Endomyocardial fibrosis associated with massive calcification of the left ventricle

This is the report of a rare case of endomyocardial fibrosis associated with massive calcification of the left ventricle in a male patient with dyspnea on great exertion, which began 5 years earlier and rapidly evolved. Due to lack of information and the absence of clinical signs that could characterize impairment of other organs, the case was initially managed as a disease with a pulmonary origin. With the evolution of the disease and in the presence of radiological images of heterogeneous opacification in the projection of the left ventricle, the diagnostic hypothesis of endomyocardial disease was established. This hypothesis was later confirmed on chest computed tomography. The patient died on the 16th day of the hospital stay, probably because of lack of myocardial reserve, with clinical findings of refractory heart failure, possibly aggravated by pulmonary infection. This shows that a rare disease such as endomyocardial fibrosis associated with massive calcification of the left ventricle may be suspected on a simple chest X-ray and confirmed by computed tomography.

Absence of the aortic valve associated with hypoplastic left-sided heart syndrome

In this report we describe the twelveth case in the literature of absence of the aortic valve cusps, associated with hypoplastic left-sided heart syndrome in a neonate. Clinical and hemodynamic conditions in our patient resemble the classical features of this syndrome except for a greater development of the ascending aorta and the left ventricular cavity, due to aortic insufficiency. A patch was unsuccessfully inserted at the aortic annulus to exclude the left ventricle from the circulation. In addition the Norwood operation was performed.

Outcome of acute renal failure associated with cardiac surgery in infants

OBJECTIVE: To analyze the impact of acute renal failure (ARF) on the evolution of infants undergoing cardiac surgery. METHODS: We assessed 15 infants undergoing cardiac surgery who developed (ARF). Their demographic, clinical and surgical data, and evolution were analyzed. RESULTS: Their mean age was 4.4±4.0 months (8 days to 24 months). Twelve infants were males, and 4 patients already had ARF at surgery. The primary cause of ARF was immediate acute cardiac dysfunction in 10 infants, cardiac dysfunction associated with sepsis in 2 infants, and isolated sepsis in 3 infants. All children depended on mechanical ventilation during their postoperative period, 14 infants used vasoactive drugs, and 11 had an infectious process associated with ARF. Thirteen infants required dialytic treatment. Eleven infants developed oluguric ARF, and all had to undergo peritoneal dialysis; of the 4 patients with non-oliguric, 2 required dialysis, the main indication being hypervolemia. Of these 13 dialyzed infants, 4 died in the first 24 hours because of the severity of the underlying cardiac disease (mean urea level of 49±20 mg/dl). The mortality rate for the entire group was 60% , and it was higher among the patients with oliguria ARF (73% vs 25%, p<0.001). The cause of death was acute cardiac dysfunction in 6 infants (early type-1ARF) and sepsis in the 3 remaining infants (late type-2 ARF). CONCLUSION: The mortality rate of ARF associated with cardiac surgery in infants was hight, being higher among children with oliguria; peritoneal dialysis was indicated due to clinically uncontrolled hypervolemia and not to the uremic hypercatabolic state.

Changes in the profile of lipoprotein subfractions associated with hormone replacement therapy

OBJECTIVE: To report the effects of 2 regimens of hormone replacement therapy during the postmenopausal period on the profile of the major lipoprotein subfractions (HDL, LDL, and VLDL). METHODS: We carried out a cohort study in 38 postmenopausal patients who were starting their hormone replacement therapy due to gynecological indications, for a period of 12 weeks. Analysis of lipoprotein subclasses was performed through nuclear magnetic resonance spectroscopy. RESULTS: Hormone replacement therapy cause an increase in the proportion of larger subfractions of VLDL and HDL (p=0.008 and 0.03, respectively) and in the proportion of larger particles of VLDL due to a 36% increase in the levels of larger particles (p=0.004), concomitantly with a 15% reduction in the levels of smaller particles (p=0.04). In regard to HDL, the increase occurred only a 17% increase in the levels of larger particles (p=0.002). No significant change occurred in the distribution pattern of LDL subfractions. CONCLUSION: The proportion of larger subfractions of VLDL and HDL increases after hormone replacement therapy. The increase in the proportion of larger particles of VLDL occurs due to an increase in the levels of the larger subclasses concomitantly with a reduction in the smaller particles. However, an increase in the proportion of larger particles of HDL occurs only due to an increase in the levels of the larger subfractions.

Characteristics associated with the dropout of hypertensive patients followed up in an outpatient referral clinic

OBJECTIVE: To determine the characteristics associated with the dropout of patients followed up in a Brazilian out patient clinic specializing in hypertension. METHODS: Planned prospective cohort study of patients who were prescribed an antihypertensive treatment after an extensive initial evaluation. The following parameters were analyzed: sex, age, educational level, duration of disease, pressure level used for classifying the patient, previous treatment, physical activity, smoking, alcohol consumption, familial history of hypertension, and lesion in a target organ. RESULTS: We studied 945 hypertensive patients, 533 (56%) of whom dropped out of the follow-up. The mean age was 52.3±12.9 years. The highest probabilities of dropout of the follow-up were associated with current smoking, relative risk of 1.46 (1.04-2.06); educational level equal to or below 5 years of schooling, relative risk of 1.52 (1.11-2.08); and hypertension duration below 5 years, relative risk of 1.78 (1.28-2.48). Age increase was associated with a higher probability of follow-up with a relative risk of 0.98 (0.97-0.99). CONCLUSION: We identified a group at risk for dropping out the follow-up, which comprised patients with a lower educational level, a recent diagnosis of hypertension, and who were smokers. We think that measures assuring adherence to treatment should be directed to this group of patients.

Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection

We report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. One of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. At the time of hospital admission, the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. The diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. The patients underwent surgery for anatomical correction of the heart disease. Only 1 patient had a good outcome.