Current status of rheumatic heart disease in India.

The rheumatic heart disease continues to be an important cause of disease burden in India, affecting the population in their prime and productive phase of the life. The prevalence of rheumatic heart disease is varied in different Indian studies, because of the inclusion of different populations at different point of times and using different screening methods for the diagnosis. The data on incidence and prevalence on a nationally represented sample are lacking. There is a need for establishing a population-based surveillance system in the country for monitoring trends, management practices, and outcomes to formulate informed guidelines for initiating contextual interventions for prevention and control of rheumatic heart disease.

The Profile of Amyotrophic Lateral Sclerosis in Natives of Western Himalayas: Hospital-Based Cohort Study.

BACKGROUND: Despite the disabling nature of amyotrophic lateral sclerosis (ALS), there are no contemporary data on clinical characteristics available from rural hilly states from India. Thus, the present study aimed at reporting clinical profile in ALS patients from natives of Western Himalayas. MATERIALS AND METHODS: A total of 32 patients of ALS were enrolled over a period of 1 year (2013-2014) in the present study. The demographic profile, clinical characteristics, and risk factors were systematically recorded, and these patients were followed for 1 year. RESULTS: The mean age of ALS patients was 53 ± 15.88 (23-90 years). Maximum number of patients of both limb onset and bulbar onset were in the age group of 40-49 years [Figure 1]. Male to female ratio was 1.46. Limb-onset was seen in 23 (72%) and bulbar-onset in 9 (28%) of patients. Bulbar-onset was more common in females as compared to males. Mean duration of symptoms were 19.06 ± 24 months (range 4-120 months). None of the studied risk factor showed statistically significant association with outcome of the disease. No familial association was found. The most common site of weakness was upper limb distal weakness. Definite ALS was seen in 13 (40.6%) patients. Mean ALS functional rating scale (ALSFRS) at presentation is 35.7 ± 7.9. All patients were started on riluzole. Mean ALSFRS at 9-month follow-up was 32.9 ± 7.4. After 1 year of follow up, 5 out of 32 patients died and among them, 4 were of limb onset and 1 was of bulbar onset ALS. Mean age at death in males was 66 ± 16 years and in females was 56.33 ± 24.8 years; mean survival in these patients was 25 months. CONCLUSION: This present study highlights following findings: (1) Male preponderance is less common in our patients as compared to earlier reports from India. Bulbar onset is more common in elderly (age >60 years) females. (2) As per previous reports from India, when compared to Western population present study supports the fact of the younger age of onset and longer duration of symptoms and slow course of disease in Indian patients.

Aortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly.

Aortopulmonary window (APW) is rare a congenital heart disease accounting for 0.1%-0.2% of all congenital heart defects. The 35% of the APW has been associated with wide variety of other structural heart diseases such as ventricular septal defect, persistent ductus arteriosus, arch anomalies and coronary artery anomalies. To the best of our knowledge, only six cases of APW with pulmonary atresia with ventricular septal defect has been described in the literature. It resembles the type 1 truncus arteriosus, and differentiation from this condition is important prior to surgical correction. We present a case of 14-year-old girl child; she was diagnosed with APW with pulmonary atresia with ventricular septal defect and D transposition of great arteries with the help of echocardiography, cardiac catheterisation and cardiac CT.