Pulmonary heart disease (cor pulmonale)

In various lung diseases an obstruction to blood flow through the network of vessels in the lungs develops. This places a burden on the right side of the heart, which normally pumps against a low-pressure load with little resistance to blood flow. Pulmonary-artery pressures are normally low compared with those in the aorta.

Pulmonary heart disease may be divided into acute and chronic forms. The classic form of acute pulmonary heart disease (acute cor pulmonale) occurs when there is a sudden obstruction to the pulmonary blood-flow pattern, as occurs with a massive embolus—a blood clot that has broken loose from its point of formation. This impairs blood flow through the lungs, causes additional reflex changes that add to the heart’s burden, and creates an acute form of high blood pressure in the pulmonary artery, with dilation and failure of the right ventricle. The right ventricle’s pumping ability is acutely reduced, and, therefore, the amount of blood available for the left side of the heart is also restricted, so that systemic circulatory failure occurs.

Respiratory symptoms are not prominent, and the disorder in its early stages is not accompanied by edema (the accumulation of excess fluid) in the lung. The clinical picture in the more severe form is one of shock, with cold, pale, and clammy skin, low arterial pressure, and a high pulse rate. Oxygen transfer in the lungs is severely impaired, and the heart may be acutely dilated. Treatment is with anticoagulant drugs (such as streptokinase) and oxygen, which relieve the hypoxia (low serum oxygen levels), or, in some instances, surgical removal of the obstruction.

Chronic cor pulmonale may be caused by a form of pulmonary disease—such as chronic bronchitis or emphysema—in which lung tissue is destroyed and replaced with air spaces, causing a loss of pulmonary blood vessels, or it may be caused by multiple blood clots in the vessels of the lung or by a primary disorder of the pulmonary blood vessels. The result is a form of heart failure partly based on an obstruction to blood flow through the pulmonary vessels, producing high blood pressure in the pulmonary artery. Cyanosis (bluish discoloration of the skin) may be evident, indicating that the arterial blood is not saturated with oxygen. In patients with chronic bronchitis and emphysema, the lack of oxygen contributes to pulmonary hypertension. The manifestations of heart failure are present—particularly where there is edema—except that shortness of breath is often due to the underlying lung disease. The right side of the heart is enlarged, the valve sounds from the pulmonic valve may be loud, and there may be electrocardiographic evidence of chronic strain on the right side of the heart. Drugs that dilate the pulmonary blood vessels or relieve the edema and drugs with anticoagulant effects can be useful in the treatment of chronic pulmonary heart disease. However, the course that affords the best chance of improvement in patients with cor pulmonale due to chronic bronchitis and emphysema includes prompt treatment of infection, termination of smoking, and correction of the lack of oxygen.

Hypertensive heart disease

Arterial hypertension is a disease in which the regulation of blood pressure is abnormal, resulting in arterial pressure that is chronically higher than normal. Hypertension results from several causes, but the cause of the most common form (essential hypertension) is not understood. A family tendency to hypertension has been found in persons with the disease, and there may be a basic genetic abnormality involving the permeability of cell membrane in the blood vessels. This defect might make such persons less able to tolerate salt and in turn more responsive to hormonal or nervous stimulation.

Excessive dietary intake of salt has long been held to be responsible for hypertension in certain people. Stress has also been shown to cause hypertension, and fear and anxiety can induce a rise in blood pressure owing to increased activity in the sympathetic nervous system. Hormones and other vasoactive substances (substances that relax or contract the blood vessels) have a direct effect on blood pressure, but the interaction of these factors remains unclear. Hypertension also results from a number of types of chronic renal (kidney) diseases and from some tumours of the adrenal gland. In certain structural abnormalities of the aorta, such as coarctation, in which the artery’s middle coat is deformed with resultant narrowing of the channel, arterial pressure in the upper half of the body is abnormally high.

Regardless of the cause but in some ways coloured by it, the effects on the cardiovascular system are similar. The impact on the vascular system varies from person to person. In some persons, for unknown reasons, the body withstands the abnormal elevation of blood pressure with minimum change in the heart and blood vessels. In other persons, blood vessel damage is early and severe, coupled with serious deterioration of heart function. In general, the rule is that the higher the blood pressure, the higher the degree of cardiovascular damage, though there are many exceptions. Rarely, a vicious and damaging form of hypertension occurs, often called malignant hypertension, that results in damage to small blood vessels throughout the body but particularly affecting the heart, brain, and kidneys.

People with hypertensive disease have an increased susceptibility to atherosclerosis of the coronary arteries, thus making it difficult to separate the cardiac manifestations from those actually caused by hypertension. Hypertensive people, therefore, may eventually have congestive heart failure following enlargement of the heart caused by the chronic increase in arterial pressure. In addition they may suffer the effects of a decline in blood supply to the heart because of coronary artery disease and the classic manifestations of coronary arteriosclerosis, such as angina pectoris or myocardial infarction. Hypertensive cardiovascular disease may also become manifest through defects in the vessels supplying the brain, leading to stroke. Furthermore, hypertensive cardiovascular manifestations may be complicated by the development of kidney failure and the resultant abnormal retention of fluid in the tissues, adding to the problems of congestive heart failure.

Before the use of antihypertensive drugs, high blood pressure was associated with a greatly increased mortality, with survival measured in months in the most severe cases. Antihypertensive drugs have dramatically increased the life expectancy of patients with severe hypertension; stroke and kidney failure are now relatively uncommon in treated hypertensive patients. The reduction in coronary heart disease among this group of patients, however, has not been as substantial. Other factors, such as smoking and diet, are important in this aspect of therapy.

Other diseases of the aorta and the pulmonary artery

Arteriosclerosis may involve the aorta and its major branches. Indeed, it seems to be an almost inevitable process with increasing age, but the rate of development and the extent of involvement vary greatly. The process may merely limit the elasticity of the aorta and allow for some dilation and increased complexity of the path of the blood flow as age advances. In more severe instances, there may be a major degree of dilation or localized formation of aneurysms (bulging of the vessel wall at a point of weakness), generally in the abdominal portion of the aorta. These aneurysms may result in pain and may occasionally rupture, causing sudden death. The arteriosclerotic process may impair the flow of blood to the tributaries of the aorta and lead to a variety of ischemic states—i.e., result in various types of damage that come from an insufficient supply of blood. This condition is particularly notable when the renal vessels are involved, creating a state of renal ischemia, occasionally creating hypertension, and possibly terminating in renal failure.

Medial necrosis is a lesion of the aorta in which the media (the middle coat of the artery) deteriorates, and, in association with arteriosclerosis and often hypertension, it may lead to a dissecting aneurysm. In a dissecting aneurysm a rupture in the intima, the innermost coat of the artery, permits blood to enter the wall of the aorta, causing separation of the layers of the wall. Obstruction to tributaries may occur, which is usually associated with severe chest pain. In many instances there is a secondary rupture of the exterior wall, which may lead to fatal internal bleeding. The aortic wall may become inflamed as an isolated process.

Calcium salts that deposit in the aorta wall may occur as a part of the arteriosclerotic process or of other disease involvement. In certain conditions, such as congenital heart disease, blood clots (thrombi) may form in the pulmonary artery, and these may break loose. Blood clots in the lungs (pulmonary emboli) may arise from this and other sources in the systemic venous circulation. These fragments of clot may be small, causing no detectable manifestations, or large, causing obstruction of either the total pulmonary arterial flow or of flow to an area of lung.