E-case #16

April 23-27, 2001

Elizabeth Gauer, M.D.

A 68-year old man presents with worsening dyspnea, LE edema, increasingabdominal girth and fatigue.  He also reports orthopnea (currently sleeping in a chair at night) and PND.  He has a two-month history of thoracic aortic aneurysm (with type B dissection) which has been stable by serial CT evaluations.  Since the diagnosis of his thoracic aneurysm, he has been on antihypertensive medications with excellent control.  He denies chest pain. He reports feeling worse after taking his medications.

PMH

• Thoracic aneurysm as noted
• UGI bleed (secondary to gastric ulcer)
• Fe deficiency anemia
• Bronchitis (PFTs with mild restrictive defect)
• Venous stasis
• HTN
• H/o alcoholism

Meds

• Ferrous sulfate
• Lasix 40 bid (recently increased)
• Labetolol 400 mg tid
• Amlodipine 5 mg qd
• Lansoprazole 30 mg qd
• Albuterol MDI

PE: 106/54, HR 61, RR 26, Sats 94% on RA Weight 245 (increased from 224 pounds 2 weeks prior)

HEENT: NC/AT.  No scleral icterus or conjunctival pallor.

Neck: JVP to angle of jaw (on later exams a positive Kussmauls sign was observed)

Heart: RRR S1, S2, extra heart sound

Lungs: Decreased BS at right base; occasional bibasilar crackles

Abd: Firm, distended, +fluid wave. 

Ext: 3-4+ LE edema.  Venous stasis changes. Rectal: brown stool, heme neg. Skin: no jaundice, no telangectasias, no palmar erythema.

Given this presentation he was admitted for further care. What is you differential diagnosis at this point?  What further studies would you obtain?

Differential diagnosis:
Cardiac: systolic dysfunction, diastolic dysfunction (including restrictive disease, constrictive pericarditis), tricuspid stenosis, TR, hypertrophic cardiomyopathy.  Superior Vena caval obstruction, nephrotic syndrome, hepatic disease, intraabdominal disease due to malignancy.   Studies (prior to current presentation)

• Echocardiogram with EF > 50% with nl heart size and nl LV function. Interventricular septum is paradoxic with RA pressure of 16 mm.  Mild AI, trace MR.
• CT of thorax: type B aortic dissection, pericardial calcifications, hepatic congestion  

On admission, a repeat echocardiogram was obtained which revealed similar findings to previous echo (nl LV function, RA pressure of 18 mm).  A CXR was obtained which showed a moderate right sided pl effusion and a small left sided pl effusion.  Curvilinear calcification of the inferior heart border was suggestive of pericardial calcification. EKG showed LBBB (old) with rate of 58, no acute ST/T changes.

Labs showed mildly elevated LFTs with tbili at 1.3, alkphos at 266, OT/PT at 20/10 respective.  CBC showed nl WBC, H/H at 8.8 and 26.5 respectively (no acute change) with MCV of 68, RDW of 22.3.  Chemistry panel was WNL. Urinalysis not sent.

He was diuresed aggressively resulting in significant improvement of CHF (repeat CXR also revealed significant reduction in the size of the right pleural effusion).   Treating physicians considered the above noted differential, and constrictive pericarditits was high on the list.

The cardiology service was consulted and they agreed that the patient should undergo both right and left sided catheterizations.  Left sided cath revealed no significant coronary lesions.  Right sided cath findings were c/w constrictive pericarditis (with matching of RV and LV diastolic pressures, RV pressures demonstrated the classic dip and plateau physiology (square root sign).  PCW was measured at 18.  There was fluoroscopic evidence of pericardial calcification.  The case was discussed with the CT surgeons and patient was felt to be a good candidate for pericardial stripping; he underwent that procedure, and at a follow up appointment about 3 months after the surgery, he reported that he felt "like a new man".  His JVP, LE edema and abdominal girth were all returned to normal, and his diuretic requirements were reduced.

Discussion:
Although constrictive pericarditis is a relatively uncommon cause of heart failure, its recognition is important; its diagnosis is often missed (Ref 1).  Chronic constrictive pericarditis is a progressive disease. A minority of patients (class I sx) may initially be managed (and do well) with diuretics and low sodium diet.  However, the vast majority of these patients will eventually require pericardiectomy.   Drugs that slow the heart rate such as beta blockers and calcium channel blockers (often used in diastolic dysfunction) should be avoided because mild sinus tachycardia is a compensatory mechanism (patients have a relatively fixed stroke volume and cardiac output depends on HR).  In this patient's case, his symptoms seemed to become most apparent after he was started on an aggressive BP control regimen (using beta blockers) for his thoracic aneurysm.  Pericardiectomy carries an operative mortality reported to range from 5 to 20%.  Those patients with poor preoperative functional class are at the highest risk for perioperative death; therefore early surgical intervention is advocated (thus the importance of early diagnosis).  Over 90% of patients will report symptomatic improvement following the procedure.

Etiology of constrictive pericarditis: Tuberculosis was formerly the leading cause of constrictive pericarditis in the Western nations (accounting for 15% or less of cases); in disadvantaged nations, tuberculosis is still a common cause of constrictive pericarditis.  Today, most cases are due to unknown causes (they are usually attributed to a previous clinically inapparent viral pericarditis).  The patient discussed above reported having pericarditis in the distant past.  Constrictive pericarditis after cardiac surgery is also an important cause to remember.

Common etiologies are summarized below (table borrowed from Ref 2):

• Idiopathic
• Infectious disease
  • TB
  • Bacterial
  • Viral (coxsackie B, echovirus)
  • Fungal
  • Parasitic
• Trauma (including cardiac surgery)
• Radiation
• Inflammatory/immunologic disorder
  • RA
  • SLE
  • Scleroderma
  • Sarcoidosis
• Neoplasms
  • breast CA
  • lung CA
  • lymphoma
  • mesothelioma
  • melanoma
• ESRD

Finally, it is often difficult to distinguish constrictive pericarditis from restrictive cardiomyopathy (extensive discussion is beyond the scope of this brief report).  Please see Ref 3, 4 for ways to distinguish the two as well as for common etiologies of restrictive cardiomyopathy.  

Diagnosis:
Physical exam: JVP with Kussmaul's sign, tachycardia, pericardial knock, decreased BS at bases.  ascites, LE edema

• CXR, CT will often show sl cardiac enlargment (due to thickened pericardium, coexisting pericardial effusion).  Extensive calcification of the pericardium may be present (not specific for constrictive pericarditis - calcification does not imply constriction).  Pleural effusions are present in about 60% of patients.
• Common EKG findings include low QRS voltage, generalized T wave inversion or flattening, LA abnormalities suggestive of P mitrale.  Afib may also occur in patients (thought to be due to chronically elevated atrial pressures and atrial enlargement).
• Echocardiography may show pericardial thickening, abnormal bouncing motion of the septum, increased respiratory variation in transvalvular blood-flow velocities (please see references for further discussion of this). 
• Cardiac cath: please see ref 2,3, for further details regarding this.

References:

1. Pauker, S; Kopelman, R: Lechan, R.  Diverted by the Chief Complaint. NEJM 1995, Vol 333, no 1, 45-48

2. Marso, S; Griffin, B; Topol, E.  Manual of Cardiovascular Medicine. Philadelphia: Lippincott Williams & Wilkins, 2000. 373-383

3. Braunwald, E.  Heart Disease.  A Textbook of Cardiovascular Medicine. 5th ed, Vol. 2.  Philadelphia: W.B. Saunders, 1997. 1596-1505 Vaitkus, PT; Kussmaul WG.  Constrictive pericarditis versus restrictive cardiomyopathy: a reappraisal and update of diagnostic criteria.  Am Heart J 1991, Nov: 122(5):1431-41