to treat hypertension . Here, we only remember that the pharmacological treatments available today for the treatment of cor pulmonale do not allow recovery from the disease, but are limited only to improving the symptoms and avoiding a worsening of the situation.


If you suffer from cor pulmonale, you should follow certain rules, the most important of which are:

  • Stay at rest when you feel the need.
  • Practice regular physical activity . Obviously, this activity must be commensurate with the state of health, as excessive efforts could be fatal.
  • Not smoking
  • If you are a woman, avoid getting pregnant , because pregnancy could make the situation worse. Furthermore, the life of the fetus would also be in danger .
  • Adopt a healthy and possibly low-salt diet ( low-sodium diet ).


Cor pulmonale is a heart disease that is difficult to treat, therefore the prognosis is generally negative.
Two factors affect in particular the life expectancy of patients:

  • The severity of the triggers. The more severe the condition that causes cor pulmonale, the worse the course of the disease.
  • The moment in which the diagnosis occurs. When diagnosed late, treatments for cor pulmonale may be ineffective.

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