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265 - Pericardial Diseases
265 - Pericardial Diseases
265 - Pericardial Diseases
Gilboa SM et al: Congenital Heart Defects in the United States: Clinical Classification
Estimating the magnitude of the affected population in 2010.
I. Acute pericarditis (<6 weeks)
Circulation 134:101, 2016.
Gurvitz M et al: Emerging Research Directions in Adult Congenital A. Fibrinous
ST
PART 6
PR
II aVL V2 V5
Disorders of the Cardiovascular System
III aVF V3 V6
I aVR V1 V4
II aVL V2 V5
III aVF V3 V6
II
B
FIGURE 265-1 A. Acute pericarditis. There are diffuse ST-segment elevations in leads I, II, aVF, and V2–V6). There is PR-segment depression due to a concomitant atrial
injury current. B. Electrical alternans. This tracing was obtained from a patient with a large pericardial effusion with cardiac tamponade.
and V2–V6, with reciprocal depressions only in aVR and sometimes Diagnosis Echocardiography (Chap. 236) is the most widely used
V1. Also, there is depression of the PR segment below the TP seg- imaging technique. It is sensitive, specific, simple, noninvasive, may
ment, reflecting atrial involvement. Usually there are no significant be performed at the bedside, and allows localization and estimation
changes in QRS complexes, unless a large pericardial effusion of the quantity of pericardial fluid. The presence of pericardial fluid
develops (see below). After several days, the ST segments return is recorded by two-dimensional transthoracic echocardiography as a
to normal (stage 2), and only then, or even later, do the T waves relatively echo-free space between the posterior pericardium and left
become inverted (stage 3). Weeks or months after the onset of acute ventricular epicardium and/or as a space between the anterior right
pericarditis, the ECG returns to normal (stage 4). In contrast, in ventricle and the parietal pericardium just beneath the anterior chest
AMI, ST elevations are upwardly convex, and reciprocal depression wall (Fig. 265-2).
is usually more prominent; these changes may return to normal The diagnosis of pericardial fluid or thickening may be confirmed
within a day or two. Q waves may develop, with loss of R-wave by computed tomography (CT) or magnetic resonance imaging (MRI).
amplitude, and T-wave inversions; these changes are usually These techniques may be superior to echocardiography in detecting
seen within hours before the ST segments have become isoelectric loculated pericardial effusions, pericardial thickening, and the identifi-
(Chaps. 268 and 269). cation of pericardial masses. MRI is also helpful in detecting pericardial
4. Pericardial effusion is usually associated with pain and/or the ECG inflammation (Fig. 265-3).
changes mentioned above, and if the effusion is large with electrical
alternans (Fig. 265-1B). Pericardial effusion is especially important
clinically when it develops within a relatively short time because TREATMENT
it may lead to cardiac tamponade (see below). Differentiation from Acute Pericarditis
cardiac enlargement on physical examination may be difficult, but
heart sounds may be fainter with large pericardial effusion. The There is no specific therapy for acute idiopathic pericarditis, but bed
friction rub and the apex impulse may disappear. The base of the rest and anti-inflammatory treatment with aspirin (2–4 g/d), with
left lung may be compressed by pericardial fluid, producing Ewart’s nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibupro-
sign, a patch of dullness and increased fremitus beneath the angle of fen (600–800 mg tid) or indomethacin (25–50 mg tid), and should
the left scapula. The chest roentgenogram may show enlargement of be administered along with gastric protection (e.g., omeprazole
the cardiac silhouette, with a “water bottle” configuration, but may 20 mg/d). In responsive patients, these doses should be continued
be normal in patients with small effusions. for 1–2 weeks and then tapered over several weeks. In addition,
AO
LA
RV
LV
LV
* *
*
A B
FIGURE 265-3 Pericardial inflammation by cardiac magnetic resonance imaging. A. Short axis view. The pericardium is thickened and enhanced on T2 magnetic
images. Note thickened white line denoted by arrow. B. Long axis view. Late gadolinium enhancement of thickened, inflamed pericardium. AO, aorta; LA, left atrium;
LV, left ventricle; RV, right ventricle. (From RY Kwong: Cardiovascular magnetic resonance imaging, in Braunwald’s Heart Disease, 10th ed, Mann DL et al [eds]. Philadelphia:
Elsevier, 2015, pp 320–40.)
whereas pulmonic vein, mitral, and aortic flow velocities diminish (as injury. In developing nations, tuberculosis, often associated with
in constrictive pericarditis, see below) (Fig. 265-4). In tamponade, there HIV infection, may also cause exudative and/or bloody effusion.
is late diastolic inward motion (collapse) of the right ventricular free The pericardial fluid should be analyzed for red and white blood
wall and the right atrium. Transesophageal echocardiography, CT, or cells and cytology for neoplastic cells. Cultures should be obtained.
cardiac MRI may be necessary to diagnose a loculated effusion respon- The presence of DNA of Mycobacterium tuberculosis determined by
sible for cardiac tamponade. the polymerase chain reaction strongly supports the diagnosis of
tuberculous pericarditis (Chap. 173).
TREATMENT
Cardiac Tamponade ■■VIRAL OR IDIOPATHIC ACUTE PERICARDITIS
In many instances, acute pericarditis occurs in association with or
Patients with acute pericarditis should be observed frequently for following illnesses of known or presumed viral origin and probably
the development of an effusion. If a large effusion is present, pericar- is caused by the same agent. There may be an antecedent infection
diocentesis should be carried out or the patient watched closely for
signs of tamponade with serial echocardiography and monitoring of
Inspiration Expiration
arterial and venous pressures.
E Septum Septum
PERICARDIOCENTESIS A E
A
If manifestations of tamponade appear, pericardiocentesis using an
apical, parasternal, or, most commonly, subxiphoid approach must TV MV TV MV
be carried out at once because if left untreated, tamponade may be RV LV
Doppler
rapidly fatal. Whenever possible, this procedure should be carried transvalvular
out under echocardiographic guidance. Intravenous saline may be inflow patterns
administered as the patient is being readied for the procedure, but Thickened
the pericardiocentesis must not be delayed. If possible, intrapericar- pericardium
dial pressure should be measured before fluid is withdrawn, and RA
Pulmonary
the pericardial cavity should be drained as completely as possible. LA vein
A small, multiholed catheter may be advanced over the needle
inserted into the pericardial cavity and left in place to allow drain- DIASTOLE DIASTOLE
ing of the pericardial space if fluid reaccumulates. Surgical drainage IVC and hepatic veins
through a limited (subxiphoid) thoracotomy may be required in Apical 4-chamber views
recurrent tamponade to remove loculated effusions, and/or when it
FIGURE 265-4 Constrictive pericarditis. Doppler schema of respirophasic
is necessary to obtain tissue for diagnosis.
changes in mitral and tricuspid inflow. Reciprocal patterns of ventricular filling
Pericardial fluid obtained from an effusion may have the physical are assessed on pulsed Doppler examination of mitral valve (MV) and tricuspid
characteristics of an exudate. In developed nations, bloody fluid valve (TV) inflow. IVC, inferior vena cava; LA, left atrium; LV, left ventricle; RA, right
is most commonly due to neoplasm, renal failure, or after cardiac atrium; RV, right ventricle. (Courtesy of Bernard E. Bulwer, MD; with permission.)
cess may extend into the myocardium and cause myocardial scarring monary congestion, a heart that is not enlarged, and a paradoxical
and atrophy, and venous congestion may then be due to the combined pulse. However, in cor pulmonale, advanced parenchymal pulmonary
effects of the pericardial and myocardial lesions. disease is usually apparent and venous pressure falls during inspira-
In constrictive pericarditis, the right and left atrial pressure pulses tion (i.e., Kussmaul’s sign is negative). Tricuspid stenosis (Chap. 261)
may also simulate chronic constrictive pericarditis with congestive
Disorders of the Cardiovascular System
266 Atrial
tumor in adults, accounting for one-third to one-half of all cases at post-
Myxoma and Other mortem examination, and approximately three-quarters of the tumors
Cardiac Tumors treated surgically. They occur at all ages, most commonly in the third
through sixth decades, with a female predilection. Approximately 90%
Eric H. Awtry of myxomas are sporadic; the remainder are familial with autosomal
dominant transmission. The familial variety often occurs as part of a
syndrome complex (Carney complex) that includes (1) myxomas (car-
diac, skin, and/or breast), (2) lentigines and/or pigmented nevi, and
Cardiac tumors can be broadly classified into those that arise primarily (3) endocrine overactivity (primary nodular adrenal cortical disease
in the heart and those that reflect metastatic disease from a distant pri- with or without Cushing’s syndrome, testicular tumors, and/or pitu-
mary source. Primary cardiac tumors can be further divided into those itary adenomas with gigantism or acromegaly). Certain constellations
that are pathologically benign and those that are malignant. Overall, of findings have been referred to as the NAME syndrome (nevi, atrial
primary cardiac tumors are relatively uncommon, whereas secondary myxoma, myxoid neurofibroma, and ephelides) or the LAMB syndrome
involvement of the heart or pericardium occurs in as many as 20% of (lentigines, atrial myxoma, and blue nevi), although these syndromes
patients with end-stage metastatic cancer. While patients with cardiac probably represent subsets of the Carney complex. The genetic basis of
tumors may present with a variety of symptoms, many patients are this complex has not been elucidated completely; however, inactivating
asymptomatic at the time of diagnosis as the tumor may be identified mutations in the tumor-suppressor gene PRKAR1A, which encodes the
incidentally on imaging studies performed for other reasons. Such protein kinase A type I-α regulatory subunit, have been identified in
findings need to be differentiated from other cardiac masses such as ~70% of patients with Carney complex.
vegetation, thrombus, or myocardial hypertrophy. Echocardiography Pathologically, myxomas are gelatinous structures that consist of
is usually the initial method of evaluation of cardiac tumors; however, myxoma cells embedded in a stroma rich in glycosaminoglycans.
a variety of imaging modalities are now available and a multimodality Most sporadic tumors are solitary, arise from the interatrial septum
approach is often necessary for accurate diagnosis and clarification of in the vicinity of the fossa ovalis (particularly in the left atrium), and
treatment options (Table 266-1). are often pedunculated on a fibrovascular stalk. In contrast, familial
■■PRIMARY TUMORS or syndromic tumors tend to occur in younger individuals, are often
Primary tumors of the heart are rare. Approximately three-quarters are multiple, may be ventricular in location, and are more likely to recur
histologically benign, and the majority of these tumors are myxomas. after initial resection.
Malignant tumors, almost all of which are sarcomas, account for 25% Myxomas commonly present with obstructive signs and symptoms.
of primary cardiac tumors. All cardiac tumors, regardless of pathologic The most common clinical presentation mimics that of mitral valve dis-
type, have the potential to cause life-threatening complications. Many ease: either stenosis owing to tumor prolapse into the mitral orifice or
tumors are now surgically curable; thus, early diagnosis is imperative. regurgitation resulting from tumor-induced valvular trauma or distor-
tion. Ventricular myxomas may cause outflow tract obstruction similar
Clinical Presentation Cardiac tumors may present with a wide to that caused by subaortic or subpulmonic stenosis. The symptoms
array of cardiac and noncardiac manifestations. These manifestations, and signs of myxoma may be sudden in onset or positional in nature,