Usefulness of echocardiography and Doppler echocardiography in endomyocardial fibrosis

Objective: Our goal was to demonstrate the usefulness of echocardiography a nd cardiac Doppler echocardiography (echo-Doppler) in the diagnosis of endo myocardial fibrosis, an unusual restrictive cardiomyopathy in Argentina. Methods: Between 1980 and 1998, we studied 10 women (aged 27 to 58 years) w ith endomyocardial fibrosis confirmed by surgery and/or endomyocardial biop sy. Of the 10 cases of endomyocardial fibrothrombosis, 8 were biventricular and 2 were left ventricular. Six patients had only an echocardiographic st udy, 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 r ight heart failure at the first examination. Four patients died of heart fa ilure and 1 of overimposed sepsis. Surgery was successful in 2 patients wit h the biventricular form of the disease. In one of them, fibrotic decortica tion was performed In both ventricles together with tricuspid and mitral re placement. 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 a re 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 roo t" 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 hea rt with obliterated ventricles and dilated atria were seen in the whole gro up. 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 s ide 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 an d/or mitral regurgitation that was always mild or moderate. The fibrous thr ombus never altered the movement of the underlying myocardium. There were h ypoechoic and hyperdense echoes inside the fibrotic material (the latter co mpatible with calcium), and in all 10 patients, different grades of pericar dial effusion were found. Echo-Doppler showed the same minimal percentage o f change In mitral and tricuspid velocities as found in healthy patients, w hich clearly differentiates endomyocardial fibrosis from constrictive peric arditis. Furthermore, a restrictive pattern was observed on both atrioventr icular 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 o f the left atrium was diseased) caused by an increased end-diastolic left v entricular pressure to the same extent throughout the respiratory cycle. He patic veins showed a markedly deep diastolic forward wave throughout the re spiratory cycle and a marked reversal with inspiration. Conclusions: We showed (1) echocardiographic studies of a significant numbe r 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.