The aim of the present study was to describe the clinical and echocardiographic findings of ventricular noncompaction in adult patients. Fifty-three patients underwent complete clinical history, electrocardiogram, Holter and transthoracic echocardiogram. Forty patients (75%) were in class I/II of the New York Heart Association, and 13 (25%) in class III/IV. Ventricular and supraventricular escape beats were found in 40% and 26.4%, respectively. Holter showed premature ventricular contractions in 32% and sustained ventricular tachycardia in 7.5%. Ventricular noncompaction was an isolated finding in 74% of cases and was associated with other congenital heart disease in 26%. Noncompacted ventricular myocardium involved only left ventricle in 62% of the patients and both ventricles in 38%. The mean ratio of noncompacted to compacted myocardial layers at the site of maximal wall thickness was 3.4 ± 0.87 mm (range 2.2–7.5). The presence of ventricular noncompaction in more than three segments was associated with a functional class greater than II and ventricular arrhythmia with demonstrable statistical significance by χ2(p < 0.003).Conclusiona) Noncompacted cardiomyopathy is a congenital pathological entity that can occur in isolated form or associated with other heart disease and often involves both ventricles. b) A ratio of noncompacted to compacted myocardium greater than 3 and involvement of three or more segments are indicators of poor prognosis. c) Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular noncompaction and establish prognostic factors.
Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line threedimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens.
We undertook our study in order to evaluate right ventricular function and perfusion by conventional and contrast echocardiography in adults with congenitally corrected transposition who had not undergone cardiac surgery, comparing the echocardiographic findings with those obtained using equilibrium radionuclide ventriculography and gated single-photon emission computed tomography with Technetiumc-99 m sestamibi. We discovered severe tricuspid regurgitation in 8 patients (61%). Right ventricular ejection fraction, as calculated by nuclear medicine, had a correlation of 0.67 (p = 0.059) with area fractional shortening and 0.84 (p = 0.01) with ejection fraction calculated by the method depending on descent of the tricuspid ring. All patients with severe tricuspid regurgitation also had right ventricular dysfunction. Of these 8 patients, 7 had persistent perfusion defects, while 6 also had ischemic perfusion defects. Echo contrast had a high sensitivity, at 91%, and also specificity and positive predictive value, both at 100%, for persistent defects, and a negative predictive value of 66% compared to methods depending on nuclear medicine. The sensitivity of contrast echocardiography for detection of ischemic defects was 66%, the specificity 100%, the positive predictive value 100%, and the negative predictive value 77% compared to the methods involving nuclear medicine. The method depending on descent of the tricuspid ring had the highest correlation with equilibrium radionuclide ventriculography in evaluation of right ventricular function in patients with congenitally corrected transposition. We conclude that contrast echocardiography is extremely valuable when assessing right ventricular myocardial perfusion, having high sensitivity and specificity for detecting persistent defects, although sensitivity was less for detection of ischemic defects than that of gated single-photon emission computed tomography with Technetium-99 m Sestamibi. Persistent and ischemic perfusion defects, together with chronic volume overload from tricuspid regurgitation, are the determining factors of right ventricular dysfunction.
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