Biswajit Das

Late survival in Ellis–van Creveld syndrome – A case report

Ellis-van Creveld syndrome (EVC) is an autosomal recessive disorder characterized by chondrodystrophy, polydactyly, ectodermal dysplasia, and cardiac anomalies. Acromelic shortening of upper and lower limbs, genu valgum, multiple frenula, deformed teeth, short ribs and narrow thorax and congenital heart diseases complete the picture. The patients with the syndrome rarely survive into adulthood. Here, we report a lady with EVC presenting for the first time in middle age.

Multivessel giant coronary aneurysms: Case report and literature review

Abstract Coronary aneurysms have frequently been reported having varied incidence and etiology. Atherosclerotic coronary artery disease (CAD) is the predominant cause particularly in adults. Other causes include Kawasaki disease, Marfan syndrome, Behcet disease, and use of stents. Giant coronary aneuryms, measuring ≥8 mm in diameter, are rare entities. Multivessel coronary artery involvement is still rare. Exact pathogenetic mechanisms have not been defined although many hypotheses have been proposed. Prognosis is generally favorable. Management includes control of risk factors and surgical intervention. No consensus is there regarding exact management strategy. Here, we report a case of giant coronary aneurysms involving multiple coronary arteries, who presented with ST-segment elevated acute myocardial infarction.

Glycemic Control and Cardiovascular Outcomes in Diabetes

While type 1 Diabetes Mellitus (DM) is characterized by insulin deficiency due to pancreatic beta cell destruction, type 2 DM is characterized by a state of long standing insulin resistance (IR), compensatory hyperinsulinemia and varying degrees of elevated plasma glucose (PG), associated with clustering of cardiovascular (CV) risk and development of macrovascular disease prior to diagnosis of DM. Coronary artery disease (CAD) accounts for 70% of mortality and morbidity in patients with diabetes. Studies made in diabetes care have helped prevent or reduce microvascular complications in type 1 and 2 diabetes. However the same cannot be said about macrovascular disease. Despite all data concerning the association of diabetes and cardiovascular disease (CVD), the exact mechanism by which diabetes is linked to atherosclerosis is incompletely understood, this is especially true in case of hyperglycemia. The positive effect of intensive glucose management in comparison to non intensive glucose control is far from proven. DCCT and UKPDS studies have shown that while a glycemic control is important for reaching long term macrovascular complications, early glucose control is far more rewarding (metabolic memory). Later trials like ACCORD, ADVANCE and VADT don’t advocate tight glycemic control. In fact, ACCORD trial has shown increased mortality with tight glucose control. Tight glucose control may be beneficial in selected patients with short disease duration, long life expectancy and no CVD. In critically ill patients a blood glucose target of 140-180 mg% is fairly reasonable and achievable. The ESC/EASD guidelines of October 2013,Iike those of ADA, AHA and ACC continue to endorse a treatment target for glucose control in diabetes of HbAlc <7%, based predominantly on microvascular disease with acknowledged uncertainty regarding the effect of the intensive glucose control on CVD risk. Management of hyperglycemia in diabetics should not be considered in isolation; diabetics require multifactorial intervention for hypertension, dyslipidemia and microalbuminuria besides hyperglycemia. In fact combined use of antihypertensives, aspirin and lipid lowering agent makes it difficult to discern salutary effects of anti hyperglycemic therapy.

Percutaneous coronary intervention in a patient with single coronary artery

Coronary artery anomalies are found in 1-5% of all coronary angiograms. Single coronary artery is a rare congenital anomaly. The prevalence of the anomaly is 0.024-0.066% of the general population and percutaneous coronary intervention in this anomaly is performed infrequently. The highest incidence of this condition is reported from India. We report a case of a 55 year old patient of anterior wall ST elevation myocardial infarction with L1 group of single coronary artery who underwent successful angioplasty and stenting to left anterior descending artery. The unique features and inherent risks of percutaneous coronary intervention to single coronary artery are discussed.

Management of acute rheumatic fever—a re-appraisal

Abstract Rheumatic fever (RF) has fallen off the radar of many physicians, yet remains a daily challenge to many working in less developed areas of India. Rheumatic fever and rheumatic heart disease (RHD) are diseases of poverty. Overcrowding, low socio-economic status, and illiteracy contribute to the high prevalence. Treatment of the disease is mostly supportive as there is no disease-modifying therapy. Nonsteroidal anti-inflammatory drugs should be withheld until the diagnosis is confirmed, and corticosteroids are an option in severe and acute carditis. Most cases of chorea do not require medication, but use of carbamazepine or sodium valproate is recommended in severe cases. New approaches to primary prevention are needed given the limitations of primary prophylaxis as a population based strategy. The most effective approach for control of RF is secondary prophylaxis, which is best delivered as part of a coordinated control program. Group A streptococcal vaccines are still years away from being available.

Perspective on cardiorenal syndrome

Abstract The heart and kidneys share responsibility for maintaining hemodynamic stability and end-organ perfusion through a close relationship that controls volume status, cardiac output, and vascular tone. Cardiorenal syndrome is generally defined as a pathophysiologic disorder of the heart and kidneys whereby acute or chronic dysfunction of one organ induces acute or chronic dysfunction of the other. Five subtypes of cardiorenal syndrome are recognized, use of which helps characterize group of patients and provide the rationale for specific management strategies. Underperfusion from reduced cardiac output, venous congestion, increased intra-abdominal pressure, and activation of neurohormonal mediator play significant role in the pathophysiology of the disease. Treatment is often empirical and includes use of diuretics, natriuretic peptides, renin-angiotensin-aldosterone system inhibitors, inotropes, and ultrafiltration.

Oxidized-low density lipoprotein—a new target in the management of dyslipidemia and coronary artery disease

Abstract Role of cholesterol in alterations of normal blood flow and damage to the endothelium is a well-recognized phenomenon. Data from meta-analysis suggest that many individuals continue to demonstrate disease progression and develop clinical end-points in spite of LDL-C levels that are at target level. This has sparked considerable interest on the contribution of oxidative stress to cardiovascular events. According to the oxidation hypothesis of atherosclerosis, the LDL particle in its nature form is not atherogenic. However, oxidatively modified LDL can be taken up by macrophages via scavenger receptors, subsequently leading to formation of foam cells, endothelial dysfunction and progression of atherosclerosis. Several studies have shown strong correlation between ox-LDL and risk for subclinical atherosclerosis as well as myocardial infarction. Efforts are on to reduce ox-LDL, which will hopefully reduce the burden of atherosclerosis.