Usefulness of Echocardiography and Doppler Echocardiography in Endomyocardial Fibrosis

doi:10.1016/S0894-7317(00)70008-3

Journal of the American Society of Echocardiography

Volume 13, Issue 5, May 2000, Pages 385-392

https://doi.org/10.1016/S0894-7317(00)70008-3 Get rights and content

Abstract

Objective: Our goal was to demonstrate the usefulness of echocardiography and cardiac Doppler echocardiography (echo-Doppler) in the diagnosis of endomyocardial fibrosis, an unusual restrictive cardiomyopathy in Argentina. Methods: Between 1980 and 1998, we studied 10 women (aged 27 to 58 years) with endomyocardial fibrosis confirmed by surgery and/or endomyocardial biopsy. Of the 10 cases of endomyocardial fibrothrombosis, 8 were biventricular and 2 were left ventricular. Six patients had only an echocardiographic study, and the last 4 patients (after 1987) had an echo-Doppler study also; 3 had a transesophageal echocardiography examination as well. Seven patients had grade III-IV dyspnea, 2 had an edematous-ascitic syndrome, and 1 had right heart failure at the first examination. Four patients died of heart failure and 1 of overimposed sepsis. Surgery was successful in 2 patients with the biventricular form of the disease. In one of them, fibrotic decortication was performed in both ventricles together with tricuspid and mitral replacement. In the other, the right side was not surgically treated because of its mild engagement. One patient was lost to follow-up, and 3 patients are awaiting surgery at this writing. Results: In all 10 patients, echocardiography was the first diagnostic tool used. In M-mode echocardiography, the typical image showed the “square root” sign in the septum and posterior wall in addition to the “merlon” sign, characterized by a hypercontractile basal ventricle opposing an obliterated apex. In 2-dimensional echocardiography, inversion of the normal sized heart with obliterated ventricles and dilated atria were seen in the whole group. In 1 patient, the fibrous thrombus was limited to the apex of the right ventricle (Shaper’s type 1) in a biventricular form, whereas in the left side of this patient and in the other 9 patients, the fibrous thrombus that initially occupied the apex engaged the posterior papillary muscle, pulling the posterior valve downward (Shaper’s type 2) and generating tricuspid and/or mitral regurgitation that was always mild or moderate. The fibrous thrombus never altered the movement of the underlying myocardium. There were hypoechoic and hyperdense echoes inside the fibrotic material (the latter compatible with calcium), and in all 10 patients, different grades of pericardial effusion were found. Echo-Doppler showed the same minimal percentage of change in mitral and tricuspid velocities as found in healthy patients, which clearly differentiates endomyocardial fibrosis from constrictive pericarditis. Furthermore, a restrictive pattern was observed on both atrioventricular valves when both sides were engaged with a markedly short tricuspid deceleration time. Pulmonary veins showed a markedly diastolic D wave and a broad reversal A wave (the latter presented a low velocity when the wall of the left atrium was diseased) caused by an increased end-diastolic left ventricular pressure to the same extent throughout the respiratory cycle. Hepatic veins showed a markedly deep diastolic forward wave throughout the respiratory cycle and a marked reversal with inspiration. Conclusions: We showed (1) echocardiographic studies of a significant number of patients with this unusual disease, (2) the characteristic diagnostic signs in M-mode and 2-dimensional echocardiography, and (3) the common echo-Doppler patterns shared by all subjects studied with this technique. (J Am Soc Echocardiogr 2000;13:385-92.)

Section snippets

Patients

Between 1980 and 1998, we studied 10 women (aged 27 to 58 years) with endomyocardial fibrosis confirmed by surgery and/or endomyocardial biopsy. Of the 10 cases of endomyocardial fibrothrombosis, 8 were biventricular and 2 were left ventricular. Six patients had only an echocardiographic study, and the last 4 patients (after 1987) had an echo-Doppler study also; 3 had a transesophageal echocardiography examination as well. Seven patients had grade III-IV dyspnea, 2 had an edematous-ascitic

RESULTS

In all 10 patients, echocardiography was the first diagnostic tool used. In M-mode echocardiography, the typical image was the “square root” sign in the interventricular septum and posterior wall together with the “merlon” septal sign (Figure 1) characterized by a hypercontractile basal ventricle opposing an obliterated apex.

. M-mode echocardiogram of the right and left ventricles (level 1). A “merlon” septal movement is seen in an endomyocardial disease. RV, Right ventricle; S, septum; LV, left

DISCUSSION

Echocardiography is the most useful tool with which to study endomyocardial disease because it establishes the diagnosis of restrictive cardiomyopathy and suggests the cause of this type of cardiomyopathy. M-mode echocardiography provides evidence of restriction by the square root sign shown in the movement of the interventricular septum and the posterior wall,7, 8, 9, 10 which resembles the dip-plateau pattern of the left ventricular pressure curve. This sign is shared by restrictive

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Cited by (53)

Endomyocardiofibrosis in the Americas Collaborative Study: The EMF-SIAC Registry
2023, Current Problems in Cardiology
Endomyocardiofibrosis (EMF) is a restrictive cardiomyopathy characterized by subendocardial fibrosis due to eosinophilic myocardial infiltration. EMF may commonly present with heart failure (HF) or atrial fibrillation (AF). Immunosuppression can be effective in early stages, but not in the chronic phase. Our objective was to describe the characteristics of EMF patients in the Americas. This registry is a retrospective multicenter cross-sectional study including patients ≥18 years-old with EMF diagnosed by imaging methods, according to the Mocumbi criteria. Clinical, biochemical, and imaging variables were analyzed. On the 54 patients included, 28 (52%) were male with an age of 47 years. The etiology was idiopathic in 47 (87%) patients, familial in 4 (7%), and secondary to chemotherapy in 2 (3.5%). We detected a history of HF in 41 patients (76%), AF in 19 (35%), and ischemic stroke in 8 (15%). The diagnosis was made by echocardiography in all patients, and 38% had Cardiac Resonance or Computed Tomography. Thirty-five patients (65%) presented a left ventricular ejection fraction ≥50%, 11 (21%) severe mitral regurgitation, and 18 (33%) severe tricuspid regurgitation. In 17 patients (32%) the diagnosis was confirmed by endomyocardial biopsy. Among medical therapy, 72% received beta-blockers, 63% vasodilators, 65% mineralocorticoid antagonists, 7.4% SGLT2 inhibitors, and 11% corticosteroids. Subendocardial resection was performed in 9 (16%) patients and mitral valve replacement in 11 (20%) patients. In conclusion, EMF patients had a high prevalence of HF, AF, and embolic events. The diagnosis was frequently made in an advanced stage when HF management and surgery were the only effective treatments.
Endomyocardiofibrosis: A Systematic Review
2021, Current Problems in Cardiology
Citation Excerpt :
A restrictive filling pattern is commonly found, with prominent E-wave, short deceleration time of E-wave, and a small A-wave.54 Pericardial effusion is frequent.47-51 Cardiac magnetic resonance imaging, if available, confirms structural and functional findings of TTE, and late gadolinium enhancement technique detect apical fibro-thrombotic obliteration and mild apical hypertrophy better than TTE, useful to guide medical and surgical treatment when TTE is inconclusive (Figs. 5 and 6).52-56
Endomyocardiofibrosis was described first time in Uganda as an infrequent restrictive cardiomyopathy with a poor prognosis, characterized by fibrosis of the ventricular subendocardium and severe restrictive physiology leading to difficult therapeutic management and frequently associated with hypereosinophilic syndrome. Its higher prevalence in the tropics and its relationship in some cases with hypereosinophilic endocarditis has led to the search for genetic, infectious, autoimmune and nutritional causes, but its etiology remains unclear. It is a rare cardiomyopathy, difficult to diagnose and with a nonexistent effective treatment. Imaging methods such as echocardiography and cardiac magnetic resonance are essential for the initial diagnosis, although endomyocardial biopsy establishes the definitive diagnosis. Immunosuppressive treatment is only useful in the early stages of the disease and usually ineffective if installed late when signs of heart failure are present. Surgical treatment is generally palliative.
Loeffler's Endocarditis: An Integrated Multimodality Approach
2020, Journal of the American Society of Echocardiography
Citation Excerpt :
Transesophageal echocardiography may be a valid option in patients with poor transthoracic acoustic window, especially in the presence of a high suspicion of LE and when other second-level imaging modalities are not promptly available.31 Transesophageal echocardiography is the most accurate tool to assess the severity and mechanisms of MV regurgitation.19 In addition, TEE allows a more precise study of pulmonary veins; in the advanced stages of LE, pulmonary veins show a high diastolic (“D”) wave and a broad atrial reversal (“A”) wave caused by an increased end-diastolic LV pressure.
Loeffler's endocarditis (LE) is the cardiac manifestation of hypereosinophilic syndrome, a rare systemic disease characterized by the sustained production of eosinophils leading to organ damage. Few data, principally by case reports, are available regarding the diagnostic workup in patients with suspected LE. Thus, we have performed a systematic search of the literature dealing with imaging in LE and propose an integrated multimodality imaging approach in the cardiac diagnostics of LE patients. The aim is to provide an updated state-of-the-art review focused on noninvasive and invasive imaging modalities for this rare and underdiagnosed disease. Standard and advanced echocardiography are typically the first cardiac imaging examinations when LE is suspected and they are also used later in follow-up for prognostic stratification and assessing response to treatment. Cardiac magnetic resonance provides a more detailed anatomical and functional evaluation of cardiac chambers, tissue characterization for the presence and extension of myocardial edema and fibrosis, and ventricular thrombi identification. Computed tomography scan and [18F]-fluoro-deoxy-glucose positron emission tomography may be helpful in selected cases to evaluate the cardiac involvement of LE as well as the other noncardiac manifestations of hypereosinophilic syndrome. Endomyocardial biopsy may be considered in patients with high clinical suspicion of LE if noninvasive imaging findings are confusing or not conclusive. The appropriate use of invasive and noninvasive imaging modalities, combining the available techniques with the patients' clinical features, will hopefully lead to early diagnosis, more accurate staging of disease, and timely treatment of LE that may prevent the irreversible myocardial damage of LE and adverse cardiovascular events.
Right Heart-Pulmonary Circulation Unit in Cardiomyopathies and Storage Diseases
2018, Heart Failure Clinics
Tropical endomyocardial fibrosis: Perspectives
2018, Annales de Cardiologie et d'Angeiologie
La fibrose endomyocardique tropicale (FET) est la première cause de cardiomyopathie restrictive dans le monde. Elle se caractérise par une fibrose diffuse de l’endocarde ventriculaire responsable d’une déformation des ventricules et d’une insuffisance cardiaque restrictive. Confinée à certaines régions du monde, l’étiologie de la FET demeure mystérieuse. Les formes sévères sont greffées d’un très mauvais pronostic. Les changements épidémiologiques récemment constatés dans certains foyers endémiques et l’amélioration des moyens diagnostics ouvrent des perspectives dans la compréhension et le traitement de la maladie.
Tropical endomyocardial fibrosis (FET) is a leading cause of heart failure and the most common restrictive cardiomyopathy worldwide. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling and valvular insufficiency define the disease. Confined to peculiar and limited geographical areas, the aetiology remains blurred and it carries a grim prognosis. The changing burden reported recently in some endemic areas and the refinement of diagnostic tools have emphasized alternative routes for understanding and treatment of the disease.
Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series
2018, Journal of the American College of Cardiology
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, particularly primary and secondary forms of iron overload, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.

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^☆: Reprint requests: Clotilde Sara Berensztein, MD, FACC, FASE, Avenida Pueyrredón 1643, 3 ° A, 1118, Buenos Aires, Argentina (E-mail: [email protected] ).

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Original ArticlesUsefulness of Echocardiography and Doppler Echocardiography in Endomyocardial Fibrosis☆

Abstract

Section snippets

Patients

RESULTS

DISCUSSION

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Mayo Clin Proc

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Miocardiopatías restrictivas

Primary restrictive cardiomyopathy: nontropical endomyocardial fibrosis and hypereosinophilic heart disease

Br Heart J

Cardiovascular features of eleven patients with eosinophilic endomyocardial disease

QJM

The Cardiomyopathies and myocarditides: toxic, chemical and physical damage to the heart

Original Articles
Usefulness of Echocardiography and Doppler Echocardiography in Endomyocardial Fibrosis☆