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Case presented by: Dr.Aris Anastasakis
Authors: George Lazaros, Aris Anastasakis, 1st Department of Cardiology, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
A 49-year-old woman was referred to the emergency department of our hospital by her attending physician due to progressively worsening dyspnea of 3-week duration. Two days before admission the patient became orthopneic and on the day of admission she finally sought medical advice.
The patient’s past medical history was notable for moderate mental retardation along with a long-standing severe bipolar affective disorder under close psychiatric monitor. Moreover, being a heavy smoker (about 3 packs a day or 135 pack years), she was also suffering from chronic obstructive pulmonary disease under occasional treatment with inhaled bronchodilators. Finally, it was reported that her body-weight was above normal since her childhood, with her body mass index in last years being steadily around 40.
On admission, the patient appeared in overt respiratory distress and very anxious. The blood pressure was 115/75mmHg without pulsus paradoxus. On cardiac auscultation the heart sounds appeared distant and muffled, but the overall evaluation was difficult due to the underlying chronic lung disease and her chest configuration. However, no pericardial friction rubs or other remarkable findings were noticed. Auscultation of the lungs revealed decreased breath sounds, wheezing and prolonged expiration in all fields. Moreover, jugular vein distension, mild ankle edema and palpable liver were detected as well.
Initial laboratory evaluation was remarkable for mildly elevated transaminases and creatinine. C-reactive protein levels and high sensitivity troponin testing were both normal (3.5mg/dL with normal values <5.0 and 8.9pg/mL with normal values<15.6, respectively). In contrast, B-type natriuretic peptide levels were significantly elevated (~3.500pg/mL). An ECG revealed sinus tachycardia (~100bpm), along with non-specific ST-T wave changes (Figure 1) whereas a chest x-ray showed markedly increased cardiothoracic ratio, with water bottle configuration of the cardiac silhouette and redistribution of pulmonary blood flow in the lung fields. Notably no pleural effusion was detected (Figure 2).
Figure 1: ECG obtained on admission disclosing sinus tachycardia with non-specific ST-T changes
Figure 2: Chest x-ray showing water bottle shape of the cardiac silhouette and blood redistribution in the lung fields
A bedside echocardiography performed in the emergency department revealed a moderate pericardial effusion in the infero-posterolateral pericardial space and adjacent to the right atrium (maximal diameter in diastole ~1.7cm) and small effusion adjacent to the right ventricle and apex (Figure 3A to D). A mild and brief in duration telodiastolic deep of the right atrium was noted as well.
Figure 3: Two dimensional echocardiography revealing dilated and hypokinetic left ventricle along with moderate pericardial effusion (see text for details)
The left ventricle was dilated (~59mm, Figure 3) with diffuse hypokinesis which however appeared more prominent in the territory of left anterior descending artery distribution and severe impairment of the ejection fraction (~20%). The left atrium was dilated as well. In contrast, right ventricular size and contractility as detected by Tissue Doppler imaging and tricuspid annular plane systolic excursion were both normal (SRV=11.8cm/sec and 2.2cm respectively) (Figure 4A and B). Pulse-wave Doppler interrogation of the mitral valve revealed merged E and A waves due to high heart rate with <25% decrease in E velocity of the mitral inflow during inspiration (Figure 4C). Color flow Doppler revealed moderate regurgitation of the atrioventricular valves. Inferior vena cava was dilated with a <50% reduction in its diameter during inspiration (Figure 5A and B). Combining the tricuspid regurgitation peak velocity and the inspiratory variation of inferior vena cava, pulmonary artery systolic pressure was estimated at ~65mmHg (Figure 4D).
Figure 4: Tissue Doppler imaging study of the right ventricle (A and B), transmitral flow (C) and pulmonary artery systolic pressure (D, see text for details)
Figure 5: Inferior vena cava appeared dilated with reduced respiratory variation
1. Heart failure with hydropericardium? 2. Acute pericarditis in a patient with heart failure? 3. Acute perimyocarditis?
1. In the context of heart failure with hydropericardium it is of paramount importance concerning the overall management to exclude ischemic heart failure as the underlying cause of the hydropericardium. In favour of this eventuality is the history of heavy smoking, namely 195 pack-years, which is an exceedingly high number. On the other hand the absence of a history of chest pain and the normal values of high sensitivity troponin exclude an evolving myocardial infarction, whereas the electrocardiogram is not diagnostic of acute coronary syndrome. In order to exclude or ascertain coronary disease coronary arteriography was performed after initial patient’s stabilization, which showed absence of significant coronary arteries stenoses. (Figure 6A and B). Nevertheless, the possibility of new onset non-ischemic (dilated) cardiomyopathy is still included in the differential diagnosis.
Figure 6: Coronary arteriography depicting left anterior descending coronary artery (6A) and right coronary artery (6B) without significant obstructions
2. Upon the exclusion of coronary artery disease the diagnosis of acute pericarditis should be excluded. According to the recently released guidelines of the European Society of Cardiology for the diagnosis and management of pericardial diseases, at least two of the following four criteria are required for establishing the latter diagnosis, namely: i. Typical (pleuritic) chest pain, ii. Pericardial friction rub, iii. Widespread ST-segment elevation in the electrocardiogram, and iv. New-appearing or worsening pericardial effusion (1). C-reactive protein elevation is a confirmatory finding rather than a main diagnostic criterion, since it can be within normal limits at presentation in 22% of cases (2). Similarly, the detection of pericardial inflammation by an imaging technique (either computed tomography of cardiac magnetic resonance imaging) is another confirmatory finding. In the case described, the patient presents only one of the above-mentioned criteria and specifically, new appearing pericardial effusion. Thus, the diagnostic criteria of acute pericarditis are not fulfilled and accordingly this patient is supposed to have a pericardial effusion without evidence of ongoing inflammation (namely hydropericardium). However, it is rather surprising that pericardial effusion appeared in the absence of right ventricular dysfunction, which is quite the rule in this setting (3,4).
3. Concerning the diagnosis of pericarditis with associated myocarditis having excluded coronary artery disease, endomyocardial biopsy (gold standard) would be indicated in this specific context to rule out or rule in infectious or immune-mediated myocarditis as a cause of non-ischemic dilated cardiomyopathy according to the 2013 Myocarditis Task Force criteria set in the Myocardial and Pericardial Working Group European Society of Cardiology (ESC) scientific Position Statement (5). Conversely according to the oldest 2007 scientific statement released by the American Heart Association, American College of Chest Physicians and ESC (6) on the role of endomyocardial biopsy in the management of cardiovascular disease, this case actually does not constitute a class of recommendation I for the above procedure. We wish to remind that such a recommendation is given in the following two clinical scenarios: i. New-onset heart failure of 2-weeks duration associated with a normal-sized or dilated left ventricle and hemodynamic compromise and ii. New-onset heart failure of 2-week to 3-month duration associated with a dilated left ventricle and new ventricular arrhythmias, second- or third-degree heart block, or failure to respond to usual care within 1 to 2 weeks. Endomyocardial biopsy is recommended in case of progressive worsening of left ventricular ejection function and/or development of hemodynamic instability.
Cardiac MRI with its potential for tissue characterization would be a valuable non-invasive tool for further refinement of the clinical suspicion of myocarditis. Features of myocarditis with cMR include: i. Intracellular and interstitial edema, ii. Hyperemia and capillary leakage, and iii. Subepicardial necrosis and fibrosis as unveiled by late gadolinium enhancement (6,8). CMR does not identify the aetiology of myocarditis and its sensitivity is lower in heart failure presentations (9-10) . However, the patient and her family denied this imaging modality in view of her psychiatric background.
In conclusion, the patient’s condition was attributed to (new onset) non-ischemic dilated cardiomyopathy, possibly clinically suspected myocarditis with hydropericardium.
Question 2: Is pericardiocentesis an indicated treatment option for this patient?
According to the simplified algorithm for pericardial effusion triage and management (Figure 7), which is included in the already mentioned recent ESC guidelines on pericardial diseases (1), pericardiocentesis or cardiac surgery constitute a class IC indication in case of cardiac tamponade, or in symptomatic moderate to large pericardial effusions not responsive to medical therapy, or in case of suspicion of unknown bacterial or neoplastic aetiology. Moreover, pericardiocentesis could be also considered in patients with large effusions in the absence of an established etiology if chronic (>3months).
Figure 7: Simplified algorithm for pericardial effusion triage and management proposed by ESC guidelines on pericardial diseases.
In the case presented no clear indication (class I) for pericardiocentesis was present, since the patient was hemodynamically stable without pulsus paradoxus. Actually, the diagnosis of tamponade requires integration of clinical symptoms, signs, and echo findings (11). In this patient some echocardiographic findings could be compatible with ‘near’ tamponade, such as inferior vena cava dimension and respiratory variation, however, the diagnosis of tamponade is mainly a clinical one and in the absence of hypotension and pulsus paradoxus (which is the hallmark of tamponade) such diagnosis cannot be supported. With respect to the other indications proposed in the guidelines for emergent pericardiocentesis, no history or suspicion of underlying neoplastic disease emerged based on the physical evaluation and overall laboratory and imaging tests findings. Purulent pericarditis is a severe and potentially lethal disorder and was very unlikely in the absence of fever and elevation of the inflammatory markers (11). Similarly, tuberculous pericarditis is a remote probability since we deal with a non-immunocompromised patient in a non-endemic area for the latter infection and the negativity of the tuberculin skin test.
A possible feature which could eventually justify the accomplishment of pericardiocentesis was the patient’s symptoms (dyspnea-orthopnea) in the presence of moderate pericardial effusion. However, it is not clear whether the symptoms were due to heart failure or to the pericardial effusion and we cannot state (as required by the guidelines) that symptoms were not responsive to medical therapy since no therapy had been administered.
Last but not least, an eventual percutaneous pericardiocentesis was technically demanding and not free of potential complications, since pericardial fluid was mainly localized in the posterior pericardial space which is not easily accessible with pericardiocantesis (Figure 3).
A first question to be answered is whether this patient should receive a trial with anti-inflammatory medications (aspirin or non-steroidal anti-inflammatory drugs-NSAIDs, or corticosteroids) plus colchicine in an effort to reduce or abolish pericardial effusion. The answer is negative since in the absence of inflammation as it is suggested by the normal CRP serum levels, anti-inflammatory treatment is not efficacious (12,14). In addition, NSAIDs administration may account for further decompensation of heart failure due to fluid retention and can deteriorate renal function in the context of cardio-renal syndrome.
Having clarified the reasons that prevented us from administering anti-inflammatory medications, heart failure treatment (including carvedilol, ramipril, furosemide and spironolactone) was given in this patient with careful titration up to the maximally tolerated dose. The patient improved substantially and was discharged in stable clinical condition 5 days after admission, being ensured that pericardial effusion was not increasing and the pulmonary arterial systolic pressure (PASP) decreased at 40mmHg. An echocardiogram performed in an outpatient basis 6 weeks later revealed a minimal pericardial effusion adjacent to the atrioventricular groove (appearing only in the systolic phase of the cardiac cycle) and adjacent to the right atrium. The ejection fraction increased from 20% to 35%, mitral regurgitation decreased and tricuspid regurgitation practically vanished. Inferior vena cava diameter was normal and the vessel was completely collapsing during inspiration. Finally, PASP dropped to 35mmHg.
Figure 8: Follow-up echocardiographic study revealing practically regression of the pericardial fluid with persistence of a negligible amount adjacent to the atrioventricular groove (panel A, systolic frame) and to the right atrium (panel B)
Figure 9: Inferior vena cava depicting normal diameter and collapsing during inspiration
The next follow-up visit was set at 1.5month later, namely 3 months after initial presentation, a time where the implantation of a cardioverter defibrillator (ICD) should be taken into account in cases of new-onset, non-ischemic cardiomyopathy cases, according to the recommendations of the 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death (15).
In cases of chronic heart failure the presence of even hemodynamically irrelevant pericardial effusion is associated with increased mortality. Indeed, in the latter population pericardial effusion along with age and ejection fraction emerged as independent predictors of 1-year mortality in this setting (16). Nevertheless according to another recent investigation the latter data do not seem to extrapolate in acute heart failure cases, as it was our patient’s case (17). Indeed, it seems that what really matters in acute heart failure, is the size of the effusion rather than its simple presence. Patients with at least moderate pericardial effusion, even if non-hemodynamically relevant, appear to have a poor 1-year outcome (figure 10).
Figure 10: Survival analysis depicting the 1-year mortality across PE severity (Modified from Santas E, et al. Int J Cardiol 2015;184:259-261)
In summary, this was a case of a patient with new-onset non-ischemic dilated cardiomyopathy accompanied by moderate pericardial effusion due to hydropericardium. In the absence of evidence of systemic inflammation the patient was treated exclusively with conventional heart failure treatment and improved rapidly. Since moderate pericardial effusion seems to carry an adverse prognosis in the setting of acute heart failure this patient should be closely monitored.
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