Anatomically corrected malposition of the great arteries.

A rare case of anatomically corrected malposition of the great arteries with bilaterally absent conus and ventricular septal defect is presented. Embryogenic mechanism responsible for the malformation, diagnostic problems of imaging and the increased risk posed for intraventricular surgical repair of associated defect are discussed here.

Estimation of pulmonary vascular resistance with Doppler diastolic gradients.

This study was undertaken to determine the diastolic Doppler echocardiographic correlates of pulmonary vascular resistance calculated on cardiac catheterization in patients with secondary pulmonary arterial hypertension. Thirty-eight consecutive patients with congenital heart disease, pulmonary artery hypertension and pulmonary regurgitation were studied. Continuous-wave Doppler-derived pulmonary artery diastolic gradients were measured at 3 points on the pulmonary regurgitant diastolic velocity slope: peak diastolic, end-diastolic (at the R wave on the electrocardiogram), and mid-diastolic (midway between the peak and end-diastolic points). Catheterization data included oximetry, measurements of pressure in the cardiac chambers and great arteries, and calculation of pulmonary vascular resistance index. Doppler-derived peak, mid, and end-diastolic pulmonary regurgitation gradients correlated best with catheterization-measured pulmonary artery systolic, mean and diastolic pressures, respectively. The best Doppler correlate of pulmonary vascular resistance index was the pulmonary artery end-diastolic gradient. Clinically useful information can be obtained from Doppler pulmonary artery diastolic gradients measured on the pulmonary regurgitant diastolic velocity slope, which can estimate the pulmonary arterial pressure as well as pulmonary vascular resistance obtained on cardiac catheterization.

High rates of obesity and cardiovascular disease risk factors in lower middle class community in Pakistan: the Metroville Health Study.

OBJECTIVES: To describe the distribution of over weight and body mass index, waist circumference and waist/ hip ratio, correlate obesity measures to coronary heart disease risk factors in comparison to Pakistan National Survey (PNS). METHODS: The Metroville Health Study (MHS) was an urban risk factor reduction intervention study in Metroville Karachi. Base line data was used which was not a random sample. Demographic data including serum cholesterol, glucose, haemoglobin, and blood pressure were collected. RESULTS: In MHS high cholesterol was 16% and 24% in men and women respectively, and 25% had hypertension. Self-reported diabetes was 8%, over-weight/obesity 34% and 49% for men and women, compared to 16% and 25% for PNS, while high risk waist-hip ratio (WHR) was present in 41% and 72% of men and women respectively. Under-weight in Metroville men was 12% and 9% in women, compared to 26% and 27% in PNS. The anthropometry variables were significantly correlated with each other while weight was significantly correlated with TC and waist circumference (WC). CONCLUSION: Obesity was alarmingly prevalent in urban Metroville in comparison to PNS. Cardio Vascular Disease (CVD) risk factors were prevalent in Metroville and TC and WC were significantly correlated with obesity measures. For prevention of increasing CVD in urban communities, targeted programs of intervention are required.

An echocardiographic index for decompensation of the chronically volume-overloaded left ventricle in children.

AIMS: The criterions for the timing of surgical intervention in children with rheumatic mitral or aortic valvar regurgitation are not defined. I hypothesized that, in children with chronic mitral or aortic regurgitation, an index for decompensation could be created by using the ratio of the diastolic left ventricular wall thickness to the radius, and that such an index could prove useful in determining the optimal time for surgical intervention. METHODS: The left ventricular echocardiograms were obtained at the tips of the leaflets of the mitral valve by M-mode echocardiography. The diastolic septal wall thickness was measured between the right and left ventricular endocardial layers, and the posterior wall thickness between the endocardium and the interphase between the epicardium and the myocardium. The left ventricular diastolic dimension was then measured, between the posterior and septal wall endocardial layers, and systolic dimension as the smallest distance detected between these layers. All diastolic measurements were made at the time of the R wave of electrocardiogram, using the leading edge technique. The ratio of wall thickness was measured using the mean of septal and posterior wall thicknesses divided by half the diastolic dimensions, the normalized thickness of the wall previously referred to as the h/r ratio and relative mural thickness. RESULTS: The ratio of wall thickness to left ventricular radius, and its relation to systolic left ventricular pressure or systolic blood pressure, was found to be linear in 89 normal school children, and 39 children with aortic stenosis. For future predictions, I calculated the 95th percentile limits and the 95th percentile confidence bands for this relation. Using the same data, it proved possible to calculate ratios of wall thickness for various ranges of either systolic blood pressure or left ventricular peak pressure. By using the normal limits of 0.356 plus or minus 0.0316 of the ratio, appropriate for the systolic blood pressure of children with mitral regurgitation, I determined the adequacy of the ratio of wall thickness. Of the children, 51 were in ventricular failure, and these had an inadequate ratio, below two standard deviation. Of the others, 21 had an inadequate ratio to within minus one to minus two standard deviations, and 12 of these were asymptomatic, 8 were symptomatic, but only one was in ventricular failure. For 18 children with aortic regurgitation, using the same limits, one child was within 1 standard deviation and was asymptomatic, 8 fell within minus 1 to minus 2 standard deviations and 2 of these were symptomatic, 5 were in ventricular failure, and 1 was asymptomatic, while the other 9 had ratios falling less than minus 2 standard deviations, and all were in ventricular failure. CONCLUSION: I conclude that the index of normalized wall thickness defined as the ratio of the left ventricular wall thickness to its radius is adequate, and within normal limits, when there is compensated volume overload, but is inadequate and below normal limits when the volume overloaded left ventricle becomes decompensated. My data suggests that the persistently decreasing ratio of wall thickness below the limits of normality serves as an indicator of ventricular decompensation, and thus can be used as a new criterion for determining the optimal time for surgical intervention.

Evaluation and comparison of blood pressure profiles of Pakistani cohort of children.

OBJECTIVE: To determine the blood pressure profile of children in Metroville, a lower middle class urban community of Karachi, Pakistan, and compare it to Pakistan National Health Survey of children. DESIGN: A research survey. PLACE AND DURATION OF STUDY: The study was conducted by National Institute of Cardiovascular Diseases, Karachi from 1996 2002. SUBJECTS AND METHODS: The blood pressure data was generated at the base line examination of MHS and was used to define the BP profile of urban Metroville children, age 2-17 years. Similar data of Pakistan National Health Survey (PNHS) undertaken by Pakistan Medical Research Council (PMRC), was used for comparison. RESULTS: A comparison of the blood pressure profile of the MHS with that of the PMRC showed that blood pressure was lower in the MHS. Additionally, comparison of the PMRC profile with USA data showed higher diastolic pressure in the PMRC. Comparison of Pakistani profiles with European data also showed higher blood pressure in Pakistani children. CONCLUSION: It is concluded that the MHS represents the BP profile of children in newly emerging lower middle class urban communities in Pakistan. PMRC data represents national average and can be used to define the blood pressure characteristics of urban communities similar to Metroville. The comparison with affluent countries highlighted the urgent need for community based preventive programs to combat hypertensive diseases in Pakistan.

Clinical profile of acute rheumatic fever in Pakistan.

We designed a multi-hospital prospective study of children less than 12 years to determine the comparative clinical profile, severity of carditis, and outcome on follow up of patients suffering an initial and recurrent episodes of acute rheumatic fever. The study extended over a period of 3 years, with diagnosis based on the Jones criteria. We included 161 children in the study, 57 having only one episode and 104 with recurrent episodes. Those seen in the first episode were differentiated from those with recurrent episodes on the basis of the history. The severity of carditis was graded by clinical and echocardiographic means. In those suffering their first episode, carditis was significantly less frequent (61.4%) compared to those having recurrent episodes (96.2%). Arthritis was more marked in the first episode (61.4%) compared to recurrent episodes (36.5%). Chorea was also significantly higher in the first episode (15.8%) compared to recurrent episodes (3.8%). Sub-cutaneous nodules were more-or-less the same in those suffering the first (7%) as opposed to recurrent episodes (5.8%), but Erythema marginatum was more marked during the first episode (3.5%), being rare in recurrent episodes at 0.9%. Fever was recorded in approximately the same numbers in first (45.6%) and recurrent episodes (48.1%). Arthralgia, in contrast, was less frequent in first (21.1%) compared to recurrent episodes (32.7%). A history of sore throat was significantly increased amongst those suffering the first episode (54.4%) compared to recurrent episodes (21.2%). When we compared the severity of carditis in the first versus recurrent episodes, at the start of study mild carditis was found in 29.8% versus 10.6%, moderate carditis in 26.3% versus 53.8%, and severe carditis in 5.3% versus 31.8% of cases, respectively. At the end of study, 30.3% of patients suffering their first episode were completely cured of carditis, and all others showed significant improvement compared to those with recurrent episodes, where only 6.8% were cured, little improvement or deterioration being noted in the remainder of the patients. We conclude that the clinical profile of acute rheumatic fever, especially that of carditis, is milder in those suffering their first attack compared to those with recurrent episodes.