A 73-year-old man with a past medical history of idiopathic recurrent pericarditis over the last 25 years, atrial fibrillation with pulmonary vein isolation, and mild to moderate mitral regurgitation presents to clinic with progressively worsening dyspnea on exertion, orthopnea, and NYHA class II symptoms. On physical exam, jugular venous distention is noted to the jaw at 45 degrees, a pericardial knock is auscultated, and 2+ peripheral edema with hepatomegaly is observed.
ECG with normal sinus rhythm and low voltage. Transthoracic echocardiogram shows normal left ventricular systolic function with an ejection fraction of 55% (Figure 1). There is a diastolic bounce of the interventricular septum noted. The IVC is dilated at 3.5 cm and does not collapse with respiration.
Figure 1: ECG demonstrates NSR with low voltage.
Cardiac MRI is obtained and confirms a diastolic septal bounce, respirophasic septal shift, and diastolic restraint (Figures 2 and 3). There is no pericardial delayed gadolinium enhancement to suggest acute or sub-acute pericarditis. Invasive catheterization demonstrated findings consistent with constriction.
Figure 2: Cardiac MRI demonstrated a diastolic septal bounce, respirophasic septal shift and diastolic restraint.
Figure 3: There is no pericardial hyper-enhancement and no evidence of acute pericarditis.
Pericardiectomy was performed at a high volume surgical center and post-operatively the patient's symptoms improved. He completed cardiac rehabilitation and was able to return to golfing without symptoms within 6 months of the surgery.
Which of the following patient characteristics is not associated with improved long term outcomes after pericardiectomy in patients with chronic pericardial constriction?
Show Answer
The correct answer is: A. NYHA Class IV.
Based on large surgical center registry analysis, all of the above are associated with improved long term mortality except New York Heart Association class IV symptoms (A).1
Historically, pericardiectomy carried a high mortality for constrictive pericarditis (estimated ~14%).2 Currently, postoperative mortality after pericardiectomy can be accomplished with much better outcomes and lower morbidity and mortality at experienced centers of excellence.3
Constrictive pericarditis is a known complication of pericarditis. The thickened, scarred and inelastic (often calcified) pericardium limits diastolic filling of the ventricles. Symptoms subsequently result from equalization of cardiac pressures and right heart failure, given that total cardiac volume is determined by the fixed pericardium. Fluid retention (ascites, leg edema), dyspnea, and fatigue are common presenting symptoms. There are a wide range of etiologies for constrictive pericarditis including viral pericarditis, cardiac surgery, collagen vascular disease, radiation and idiopathic.1
One should keep a high index of suspicion for underlying constrictive pericarditis, especially if heart failure symptoms are out of proportion to the examination findings. Transient constrictive pericarditis is increasingly recognized as a distinct sub-type of constrictive pericarditis.4 If a patient is hemodynamically stable, a trial of anti-inflammatory medical management (NSAID, steroid, colchicine and diuretic) for 6 months before pericardiectomy is recommended.3-5 If there is no improvement in symptoms then pericardiectomy is pursued. Pericardiectomy is the definitive treatment option for patients with chronic constrictive pericarditis.
Long term outcomes for pericardiectomy was examined in 135 patients from 1985-1995 which showed that younger patients, who were NYHA 1 and 2 status preoperatively and had no history of chest radiation did best with pericardiectomy.6 In another series, long term survival after pericardiectomy for constrictive pericarditis was related to the underlying etiology, LV systolic function, renal function and pulmonary artery systolic pressure in 163 patients over 24 year follow-up.1
"End stage" constrictive pericarditis is manifested by cachexia, reduced resting cardiac output, hypoalbuminemia, and liver dysfunction from chronic congestion. There should be a risk and benefit discussion with these advanced patients before pursuing pericardiectomy due to the high risk nature of surgery in this patient group.3 Pericardiectomy should be pursued at experienced pericardial focused centers, whenever possible, for improved surgical outcomes.1
References
Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol 2004;43:1445-52.
McCaughan BC, Schaff HV, Piehler JM, et al. Early and late results of pericardiectomy for constrictive pericarditis. J Thorac Cardiovasc Surg 1985;89:340-50.
Cremer PC, Kumar A, Kontzias A, et al. Complicated pericarditis: understanding risk factors and pathophysiology to inform imaging and treatment. J Am Coll Cardiol 2016;68:2311-2328.
Gentry J, Klein AL, Jellis CL. Transient constrictive pericarditis: current diagnostic and therapeutic strategies. Curr Cardiol Rep 2016;18:41.
Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004;43:271-5.
Ling LH, Oh JK, Schaff HV, et al. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999;100:1380-6.