Tropical endomyocardial fibrosis in India: A vanishing disease.

Tropical endomyocardial fibrosis in India was a common medical problem in the coastal districts of south India, especially the Kerala State. The clinical and autopsy studies have shown left and right ventricular apical fibrosis, with varying degree of atrioventricular valve regurgitation. Left ventricular endomyocardial fibrosis presents with severe pulmonary hypertension and right ventricular endomyocardial fibrosis presents very high systemic venous pressure and congestive cardiac failure. Surgical management improved the natural history of the disease to some extent. Various infectious and toxic factors were postulated regarding its aetiology. During the last few years, incidence of the disease has decreased considerably. The only explanation identified is the significant improvement in the living standards of the people with the corresponding decline in the childhood malnutrition, infections, worm infestation and associated eosinophilia.

Addressing healthcare needs of people living below the poverty line: A rapid assessment of the Andhra Pradesh Health Insurance Scheme.

Background. Families living below the poverty line in countries which do not have universal healthcare coverage are drawn into indebtedness and bankruptcy. The state of Andhra Pradesh in India established the Rajiv Aarogyasri Community Health Insurance Scheme (RACHIS) in 2007 with the aim of breaking this cycle by improving the access of below the poverty line (BPL) families to secondary and tertiary healthcare. It covered a wide range of surgical and medical treatments for serious illnesses requiring specialist healthcare resources not always available at district-level government hospitals. The impact of this scheme was evaluated by a rapid assessment, commissioned by the government of Andhra Pradesh. The aim of the assessment was to explore the contribution of the scheme to the reduction of catastrophic health expenditure among the poor and to recommend ways by which delivery of the scheme could be improved. We report the findings of this assessment. Methods. Two types of data were used for the assessment. Patient data pertaining to 89 699 treatment requests approved by the scheme during its first 18 months were examined. Second, surveys of scheme beneficiaries and providers were undertaken in 6 randomly selected districts of Andhra Pradesh. Results. This novel scheme was beginning to reach the BPL households in the state and providing access to free secondary and tertiary healthcare to seriously ill poor people. Conclusion. An integrated model encompassing primary, secondary and tertiary care would be of greater benefit to families below the poverty line and more cost-effective for the government. There is considerable potential for the government to build on this successful start and to strengthen equity of access and the quality of care provided by the scheme.

The cardio-protective diet.

Globalization has made calorie rich, cheap, convenient marketed foods the main menu for the common man. Indians are particularly susceptible to the adverse outcomes of this dietary change because of ethnic, epigenetic reasons and sarcopenic adiposity (less muscle more fat for the same body weight). Children have smaller body frame making them more susceptible to adverse effects of hyperglycaemia leading to stress on beta cells and their damage. This has resulted in escalation of lifestyle diseases by three-fold, that too at our younger age group at lower body mass indices. Preventive measures are necessary in early life to protect the beta cells, to achieve a metabolically healthy society. This will help in sustaining optimal beta cell function throughout a person’s life. Modification in dietary habits by educating the society, proper food labelling and legal regulation, restricting calorie, sugar, saturated fat, trans-fat and salt intake has proved its benefits in the developed world. Changes in the quality of food is as important as restricting calorie intake. This includes facilitation of increased consumption of dietary fiber, complex carbohydrates, nuts, fruits and vegetables. Restrictions are needed to reduce trans-fats, saturated fats and cooking habits such as deep frying which oxidizes cholesterol and lipids. Foods with long shelf-life shorten the life line because of their salt, sugar or trans-fat content. Individual meals need to be targeted in the general dietary guidelines, to minimize the post-prandial metabolic insult. In general, we need healthy start to early life particularly the first twenty years of life so that the habits cultured during childhood are sustained for the rest of productive years.

Prevalence-correlates-awareness-treatment and control of hypertension in kumarakom, kerala: baseline results of a community-based intervention program.

BACKGROUND, Hypertension is one of the major causes of cardiovascular morbidity and mortality. However, awareness, treatment, and control of hypertension remain major challenges worldwide. In this article, we present the baseline prevalence of hypertension from an ongoing intervention program for its control in a community-based sample in Kerala, Southern India. METHODS, We measured blood pressure, body weight, and height of 4955 individuals above the age of 30 yers (men;2159:mean-age: 50 years) and collected information on alcohol use, tobacco use, and other demographic variables using a pre-tested structured questionnaire. RESULTS, The overall prevalence of hypertension (JNC-VII) was 36.7% ( 95% CI:35.5-38.0; men: 36.0% and women 37.2% ) in multipile logistic regression analysis, a body mass index of >/=25 kg/m(2) was associated with a 1.65-fold (95% CI:1.37-1.98) prevalence of hypertension compared to a body mass index <25kg/m(2). Individuals with diabetes mellitus had 2.10 higher odds of hypertension prevalence (95% CI: 1.62-2.73) compared to people wihtout diabetes mellitus. Participants with increased waist circumference (90 cm in men, 85 cm in women) were 1.84 times more likely to be hypertensive compared to those with normal waist circumference (95% CI: 1.55-2.19). Among hypertensives, 24% were aware of the condition, 20% were on treatment, and 6.4% achieved effective blood pressure control. CONCLUSION, A higher body mass index, increased waist circumference, and self-reported diabetes mellitus were the important correlates of hypertension in our community-based sample. Our data emphasize the importance of educational interventions and appropriate lifestyle modifications that target increased body mass index and waist circumference to reduce the community burden of hypertension.

Long-term outcome of patients operated for large ventricular septal defects with increased pulmonary vascular resistance.

BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation.

Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.

Coexistence of congenital subaortic and sinus of valsalva aneurysms.

We report a rare association of an aneurysm of the sinus of Valsalva and a subaortic aneurysm. Congenital defective union of the aortic wall and ventricular muscle with the fibrous ring is probably the explanation for this condition.

Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study.

BACKGROUND: Coronary anomalies should be recognized to avoid problems during coronary intervention and cardiac surgery. METHODS AND RESULTS: We retrospectively reviewed 7400 coronary angiograms to find out the pattern and incidence of coronary anomalies of origin and distribution. We excluded patients with congenital heart diseases, coronary artery fistulae and patients with separate origin of the conus artery. and found 34 cases (0.46%) (22 males), mean age 50.7 +/- 12 years with coronary anomalies. Six cases underwent angiography prior to valve replacement and the rest were part of the evaluation for atherosclerotic coronary artery disease. The most common anomaly was separate origins of the left anterior descending coronary artery and left circumflex coronary artery [n=12 (35.3%)]. The next most common anomalies were origins of the right coronary artery from the left coronary sinus [n=7 (20.6%)] and left circumflex artery from the right sinus [n=6 (20%)]. A single coronary artery was seen in 3 cases (8.8%) which included one case of postmyocardial infarction ventricular septal rupture with triple-vessel disease, and another with two small coronary fistulae. One case each of the following coronary anomalies was found: (i) double right coronary artery, (ii) left anterior descending coronary artery from the right coronary sinus, (iii) all three coronary arteries originating separately from the right sinus, and (iv) left main coronary artery from the right sinus. Of these 34 patients, 11 (32.4%) had significant atherosclerotic disease in the anomalous vessel. CONCLUSIONS: The incidence of primary coronary anomaly seems to be less than that in earlier reports, but the pattern of anomalies appears to be similar.

Left ventricular thrombi in the presence of normal left ventricular function.

We report two cases of left ventricular thrombi identified by routine echocardiography in the presence of normal ventricular function to highlight the rarity and clinical significance of this condition. A 14-year-old boy, positive for anticardiolipin and antinuclear antibodies, was found to have a left ventricular thrombus. A 30-year-old male, who presented with a transient ischemic attack, was found to have hypereosinophilic syndrome and a mobile left ventricular thrombus. The thrombi disappeared in both patients after a few days of anticoagulant therapy without symptoms of embolization.

Serological diagnosis of human brucellosis: analysis of seven cases with neurological and cardiological manifestations.

Serum Tube Agglutination (STA) test was used as routine test to detect antibrucellar antibodies in diagnosis of brucella infection in sera (n = 75) and CSF (n = 14) from 78 patients with neurological (n = 60) and cardiological (n = 15) complaints in whom brucellosis was suspected, over a period of two and a half years from January, 1997 to July 1999. Seven (neurological-six and cardiac-one) serum samples (9.33%) were positive by STA, while none of the CSFs were positive. STA titres ranged from 1:10 to 1:1280. We report the findings of these seven cases with neurological and cardiac manifestations in whom STA were found positive. Treatment was accomplished in two cases (neurological-one and cardiac-one), while remaining five cases either were treated empirically with antitubercular treatment or lost for follow up. However these reported cases should alert clinicians to investigate for Brucella infection in cases of pyrexia of unknown origin and this condition in cases of chronic meningitis with unproven aetiology.

Coronary artery ectasia: angiographic, clinical profile and follow-up.

Out of 3200 coronary angiograms we reviewed, there were 144 cases of coronary ectasia--an incidence of 4.5 percent. Among these, 122 were associated with atherosclerotic coronary artery disease, i.e. coronary stenosis more than 50 percent (group A) and 22 not associated with coronary artery disease (group B). The patients in groups A and B were compared with age- and sex-matched patients (group C) (n=100) who had coronary artery disease alone without ectasia. The incidence of ectasia was not increased in patients with thoracoabdominal aortic aneurysm i.e. 2/154 (1.8%) or in patients with peripheral occlusive vascular disease i.e. 5/161 (3.1%). Ectasia was typed according to a modified version of the criteria proposed by Markis et al. Type II was the commonest, followed by type I, III and IV. Right coronary artery was the most commonly involved vessel by ectasia followed by left circumflex, left anterior descending artery and left main coronary artery. Diffuse ectasia was seen more frequently in right coronary artery and localised ectasia in left anterior descending artery. Patients in groups A and B had similar epidemiological characteristics, though more patients with ectasia alone (group B) had better left ventricular function and negative stress tests. The patients in group A had a similar incidence of previous myocardial infarction, coronary risk factor profile, treadmill exercise test status and severity of coronary artery disease when compared to group C. On a mean follow-up of 3+/-1.2 years, all the three groups had similar event rates.

Saccular coronary aneurysms: angiographic and clinical profile and follow-up of 22 cases.

Saccular coronary aneurysms are defined as aneurysms with the transverse dimension at least 1.5 times the longitudinal dimension. Out of 3,200 coronary angiograms reviewed, there were 22 patients (20 males) with saccular coronary aneurysms (totalling 25 aneurysms). The morphology of the aneurysms, the extent and severity of associated coronary lesions, the clinical profile and follow-up data of these patients were analysed. Aneurysms were located in left main coronary artery 3 (12%), left anterior descending coronary artery 13 (52%), right coronary artery 5 (20%) and left circumflex 4 (16%). There were 5 large aneurysms (> 15 mm in diameter) (1 in left main coronary artery, 2 each in right coronary artery and left anterior descending coronary artery) averaging 32 mm in size. Fifteen aneurysms had significant coronary artery stenosis located proximal to it, supporting the theory of post-stenotic dilatation as the aetiology of aneurysm formation. Two patients had associated muscle bridges distal to the aneurysm; 20 had atherosclerotic coronary artery disease and one had coronary artery ectasia. Five patients were lost to follow-up, which ranged from 1 year to 19 years (mean 5.3 +/- 4.1 years). No patient had history suggestive of rupture of the aneurysm on follow-up. Two patients had myocardial infarction in the territory of the vessel with the aneurysm. Rest of the patients were in NYHA class I/II. One large right coronary artery aneurysm was subjected to aneurysmectomy due to symptoms of tricuspid valve obstruction. One left main coronary artery aneurysm measuring 12 mm, on follow-up of 19 years increased in size to 45 mm, in addition the patient developed a right coronary artery aneurysm. Coronary risk factor profiles in the 20 patients with atherosclerotic coronary artery disease and aneurysms were similar to age- and sex-matched control population with atherosclerotic coronary artery disease without aneurysms.

Newborn screening in Singapore.

Neonatal screening in Singapore for G6PD deficiency started in 1965. Screening for congenital hypothyroidism started in 1981 as a pilot research program and by 1990, it became nationwide. Screening for congenital hypothyroidism is by the measurement of TSH in the cord serum with recall of those exceeding the 99th percentile, by about 3-4 days of life. Treatment is usually started within a week of life. Over 400,000 newborns have been screened and the screening rate has been about 99.95%. The incidence rate is about 1 in 3,000. G6PD activity in the cord blood is measured using semi-quantitative rapid screening tests. Those identified are physically protected from environmental triggers by keeping them in hospital for a variable period of time. Parents are counseled. Data obtained from 22,830 newborns from the National University Hospital revealed incidence rates of 1.62% in all newborns, 3.15% in males and 0.11% in females. The Chinese and Malay males had a higher (3.94% and 2.95%) incidence respectively when compared to the Indian males with (0.66% incidence). The application of the preventive measures has resulted in no report of kernicterus in the last 20 years. Our efforts are now focused on minimizing the recall rate in the case of hypothyroidism screening, reducing the period of stay in hospital in those with G6PD deficiency and considering the introduction of a PKU screening program in Singapore.

Mass newborn screening for glucose-6-phosphate dehydrogenase deficiency in Singapore.

Recognition of kernicterus as a significant contributor to newborn mortality and morbidity in G6PD deficient babies and the ease with which G6PD deficiency could be detected and kernicterus prevented by avoidance of triggers, led to the establishment of mass newborn screening for G6PD deficiency in 1965. G6PD deficient newborns are identified within a day of birth by measuring the enzyme activity in cord blood. They are then physically protected from triggers by keeping them in the hospital for the first 2 weeks of life after their parents are counseled. Enzyme activity is measured using Wong's in-house modification of the Bernstein's technique or the BM G6PD Deficiency Screening test based on the Beutler assay. Close to 1.6 million newborns, representing practically 100% of all births have been screened. Analysis of data from 22,830 newborns at the National University Hospital reveals an incidence of 1.62% in all newborns. 3.15% in males and and 0.11% in females. A distinct racial variation in the incidence of deficiency was observed in males: Chinese 3.94%, Malays 2.95% and Indians 0.66%. Intermediate deficiency was most frequently identified (1.83%) in Chinese females. With the preventive measures. the incidence of kernicterus has dropped dramatically and there has been, over the last 20 years, no reported cases of kernicterus in newborns with G6PD deficiency. We are now looking at issues like comparing different assay techniques and determining a shorter period of stay in hospital. We believe that all Asians, especially Chinese babies, should be screened for G6PD deficiency, irrespective of which country the child is born.

Clinical and angiographic profile and follow-up of myocardial bridges: a study of 21 cases.

Myocardial bridging describes an angiographic entity, which is any degree of systolic narrowing of a coronary artery observed in at least one angiographic projection. Among the cineangiograms of 3200 patients reviewed, there were 21 cases (19 males) of myocardial bridges--incidence of 0.6 percent. Of these, seven had hypertrophic cardiomyopathy, six had atherosclerotic coronary artery disease and remaining eight had no evidence of either. All 21 patients had myocardial bridges in proximal or mid left anterior descending coronary artery. In addition, one case of hypertrophic cardiomyopathy had whole posterior descending coronary artery under a myocardial bridge. Another case of hypertrophic cardiomyopathy had a short normal segment of 5 mm inside a long myocardial bridge of 35 mm (tandem myocardial bridges). The length of the bridges varied from 10 to 35 mm (mean 24.5 +/- 4.5 mm) and diameter stenosis during systole varied from 40-90 percent (mean 70 +/- 8%). Two patients had large saccular coronary aneurysms proximal to the muscle bridge. Four of the eight patients who had neither hypertrophic cardiomyopathy nor coronary artery disease presented with acute anterior wall myocardial infarction and three of them had regional wall motion abnormality of left descending territory. Of the six patients who had coronary artery disease, one had 60 percent left descending artery lesion and two had recanalized segments proximal to the bridge. Five of the above six patients had significant stenosis of other coronary vessels. Four patients were lost to follow-up (mean period 3.4 +/- 2 years). In the coronary artery disease group, one patient underwent coronary artery bypass graft surgery for 3-vessel disease including graft to left descending artery and one developed inferior wall myocardial infarction. The patients in the hypertrophic cardiomyopathy group and "no hypertrophic cardiomyopathy-no coronary artery disease" group were free of events at last follow-up. Long-term prognosis of isolated myocardial bridges appears to be excellent. Degree of systolic narrowing or length of myocardial bridge does not correlate with event rates on follow-up.