DK2985_C000 1..28 - AlSharqia Echo Club

Cardiomyopathy 237than moderate MR (or TR) or increased wall thicknessmay all suggest the presence of RCM. In contrast,finding a thickened or calcified pericardium rather suggestsconstrictive pericarditis.Besides echo, other diagnostic tools are available tohelp identify the correct diagnosis: hemodynamic pressuretracings from left and right heart catheterization, clinicalclues, thoracic imaging (MRI, CT), and endomyocardialbiopsy. An integrated approach is needed to find thecorrect diagnosis in the majority of the cases.E. AmyloidosisCardiac amyloidosis is the most frequent RCM. It iscaused by amyloid fibrils deposition in the myocardium,valve tissue, atrial septum, pericardium, and conductionsystem. Cardiac involvement is usually associated withthe primary and senile forms of amyloidosis.Echocardiographic features include a progressiveincrease in right and left ventricular wall thickness [discordantwith the presence of low QRS voltage on electrocardiography(ECG)], typically symmetrical with acharacteristic “speckled” appearance and normal-sized oreven small ventricular cavities (Fig. 10.24). The severityof the wall thickening is a prognostic marker. In addition,the systolic function is preserved until late in the course ofthe disease. Small to moderate pericardial effusion is alsocommonly encountered (Fig. 10.24). Of note, SAM andLVOT obstruction can sometimes coexist and be confusedwith HCM (39). A thickened atrial septum with multivalvularinvolvement (multiple regurgitation, usuallymild), again with low QRS voltage on ECG are usefulclues to the diagnosis of amyloidosis.Although a restrictive filling pattern is typicallyencountered in advanced disease, observational studieshave shown that in some patients the degree of diastolicdysfunction, which parallels disease progression mayevolve in time. The patients initially exhibit a delayedrelaxation pattern (“E/A” ,1, prolonged DT), progressingto a pseudonormal and finally to the typical restrictivefilling profile. The severity of diastolic dysfunctionencountered is highly correlated to the prognosis: theshorter the DT, the worse the survival (40).F. SarcoidosisSarcoidosis is a granulomatous disorder involving multiplesystems of unknown etiology. The lungs and mediastinallymph nodes are the most commonly involved, althoughvirtually any organ can be infiltrated by these noncaseatinggranulomas. Heart involvement is clinically present in5–10% of patients, but reaches 25% in autopsy series,suggesting that a high degree of suspicion is necessary inthese patients. Cardiac sarcoidosis may affect the ventricularseptum with secondary conduction abnomalities, thepapillary muscle with associated MR or the pericardium.Even more troublesome is the fact that sudden death canbe the first cardiac manifestation of the disease (41).Echocardiography is a useful tool for the assessment ofcardiac involvement and its severity. Myocardial sarcoidosisleads to fibrosis, resulting in increased stiffness, likeRCM, but with earlier decrease in systolic function andventricular dilatation as a rule. Regional wall motionabnormalities are frequent even in the absence of coronaryartery disease, and tend to involve the basal and midventricularsegments. This finding is believed to resultfrom the patchy involvement by granulomas. Ventricularaneurysm can also be found. Severe pulmonary sarcoidosiscan also lead to pulmonary hypertension and secondaryright ventricular hypertrophy with variable degree ofdysfunction, without myocardial involvement per se.(A)(B)PERARVLALVAoPEFigure 10.24 Transthoracic subcostal view of a patient with amyloidosis. Note the biatrial dilatation, the increased ventricular wallthickness with the characteristic sparkling appearance and the small circumferential pericardial effusion (PE) (Ao, aorta; LA, left atrium;LV, left ventricle; RA, right atrium; RV, right ventricle).