An Assessment of Non-Communicable Diseases, Diabetes, and Related Risk Factors in the Territory of Guam: A Systems Perspective

Constrictive pericarditis is only diagnosed once in every 10,000 admissions. It is characterized by inflammation and fibrous scarring of the pericardium resulting in a thickened, rigid sac that impairs diastolic filling. Approximately 50% of cases are idiopathic, while post cardiac surgery and post radiation therapy account for 37% and 9% respectively. The scarcity of presenting symptoms and the rarity of diagnosis make it a challenge to recognize. A 57 year-old man with no past medical history presented to the emergency room with progressing fatigue for two weeks and worsening lower extremity edema for one week, associated with dyspnea on exertion and decreased exercise tolerance. He was noted to be in atrial fibrillation with RVR upon admission to ER. Physical exam was significant for an 8 centimeter elevated JVP, and a Kussmaul's sign, which was noted sitting upright. Mild abdominal distention, hepatomegaly, and 3+ bilateral pitting edema to the upper thigh were also noted. Electrocardiogram showed non-specific low voltage QRS. A lateral chest x-ray showed significantly abundant calcifications along the anterior, inferior aspect of the cardiac silhouette. A subsequent tissue Doppler echocardiogram noted a septal bounce and an elevated early diastolic mitral annular velocity or E'. Cardiac catheterization showed an equalization of pressures, a dip and plateau sign, and ventricular interdependence. A phrenic-to-phrenic nerve pericardectomy was performed, removing a 5 millimeter thick, firm, calcified pericardium. A final diagnosis of idiopathic constrictive pericarditis was diagnosed as tissue pathology showed no abnormalities other than severe calcification. Five days post-operatively, significantly decreased leg edema, heart rate, dyspnea, abdominal distention, and JVP were noted. He was subsequently discharged two weeks post-operative with resolution of his symptoms. This case illustrates the rarity of having multiple signs and symptoms associated with constrictive pericarditis as well as the new diagnostic capabilities of tissue Doppler. In constrictive pericarditis, elevated JVP is found in 86% of patients, dyspnea on exertion in 78%, edema in 54%, abdominal fullness in 68%, fatigue in 25%, a Kussmaul's sign in 21%, calcifications on chest x-ray in only 20%, and atrial fibrillation in only 10%. Newer data has also shown excellent specificity, 97%, for constrictive pericarditis when an elevated E' of greater than 12 cm/sec is noted. The presence of multiple rare signs and symptoms and the use of new diagnostic tissue Doppler signs make this case extremely unusual. Recognizing these signs and the proper use of cardiac catheterization and tissue Doppler is critical in differentiating constrictive pericarditis from restrictive cardiomyopathy and pericardial tamponade. It is also critical for the initiation of pericardectomy to prevent further deterioration or cardiac death.