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A 61-Year-Old Man With Shortness of Breath, Ascites, and Lower Extremity Edema FREE TO VIEW

Ali Ataya, MD; Jessica M. Cope, PharmD; Diego Moguillansky, MD; Tiago N. Machuca, MD, PhD; Hassan Alnuaimat, MD
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CORRESPONDENCE TO: Ali Ataya, MD, 1600 SW Archer Rd, M452, PO Box 100225, Gainesville, FL, 32610

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(6):e195-e199. doi:10.1016/j.chest.2015.12.037
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A 61-year-old man presented with an 18-month history of progressive shortness of breath on exertion, fatigue, worsening bilateral lower extremity edema, abdominal swelling, and increased assistance with activities of daily living. Pertinent past medical history included right-sided pneumonia secondary to Streptococcus pneumoniae that was complicated by empyema, requiring right-sided video-assisted thoracoscopic surgery with decortication 2 years earlier. He had a negative cardiac history, no recent travel in the last 3 years, and no known exposure to tuberculosis. His medications included aspirin and daily furosemide. His symptoms appeared to be refractory to diuretic therapy. Previous workup 6 months earlier included an echocardiography (ECHO) showing enlarged left and right atria with a normal ejection fraction, and a catheterization of the left side of the heart with reported normal left ventricular function and unobstructed coronary arteries.

Figures in this Article

The patient’s heart rate was 74 beats/min, blood pressure was 121/82 mm Hg, temperature was 36.8°C, respiratory rate was 14 breaths/min, and oxygen saturation was 99% on room air. Physical examination revealed jugular venous distention to the level of the mandible and paradoxical venous distention with inspiration. The lungs were clear to auscultation bilaterally. Cardiac examination revealed regular rhythm with normal S1 and S2 intensity and no evidence of murmurs or added sounds. Abdominal examination was positive for hepatomegaly and a distended abdomen with a positive fluid thrill consistent with ascites. There was prominent bilateral lower extremity edema.

Chest radiograph showed pulmonary vascular congestion. The troponin T, complete blood count, and complete metabolic profile were normal. The ECG showed sinus rhythm, with low voltage in the extremity leads, and nonspecific T-wave abnormalities in the anterolateral leads. A contrast CT image of the chest showed right lung pleural thickening from a previous surgical decortication. ECHO showed a normal left ventricle size and function (ejection fraction, 55%-60%), no pericardial effusion, prominent biatrial enlargement, a normal right ventricle (RV) with a tricuspid annular plane systolic excursion of 16 mm (normal ≥ 17 mm). RV systolic pressure could not be estimated, but there was no indirect evidence of elevated RV pressures. Pulmonary artery catheterization (PAC) was performed, with the results shown in Table 1.

Table Graphic Jump Location
Table 1 PAC Hemodynamic Measurements

PA = pulmonary artery; PAC = pulmonary artery catheterization.

The patient underwent a cardiac magnetic resonance imaging (CMR), which showed increased pericardial thickening, most significant over the right side of the heart (Fig 1). A prominent septal shift with inspiration, consistent with increased ventricular interdependence, and decreased pericardial mobility were present.

Figure 1
Figure Jump LinkFigure 1 MRI still frame obtained at end diastole, showing biatrial enlargement, normal biventricular size, and a thickened pericardium, more prominent adjacent to the right ventricle.Grahic Jump Location

What is the diagnosis?

Diagnosis: Constrictive pericarditis

Constrictive pericarditis is an uncommon diagnosis. It can occur secondary to multiple etiologies (Table 2), with idiopathic being the most common in developed countries. Tuberculosis accounts for most of the cases in underdeveloped countries.

Table Graphic Jump Location
Table 2 Causes of Constrictive Pericarditis

The predisposing etiology results in an inflammatory response that leads to fibrosis, calcification, thickening, and stiffening of the pericardium along with loss of elasticity. The pericardium may be of normal thickness in 18% of cases. Owing to the lack of pericardial elasticity, the ability of the cardiac ventricles to expand is compromised during diastole. This results in increased ventricular pressures that eventually equalize to the pressure exerted by the noncompliant pericardium. Because the myocardium itself is not affected, a greater degree of interventricular dependence is observed.

Physical examination will reveal signs of right-sided heart failure, including jugular venous distention with positive Kussmaul’s sign, ascites, lower extremity edema, hepatomegaly, and pericardial knock in half of patients as a result of abrupt interruption of ventricular diastolic filling. Patients may complain of fatigue because of a low cardiac output state. B-type natriuretic peptide is low in this condition, whereas in restrictive cardiomyopathy it is almost always > 200 pg/mL. The ECG is almost always abnormal. Low voltage and nonspecific T-wave abnormalities are commonly observed, such as T-wave inversion or flattening.

Pericardial calcifications on chest radiography are highly suggestive of the disease and are best observed on lateral films, but are present in 27% of all cases. Cardiac size is often normal, and pleural effusion can be seen in up to 50% of patients, occurring bilaterally or on either side.

CT chest imaging improves visualization of pericardial thickening (>4 mm) and inferior vena cava congestion, and rules out pericardial effusions. However, normal pericardial thickness is present in 20% of patients. Changes in the cardiac chambers may be seen such as right and left ventricular narrowing with tubular deformation, and straightening of the interventricular septum. CT imaging is also more sensitive in detecting pericardial calcifications when not seen on chest radiography, and serves as a tool to assess thoracic anatomy and patient candidacy for pericardiectomy, especially in those who have previously undergone other thoracic surgeries.

ECHO allows examination of the cardiac chambers, right and left ventricular function, valvular disorders, and vena cava and hepatic vein congestion. A constrictive protocol allows the study of different ECHO parameters with respiratory variations that differentiates constriction from restriction. Variables consistent with constrictive pericarditis also include a normal ejection fraction, biatrial enlargement, normal ventricle sizes, presence of ventricular septal shift, increased ratio of early (E) to late (A) transmitral filling velocity flow (E/A > 0.8), high early diastolic mitral annular relaxation velocity (e′ > 8 cm/s), inferior vena-cava distention, and hepatic vein expiratory diastolic reversal ratio.

The diagnosis of constrictive pericarditis is suggested on the basis of PAC findings such as diastolic equalization, prominent x- and y-wave descents in the right atrium, and a dip-plateau shape (square-root sign) on RV tracings. The dip-plateau tracing occurs as a result of early diastolic filling followed by an abrupt cessation of flow secondary to inability of the ventricle to expand beyond the stiffened pericardium. Pulmonary hypertension may be present; however, the systolic pulmonary artery pressures are often less than 55 mm Hg, in contrast to higher pressures seen in restrictive cardiomyopathy. The hemodynamic features in constrictive pericarditis may not be appreciated in hypovolemic patients, especially in those receiving aggressive diuretic therapy. A rapid volume challenge during PAC can unmask the hemodynamic findings of constrictive pericarditis. Without a right-sided heart catheterization, performing a left-sided heart catheterization alone will preclude detecting diastolic pressure equalization of the cardiac chambers and will result in missing a diagnosis of constrictive pericarditis. A dip-plateau tracing is normally absent in 23% of cases as well as in patients who are tachycardic.

CMR serves as an adjuvant to the diagnosis of constrictive pericarditis. It allows more accurate visualization of the pericardium thickness in comparison to CT imaging and reveals signs of enhanced interventricular dependence such as septal bounce using gated sequences. The inferior vena cava dilatation can also be seen. Increased gadolinium-delayed enhancement on MRI of the pericardium may indicate abnormal pericardium inflammation in the absence of thickening; this may help guide nonsurgical management with antiinflammatory agents.

Other forms of pericardial diseases that warrant mentioning are effusive-constrictive pericarditis and occult constrictive pericarditis. In effusive-constrictive pericarditis, the concurrent presence of pericardial thickening and pericardial effusion, often with cardiac tamponade, results in a constrictive physiology and a mixed hemodynamic finding of both cardiac tamponade and constrictive pericarditis. Occult constrictive pericarditis is characterized by the nonspecific presentation of shortness of breath and fatigue in patients who are volume depleted with normal resting hemodynamics but on infusion of a 1 L fluid bolus they develop hemodynamic findings characteristic of constrictive pericarditis.

Some cases of early pericarditis, especially those after cardiac surgery, are inflammatory in nature and usually resolve spontaneously or with the use of steroids or other antiinflammatory agents. In general, constrictive pericarditis is a chronic and debilitating disease and surgical pericardiectomy is warranted. Surgical mortality is approximately 7%, depending on the etiology. Long-term surgical outcomes are favorable, with most patients achieving long-term survival and New York Heart Association functional class I or II.

Clinical Course

The finding of diastolic pressure equalization together with the CMR findings confirms the diagnosis of constrictive pericarditis. The patient underwent an uneventful off-pump pericardiectomy through a median sternotomy. The pericardium was found to be tight and thickened, particularly over the areas in direct contact with the abnormal right mediastinal pleura. There was evidence of significant right-sided adhesions from the previous right empyema, which were released from the pericardium. Left-sided adhesions were significantly less. Bulge of the heart was experienced on releasing it from the pericardium (Video 1) with an observed drop in right atrial pressure. On 1-month follow-up, he had lost more than 9 kg in weight and was back to his baseline with complete resolution of his symptoms.

  • 1.

    The finding of biatrial enlargement on ECHO in the setting of normal valvular and cardiac function should raise suspicion of constrictive pericarditis.

  • 2.

    Hemodynamic finding of diastolic pressure equalization is present in 81% of cases of constrictive pericarditis, deep x and y descents are present in 77%, and respiratory variations are present in only 40%.

  • 3.

    The presence of pericardial thickening, inferior vena cava dilation, and changes in respiratory variation on CMR can confidently make the diagnosis without requiring PAC.

  • 4.

    Treatment of chronic constrictive pericarditis is almost always surgical; however, in cases of early inflammatory constrictive pericarditis, antiinflammatory agents can aid in the resolution of the disease.

Financial/nonfinancial disclosures: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Additional information: The Video can be found in the Multimedia section of the online article.


Figure Jump LinkFigure 1 MRI still frame obtained at end diastole, showing biatrial enlargement, normal biventricular size, and a thickened pericardium, more prominent adjacent to the right ventricle.Grahic Jump Location


Table Graphic Jump Location
Table 1 PAC Hemodynamic Measurements

PA = pulmonary artery; PAC = pulmonary artery catheterization.

Table Graphic Jump Location
Table 2 Causes of Constrictive Pericarditis


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