Generalized Cardiomegaly

Examination of the shape of the heart on a chest x-ray can sometimes provide clues to the type of cardiac disease present. If the heart appears large in most dimensions, it is often difficult to tell whether multichamber enlargement, myocardiopathy, or pericardial effusion is present. If acute (within several days) and marked enlargement of the cardiac silhouette is seen, the most likely diagnosis is a pericardial effusion. Under these circumstances, the heart has a pendulous appearance and is much wider at the base. This is often referred to as a water bag appearance ( Fig. 5.3 ). Pericardial effusions must be greater than 250 mL to be detectable radiographically. Effusions are sometimes visible on CT scans of the chest, but if you suspect a pericardial effusion, the imaging procedure of choice is echocardiography. After penetrating trauma or surgery, air may be seen within the pericardium ( Fig. 5.4 ).

Pericardial Effusion.

(A) In a patient with a viral syndrome, a posteroanterior chest x-ray shows mild cardiomegaly with prominence of the left cardiac border. (B) One week later, a marked and sudden increase in the transverse diameter of the heart due to a pericardial effusion is apparent. A definitive diagnosis is best made by using cardiac ultrasound. (C) A pericardial effusion also can be seen on computed tomography scan as a fluid density (arrows) surrounding the heart.

Pneumopericardium.

On this chest x-ray, the pericardium (arrows) is outlined by air between it and the heart and air in the lungs. Note that the pericardium does not extend above the level of the pulmonary arteries. This helps distinguish pneumopericardium from pneumomediastinum.

Constrictive pericarditis is most commonly due to tuberculosis and viral and pyogenic infections. It also may occur after radiation therapy. Of patients with constrictive pericarditis, 90% will have pericardial calcification, which may be visible only on CT, and 60% will have a pleural effusion. Of those patients with pericardial calcification, 50% also will have constrictive pericarditis.

Cardiomegaly can be due to valvular disease, cardiomyopathy, congenital heart disease, pericardial effusion, or mass lesions. Cardiomyopathy and pericardial effusion both generally lead to symmetric enlargement, whereas valvular disease and congenital heart disease often have specific chamber enlargement. Dilated cardiomyopathy is caused by ineffective contraction during systole and most commonly results from an infection or a metabolic disorder. It also may be caused by collagen vascular disease or toxic agents such as alcohol and chemotherapeutic drugs. An example of the latter is doxorubicin (Adriamycin), one of the most widely used chemotherapeutic agents ( Fig. 5.5 ).

Cardiomyopathy.

In this case, cardiomyopathy is due to cancer chemotherapy with doxorubicin. (A) An initial chest x-ray demonstrates a normal-sized heart. (B) After several therapeutic courses of doxorubicin, marked enlargement in the cardiac silhouette is due to multichamber dilatation.

Use of the cardiothoracic ratio to assess heart size and the effect of cardiac failure on the appearance of the pulmonary vessels and lungs was discussed in Chapter 3 . The width of the heart should not exceed half the width of the chest at its widest point. This measurement is reliable only on an upright posteroanterior (PA) chest image; on an anteroposterior (AP) chest x-ray, the heart will often exceed this measurement, owing to magnification. On a supine image, there is even more magnification, and high position of the hemidiaphragms occurs. This high position will push the heart upward and outward, making it appear wide. A note of caution should be inserted here about patients who have chronic obstructive pulmonary disease (COPD). The shape of the heart is determined by external forces and internal factors. One factor relates to the level of the hemidiaphragms. The measurement of cardiothoracic ratio assumes that the hemidiaphragms are in normal position. With COPD, the hemidiaphragms are driven inferiorly (often to the level of the posterior 12th rib). The heart then sags and elongates. This can make an enlarged heart appear normal in size, especially considering the cardiothoracic ratio. It follows, then, that if the heart appears too wide in a patient who has COPD, it is really very large.

Because assessment of cardiac function by chest x-ray is rather poor, quantitative evaluations of cardiac ejection fraction are usually made by nuclear medicine multiple gated acquisition (MUGA) scanning, gated single-photon emission CT (SPECT) studies, or echocardiography. In the nuclear medicine MUGA procedure, red cells are labeled with radioactive material, and images of the heart are obtained in a gated fashion. Computer analysis enables construction of a time-activity curve showing the amount of activity in the left or right ventricle in both diastole and systole, allowing for the calculation of an ejection fraction. The normal left ventricular ejection fraction (LVEF) is between 55% and 75%. In older persons, the lower limit of LVEF is probably about 50%. Both nuclear medicine gated SPECT ( Fig. 5.6 ) and echocardiography produce visualization of the myocardium. The ventricular cavity is then measured at various portions of the cardiac cycle, and the ejection fraction is calculated. All these studies also can detect regional wall motion abnormalities.

Measurement of the Cardiac Ejection Fraction and Regional Wall Motion.

A nuclear medicine technique in which the myocardium is visualized allows the computer to construct a view of the heart and, by inference, the cavity of the left ventricle. When the data are acquired in conjunction with an electrocardiogram, an image can be derived that shows the outline of the ventricular cavity at diastole (mesh outline) and at systole (gray center) . In this patient, poor wall motion of the distal anterior wall and apex is seen (arrows) .

Left Atrial Enlargement

Isolated left atrial enlargement occurs most commonly in mitral stenosis. The earliest sign is displacement of the esophagus posteriorly ( Fig. 5.7A ). Enlargement of the left atrial appendage on the PA view is the next sign to appear (see Fig. 5.7B ). As the left atrium enlarges further, the esophagus becomes more posteriorly displaced, splaying or widening of the inferior carinal angle is seen, and the enlarged left atrium can be seen on the PA x-ray as a double density behind the heart and below the carina (see Fig. 5.7C ). The normal inferior carinal angle should not exceed 75 degrees.

Mitral Stenosis.

(A) One of the earliest signs of mitral stenosis is left atrial (LA) enlargement, which can be seen on the lateral view of the chest. The left atrium can be seen displacing the barium-filled esophagus posteriorly. (B) A somewhat later finding on a posteroanterior chest x-ray is the four-bump or so-called ski mogul heart. The four bumps are created by the aorta (Ao) , pulmonary artery (PA) , left atrial appendage (LAA) , and left ventricle (LV) . The LA appendage normally does not bulge out. (C) Very late findings of LA enlargement on the posteroanterior chest x-ray are a double density behind the heart (arrows) and splaying of the subcarinal angle, which normally does not measure more than 75 degrees.

Rheumatic heart disease most often affects the mitral valve and, to a lesser extent, the aortic valve. The classic appearance of a mitral heart on a PA chest x-ray is easily recognized by four bumps along the left cardiac border. This also is sometimes called the ski mogul heart . Going from superior to inferior, the bumps represent the aortic arch, pulmonary artery, left atrial appendage, and left ventricle (see Fig. 5.7B ). Left atrial enlargement also is seen with congenital cardiac lesions that have intracardiac shunts, as well as in patients who have left ventricular failure.

As rheumatic heart disease progresses, mitral stenosis and mitral insufficiency develop. The heart becomes very large because of dilatation and hypertrophy of the left ventricle. In the combined form of mitral disease, the left atrium becomes even larger than what is seen in mitral stenosis alone. Typical findings are a straightening of the left cardiac border due to left atrial enlargement, left ventricular enlargement, as evidenced by leftward and downward displacement of the cardiac apex and, if left ventricular dilatation becomes massive, rightward displacement of the right ventricle. On the lateral view, in addition to the obvious left atrial enlargement, the posterior displacement of the heart continues down inferiorly, indicating left ventricular enlargement as well. In severe mitral disease, the mitral valve and aortic and tricuspid valves are affected. In general, the manifestations of the more proximal valve lesion are the most prominent. With mitral and tricuspid disease, enlargement of the left atrium, left ventricle, right atrium, and right ventricle occurs.

Many radiologists say that you cannot differentiate right atrial from right ventricular enlargement on a chest x-ray, and this is probably true. Fortunately, in most adults, when one of the right chambers is enlarged, so is the other. On the frontal chest x-ray, right atrial enlargement is suggested by an increased convexity of the right heart border. Isolated right ventricular enlargement is very difficult to appreciate because it overlaps the right atrium and left ventricle on the frontal view. On the lateral view, both right ventricular and right atrial enlargement will cause a filling in of the anterior clear space behind the sternum ( Fig. 5.8 ). Normally, on the lateral view, the anterior portion of the heart fills in only approximately one-third or less of the anterior clear space unless enlargement of the right atrium or right ventricle is found.

Mitral and Tricuspid Insufficiency.

As a late finding in rheumatic heart disease, mitral and sometimes tricuspid insufficiency develop. (A) On the posteroanterior chest x-ray, there is marked enlargement of not only the left atrium (LA) but also the left ventricle (LV ; seen as straightening of the left cardiac border), as well as right-sided enlargement, particularly of the right atrium (RA ; seen by marked prominence of the right cardiac border). (B) On the lateral view of the chest, the LV can be seen overlapping the spine, and the RA and right ventricle (RV) have filled in the retrosternal space to more than the usual lower one-third.

Prosthetic tricuspid and mitral valves are often used for the treatment of rheumatic heart disease. You should be able to recognize these valves by their size, location, and orientation ( Fig. 5.9 ). The valve with the largest area is the tricuspid valve, the mitral valve is intermediate in size, and the smallest is the aortic valve. You also should be able to recognize prosthetic mitral and tricuspid valves. As expected, the mitral valve is located posteriorly and to the left in the heart, and the tricuspid valve is anterior and toward the right side.

Prosthetic Mitral and Tricuspid Valves.

(A) Both the mitral (M) and tricuspid (T) valves were difficult to appreciate on the posteroanterior view of the chest and were therefore drawn in. (B) They are easily seen on the lateral view in the expected regions between the left atrium (LA) and left ventricle (LV) , and the right atrium (RA) and right ventricle (RV) have filled in the retrosternal space to more than the usual lower third. Also note that a cardiac pacer comes down the superior vena cava through the right atrium and into the right ventricle. These two valves are normally relatively large because of relatively low pressure gradients across them.

Left Ventricular Enlargement

On a frontal chest x-ray, left ventricular enlargement, as already mentioned, produces a round left cardiac border as well as downward displacement of the apex. On the lateral view, the posterior aspect of the heart, where it intersects the hemidiaphragm, is usually posteriorly displaced behind the inferior vena cava. The Hoffman-Rigler sign also can be used. To use this, find the intersection of the inferior vena cava with the hemidiaphragm on the lateral image, and then measure 2 cm up and 2 cm back. If the heart projects posteriorly, left ventricular enlargement probably is present. A note of caution should be inserted here because an enlarged right heart can sometimes push the left ventricle back. To exclude this, look at the space behind the sternum: only the lower one-third should be filled by soft tissue.

Left ventricular dilatation can be due to a number of causes, including coronary artery disease, aortic stenosis, and aortic regurgitation. You should take care to consider the possibility of a left ventricular aneurysm before you suggest left ventricular enlargement and quit. Ventricular aneurysms most commonly occur near the apex and anteriorly and have a high rate of mortality. Left ventricular hypertrophy is difficult to detect radiographically. It may be present in patients who have a normal cardiac configuration on chest x-ray. If this is suspected, an echocardiogram (ultrasound) is the test of choice.

Aortic Stenosis and Insufficiency

Aortic stenosis is most commonly valvular, although in a smaller number of patients, it may be either subvalvular or supravalvular. Valvular stenosis can be due to rheumatic heart disease, a bicuspid (rather than tricuspid) aortic valve, or degenerative changes (usually in patients older than 70 years). It may be difficult to detect this condition from findings on a plain x-ray of the chest, and sometimes the only finding is a calcified aortic valve. Initially, with aortic stenosis, left ventricular hypertrophy is noted. The heart will be normal in size and may show a slight rounding of the cardiac apex. When left ventricular dilatation occurs, the left cardiac border elongates and the apex of the heart moves downward toward the left hemidiaphragm. The aortic knob will be normal in size, although the ascending aortic arch is enlarged, causing a convexity of the right upper cardiac margin. The enlargement of the ascending aorta is due to poststenotic dilatation.

With aortic insufficiency, the left ventricle becomes much larger and, on a PA chest x-ray, the apex of the heart may project below the most superior portion of the left hemidiaphragm. The ascending aorta still shows some enlargement ( Fig. 5.10 ). Prosthetic aortic valves are relatively easy to recognize by their relatively small size and their location at the root of the ascending aorta ( Fig. 5.11 ).

Aortic Insufficiency.

A prominent left ventricle (LV) is seen on both the posteroanterior view (A) and lateral view (B). In addition, a convexity appears in the region of the ascending aorta (arrows) because of poststenotic dilatation.

Prosthetic Aortic Valve.

(A) The prosthetic valve was not easily visible on the posteroanterior view and was therefore drawn in. (B) On the lateral view, the valve is easily seen in the expected region between the left ventricle (LV) and the ascending aorta (Ao) . Also note its relatively small size. The arrows indicate the direction of blood flow.

RV, Right ventricle.

Pulmonary Artery Enlargement

Enlargement of the pulmonary artery is fairly easy to recognize on the PA chest x-ray by a bulging along the left cardiac border, just below the aortic arch ( Fig. 5.12 ). Pulmonary artery enlargement can be due to a number of causes, but probably the three most common are pulmonic stenosis (with poststenotic dilatation), pulmonary artery hypertension, and abnormalities in which increased flow through the pulmonary artery occurs, such as a patent ductus arteriosus or an atrial septal defect (ASD).

Progressive Pulmonary Arterial Hypertension.

(A) This patient initially was seen with a relatively normal chest x-ray. (B) However, several years later, increasing heart size, as well as marked dilatation of the main pulmonary artery (MPA) and right pulmonary artery (RPA) , are noted. Rapid tapering of the arteries as they proceed peripherally is suggestive of pulmonary hypertension and is sometimes referred to as pruning .

If enlargement of both the left and right main pulmonary arteries is present, consider the diagnosis of pulmonary arterial hypertension. In this entity, in addition to the very large central pulmonary arteries, rapid “pruning” of the vessels occurs as they proceed peripherally in the lung. Even though the central vessels are extremely large, it is unusual to be able to see vessels at the edge of the lung. The chest x-ray is relatively insensitive for detecting mild or moderate pulmonary hypertension, but it is still the recommended initial recommended imaging test. Pulmonary hypertension is a complex disorder and may be idiopathic or due to a number of causes, including ASD or ventricular septal defect (VSD), patent ductus arteriosus, arteriovenous shunt, left ventricular failure, mitral valve disease, pulmonary emboli, parenchymal lung disease, COPD, and other less common entities. Exertional dyspnea is the most frequent presenting symptom but also may be accompanied by syncope, fatigue, weakness, angina, and peripheral edema.