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Objective: To determine the outcome of early and late-onset Fontan operation in patients with univentricular heart repair. Study Design: Quasi-experimental study. Place and Duration of the Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jan 2019 to Dec 2021. Methodology: During the study period, 121 patients of both genders, up to any age, with functionally univentricular hearts and who planned to undergo Fontan procedures were included. All cases were divided into two groups, Group-A had patients who underwent extra-cardiac Fontan at age ≤6 years, while Group B had patients who underwent extra-cardiac Fontan at age >6 years. Pre-procedure diagnosis, demographic, anatomic and hemodynamic characteristics, post-Fontan procedure morbidity and mortality were noted. Results: As per inclusion/exclusion criteria, 121 cases underwent Fontan surgery during the study period. There were 61(50.4%) male patients. Overall, the mean age was 7.02±4.6 years. Seventy-six 76(61.2%) cases were in Group-A and 45(38.8%) in Group-B. Mortality (within 30 days post-surgery) was reported in 11(9.1%) patients. Except for the mean duration of chest tube drainage ((0.010), there was no difference in terms of post-Fontan surgery outcomes between both study groups (p>0.05). Conclusion: The age of the patients at the time of the Fontan procedure did not affect the outcomes.

Objective: To determine the risk factors for early post-operative arrhythmias in children undergoing congenital heart surgeries at a tertiary care hospital. Study Design: Prospective longitudinal study. Place and Duration of Study: National Institute of Cardiovascular Disease, Karachi Pakistan, from Jan to Jul 2020. Methodology: We included 143 patients of both genders undergoing open heart surgery for congenital heart diseases. We noted all patients' pre-operative, intra-operative, and post-operative clinical characteristics. Patients were monitored in the pediatric cardiac intensive care unit. We analyzed the development of postoperative arrhythmias and the factors associated with them. Results: In 143 patients, the mean age at the time of surgery was 7.52±6.0 years. There were 83(58.0%) male patients. Tetralogy of Fallot was the most common type of congenital heart disease noted in 57(39.0%) patients. The mean cardiopulmonary bypass time was 83.9±25.5 minutes, while the mean aortic cross-clamp time was 58.1±21.9 minutes. Post-operatively, arrhythmia was observed in 39(27.3%) children. Intra-operative arrhythmias (p

Objective: To find out the mode of presentation and severity of cardiac involvement in children presented with established rheumatic heart diseases (RHD) at first presentation at a tertiary care setup. Study Design: Cross-sectional study. Place and Duration of the Study: Department of Pediatric Cardiology, National Institute of Cardiovascular Disease, Karachi Pakistan, from Jul 2020 to Jun 2021. Methodology: One hundred seven children of genders aged 3 to 18 with a confirmed diagnosis of RHD were included. Demographic features, chief presenting complaints, possible reasons for late presentation, and echocardiographic findings were noted. Results: In 107 patients with RHD, 63(58.9%) were male. The sensation of the rapid pounding of heartbeat (palpitation) was observed among 72(67.3%) children, while a similar number of cases, 72(67.3%), presented with breathlessness. There were 33(30.8%) children who were diagnosed for the first time with RHD. The most common reason for late diagnosis was socioeconomic factors in children (18, 26.1%). Primary prophylaxis for acute rheumatic fever (ARF)/RHD was noted to be given in 11(10.3%) patients. Mitral regurgitation was the most common echocardiographic finding in 103(96.3%), followed by aortic regurgitation in 99(92.5%). Conclusion: The majority of children with RHD are diagnosed late, which exposes them to an increased risk of undiagnosed valvular disorders. Palpitation, orthopnea and breathlessness were RHD most frequent presenting clinical features. Mitral regurgitation and aortic regurgitation were the most common echocardiographic findings.

Objective: To determine the relationship between transthoracic and transesophageal echocardiography in the anatomic and hemodynamic assessment of secundum atrial septal defects in children and adults. Study Design: Cross-sectional study. Place and Duration of Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jul 2020 to Jun 2021. Methodology: Seventy-seven patients above three years of age with secundum atrial septal defects were included. Linear regression was used to determine any statistically significant difference between transthoracic and transesophageal echocardiography approaches. Results: Of 77 patients, there were 37(48.1%) male and 40(51.9%) female. No significant difference was noted in terms of any of the clinical parameters during transthoracic echocardiography and transesophageal echocardiography. As per transthoracic echocardiography, 35(45.5%) were noted to be deviceable, while in transesophageal echocardiography, 36(46.8%) patients were found to be deviceable (p=0.872). No emergency exploration or deaths occurred. Linear regression showed no statistically significant difference between transthoracic echocardiogram and transesophageal echocardiography findings in terms of atrial septal defects size (p=0.226), total septal length (p=0.930), RV systolic pressure (p=0.440), RV functions and TAPSE (0.748) except LV ejection fraction function findings (p