In the developed world the cause is most commonly: (1 – 3). idiopathic, postsurgical, or radiation injury. Tuberculosis was the most common cause of constrictive pericarditis in the developed world before development of effective drug therapy. It remains important in developing countries. 1. Troughton RW, Asher CR, Klein AL: Pericarditis. Lancet 2004; 363:717. 2. Hoit BD: Management of effusive and constrictive pericardial heart disease. Circulation 2002; 105:2939. 3. Little WC, Freeman GL: Pericardial disease. Circulation 2006; 113:1622.
Although constriction can follow an initial insult by as little as several months, it usually takes years to develop. The end result is dense fibrosis, often calcification, and adhesions of the parietal and visceral pericardium. Usually scarring is more or less symmetrical and impedes filling of all heart chambers. In a subset of patients the process develops relatively rapidly and is reversible. This variant is seen most commonly following cardiac surgery. 1 1. Haley JH, Tajik AJ, Danielson GK, et al: Transient constrictive pericarditis: Causes and natural history. J Am Coll Cardiol 2004; 43:271.
The clinical presentation is usually dominated by signs and symptoms of right-heart failure. The pathophysiological consequence of pericardial scarring is markedly restricted filling of the heart. This results in elevation and equilibration of filling pressures in all chambers and the systemic and pulmonary veins. In early diastole the ventricles fill abnormally rapidly because of markedly elevated atrial pressures and accentuated early diastolic ventricular suction, the latter related to small end-systolic volumes. 1. Troughton RW, Asher CR, Klein AL: Pericarditis. Lancet 2004; 363:717. 2. Hoit BD: Management of effusive and constrictive pericardial heart disease. Circulation 2002; 105:2939. 3. Little WC, Freeman GL: Pericardial disease. Circulation 2006; 113:1622.
Systemic venous congestion results in hepatic congestion, peripheral edema, ascites, and sometimes anasarca and cardiac cirrhosis. Reduced cardiac output is a consequence of impaired ventricular filling and causes fatigue, muscle wasting, and weight loss. In “pure” constriction, contractile function is preserved, although ejection fraction can be reduced as a consequence of reduced preload. The myocardium is occasionally involved in the chronic inflammation and fibrosis, leading to true contractile dysfunction that can at times be quite severe. The latter predicts a poor response to pericardiectomy. Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
Schematic representation of transvalvular and central venous flow velocities in constrictivepericarditis. During inspiration the decrease in left ventricular filling results in a leftwardseptal shift, allowing augmented flow into the right ventricle. The opposite occurs duringexpiration. EA = mitral inflow; HV = hepatic vein; LA = left atrium; LV = left ventricle; PV =pulmonary venous flow; RA = right atrium; RV = right ventricle.
At a relatively early stage these signs and symptoms include lower extremity edema, vague abdominal complaints, and some degree of passive hepatic congestion. Signs and symptoms ascribable to elevated pulmonary venous pressures such as exertional dyspnea, cough, and orthopnea may also appear with progressive disease. Atrial fibrillation and tricuspid regurgitation, which further exacerbate venous pressure elevation, may also appear at this stage. In the end stage of constrictive pericarditis, the effects of a chronically low cardiac output are prominent including severe fatigue, muscle wasting, and cachexia. Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
El hallazgo semiológico más propio de la pericarditis constrictiva es el signo de Kussmaul o aumento de la presión venosa yugular (ingurgitación yugular) durante la inspiración, como consecuencia de un aumento del retorno venoso, secundario a la presión negativa intratorácica, pero con restricción al llene ventricular derecho y sin que haya un aumento simultáneo del gasto cardíaco. Dado que el corazón está recubierto por una coraza rígida, la distensibilidad de este se ve muy disminuida y cualquier aumento de volumen se transmite en forma retrógrada, es por eso que las yugulares se ingurgitan al inspirar (no como normalmente se esperaría: al inspirar, las yugulares colapsan) 1. Haley JH, Tajik AJ, Danielson GK, et al: Transient constrictive pericarditis: Causes and natural history. J Am Coll Cardiol 2004; 43:271.
Constrictive pericarditis Doppler schema of respirophasic changes in mitral and tricuspid inflow. Reciprocalpatterns of ventricular filling are assessed on pulsed Doppler examination of mitral (MV) and tricuspid (TV)inflow. (Courtesy of Bernard E. Bulwer, MD; with permission.)
No specific ECG findings exist. Nonspecific T wave abnormalities are often observed, as well as reduced voltage. Left atrial abnormality may also be present. Atrial fibrillation is present in significant numbers of patients. Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
The cardiac silhouette can be enlarged secondary to a coexisting pericardial effusion. Pericardial calcification is seen in a minority of patients and should raise the suspicion of tuberculous pericarditis, but calcification per se is not diagnostic of constrictive physiology. The lateral chest film is useful to detect pericardial calcification along the right heart border and in the atrioventricular groove. Isolated calcification of the LV apex or posterior wall suggests ventricular aneurysm rather than pericardial calcification. Pleural effusions are occasionally noted and can be a presenting sign of constrictive pericarditis. When left heart filling pressures are markedly elevated, pulmonary vascular congestion and redistribution can be present. Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
Chest radiograph showing marked pericardial calcificationsin a patient with constrictive pericarditis.
CT provides detailed images of the pericardium and is especially helpful in detecting even minute amounts of pericardial calcification. Its major disadvantage is the frequent need for iodinated contrast medium administration to best display findings of pericardial pathology. The thickness of the normal pericardium measured by CT is less than 2 mm. MRI provides a detailed and comprehensive examination of the pericardium without the need for iodinated contrast or ionizing radiation. It is somewhat less sensitive for detecting calcification than CT. 1. Wang ZJ, Reddy GP, Gotway MB, et al: CT and MR imaging of pericardial disease. Radiographics 2003; 23:S167-S180. 2. Oyama N, Oyama N, Komuro K, et al: Computed tomography and magnetic resonance imaging of the pericardium: Anatomy and pathology. Magn Res Med Sci 2004; 3:145.
Panel A. Echocardiographic transmitral flow pattern with exaggerated respiratory variation in inflow velocities (>25%). Panel B. Transaxial CT image (slice thickness 3.0 mm) of the heart at mid-ventricular level, demonstrating severe calcification of the pericardium (arrows). In addition, bilateral pleural effusion is seen. AO, descending aorta; PE, pleural effusion. Panel C. Three-dimensional volume rendering technique reconstruction of the whole chest. Nearly circumferential pericardial calcification is seen (structures of high CT density, such as bone and calcification, are rendered in white colour). Panel D. Three-dimensional volume rendering technique reconstruction of the heart. To exclude the non-calcified part of the heart, a threshold of 130 Hounsfield units (common threshold for coronary calcification in CT imaging) was chosen.
In mid-diastole, the thickened pericardium begins to restrict right ventricular filling, causing a rapid increase in ventricular pressure. Early changes of septal flattening and bowing of the interventricular septum toward the left ventricle (normally concave in shape toward the left ventricle during diastolic filling) are seen. This pressure change results in diastolic septal dysfunction, the septal bounce described in echocardiography. Frontiers in Bioscience 14, 2688-2703, January 1, 2009
M-mode and two-dimensional echocardiography findings include: Pericardial thickening, Abrupt displacement of the interventricular septum during early diastole (septal “bounce”), and Signs of systemic venous congestion such as dilation of hepatic veins and distention of the inferior vena cava with blunted respiratory fluctuation. Premature pulmonic valve opening as a result of elevated RV early diastolic pressure may also be observed. Exaggerated septal shifting during respiration is often present. 1 1. Maisch B, Seferovic PM, Ristic AD, et al: Guidelines on the diagnosis and management of pericardial diseases executive summary; the Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2004; 25:587.
Cardiac catheterization in patients with suspected constrictive pericarditis provides documentation of the hemodynamics of constrictive physiology and assists in discriminating between constrictive pericarditis and restrictive cardiomyopathy. Although there is limited need for contrast ventriculography, coronary angiography is used to detect occult coronary artery disease in those being considered for pericardiectomy. In addition, on rare occasions external pinching or compression of the coronary arteries or outflow tract regions by the constricting pericardium is detected.
Constriction RestrictionProminent y descent in venous pressure Present VariableParadoxical pulse ≈⅓ cases AbsentPericardial knock Present AbsentEqual right-left side filling pressures Present Left at least 3-5 mm Hg > rightFilling pressures >25 mm Hg Rare CommonPulmonary artery systolic pressure >60 No Commonmm Hg“Square root” sign Present VariableRespiratory variation in left-right Exaggerated Normalpressures/flowsVentricular wall thickness Normal Usually increasedAtrial size Possible LA enlargement Biatrial enlargementSeptal “bounce” Present AbsentTissue Doppler E′ velocity Increased ReducedPericardial thickness Increased Normal Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
Constrictive pericarditis is a progressive disease. With the exception of patients with transient constrictive pericarditis, surgical pericardiectomy is the only definitive treatment. Libby: Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.