Cardiology Intensive Board Review, 3 ed.

Pericardial Disease

Wael A. Jaber • Parag R. Patel

ANSWERS

1.a. Presence of an ASD. The presence of the iatrogenic ASD after the transseptal puncture for the radiofrequency ablation/pulmonary vein isolation procedure equates right atrial (RA) and left atrial (LA) pressures with inspiration. The predicted decrease in LV filling during inspiration due to interventricular dependence and exaggerated RV filling and septal shift toward the LV is mitigated by the presence of an ASD. With inspiration, the decrease in intrathoracic pressure is transmitted to both atria and thus preload to the LV is maintained and interventricular dependence is not as pronounced. Thus, the variation in systolic blood pressure is not as prominent, resulting in minimal to no pulsus paradoxus. Administration of excess fluid would stave off circulatory collapse in tamponade; however, it would not diminish the pulsus. Answer b is incorrect as with severe LV dysfunction, patients can have a pulsus alternans (variation in peak systolic pressure with every other beat) and Answer c is incorrect as obstructive lung disease can lead to the presence of a pulsus due to exaggerated inspiratory effort and negative intrathoracic pressure.

2.a. Ibuprofen 600 TID for 2 weeks followed by taper and colchicine 0.5 mg BID for 3 months. The patient presents with an initial attack of acute pericarditis without any high-risk features (small effusion, negative troponin, no fever/trauma, or anticoagulant use). The appropriate regimen in this case would be an NSAID (ibuprofen 600 to 800 mg TID or indomethacin 50 mg TID) for a course of 1 to 2 weeks with physician follow-up. In addition, the use of colchicine has been studied in two trials with improvement in symptom resolution and maintenance of remission at a dose of 0.5 mg (daily for <70 kg; BID for >70 kg) for a fixed period of 3 months.

Aspirin and colchicine can be used together; however, the dosing is incorrect for Answer b (650 to 1,000 mg TID) and the colchicine should still be continued for 3 months. Prophylactic Proton-pump inhibitor (PPI) should be utilized during the high-dose NSAID use to prevent gastric ulcer. Answer c is incorrect (dosing of ibuprofen is incorrect). Answer d is incorrect since steroid therapy is only reserved for patients with NSAID or acetylsalicylic acid (ASA) contraindication or patients having relapsing pericarditis that is refractory to NSAID/ASA therapy.

3.a. Aspirin 650 TID for 2 weeks with taper to 81 mg daily + colchicine 0.5 mg BID for 3 months. The patient has postinfarction pericarditis with a typical presentation after reperfusion for late-presenting MI. Although not as frequent, postinfarction pericarditis (Dressler syndrome) is still seen in a small percentage of patients after large MI, and cardiac/pericardial trauma. The regimen used in these patients is modified to include aspirin (instead of NSAIDs) for two reasons: (a) aspirin is required for patients with CAD, with or without recent stenting; and (b) NSAIDs are postulated to impair scar formation and wound healing after an MI. Colchicine is still part of the regimen despite the recent MI and helps with symptom resolution. Correct answer is a—with high-dose aspirin initially with gradual taper once symptoms improve. The clopidogrel is continued despite high doses of aspirin, due to the placement of a recent intracoronary stent.

4.a. Initiate steroid therapy (0.25 to 0.5 mg/kg/day) along with colchicine, and initiate PO diuretic. The patient had acute pericarditis, which transformed into a chronic effusive constrictive pericarditis. There is evidence of therapy failure (persistent symptoms and elevated biomarkers) and ongoing inflammation, leading to symptomatic constrictive pericarditis with increasing hemodynamic significance (as demonstrated by symptoms/physical examination and echo findings). The next step would be to escalate anti-inflammatory therapy to include glucocorticoids (prednisone) to help abate the ongoing symptoms and pericardial inflammation. Glucocorticoids are generally not first-line therapy since patients who receive them early in the course of the disease process are more likely to have relapsing pericarditis and eventually develop constrictive pericarditis. In the case of this patient, he does not have any other treatment options due to his NSAID allergy, so steroids should be initiated at a low dose and maintained with very gradual taper (weeks to months) that involves assessment of his symptoms, biomarker trend (ESR/hsCRP), as well as MRI findings to assess for inflammation/edema within the pericardium to help quell the disease process.

Admission for IV diuresis is not necessary as the patient has not proven resistance to PO diuretics and although a cardiac catheterization may be required alternative noninvasive diagnostic modalities should be performed prior to catheterization to make the diagnosis. Answer c is incorrect since the patient has demonstrated treatment failure with progression of symptoms in the interim. Answer d is incorrect as medical therapy options (steroids, diuresis) are still available. Pericardiectomy is generally reserved in medically refractory cases.

5.a. Tuberculous pericarditis. The patient presents with acute tuberculous pericarditis with large exudative effusion. Indolence of the effusion is likely over months; however, the salient findings in the fluid analysis are the elevated interferon gamma, ADA, and normal peripheral white blood cell count with pericardial lymphocyte predominance. The presence of interferon gamma elevation had a 92% sensitivity, 100% specificity, and 100% positive predictive value for tuberculous pericarditis. ADA was also linked to tuberculous pericarditis; however, it was not as sensitive or specific (87%/92%).

Although Answers b and c are epidemiologically possible, the fluid analysis is not suggestive of either. Pericardial biopsy (Answer d) is incorrect as tuberculous pericarditis can be defined by the interferon gamma and ADA elevation. Also Acid-Fast Bacilli (AFB) staining will reveal AFB + organisms confirming diagnosis. Biopsy should be reserved for patients with unrevealing fluid analysis who are still symptomatic and require further diagnostic testing to make a diagnosis.

6.a. Annulus reversus refers to reversal of septal and lateral mitral tissue Doppler velocities (E’ septal > E’ lateral) and annulus paradoxus refers to inverse correlation of E/E’ and LV end-diastolic pressure. The annulus reversus phenomenon describes a reversal of mitral lateral and septal tissue Doppler velocity. Normally, E’ lateral > E’ septal; however, in constrictive pericarditis it is postulated that tethering of the free wall prevents longitudinal motion of the annulus at the lateral border, thus decreasing the lateral E’ and the septal E’ concurrently is mildly exaggerated.

The annulus paradoxus phenomenon was initially described after data looking at the correlation between the E/E’ ratio and pulmonary capillary wedge pressure were established. In a small subset of patients with constrictive pericarditis, inversion of the correlation between E/E’ and PCWP was noted, and named annulus paradoxus.

7.a. Lack of preload due to overdiuresis. Volume loading is required to elucidate the diagnostic findings described above. Constrictive pericarditis is a preload-dependent condition and with overdiuresis and low central venous pressure, the hemodynamic findings of elevated and equal end-diastolic ventricular pressure waveforms as well as respiratory discordance of the LV/RV waveforms are not seen. Often in these cases, the patient is given a bolus of 1 to 2 L of normal saline to increase the RA pressure >12 to 15 mmHg and the study is performed once they are adequately volume loaded. Of note, in cases of atrial fibrillation, the patient may require a temporary venous pacemaker to regularize the rhythm for analysis purposes.

8.c. Reassure the patient and observe him over the next 3 months for worsening of symptoms. The natural history of acute viral or idiopathic pericarditis is usually short and self-limited. Occasionally, mild forms of constriction may develop weeks after the initial event, but they usually resolve without any specific treatment. No further treatment is indicated unless he becomes more symptomatic or develops signs of cardiac tamponade.

9.c. Give a nonsteroidal anti-inflammatory medication. The clinical presentation of a few days of severe chest pain does not favor an acute MI. Furthermore, the ECG tracing supports the diagnosis of pericarditis. Therefore, cardiac catheterization and thrombolytics are not appropriate. The only reasonable answer is to start the patient on anti-inflammatory medications and obtain a TTE to rule out pericardial effusion.

10.a. Constrictive pericardial disease. This patient did not have evidence of ischemia on a recent stress test. Furthermore, there is no evidence of obstructive disease in his coronaries or grafts. His tracings mostly support the diagnosis of constriction, given the diastolic equalization of pressures in the cardiac chambers and the typical square root sign. Amyloidosis would typically show signs of restrictive hemodynamics with no respiratory variation. Echocardiography typically shows increased LV wall thickness. Additionally, a diagnosis of tamponade should have been evident by echocardiography, which the patient had before heart catheterization. Otherwise, hemodynamic tracings of cardiac tamponade would look exactly the same as for constriction.

11.b. This patient has significant diastolic dysfunction, and his prognosis is guarded. He has evidence of restrictive LV filling (advanced diastolic dysfunction) in the absence of CAD. The differential diagnosis in his age group includes amyloidosis (especially considering concomitant renal dysfunction), hemochromatosis, and other infiltrative processes.

12.c. His symptoms are related to impairment of RV filling and pericardial disease. This patient with the main presentation of dyspnea has an increased cardiac silhouette. The nuclear image provided shows a circumferential echolucency surrounding the heart. This is consistent with a large pericardial effusion, and he most likely has RA and RV diastolic compromise. There is no evidence of a perfusion defect to suggest ischemia.

13.c. He is showing signs of early postinfarction pericarditis, and a nonsteroidal anti-inflammatory medication should be started. This patient had an MI 72 hours ago that was successfully treated with thrombolytics. The ECG shows diffuse ST elevation with PR depression. These findings support the diagnosis of post-MI pericarditis. The ECG changes are new and nonlocalizing. Most patients improve with nonsteroidal anti-inflammatory medications.

14.a. He has a pericardial cyst that is benign; no further treatment should be offered. The TTE and CXR show a pericardial cyst. Pericardial cysts are usually smooth structures containing transudative fluid. They are frequently only 2 or 3 cm in diameter, often located at the right cardiodiaphragmatic angle, and clinically silent. However, cysts can be associated with chest pain, dyspnea, cough, and arrhythmias likely caused by compression of adjacent tissues. They can also become secondarily infected. In this patient, whose nonspecific symptoms appear to be resolving, no further treatment is needed.

15.a. The patient should have immediate surgical intervention. This patient has evidence of acute type A aortic dissection with extension to the pericardium, as evidenced by the pericardial effusion on the TEE. He should be immediately referred for surgical repair. If the diagnosis were not certain based on the TEE, then CT, MRI, or aortic angiography would be needed to better define the anatomy. The safest and most efficient management of patients with aortic dissection is to carry out all diagnostic procedures in the operating room. Pericardial drainage often gives only temporary relief or no relief of the tamponade, and the subsequent increase in BP disrupts sealing clots, accelerating intrapericardial leakage.

16.a. Immediate surgical exploration of the pericardium. The TTE and TEE demonstrate a pericardial hematoma compromising RA and RV filling. This is an indication for surgical exploration and evacuation of the hematoma.

17.b. IV hydration. This patient has evidence of pericarditis likely related to uremia, as he is close to requiring dialysis. Although his TTE shows signs of tamponade (RA collapse, moderate-sized effusion, and respiratory variation across the mitral inflow), there is no jugular venous distention, and the inferior vena cava is small sized, indicating that this patient has been overdiuresed. His hypotension and tachycardia are related to dehydration. He should, therefore, be treated with IV hydration.

18.b. Admit the patient for observation on telemetry with a follow-up TTE. The ECG shows findings consistent with an anterior wall injury, and the TTE shows a small pericardial effusion. Given this patient’s history, he most likely has a cardiac contusion. Although the prognosis for recovery is generally excellent, these patients require careful monitoring and follow-up for late complications, which range from ventricular arrhythmias to cardiac rupture. Hence, the most logical answer to this question is to admit the patient to a telemetry bed with follow-up TTE.

19.d. Proceeding with chemotherapy without further cardiac evaluation. This patient’s MRI shows congenital absence of the pericardium. This is a benign condition usually found incidentally. No specific cardiac treatment is needed unless there is entrapment of one of the cardiac chambers.

20.d. A surgical evaluation for pericardiectomy is necessary because the findings on his TTE indicate that he will develop problems in the future if this is not taken care of soon. The patient is currently symptomatic with edema of the lower extremities. Furthermore, he has a pericardial friction rub suggestive of an active pericardial process likely related to his rheumatologic disease process. He is already on methotrexate and prednisone as anti-inflammatory medications. Pericardial effusions related to rheumatoid arthritis often progress to constriction despite anti-inflammatory therapy, and early management consisting of pericardial stripping is recommended.

21.b. Immediate pericardiocentesis. The next step is an immediate pericardiocentesis. This patient has signs of early sepsis. Furthermore, the CXR shows pneumopericardium that likely developed secondary to gastric perforation from the esophagogastroduodenoscopy and cauterization of the ulcer. This patient needs immediate referral to surgery for repair.

22.c. He has an iatrogenic pneumohydropericardium; immediate drainage and surgical attention are needed. This patient had a TEE that most likely resulted in an esophageal tear with communication to the pericardial sac. On the CXR, there is a lucent triangle outlining the pericardium with pericardial passage over the aortic arch.

23.a. Perform emergency dialysis. This patient has missed her dialysis session and is now presenting with hyperkalemia (note peaked T waves on ECG) and uremic pericarditis. The most essential step is to start dialysis to treat the hyperkalemia.

24.c. Continue the current management; the ECG will improve with the resolution of ketoacidosis. Patients presenting with diabetic ketoacidosis can have ECG features that are typical of stage I pericarditis and hypokalemia. The treatment is usually that of ketoacidosis. The ECG returns to normal after resolution of the acidosis.

25.c. Mesothelioma. Teratoma is the most common pericardial tumor in infancy and childhood. Neuroma and hemangioma are uncommon enough to be considered curiosities.

SUGGESTED READINGS

Buck M, Ingle JN, Giuliani ER, et al. Pericardial effusion in women with breast cancer. Cancer. 1987;60(2):263–269.

Ha JW, Oh JK, Ling LH, Nishimura RA, Seward JB, Tajik AJ. Annulus paradoxus: transmitral flow velocity to mitral annular velocity ratio is inversely proportional to pulmonary capillary wedge pressure in patients with constrictive pericarditis. Circulation. 2001;105:976–978.

Hoit BD. Management of effusive and constrictive pericardial heart disease. Circulation. 2002;105(25):2939–2942.

Imazio M, Brucato A, Cemin R, et al. A randomized trial of colchicine for acute pericarditis. N Engl J Med. 2013;369:1522–1528.

Klein AL, Asher CR. Diseases of the pericardium, restrictive cardiomyopathy, and diastolic dysfunction. In: Topol EJ, ed. Textbook of Cardiovascular Medicine, 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002.

Lange RA, Hillis LD. Clinical practice. Acute pericarditis. N Engl J Med. 2004;351:2195.

Reuss CS, Wilansky SM, Lester SJ, et al. Using mitral “annulus reversus” to diagnose constrictive pericarditis. Eur J Echocardiogr. 2009;10:372–375.

Reuter H, Burgess L, van Vuuren W, Doubell A. Diagnosing tuberculous pericarditis. QJM. 2006;99:827–839.

Spodick DS. The Pericardium: A Comprehensive Textbook. New York: Marcel Dekker; 1997.