Background: Congenital heart diseases (CHD) are relatively common with a prevalence ranging from 3.7 to 17.5 per 1000 live births. Little is known about genetic link with respect to congenital heart disease. Iroquoise (Irx) homeobox genes have been widely studied and their expression in both developing and adult heart. Author tried to study the role of irx4 and irx5 genes in structural congenital heart disease, keeping the focus on study reported by Cheng Z et al.Methods: Author studied reported mutation site sequences in 25 various congenital heart disease patients and control healthy relatives of patients. It is a unique study and there has not been such a study reported in literature till date. Besides comparison with healthy related controls, author took cardiac tissue biopsy in patients while doing corrective cardiac surgery. However, blood samples were taken from controls due to ease of feasibility.Results: Although, there were no sequence variations in the studied exon regions, but author got a base pair sequence change at 6 bp intron region, which is near the exon splice site in irx4 gene. Besides two ASD patient’s male children (one child each) had ASD prompting us to believe some role of sex linkage. However later needs pedigree analysis and sex chromosome studies for further analysis.Conclusions: Gene sequence in the Kashmiri population is unique. There is possibility of role of irx genes in CHD. ASD might have sex linkage in some.

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Background: Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of RVOT obstruction including tetralogy of Fallot (TOF). Transatrial repair avoids a ventriculotomy (in contrast to the transventricular approach) emphasizing maximal preservation of RV structure and function. We have adopted this technique as less traumatic for the right ventricle. This study evaluates the early surgical results of our approach.Methods: Between January 2005 to January 2014, 77 consecutive patients with RVOT obstruction were referred to our unit for surgical therapy. Of these, 14 were unsuitable for repair and underwent aortopulmonary shunting. In the remaining 63 patients (mean age of 2.67±0.38 years), complete transatrial/transpulmonary repair was performed. Previously placed shunts (four patients) were taken down. In all cases, subpulmonary resection and ventricular septal defect (VSD) closure were accomplished transatrially. In 51 patients, the main pulmonary artery was augmented with an autologous pericardial patch.Results: There were 7 (9%) deaths in this series. No patient required permanent pacemaker. Median ICU and hospital stay were 91 hours and 14 days, respectively. At median follow up of 54 (mean 51±12) months, all patients are asymptomatic, with no significant residual lesion.Conclusions: Transatrial/transpulmonary repair of TOF is associated with remarkably low morbidity and mortality in our early experience.

Introduction: Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. Immediate surgical correction on diagnosis with the aim of restoring a twocoronary system circulation is the current standard of treatment. However, the management of the mitral valve regurgitation in these patients is controversial. Methods: Fifty one patients of ALCAPA underwent coronary reimplantation over a 10-year period. The mitral valve was not addressed (except one patient with an organic basis) at the time of coronary implantation even in patients with moderate to severe mitral regurgitation. These patients were followed postoperatively with with serial echocardiography at six months and at two years thereafter to assess the improvement in ventricular and mitral valvular function. Result: A total of 53 cases were studied. Mitral valve regurgitation of varying severity was seen in 51 (96.5%) of the 53 patients preoperatively. There were 5 postoperative deaths (9.6%). Postoperatively, after 6 months, mitral regurgitation was none to mild in 38 patients (84.4%, n= 45). Moderate to severe mitral regurgitation was seen in 7 patients (15.6%). At 2 years follow up, 43 patients (95.5%, n= 45) had none to mild mitral regurgitation. Only two patients had moderate to severe mitral regurgitation. Conclusion: Reimplantation of anomalous coronary artery to the aorta must be the procedure of choice in patients with ALCAPA. Mitral valve repair is not required even in severe regurgitation, except when there is an organic basis of regurgitation.

Background: The St. Jude Medical bileaflet mechanical valve was designed by Xinion (Chris) Posis and Donald Hanson who came up with the concept of a leaflet-tab rotating in a "butterfly recess" in the inner wall of the housing with the hinge mechanism located near the central axis of the housing. The bileaflet configuration provided central, evenly distributed flow patterns with small areas of flow separation, minimal flow resistance, low profile, large effective orifice area, and ease of surgical implantation. The valve leaflets and housing were made of pyrolitic carbon and the sewing cuff of double velour dacron. The valve satisfies many of the features of an ideal valve such as long-term durability, non thrombogenicity and hemodynamic efficiency. However, its clinical dependence on chronic anticoagulation persists. Methods: Between January 2003 to December 2014, 430 patients were studied who had undergone or underwent mitral valve, aortic valve or double valve (both aortic and mitral) replacement with the St. Jude Medical bileaflet valve. Results: A total of 430 cases were studied. The mean age of operation was 39.16± 9.7 years. The operative mortality rate was 5.5% overall. The average period of follow-up was 4.21± 3.2 years. Structural valve deterioration was not seen in any patient in the series. Nonstructural valve dysfunction was reported in 7 patients. Clinically significant hemolysis was not seen in any patient. Thromboembolism was reported in 22 patients. Valve thrombosis was reported in 3 patients. Major bleeding events requiring hospitalization or transfusion were reported in 17 patients. PVE was reported in 4 patients. There were 36 late deaths. 21 patients died of valve related causes, 9 from cardiac causes and 6 patients from non-cardiac causes. Overall survival rate was 90.66%. Conclusions: Even though the St. Jude Medical bileaflet mechanical valve satisfies most of the features of an excellent mechanical valve substitute, the late valve related complications (especially thromboembolism and bleeding) and mortality, although low, illustrate that our quest for a "perfect" prosthesis remains.