Background: Diphtheria remains an important cause of paediatric mortality in developing countries. The mortality rate is still ∼10% and has changed little over the past 20 years with particular reference to developed world. The objective of this study is to examine clinical spectrum of diphtheric cardiomyopathy and by using tools (serum markers, ECG, Echocardiography) to predict the cardiomyopathy and role of steroids in these predicted patients. Methods: For this cohort study, 67 patients having diphtheria presenting for the first time in a 3-year period were enrolled after obtaining informed verbal consent from the guardian of each child. Demographical profile, vaccination status, clinical spectrum, ECG interpretation and echocardiographic findings were recorded that predicted the occurrence of diphtheric cardiomyopathy and used intravenous methylprednisolone pulses in these predicted patients to look for their outcome regarding change in severity or the fate in the form of mortality. Results: Among the 67 enrolled children (M: F 2.3:1) with age ranging from 24 to 172 months (median 106 months), 56.7% subjects presented with diphtheria were non-vaccinated. 37.3% had a cardiac involvement in the form of diphtheria cardiomyopathy or arrhythmia.7.5% patient expired on follow up. Presence of septal paradoxes on echo had association with the cardiac involvement (OR 10.1: 95% CI 1.2-84.6; p=0.0005). IV methyl prednisolone was given in all 37.3% (n=25) patients predicted as diphtheric cardiomyopathy (Asymptomatic) and 88 %(n=22) had a favourable outcome with no morbidity and mortality. 12% (n=3) were expired and they presented with shock and VT as their first presentation (symptomatic). Conclusion: Early prediction by alone or in combination of ECG and echocardiographic markers and early use of IV methyl prednisolone in these predicted patients before symptoms, can reduce the mortality related to diphtheric cardiomyopathy and can decrease the burden of the disease in the community. Further randomized controlled trials with a larger sample size are required to unambiguously delineate the prognostic value of steroids in early predicted diphtheric cardiomyopathy.

Objective: To describe the prevalence, clinical spectrum (Particularly cardiac) and treatment strategies of MIS-C. Study Design: Case Control study (prospective and retrospective surveillance). Setting: Children Hospital and Pediatric Medicine Department DHQ/Allied Hospital Faisalabad, Pakistan. Period: 15th March, 2017, to March 14th, 2020. Material & Methods: All patients with confirmed MIS-C/KD were enrolled and made two groups before and after the pandemic of SARS CoV-2. Results: A total of 6 patients before pandemic (group 1) and 18 patients after the pandemic (group 2) were studied over a period of 3 years. There is female dominance in group 1 and male dominance in group 2. Patients in group 2 presented at higher age (median 8y). Gastrointestinal symptoms (72.2%), Shock (11.2%) and LV dysfunction (11.1%) were more different features of MIS-C as compared to KD. There was more intense hyperinflation response was in MISC as compared to KD (CRP median 15.5 vs 78 and S/Ferritin median 725 vs 1889). Conclusion: MIS-C is on the Kawasaki disease spectrum in many ways both clinically and biochemical markers but the level of inflammation and immune response and severity of cardiac involvement were much bigger than KD. This should be in high consideration in perspective of new waves of SARS CoV-2.

Combined congenital aortic and pulmonary valvular stenosis is a rare congenital heart defect. Prevalence of severe combined valvular stenosis of aortic and pulmonary valve accounts about 0.01% and also has association with many syndromes.  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 and its management. We present this rare combination in a 9 year old boy which was promptly managed with the balloon valvoplasty without any complications in the same setting under local anesthesia with sedation.

Background and Objective: The long QT syndrome (LQTS) is a repolarization defect of heart involving potassium linked channels and it usually manifests clinically as seizures, syncope, or sudden cardiac death syndrome in children secondary to its characteristic ventricular tachy-arrhythmia like torsades de pointes. The reason behind epilepsy or seizures like activity in this disease is the sequelae of prolonged cerebral hypoperfusion secondary to the cardiac dysrhythmia. The aim of study is to look for clinical spectrum and risk factors associated with LQTS among children presenting with epilepsy, which can predict the early diagnosis of LQTS. Materials and Methods: For this observational study, 422 patients having epilepsy presenting for the first time in a 3-year period were enrolled. Demographical profile, LQTS measures, and various factors under observation were recorded. Results: Among the 422 enrolled children (M: F 1.8:1) with age ranging from 4 to 87 months (median 23 months), 8 (1.9%) children who presented with epilepsy had LQTS. Among those, mean QTc on electrocardiogram was 454 ± 31 msec and mean Schwartz score >3. Half of the patients with LQTS had deafness (P = 0.002) and 37.5% had a positive family history (P = 0.0045). Nearly a third (37.5%) presented with syncope and 87.5% patients with LQTS had no postictal drowsiness or sleep (P ≤ 0.004). Conclusions: LQTS is underestimated in children presenting with epilepsy and LQTS should be considered as an alternate diagnosis in children with recurrent seizures or syncopal attacks. The brief period of seizures with no postictal drowsiness, syncope, and strong family history are the features which may help in segregating LQTS from epilepsy.