Only a few years ago my main complaint was the great dearth of medical publicat ions and indigenously generated medical data. I cannot make that complaint now. It seems that I do not even have time to read through the various small booklets and throw-away medical ‘newspapers’ that clutter my desk every week without end. Iut was that one ‘s complaint when there were so few publications and is everyone satisfied with the situation now? A shameful situation is it not for a country with only one Journal listed by the international Index Medicus and only three Journals recognized by the Pakistan Medical and Dental Council.

INTRODUCTION: Cariomyopathies are a heterogenous group of disorders characterized by heart muscle dysfunction (1, 2). Hypertrophic cardiomyopathi which is the second most common type of cardiomyopathy is. Now also known to have many clinico-pathological types including the rarest and the most recently recognized variety called “Apical Hypertrophic Cardiomyopathy” describe d by the Japanese 13). This variety which was thought to be peculiar to the Far East only has been noted to occur sporadically, in the western populations (4, 5, 6). This paper describes the occurrence of this relatively rare form of disease in Pakistan. DISCUSSION: Cardiomyopathies are a group of disorders affecting the heart muscle and of unknown or ill understood etiology and not secondary tb any known disease (I). As characterized by John Goodwin (2) and the W.H.O. (1) the two commonest varieties are the Dilated Cardiomyopathy which as the name implies is characterized by dilatation of the ventricles usually without any hypertrophy and a marked diminution in the systolic contractile function resulting in congestive heart failure and ultimately death – in simple terms systolic function failure. The use of amiodarone would appear to be most appropriate if significant arrythmja are documented. The natural history of this condition is not fully elucidated but may be better than some other forms of Hypertrophic Cardiomyopathy. However, in view of the serious nature of this class of diseases (15) these patients should be closely followed until further data is forthcoming.

In order to manage cardiac failure we need to classify cardiac failure into categories: a) Acute; history of onset in days or hours. • b) Chronic or recurrent; history of onset of more than 3 month duration. c) Sub acute; onset of symptoms of less than 3 month duration. Common causes in children are: . . • . a) Rheumatic valvular heart disease. b) Myocarditis (1% of all new Paediatric out patients at the National Institue of Cardiovascular diseases). • Common causes in adults are: a) Ischemic heart diseases and its complicat ions. b) Hypertensive he art disease. c) Rheumatic heart disease. d) Cardiomyopathies.

Cardiac rehabilitation (C.R.) should be part of routine management of all patients diagnosed to be suffering from a cardiovascular disorder. The WHO recognizes and endorses this. Cardiac rehab does not necessarily involve setting up of a sophisticated physical facility with expensive equipment. It is quite possible to diagnose, prognosticate, give a risk stratification, carry out a physical reconditioning program, educate patients and their families about the ailment, and, in short help rehabilitate the patient back into their environment with very little equipment or expense. What is sad is that while the developing countries and their population need such programs the most, these programs are most lacking in these very countries. Let no healthcare system in any country ofthe world enter the twenty first century without cardiac rehabilitation as part of routine management of all patients sufferingfrom any cardiovascular ailment.

Material and Methods: The study includes patients operated during period October, 1980 to November, 1981. The diagnosis was made on clinical, none—invasive echocardiographic and Cardiac Catheterization studies. Haemodynamic and angiographic studies were obtained in all patients undergoing Cardiopulmonary Bypass Surgery. The mortality was defined as intra-operat1e when patient died in the operating room, early if death occurred during the period of hospitalization and late, when death occurred 3 months postoperatively following discharge from the hospital. Results: A total of 507 patients were operated. One hundred and fifty eight of these had congenital cardiac malformations. I he age ranged between 0.3—60 years. One hundred and three patients were less than 1 2 years of’ age at the time of operation and fifty five were (Tale I) greater than 12 years. Discussion: Our data show that significant number of our patients with congenital cardiac defects arc adolescent or adults. This is due to lack of early detection. In most medically organized SOCICtICS the diagnosis and treatment of Con genital Cardiac defects is undertaken within the first year of life. IntraCardiac repairs of congenital defects the newborn and infants is not yet done at 0ur Institute. Even in Western Countries only a few cent res are aquipped to deal with this group of patientsl3. We however perform shunt surgery for Cyanotic infants who have pulmonary stenosis and reduced Pulmonary blood flow. Our present experience gives us a frame work to develop increased expertise and high-lights areas needing improvement.

The era of balloon valvuloplasty has started in Pakistan (Pakistan Heart Journal Vol. 20 No. 4, Oct. —. Dec. 1987. Percutaneous Balloon Pulmonary Valvuloplasty: A. La!, A. Faruqui and K. Aziz). Presently, balloon valvuloplasties are being routinely done at least in Karachi and Lahore. What then appears the status and future of balloon valvuloplasty in our environment? As Tricuspid Stenosis is a rarer situation, for the most part we are talking about Aortic Stenosis, Mitral Stenosis and Pulmonic Stenosis. Let us examine each of these situations. Balloon dilatation of Aortic Stenosis in adults can be done reasonably safely and produces adequate immediate relief of gradient. However, the long term followup has shown a very high rate of recurrence of Aortic Stenosis within six months. While balloon dilatation for Aortic Stenosis remains a viable alternative, just like Aortic Valvotomy in adults, it may prove increasingly disappointing. In conclusion, Balloon Valvuloplasty has made a significant impact in the way purely stenotic valve lesions are being treated. Ballooning is the treatment ofchoice for Pulmonary Stenosis. It is an option in some cases of Aortic Stenosis. Finally, despite the theoretic advantage, Ballooning is not cost effective in our circumstances for the treatment of Mitral Stenos is.

There can be no two opinions on a simple assertion that education should be in one’s mother tongue. As such there should have been no necessity of a debate on whether our higher education including Medic /ne should be fri !Jrdu or not. Why then does this issue crop up every n o w an d th en? Firs t/y, a t pres en t the s ys tern o f higher edu ca tio n is in English, th e tea ch ers ha ye been train ed in English speaking c o Un tn es, the textbooks are in English and any big and fundamental change is opposed by the very magnitude of the undertaking. Finally then should Medicine be taught in Urdu? The answer is a very definite “YES”. But for now we must ask a more immediately pertinent question “Can Medicine be taught in Urdu presently?”. To this the sad answer is “No”!

No other field in modern medicine is perhaps as labour intensive as well as capital intensive as Cardiology. Also, no other field of medicine is anywhere as innovative and as rapidly evolving as Cardiology. To top this Hi-Tech explosion is the fact that for developing countries like Pakistan everything from drugs and other consumables to hard-ware to experties is imported.The problems are defined enough and to an extent know to all and sundry.If we fail to solve our problem and evolve a cardiology and priorities best suited to us, then some one else will impose it on us or even worse: chaos will reign. I still have hope.

This writer had the opportunity recently to review most of the Cardiology related data that has been pub//shed in Pakistan. It was surprising to find isolated pieces of works of excellence that are not well known and never quoted. However, much o f what is available represents fragmented data lacking in scientific design and therefore open to criticism and unreliable. Constant. self criticism and peer review are going to be the only way we shall ever be able to achieve an objective and scientific approach to our problems. • Petty considerations should not be a//owed to keep us from achieving• this end.

MATERIAL AND METHODS: Bet ween Dec. ‘78 and Feb. ‘80 270 patient seen in the OPD and wards of N.J .C.V.D. had Echocardiography evaluatior 38 patients were found to have isolatei mitral valve prolapse (MVP). Also, 18 patient had MVP with some other associated abnor mality. Of the Patients with MVP, 20 (53 had a pre-echo suspicion of MVP. There is some controversr regarding tie exact echo diagnosis of MVP, as a iarge spectrum of abnor?naiities mis been reported. v”v e rpade a definitive diagnosis of MVP 0fli when one of the above two patterns were recorded. Care was taken to eliminate the chances of artifactually producing the prolapse pattern by steep angulat ion upwards from the apex. Results: Total No. of patients 38. Age : ranged from 3-55 years, mean 24 years. Majority of the patients were between 17-30 years. Sex.’ Males 17, Females 21. Discussion: Mtral Valve Prolapse syndrome as anecdo-.. tally considered to be rare in Pakistan and a monograph was read at one of the local syrn posium presenting a few cases confirmed by phonocardiography (7). Treatment involves mostly reassurance and permission to lead a full life, occasionally use of propranolol for symptoms and bacterial endocaditis prophylaxis in those with a clear cut syctnlic murmur. A lot ciisabilitv and anxIety can be relieved by recognition of this disease which s compatible with long term survival and good prognosis.

A design of the Planning of Cardiovascular disease (CVD) risk factor modification study in a lower-middle urban community in Pakistan is presented. The objective of the planned study is to determine whether known strategies for CVD risk factor modification can be implemented in lower middle class communities in Pakistan and whether increased knowledge and awareness of CVD can be affected by education. The secondary goal is modification of behavior by affecting 33% reduction in cooking fats and 25% reduction in salt consumption. The study design involves registration, clusterization and assignment of ccontrol and intervention cluster groups by computer randomization process. Intervention would be instituted by household visits of social workers of the National Institute of Cardiovascular Diseases. Composition of Sample size, power of the study and methods of data collection and analysis are discussed. In conclusion methodology of planning of a CVD risk factor modification study for a low middle class urban community is presented.

Objective— To examine variants at the 9p21 locus in a case-control study of acute myocardial infarction (MI) in Pakistanis and to perform an updated meta-analysis of published studies in people of European ancestry. Methods and Results— A total of 1851 patients with first-ever confirmed MI and 1903 controls were genotyped for 89 tagging single-nucleotide polymorphisms at locus 9p21, including the lead variant ( rs1333049 ) identified by the Wellcome Trust Case Control Consortium. Minor allele frequencies and extent of linkage disequilibrium observed in Pakistanis were broadly similar to those seen in Europeans. In the Pakistani study, 6 variants were associated with MI ( P <10 −2 ) in the initial sample set, and in an additional 741 cases and 674 controls in whom further genotyping was performed for these variants. For Pakistanis, the odds ratio for MI was 1.13 (95% CI, 1.05 to 1.22; P =2×10 −3 ) for each copy of the C allele at rs1333049 . In comparison, a meta-analysis of studies in Europeans yielded an odds ratio of 1.31 (95% CI, 1.26 to 1.37) for the same variant ( P =1×10 −3 for heterogeneity). Meta-analyses of 23 variants, in up to 38 250 cases and 84 820 controls generally yielded higher values in Europeans than in Pakistanis. Conclusion— To our knowledge, this study provides the first demonstration that variants at the 9p21 locus are significantly associated with MI risk in Pakistanis. However, association signals at this locus were weaker in Pakistanis than those in European studies.

Background— Evidence is sparse about the genetic determinants of major lipids in Pakistanis. Methods and Results— Variants (n=45 000) across 2000 genes were assessed in 3200 Pakistanis and compared with 2450 Germans using the same gene array and similar lipid assays. We also did a meta-analysis of selected lipid-related variants in Europeans. Pakistani genetic architecture was distinct from that of several ethnic groups represented in international reference samples. Forty-one variants at 14 loci were significantly associated with levels of HDL-C, triglyceride, or LDL-C. The most significant lipid-related variants identified among Pakistanis corresponded to genes previously shown to be relevant to Europeans, such as CETP associated with HDL-C levels (rs711752; P <10 −13 ), APOA5/ZNF259 (rs651821; P <10 −13 ) and GCKR (rs1260326; P <10 −13 ) with triglyceride levels; and CELSR2 variants with LDL-C levels (rs646776; P <10 −9 ). For Pakistanis, these 41 variants explained 6.2%, 7.1%, and 0.9% of the variation in HDL-C, triglyceride, and LDL-C, respectively. Compared with Europeans, the allele frequency of rs662799 in APOA5 among Pakistanis was higher and its impact on triglyceride concentration was greater ( P -value for difference <10 −4 ). Conclusions— Several lipid-related genetic variants are common to Pakistanis and Europeans, though they explain only a modest proportion of population variation in lipid concentration. Allelic frequencies and effect sizes of lipid-related variants can differ between Pakistanis and Europeans.

The burden of coronary heart disease (CHD) is increasing at a greater rate in South Asia than in any other region globally, but there is little direct evidence about its determinants. The Pakistan Risk of Myocardial Infarction Study (PROMIS) is an epidemiological resource to enable reliable study of genetic, lifestyle and other determinants of CHD in South Asia. By March 2009, PROMIS had recruited over 5,000 cases of first-ever confirmed acute myocardial infarction (MI) and over 5,000 matched controls aged 30–80 years. For each participant, information has been recorded on demographic factors, lifestyle, medical and family history, anthropometry, and a 12-lead electrocardiogram. A range of biological samples has been collected and stored, including DNA, plasma, serum and whole blood. During its next stage, the study aims to expand recruitment to achieve a total of about 20,000 cases and about 20,000 controls, and, in subsets of participants, to enrich the resource by collection of monocytes, establishment of lymphoblastoid cell lines, and by resurveying participants. Measurements in progress include profiling of candidate biochemical factors, assay of 45,000 variants in 2,100 candidate genes, and a genomewide association scan of over 650,000 genetic markers. We have established a large epidemiological resource for CHD in South Asia. In parallel with its further expansion and enrichment, the PROMIS resource will be systematically harvested to help identify and evaluate genetic and other determinants of MI in South Asia. Findings from this study should advance scientific understanding and inform regionally appropriate disease prevention and control strategies.

The emerging Cardiovascular Diseases are becoming leading cause of death in the developing countries. The incidence of coronary heart disease in Pakistan is not well established. The aim was to report the prevalence of coronary heart disease in an urban Pakistani Community and to evaluate the awareness of coronary heart disease and to determine life styles of the community.Material: Metroville a suburb of Karachi was selected, it has 4296 household population. After open invitation 398 households agreed to participate in an intervention study. The data obtained at baseline is basis of this report. Subjects >= 18 years age were 1078 while 382 males and 343 females were > 30 years age. Physical exam height, weight, BMI, ECG, waist circumference, blood pressure were determined. Questionnaire was administered to evaluate life styles and awareness in face to face interviews.Results: Household data showed 1.24 families per household with 3.98 adults and 4.26 children. Uneducated were 27.5% while 26.3% had 10 years as more schooling. Most had job. By history the prevalence of heart attack was 8.2% in women and 4.5% in men, Over all 6.2%, Stroke 2.6, hypertension 26.7% and diabetes 9.5%.Abnormal ECG suggesting myocardial infarction or ishaemia prevalence rate was 4.4 percent. awareness that heart attack was major problem was reported in 40% men and 25% women who strongly agreed while 31% men and 35% agreed that heart attack can not be prevented. Food and its linkage to coronary heart disease showed majority were aware of organ meat, fat and obesity linkage to coronary heart disease.Life Styles: Physical activity was mostly confined to walking stairs at home and shopping trips. Tobacco was used by 34.3% men and 6.2% women.Conclusion: Coronary heart disease prevalence was significant in an urban Karachi community and the prevalence had increased over the past decades. Smoking, obesity were prevalent. The community had sedentary life style.

Background: We conducted an observational study to determine the delay in presentation to hospital, and its associates among patients experiencing first Acute Myocardial Infarction ( AMI) in Karachi, Pakistan.Methods: A hospital based cross-sectional study was conducted at National Institute of Cardiovascular Disease (NICVD) in Karachi. A structured questionnaire was used to collect data. The primary outcome was delay in presentation, defined as a time interval of six or more hours from the onset of symptoms to presentation to hospital. Logistic regression analysis was performed to determine the factors associated with prehospital delay.Results: A total of 720 subjects were interviewed; 22% were females. The mean age (SD) of the subjects was 54 (+/- 12) years. The mean ( SE) and median (IQR) time to presentation was 12.3 (1.7) hours and 3.04 (6.0) hours respectively. About 34% of the subjects presented late. Lack of knowledge of any of the symptoms of heart attack ( odds ratio (95% CI)) (1.82 (1.10, 2.99)), and mild chest pain (10.05 (6.50, 15.54)) were independently associated with prehospital delay.Conclusion: Over one-third of patients with AMI in Pakistan present late to the hospital. Lack of knowledge of symptoms of heart attack, and low severity of chest pain were the main predictors of prehospital delay. Strategies to reduce delayed presentation in this population must focus on education about symptoms of heart attack.

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.