Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.

Reduction in left ventricular hypertrophy in hypertensive patients treated with enalapril, losartan or the combination of enalapril and losartan

OBJECTIVE: To compare the regression of left ventricular hypertrophy in patients with moderate hypertension treated with enalapril, losartan or a combination of the two drugs at lower doses. METHODS: Patients of both sexes with moderate hypertension confirmed by ambulatory monitoring of arte-rial blood pressure and with left ventricular hypertrophy on echocardiogram were assigned to three groups: enalapril (35 mg/day, n=15), losartan (175 mg/day, n=15) and enalapril+losartan (15 mg+100 mg/day, n=16). The patients received the drugs for 10 months. RESULTS: The three therapeutic regimens were equally effective in reducing blood pressure and left ventricular mass index (LVMI, g/m²): 141±3.9 to 123±3.6 in the enalapril group (p<0.05), from 147±3.8 to 133±2.8 in the losartan group (p<0.05), and from 146±3.0 to 116±4.0 in the enalapril+losartan group (p<0.05). However, the percent reduction of LVMI was significantly greater (p<0.01) in the enalapril+losartan group (20.5±5.0%) than in enalapril (12.4±3.2%) and the losartan (9.1±2.1%) groups. Normalization of LVMI was obtained in 10 out of the 16 patients who received enalapril+ losartan, in 6 out of the 15 patients who received only enalapril and in 4 out of the 15 patients treated with losartan. CONCLUSION: The combination of an angiotensin-converting enzyme inhibitor and an angiotensin II receptor antagonist (AT1 receptor antagonist) in patients produced an additional effect on the reduction of left ventricular hypertrophy. This finding may depend on a more complete inhibition of the cardiac renin-angiotensin.

Apical aneurysm and left ventricular hypertrophy

A 59-year-old woman presented with an embolic transient ischemic attack and a history of controlled hypertension for 16 years. Both echocardiogram and MRI showed severe biventricular hypertrophy and an apical aneurysm with a thrombus. The occurrence of an apical aneurysm in the presence of cardiac hypertrophy is a rare finding and has been described in patients with hypertrophic cardiomyopathy. However, it has not been reported in patients with systemic arterial hypertension. In this patient the lack of a relationship between the severity of the hypertrophy and the levels of blood pressure, together with the presence of histologic disorganization of myocardial cardiac muscle cells by endomyocardial biopsy suggested the diagnosis of hypertrophic cardiomyopathy.

Echocardiographic assessment of the different left ventricular geometric patterns in hypertensive patients

OBJECTIVE: To identiy left ventricular geometric patterns in hypertensive patients on echocardiography, and to correlate those patterns with casual blood pressure measurements and with the parameters obtained on a 24-hour ambulatory blood pressure monitoring. METHODS: We studied sixty hypertensive patients, grouped according to the Joint National Committee stages of hypertension.. Using the single- and two-dimensional Doppler Echocardiography, we analyzed the left ventricular mass and the geometric patterns through the correlation of left ventricular mass index and relative wall thickness. On ambulatory blood pressure monitoring we assessed the means and pressure loads in the different geometric patterns detected on echocardiography RESULTS: We identified three left ventricular geometric patterns: 1) concentric hypertrophy, in 25% of the patients; 2) concentric remodeling, in 25%; and 3) normal geometry, in 50%. Casual systolic blood pressure was higher in the group with concentric hypertrophy than in the other groups (p=0.001). Mean systolic pressure in the 24h, daytime and nighttime periods was also higher in patients with concentric hypertrophy, as compared to the other groups (p=0.003, p=0.004 and p=0.007). Daytime systolic load and nighttime diastolic load were higher in patients with concentric hypertrophy ( p=0.004 and p=0.01, respectively). CONCLUSIONS: Left ventricular geometric patterns show significant correlation with casual systolic blood pressure, and with means and pressure loads on ambulatory blood pressure monitoring.

Contribution of Doppler echocardiography to the evaluation of systolic and diastolic function of obese women versus a control group

OBJECTIVE: To study by doppler echocardiography the cardiac systolic and diastolic functions of health, uncomplicated obese subjects. METHODS: Fifty-nine obese women with an average body mass index (BMI) of 35 kg/m² were evaluated and compared with 19 subjects with an average BMI of 23 kg/m² (control group). RESULTS: In the obese group, a clear tendency was observed toward higher systolic pressure, increased wall thickness and, consequently, myocardial mass, elevation on the circumference stress of the left ventricular wall, and an indisputable presence of diastolic abnormalities. Filling abnormalities were observed with impaired relaxation, with prolonged isovolumic relaxation time (IVRT) and augmented atrium contribution representing early indexes of cardiac dysfunction when systolic performance is still normal. CONCLUSION: Obesity is generally a chronic condition, and doppler echocardiography can be used as a noninvasive instrument for early evaluation of left ventricular diastolic indexes.

Submitral left ventricular aneurysm. Case report and review of published Brazilian cases

Submitral left ventricular aneurysm is a cardiac pathology widely recognized, but relatively unknown, occurred almost exclusively in African black patients. Although still this idea of racial prevalence exists, cases have been described in patients of all the races. Ten Brazilian cases were reported. One of them was presented inside an Italian paper that refers the surgical treatment of a Brazilian patient of black race. We reported one more submitral left ventricular aneurysm case in a brown female patient, with antecedents of peripheral thromboembolism initially not identified as consequence of the cardiac pathology.

Ventricular resynchronization through biventricular cardiac pacing for the treatment of refractory heart failure in dilated cardiomyopathy

OBJECTIVE: The biventricular pacing (BVP) approach has good results in the treatment of congestive heart failure (CHF) in patients (pts) with disorders of intraventricular conduction. METHODS: We have applied BVP to 28 pts, with left ventricular pacing using minitoracotomy in 3 pts and the transvenous aproach via coronary sinus in 25 pts. The mean duration of the QRS complexes was 187 ms, in the presence of the left branch block in 22 pts, and right branch block + divisional hemiblock in 6 pts. All pts had been considerated candidates to cardiac transplantation, and were under optimized drug therapy. Sixteen pts were in Functional Class (NYHA) IV, and 12 in class III. The ejection fraction varied from 22 to 46% (average = 34%). The pacing mode employed was biventricular triple-chamber in 22 pts, and bi-ventricular dual-chamber in 6 pts (one with ICD). RESULTS: The pts were followed up for a period that ranged from 10 days to 14 months (mean 5 months). All pts presented clinical improvement after implant, chaging the NYHA Functional Class at the end of follow-up to Class I (9pts), Class II (10 pts) and Class III (6 pts). The initial mean ejection fraction have-raised to 37%. Two pts died suddenly. One patient died due to a pulmonary fungal infection. CONCLUSION: Ventricular resynchronization through BVP, improved significantly the Functional Class and, therefore, the quality of life. Assessments of myocardial function acutely performed do not reflect the clinical improvement observed.

An analysis of electrocardiographic criteria for determining left ventricular hypertrophy

OBJECTIVE: To determine the most sensitive criterion for the detection of left ventricular hypertrophy according to echocardiographically defined left ventricular mass. METHODS: The Sokolow-Lyon voltage, Sokolow-Lyon-Rappaport, Cornell voltage duration product, White-Bock, and Romhilt-Estes point scoring criteria were compared with left ventricular mass index, corrected for body surface, obtained from the echocardiograms of 306 outpatients (176 females, 130 males), of all age groups. RESULTS: The Cornell voltage duration product criteria index had the greatest sensitivity in women (54.90%), and the Sokolow-Lyon-Rappaport index was most sensitive in men (73.53%). When applied to men at the same voltage amplitude (20mm) as that in women, the Cornell index showed increased sensitivity relative to the conventional index (28mm) of 67.65% (P<=0.01) and a sensitivity similar to that of the Sokolow-Lyon-Rappaport index, with higher specificity (P<=0.01). The White-Bock and Romhilt-Estes criteria were the least sensitive in men and women, despite their high specificity. The electrocardiographic criteria were more efficient when dilatation predominated over left ventricular hypertrophy. CONCLUSION: The Cornell index had greater sensitivity in women, and the Sokolow-Lyon-Rappaport index was more sensitive in men. When applied to men at the same voltage amplitude as that of women, the Cornell index had an increase in sensitivity similar to that of the Sokolow-Lyon-Rappaport index.

Obstruction of the left ventricular outflow tract after mitral valvuloplasty with implantation of Gregori's ring

Mitral valvuloplasty is efficient for repairing mitral valve disease with few complications. In some cases, obstruction of the left ventricular outflow tract may occur due to systolic anterior motion of the mitral valve. We report the case of a patient with this complication and a pressure gradient between the left ventricle and the aorta of 130 mm Hg after mitral valvuloplasty with implantation of a Gregori's ring. The management was clinical with suspension of the vasoactive drugs and introduction of a beta-blocker. Two years after the surgery, the patient is asymptomatic and has a normal life.

Correlations Between the Collagen Content of the Human Left Ventricular Myocardium, Measured by Biochemical and Morphometric Methods

OBJECTIVE: To assess, in myocardium specimens obtained from necropsies, the correlation between the concentration of hydroxyproline, measured with the photocolorimetric method, and the intensity of fibrosis, determined with the morphometric method. METHODS: Left ventricle myocardium samples were obtained from 45 patients who had undergone necropsy, some of them with a variety of cardiopathies and others without any heart disease. The concentrations of hydroxyproline were determined with the photocolorimetric method. In the histologic sections from each heart, the myocardial fibrosis was quantified by using a light microscope with an integrating ocular lens. RESULTS: A median of, respectively, 4.5 and 4.3 mug of hydroxyproline/mg of dry weight was found in fixed and nonfixed left ventricle myocardium fragments. A positive correlation occurred between the hydroxyproline concentrations and the intensity of fibrosis, both in the fixed (Sr=+0.25; p=0.099) and in the nonfixed (Sr=+0.32; p=0.03) specimens. CONCLUSION: The biochemical methodology was proven to be adequate, and manual morphometry was shown to have limitations that may interfere with the statistical significance of correlations for the estimate of fibrosis intensity in the human myocardium.

QRS Voltage-Duration Product in the Identification of Left Ventricular Hypertrophy in Spontaneously Hypertensive Rats

OBJECTIVE - Evaluation of the performance of the QRS voltage-duration product (VDP) for detection of left ventricular hypertrophy (LVH) in spontaneously hypertensive rats (SHR). METHODS - Orthogonal electrocardiograms (ECG) were recorded in male SHR at the age of 12 and 20 weeks, when systolic blood pressure (sBP) reached the average values of 165±3 mmHg and 195±12 mmHg, respectively. Age- and sex- matched normotensive Wistar Kyoto (WKY) rats were used as controls. VDP was calculated as a product of maximum QRS spatial vector magnitude and QRS duration. Left ventricular mass (LVM) was weighed after rats were sacrificed. RESULTS - LVM in SHR at 12 and 20 weeks of age (0.86±0.05 g and 1.05±0.07 g, respectively) was significantly higher as compared with that in WKY (0.65±0.07 g and 0.70±0.02 g). The increase in LVM closely correlated with the sBP increase. VDP did not reflect the increase in LVM in SHR. VDP was lower in SHR as compared with that in WKY, and the difference was significant at the age of 20 weeks (18.2mVms compared with 10.7mVms, p<0.01). On the contrary, a significant increase in the VDP was observed in the control WKY at the age of 20 weeks without changes in LVM. The changes in VDP were influenced mainly by the changes in QRSmax. CONCLUSION - LVM was not the major determinant of QRS voltage changes and consequently of the VDP. These data point to the importance of the nonspatial determinants of the recorded QRS voltage in terms of the solid angle theory.

Doppler tissue imaging to assess systolic function in Chagas' disease

OBJECTIVE: To assess the usefulness of Doppler tissue imaging (DTI) for evaluating the systolic function of chagasic patients with and without electrocardiographic abnormalities, in comparision with echocardiographic study. METHODS: We studied 77 patients divided into 3 groups as follows: group 1 - control; group 2 - chagasic patients with normal electrocardiographic findings; and group 3 - chagasic patients with abnormal electrocardiographic findings. The following parameters were assessed: left ventricular dimensions and ejection fraction, left atrial dimensions and diastolic function on echocardiography. Systolic velocity and regional isovolumic contraction time (IVCTr) of the septal, anterior, lateral, posterior and inferior left ventricular walls were assessed on DTI. RESULTS: Left ventricular cavitary dimensions, ejection fraction and DTI systolic wave showed significant differences between groups 1 and 3 and between groups 2 and 3, which were not found between groups 1 and 2. IVCTr allowed a statistically significant discrimination among the 3 groups. CONCLUSION: DTI allowed discrimination among the different groups assessed, being superior to echocardiography in identifying early abnormalities of contractility, and, therefore, potentially useful for detecting incipient myocardial alterations in chagasic patients with normal electrocardiographic findings.

Value of the radiological study of the thorax for diagnosing left ventricular dysfunction in Chagas' disease

OBJECTIVE: To determine the value of the radiological study of the thorax for diagnosing left ventricular dilation and left ventricular systolic dysfunction in patients with Chagas' disease. METHODS: A cross-sectional study of 166 consecutive patients with Chagas' disease and no other associated diseases. The patients underwent cardiac assessment with chest radiography and Doppler echocardiography. Sensitivity, specificity, and positive and negative predictive values of chest radiography were calculated to detect left ventricular dysfunction and the accuracy of the cardiothoracic ratio in the diagnosis of left ventricular dysfunction with the area below the ROC curve. The cardiothoracic ratio was correlated with the left ventricular ejection fraction and the left ventricular diastolic diameter. RESULTS: The abnormal chest radiogram had a sensitivity of 50%, specificity of 80.5%, and positive and negative predictive values of 51.2% and 79.8%, respectively, in the diagnosis of left ventricular dysfunction. The cardiothoracic ratio showed a weak correlation with left ventricular ejection fraction (r=-0.23) and left ventricular diastolic diameter (r=0.30). The area calculated under the ROC curve was 0.734. CONCLUSION: The radiological study of the thorax is not an accurate indicator of left ventricular dysfunction; its use as a screening method to initially approach the patient with Chagas' disease should be reevaluated.