Vikas Agrawal

Tetralogy of Fallot with Holt-Oram syndrome

Holt-Oram syndrome (HOS) is characterised by mild to severe congenital cardiac defects and skeletal abnormalities of the upper limb. This syndrome is also referred to as Hand-Heart syndrome. The most common cardiac disorder is an ostium secundum detected an atrial septal defect (ASD), followed by ventricular septal defect (VSD) and ostium primum ASD. We report a case of HOS with tetralogy of Fallot (TOF). This association is very rare and is hardly reported in the literature.

Metoprolol vs ivabradine in patients with mitral stenosis in sinus rhythm

BACKGROUND Severe mitral stenosis is usually symptomatic and is treated by BMV or surgery, whereas mild to moderate mitral stenosis is usually asymptomatic or mildly symptomatic and managed medically. Patients in the later group may become symptomatic during episodes of exercise and increased heart rate. Beta-blockers are frequently used in patients with mitral stenosis to control the heart rate and alleviate exercise-related symptoms. The objective of our study was to investigate the comparative efficacy of ivabradine versus metoprolol in patients with mitral stenosis in sinus rhythm. METHODS We studied 97 patients of mitral stenosis in sinus rhythm presented with exertional symptoms. The effectiveness of Metoprolol was compared with ivabradine in alleviating these exertional symptoms in a randomized, open label non crossover study. We also assessed various stress ECG parameters, 24 hour Holter parameters and 2D Echo parameters to objectively compare the effects of ivabradine and metoprolol in these patients. RESULTS Ivabradine and metoprolol both were effective in controlling exertional symptoms. Significant improvement in objective parameters like TMT (work capacity, baseline heart rate and maximal heart rate) and 2D echocardiography (right ventricular systolic pressure) are seen with both drugs. Ivabradine controls the exertional symptoms significantly more than metoprolol. On head to head comparison there was a significant benefit of working capacity and heart rate at maximal exercise in favour of ivabradine. CONCLUSIONS Ivabradine should be strongly considered in medical management of mitral stenosis patients where beta blockers are contraindicated such as reactive airway disease. The cost of ivabradine is higher than metoprolol which might possess constraints as most of the rheumatic heat disease patients belong to low socio economic status.

Clinical Profile with Angiographic Correlation in Naïve Acute Coronary Syndrome.

INTRODUCTION Despite cardiovascular diseases having grown to epidemic proportions, there are few studies from India pertaining to Acute Coronary Syndrome (ACS), more so from the region of Purvanchal which is less developed with more poverty. Our study is first of its kind in this region of patients presenting for the first time with ACS. AIM The present study was undertaken to study the clinical and angiographic characteristics of ACS patients of Purvanchal. MATERIALS AND METHODS This was a prospective cohort study of 100 patients admitted with ACS. Patients were excluded if they had prior cardiac pathology like valvular heart disease, cardiomyopathy, pericardial disease, cor pulmonale, ischaemic heart disease or cardiac revascularisation. Patients who did not undergo angiography were excluded. Patients were divided into ST Elevation Myocardial Infarction (STEMI) and non STEMI (NSTEMI). Presentation delays as well as clinical characteristics analysed in each group were age, gender, presence or absence of diabetes mellitus, hypertension, dyslipidaemia, smoking, Body Mass Index (BMI), family history, duration of chest pain, and treatment received. RESULTS Mean age of patients was 58.9 years with 27% below 50 years. Of the total 75% were males. Patients with STEMI were 65%. Median time to reach hospital was 24 hours with only 27% patients reaching hospital within 6 hours. Among patients with STEMI only 43% received fibrinolytic therapy. 23% patients had diabetes, 21% were hypertensive, 16% were smokers, family history of cardiovascular disease present in 11% and 21% had body mass index more than 30. Mean LDL was 115mg/dl and HDL 39mg/dl with 54% of patients having at least one risk factor. Factors favouring triple vessel involvement were female sex, higher age, smoking, presence of diabetes and NSTEMI. CONCLUSION Indians develop ACS at earlier age. Precious time is lost before seeking treatment. There is a need for aggressive risk factor modification which along with health awareness will be the key to prevent premature cases of ACS and limit morbidity and mortality due to delayed treatment.

Takayasu arteritis with mid aortic dysplastic syndrome presenting as dilated cardiomyopathy

Abstract Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels particularly the aorta and its major branches. Takayasu arteritis can have various presentations, depending on site and extent of disease. We describe a case of TA with mid aortic dysplastic syndrome, which presented as dilated cardiomyopathy.

Role of ACE inhibitors in anthracycline-induced cardiotoxicity: A randomized, double-blind, placebo-controlled trial

Several measures including drugs have been tried to reduce anthracycline cardiotoxicity. The lack of randomized trials prompted this study to assess the role of an angiotensin converting enzyme (ACE) inhibitor (enalapril) in anthracycline‐induced cardiotoxicity in children with hematological malignancies.

Familial hypercholesterolemia supravalvular AS and extensive atherosclerosis.

Familial hypercholesterolemia is an autosomally dominant disorder caused by various mutations in low-density lipoprotein receptor genes. This can lead to premature coronary atherosclerosis and cardiac-related death. The symptoms are more severe in the homozygous type of the disease. Premature malignant atherogenesis leading to aortic root abnormalities causing supravalvular aortic stenosis is rare. Our case demonstrates the diagnostic imaging findings of the phenotype of patients who have severe elevated LDL with familial hypercolesterolemia.

Correlation between 2D Echocardiography and Multidetector Row CT for Early Detection of Diastolic Dysfunction in Normotensive Diabetic Patients.

INTRODUCTION Abnormalities in diastolic function are considered early signs of diabetic cardiomyopathy. Two dimensional echocardiography with Tissue Doppler Imaging (TDI) has been used most commonly to evaluate diastolic left ventricular function. AIM The study was conducted to study the prevalence of diastolic dysfunction with the help of echocardiography and MDCT and their correlation in asymptomatic diabetic people. MATERIALS AND METHODS A total of 15 diabetic, normotensive patients who had undergone 64-multidetector row CT and 2D echocardiography with TDI were included. The parameters used for assessment of diastolic dysfunction were early (E) and late (A) transmitral peak velocity (cm/s) and peak mitral septal tissue velocity (Ea; cm/s). Subsequently, LV filling pressures (E/Ea) were estimated. RESULTS Of the total 73.3% patients showed presence of diastolic dysfunction by both CT and echocardiography. Good correlations were observed between cardiac CT and 2D echocardiography for assessment of E (r = 0.992; p < 0.001), A (r=0.974, p <0.001), E/A (r = 0.979; p < 0.01), Ea (r = 0.977; p < 0.001), and E/Ea (r = 0.994; p < 0.001). CONCLUSION Doppler echocardiography is considered one of the best methods for assessment of LV diastolic dysfunction. In this study of cardiac-asymptomatic diabetic patients, MDCT and echocardiography showed good correlation for assessment of early diastolic dysfunction. Hence, cardiac CT can be an additional tool for assessing LV diastolic function.

Extensive xanthomas with severe coronary artery disease in a young patient with familial hypercholesterolemia

A 20-year-old female presented to us with recent onset unstable angina. She had a strong family history of premature coronary artery disease (two elder sisters dying at a young age due to CAD). She had large tendinous xanthomas over extensor tendons of metacarpophalangeal joints and patella ( Panels A–C ). She also had xanthelesma, gaint tuberous …

Congenital Pulmonary Vein Stenosis in an Adult Patient

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5] A 30-year-old female presented to us with exertional breathlessness for 2 years, orthopnea, and paroxysmal nocturnal dyspnea for 2 months. Electrocardiography showed sinus tachycardia and right bundle