Restrictive Myocardial Disease Research Articles

Echocardiographic Diagnosis of Constrictive Pericarditis

Background— Constrictive pericarditis is a potentially reversible cause of heart failure that may be difficult to differentiate from restrictive myocardial disease and severe tricuspid regurgitation. Echocardiography provides an important opportunity to evaluate for constrictive pericarditis, and definite diagnostic criteria are needed. Methods and Results— Patients with surgically confirmed constrictive pericarditis (n=130) at Mayo Clinic (2008–2010) were compared with patients (n=36) diagnosed with restrictive myocardial disease or severe tricuspid regurgitation after constrictive pericarditis was considered but ruled out. Comprehensive echocardiograms were reviewed in blinded fashion. Five principal echocardiographic variables were selected based on prior studies and potential for clinical use: (1) respiration-related ventricular septal shift, (2) variation in mitral inflow E velocity, (3) medial mitral annular e' velocity, (4) ratio of medial mitral annular e' to lateral e', and (5) hepatic vein expiratory diastolic reversal ratio. All 5 principal variables differed significantly between the groups. In patients with atrial fibrillation or flutter (n=29), all but mitral inflow velocity remained significantly different. Three variables were independently associated with constrictive pericarditis: (1) ventricular septal shift, (2) medial mitral e', and (3) hepatic vein expiratory diastolic reversal ratio. The presence of ventricular septal shift in combination with either medial e'≥9 cm/s or hepatic vein expiratory diastolic reversal ratio ≥0.79 corresponded to a desirable combination of sensitivity (87%) and specificity (91%). The specificity increased to 97% when all 3 factors were present, but the sensitivity decreased to 64%. Conclusions— Echocardiography allows differentiation of constrictive pericarditis from restrictive myocardial disease and severe tricuspid regurgitation. Respiration-related ventricular septal shift, preserved or increased medial mitral annular e' velocity, and prominent hepatic vein expiratory diastolic flow reversals are independently associated with the diagnosis of constrictive pericarditis.

Pericardial diseases in the era of imaging, biomarkers and molecular diagnosis

Pericardial diseases only affect a small proportion of patients with heart disease. But their diagnosis and differential diagnosis is, though too often neglected by cardiologists and internists, extremely important for the individual patient and of therapeutic and prognostic relevance in the differential diagnosis of cardiac symptoms [1, 2]: An excellent example for precordial pain is acute pericarditis, which should be differentiated from aortic dissection, myocardial infarction, pneumonia or pleuritis, pulmonary embolism, pneumothorax, costochondritis (Da Costa syndrome), gastroesophageal reflux or neoplasm and herpes zoster. In the spectrum of heart failure syndromes, it is pericardial constriction that causes dyspnoea and peripheral edema. Seferovic et al. particularly address these issues in their contribution, which was designed to update the 2004 guidelines of the European Society of Cardiology [3]. These guidelines are still the only guidelines worldwide on the management of pericardial diseases. Interventional pericardiologists nowadays use intrapericardial endoscopy and biopsy together with classic cytology to establish an etiologically based diagnosis [4, 5] by making use of techniques that have been accepted and applied to diagnose inflammatory cardiomyopathies [6] or tumors or rheumatic diseases since several decades. Pericardial access has become a vital interest for electrophysiologists, who now also ablate epicardial foci and reentry sites to treat malignant ventricular tachycardia [7, 8]. Pericardial access is also discussed for localized pharmacological treatment [9], even for stem cells or cocktails with growth factors [10]. Devices for locomotion on the epicardial surface have been designed and applied in preclinical settings [10, 11]. Apart from the patient’s symptoms, echocardiography remains the mainstay of imaging of acute pericardial syndromes but also of constrictive pericarditis, effusive–constrictive pericarditis, pericardial effusion, tamponade, absence of the pericardium and cysts or tumors. The remarkable progress, which has been made in echocardiography in the last years, is very well described by Veress, Feng and Oh from the Mayo-Clinic. Progress in cardiac tissue Doppler analysis, strain and strain rate imaging by speckle tracking imaging and three-dimensional echocardiography, the assessment of early diastolic annulus velocity and annulus reversus by TDI improves the differentiation of constriction from restrictive myocardial disease and is in the focus of their contribution. Threedimensional echocardiography may come up as a useful method for the precise assessment pericardial masses as it provides incremental value to 2D echocardiography by detecting anatomical and pathological structures with higher accuracy. Of particular interest are their contributions and that of others on effusive–constrictive pericarditis [12–14] and on the differential diagnosis of constrictive pericarditis to restrictive cardiomyopathy [15]. Syed and coworkers in this issue point out that effusive– constrictive pericarditis is best characterized in patients with tamponade who continue to have elevated intracardiac pressure after removal of pericardial fluid. The underlying causes are pericardial inflammation in conjunction with the presence of pericardial fluid under pressure, whereby the etiology is diverse. Alter et al. add valuable information on imaging by reviewing MRI and CT [16, 17] as well as scintigraphic Bernhard Maisch: Former Director of the University Hospital of Internal Medicine and Cardiology.

Echocardiography in pericardial diseases: new developments

Echocardiography is one of the most important clinical tools in the diagnosis and management of various pericardial diseases, including constrictive pericarditis, effusive constrictive pericarditis, pericardial effusion, tamponade, absence of the pericardium and cysts or tumors. During recent years, remarkable progress has been made in echocardiography: cardiac tissue Doppler analysis (TDI), strain and strain rate imaging by speckle tracking imaging (STE) and three-dimensional (3D) echocardiography. The assessment of early diastolic annulus velocity and annulus reversus by TDI improves the differentiation of constriction from restrictive myocardial disease, which can be further facilitated by STE as a complementary tool. 3D echocardiography may be useful for the more precise assessment of pericardial diseases, such as pericardial effusion or pericardial masses as it provides incremental value to 2D echocardiography by detecting anatomic structures with higher accuracy. Applications of these newer echocardiographic techniques in the assessment of pericardial diseases are discussed in this chapter.

Abstract 5932: The Differences of Echocardiography and MRI Findings in Patients with Constrictive Pericarditis According to the History of Chest Radiation

Background: Radiation therapy (RT) to the chest may induce various combined cardiac problems such as constrictive pericarditis (CP), restrictive myocardial disease, and coronary artery disease. Therefore, it was hypothesized that the MRI and transthoracic echocardiographic (TTE) findings in patients with CP may differ according to the history of RT. Methods: A total of 68 patients with CP who performed both TTE and MRI study at Mayo Clinic from 2002 to 2008 were reviewed and divided into two groups according to the history of RT; RT group (group I, n=13, 53.6±9.8 years, 8 males) versus no RT group (group II, n=55, 59.0±14.8, 45 males). Results: The results of TTE study were summarized in table . Early diastolic velocity of septal mitral annulus (E′) and deceleration time (DT) of mitral inflow was significantly lower, and the ratio of early diastolic mitral inflow velocity (E) to E′ is significantly higher in group I than in group II. Left atrial volume index (LAVI) was significantly lower and LA area and left ventricular end-diastolic dimension (LVEDD) was significantly smaller in group I than in group II. Delayed enhancement of pericardium was the only significant finding in MRI and significantly prevalent in group I than in group II (100.0% in group I vs 63.6% in group II, p=0.012). The other MRI findings including pericardial thickness, left ventricular and right ventricular ejection fraction, and the presence of pericardial and pleural effusion were not different between the groups. Conclusion: In CP patients with the history of RT compared to patients without history of RT, E′ and DT was significantly lower, LAVI and LVEDD were smaller, pericardial DE in MRI was invariably found. The lower E′ velocity and decreased chamber size and volumes may be explained by RT induced coexisting myocardial disease. TTE findings of the patients

Constrictive Pericarditis in the Modern Era: Novel Criteria for Diagnosis in the Cardiac Catheterization Laboratory

This study sought to determine the clinical utility of a new catheterization criterion for the diagnosis of constrictive pericarditis (CP). The finding of early rapid filling and equalization of end-diastolic pressures obtained by cardiac catheterization are necessary for the diagnosis of CP, but these findings are also present in patients with restrictive myocardial disease (RMD). Enhanced ventricular interaction is unique to CP. High-fidelity intracardiac pressure waveforms from 100 consecutive patients undergoing hemodynamic catheterization for diagnosis of CP versus RMD were examined. Fifty-nine patients had surgically documented CP and comprised group 1; the remaining 41 patients with RMD comprised group 2. The ratio of the right ventricular to left ventricular systolic pressure-time area during inspiration versus expiration (systolic area index) was used as a measurement of enhanced ventricular interaction. There were statistically significant differences in the conventional catheterization criteria between CP and RMD, but the predictive accuracy of any of the criteria was <75%. The systolic area index had a sensitivity of 97% and a predictive accuracy of 100% for the identification of patients with surgically proven CP. The ratio of right ventricular to left ventricular systolic area during inspiration and expiration is a reliable catheterization criterion for differentiating CP from RMD, which incorporates the concept of enhanced ventricular interdependence.

CT in tuberculous constrictive pericarditis.

Constrictive pericarditis (CP) and restrictive cardiomyopathy have the same pathophysiological disorder (impaired diastolic filling) and a similar presentation. To determine the value of a noninvasive technique, CT, in differentiating between the two, we reviewed the CT scans of 212 patients with clinical signs and symptoms of CP and/or restrictive cardiomyopathy, after 26 cases that did not proceed to surgery were excluded. Computed tomography showed pericardial thickening (greater than 3 mm) in 157 patients with proven tuberculous CP. In two children with proven constriction, the pericardium measured between 2 and 3 mm. We determined that pericardial thickening, in the correct clinical setting, is the only CT sign necessary to diagnose CP. Other CT signs, inferior vena cava dilatation (97%), and deviation of the interventricular septum (15%) were confirmatory. Computed tomography accurately differentiates between CP and restrictive myocardial disease, making more invasive modalities such as cardiac catheterization and endomyocardial biopsy, with few exceptions, unnecessary.

Idiopathic restrictive cardiomyopathy in the young: report of two cases

Two cases of idiopathic restrictive cardiomyopathy in young age are reported. This rare kind of restrictive cardiomyopathy is characterized by the absence of specific histologic features of myocardial abnormalities. In both cases (aged 12 and 9 years at diagnosis), the clinical picture was characterized by severe and slowly progressive congestive heart failure. The electrocardiogram showed biventricular hypertrophy, right bundle branch block and pseudoinfarctional Q waves. Echocardiography revealed moderate pericardial effusion, biatrial enlargement, and normal or nearly normal biventricular dimensions and systolic function. Cardiac catheterization disclosed the typically restrictive filling pattern. Right ventricular endomyocardial biopsy demonstrated moderate interstitial fibrosis and cellular hypertrophy without any evidence of infiltrative or storage myocardial disease or endocardial pathology. One patient underwent cardiac transplantation, whereas in the other, transplantation was contraindicated because of longstanding pulmonary hypertension and liver cirrhosis. The knowledge of this rare entity may correctly orient the diagnostic process in children suspected of having restrictive myocardial disease. Heart, or even heart-lung, transplantation must be considered in cases with congestive heart failure before irreversible damage occurs in many organs.

Late results after pericardectomy for constrictive pericarditis via left thoracotomy.

Pericardectomy was performed on 28 men and 8 women (mean age 56, range 18-74 years) with diagnosis constrictive pericarditis. The etiology was unknown in 16 cases. Left anterior thoracotomy was employed in all but one case, in which median sternotomy was preferred because of extensive left pleural calcifications. There were three early and 11 late deaths. Actuarial survival at 5 and 10 years was 77% and 64%, respectively. Relief of symptoms was reported by 16 of the 33 patients (49%), who survived the postoperative period. These 16 were among the 22 survivors observed for a median of 8 (range 2-17) years postoperatively. Complete pericardectomy was achieved in all 16 asymptomatic patients, but in only nine of the 17 hospital survivors (53%), with persistent heart failure (p less than 0.01). Pericardial calcifications were significantly less common in the former than in the latter group. Lack of postoperative improvement was related to severe, nonresectable calcifications, and probably in some cases to associated fibrous epicarditis or restrictive myocardial disease. Left thoracotomy permits easier and more complete left ventricular pericardectomy, but is less safe when severe calcifications involve the right ventricle and atrium.