Isolated congenital valvular stenosis of either aortic or pulmonary valve is commonly seen yet the presence of both these lesions in the same patient is rare. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves. We present here a case report of a three and half year old boy diagnosed as a combined congenital severe valvular aortic stenosis with valvular pulmonary stenosis. The patient underwent successful balloon dilatation of both these valves in the same sitting.

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Background: 5-FU a cytostatic agent is used to treat various malignancies. Cardiotoxicity is infrequent complication of 5-FU. Previously we had observed ECG changes in our patients on 5-FU viz sinus bradycardia, atrial fibrillation. We also found that 5-FU bradycardia might be a hypervagotonia as vagolytics abolished it. This study was undertaken to assess the impact of this commonly used chemotherapeutic agent on basic physiologic parameters of impulse initiation and conduction in human heart. The aim was to study effect of 5-FU on electrophysiological properties of SA node and conduction system of heart.Methods: Pre -5 FU state patients were subjected to electrophysiological study and following parameters were checked viz heart rate, CSNRT. SACT, AH and HV intervals and wenckebach point. Post 5- FU state these parameters were again checked. Response to Atropine was seen in patients who developed sinus bradycardia.Results: Sinus bradycardia was seen in 8% (n=2) of patients in post 5FUstate Their electrophysiological parameters SNRT, CSNRT, SACT, AH were mildly prolonged (above the normal) in post 5FU state. Post atropine there was increase in heart rate and the deranged electrophysiological parameters came back to normal. Atrial fibrillation was seen in two patients while performing sinus nodal functions in post 5FU state.Conclusions: Sinus bradycardia is a rare cardiotoxic side effect of 5FU, is a result of an autonomic imbalance viz hypervagotonia which is abolished by atropine and more common in infusuion form than bolus. Infra-his conduction is not altererd and threshold for inducing atrial arrhythmias is decreased in post 5-FU state.

Lymphoma involving the heart is rare. This is a case report on non-Hodgkin lymphoma where the patient presented for the first time with heart failure and severe left ventricular systolic dysfunction due to lymphoma infiltrating the heart muscle and had simultaneous bilateral involvement of kidneys. This type of presentation has never been described in world literature and is the first reported case.

Mayer Rokintansky-Kuster-Hauser syndrome is characterized by the congenital absence of the vagina with a variety of Müllerian duct anomalies. Because of normal sexual development, the syndrome usually remains undetected until primary amenorrhea and/or difficulty in attempting sexual intercourse result in the diagnosis. MRKHS frequently is associated with anomalies of the urinary tract (unilateral agenesis or ectopia of one or both kidneys, horse-shoe kidney), cervico-thoracic region (asymmetric, fused or wedged vertebrae, scoliosis and Klippel-Feil anomaly), congenital cardiac defects, hearing defects, and digital anomalies of varying severity. Congenital heart diseases in the form of atrial septal defect, HoltOram Syndrome with or without aorticopulmonary window, pulmonary stenosis etc in association with MRKHS have been described in the literature. We present a case of MRKHS with unilateral agenesis of the kidney and atrial septal defect with partial anomalous pulmonary venous connection. This combination of anomalies has not been reported in the literature so far. The various presentations and the pathogenesis of the condition are discussed. KEY WORDSMayer-Rokintansky-Kuster-Hauser syndrome, MURCS, atrial septal defect with partial