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.
BackgroundDown's syndrome (DS) is a genetic anomaly, which undergoes increased morbidity and mortality when associated with congenital heart disease (CHD). The aims of the study were to determine the prevalence of CHD and pulmonary hypertension (PH) in DS.MethodsOne hundred twenty-seven patients with DS living in Mexico City were evaluated by physical exam, electrocardiogram and echocardiogram.ResultsCHD was found in 40%. In 80% (n = 102) PH was present [systolic pulmonary artery pressure (SPAP) of 47 ± 19 mm Hg and mean pulmonary artery pressure (MPAP) of 32 ± 11 mm Hg]. Patients with CHD and PH were classified as having 1) no shunt (n = 18) with SPAP of 37 ± 9 mm Hg and MPAP of 25 ± 6 mm Hg and 2) with shunt (n = 26) with PASP of 57 ± 29 mm Hg and MPAP of 38 ± 19 mm Hg (p ≤ 0.001). In those without CHD or with CHD without shunt (n = 76), SPAP was 37 ± 19 mm Hg and the MPAP 25 ± 6 mm Hg. The prevalence of PH in DS was 5.9% at one year and 15% at 10 years. The odds ratio of PH in DS with CHD was 7.3 vs. 3 without CHD.ConclusionDS has a high prevalence of CHD and PH. PH prevalence increases when it is associated with CHD. The pathophysiology of PH in DS without CHD should be studied in the near future. Echocardiography is an indispensible tool for evaluation of DS.
Kidney transplant (KT) resolves many of the cardiac abnormalities associated with chronic kidney failure (CKF). This study analyzed cardiac alterations of kidney failure and their modification with transplant. Thirteen patients in CKF underwent conventional echocardiograms, dobutamine stress echocardiograms, and injection of contrast to examine perfusion before KT and 3 months after transplant. Nine patients had evidence of left ventricular hypertrophy and six had evidence of diastolic dysfunction. Wall thickness, left ventricular mass, and mass index diminished after KT; only two patients continued to manifest hypertrophy. Left ventricular systolic diameters and volumes diminished at 3 months, and diastolic diameters after 4 months. Left ventricular fractional shortening and ejection fraction increased 3 months after transplant. At the end of the study, only two patients continued to show diastolic dysfunction. Dobutamine echocardiograms showed no segmental wall-movement abnormalities. Myocardial perfusion was normal before and after transplant. The results suggest that KT diminishes hypertrophy and improves left ventricular systolic and diastolic function. Echocardiography provides valuable information for detection and follow-up of cardiac abnormalities in patients with kidney disease. Evaluation of segmental wall movement and myocardial perfusion aid in demonstrating that our studied patients with CKF had no indirect signs of coronary artery disease.
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