Challenges to manage primary chylopericardium in children.

Primary chylopericardium (PC) is a rare entity in the pediatric population with very few reported cases. Most cases of chylopericardium manifest after trauma or following cardiac surgery. The other etiologies which may lead to chylopericardium are malignancy, tuberculosis, or congenital lymphangiomatosis. We report two cases of PC in the pediatric population with contrasting outcomes. Both failed conservative management with dietary modification and octreotide. Surgery with pleuropericardial and pleuroperitoneal windows was performed in both. The first case had a thoracic duct ligation. The first patient died, and the second survived.

Mitral valve repair in children with rheumatic heart disease.

PURPOSE: Rheumatic heart disease is the most common acquired heart disease in children in developing countries. The heart valve lesions produce severe hemodynamic changes due to scarring of the valves over time. Around 15.6 million people are affected by rheumatic heart disease (RHD), and 230,000 die around the globe annually. Valve repair should be the primary goal, although it is technically challenging because of the fact that rheumatic process evolves making repair outcomes variable. METHODS: We reviewed the literature for the various techniques done for mitral valve repair in children with rheumatic heart disease. Early and late results of repair were compared with the results found for mitral valve repair done for such children. RESULTS: Prosthetic heart valve implantation in children has major negative impact on their immediate- and long-term survival as well as on quality of their life. Valve repair is associated with improved ventricular function because the normal valve tissue and subvalvular apparatus are preserved, reduced complications related to prosthetic valve, and lower in-hospital and late mortality. CONCLUSION: In children, the results of mitral valve replacement were found to be inferior to those of mitral valve repair. The reoperation rates are similar in patients undergoing initial repair or replacement, which favors repair as an option. In developing world, rheumatic mitral valve disease is more prevalent where adequate facilities for monitoring of prosthetic valve function and management of anticoagulation therapy are not easily available. Valve repair therefore should be the primary goal.

Neonatal ascending aorta thrombosis: A rare and lethal entity.

Ascending aorta thrombosis (AAT) in a neonate is a rare and lethal event. To date, AAT has been reported in around 25 patients, out of whom only eight patients had concomitant arch involvement. We report a case of one such patient with ascending aorta and arch thrombosis and present a brief review of the available literature.

Left isomerism with bilateral superior vena cava, interrupted inferior vena cava and tetralogy of Fallot.

The association of left isomerism with tetralogy of Fallot (TOF) is rare and reported as scattered case reports. Complex congenital cardiac and noncardiac malformations are known to be associated with left isomerism, but right-sided obstructive lesions are rare. We present our experience with a case of left isomerism with bilateral superior vena cava, interrupted inferior vena cava, and TOF managed by atrial septation and intracardiac repair.

Outcome of 40 consecutive cases of modified Ross procedure using novel Dacron valved conduit.

OBJECTIVE: The Ross procedure is an established option for aortic valve disease in children. Due to limited availability of pulmonary homograft, we devised a novel technique for right ventricular outflow tract (RVOT) reconstruction by preparing indigenous Dacron valved conduit. METHODS: Forty consecutive cases of modified Ross procedure done at our center (2013-2018) were analyzed. Thirty-seven patients (95%) were followed up with median duration of 2.5 (0.08-5.5) years. Median age was 12 (5-39) years. Nineteen (47.5%) patients had rheumatic aortic valve disease, while 19 (47.5%) had congenital aortic valve disease. Aortic root replacement with pulmonary autograft was performed in all patients. Dacron conduit for RVOT reconstruction was used with on table sewn bileaflet valve using Dacron patch (n = 22), expanded polytetrafluoroethylene (ePTFE) membrane (n = 10), bioprosthetic valve (n = 4), and pericardium (n = 4). Additional surgical procedures included mitral valve repair (n = 10), septal myectomy (n = 2), ascending aorta replacement (n = 1), ruptured sinus of valsalva (RSOV) repair (n = 1), and ventricular septal defect (VSD) closure (n = 1). RESULTS: There was one in-hospital mortality while one late death occurred at 3.5 years postoperatively. The neo-aortic valve regurgitation on echocardiographic evaluation at last follow-up was trivial (n = 28), mild (n = 7), and moderate (n = 2). Mild RVOT obstruction was present in 8 patients while 18 patients had mild pulmonary regurgitation. No patient required reintervention during follow-up. CONCLUSION: Our early results of modified Ross procedure are encouraging, however, long-term follow-up is required.

Pulmonary root translocation and Senning procedure for congenitally corrected transposition of great arteries with pulmonary stenosis: a case report.

Congenitally corrected transposition of the great arteries (CCTGAs) is a condition, which includes atrioventricular and ventriculoarterial discordant connections along with ventricular septal defect (VSD), pulmonary stenosis (PS), or pulmonary atresia (PA). Without treatment, progressive systemic ventricular failure begins, which is followed by sudden cardiac death by the fourth or fifth decade. We report a case of a 4-year-old with CCTGA, VSD, and PS operated by Senning procedure and pulmonary root translocation (PRT) with uneventful postoperative recovery. PRT overcomes problems with the right ventricle to the pulmonary artery conduit and maintains pulmonary valve function and growth capacity. Our initial experience with PRT in CCTGA indicates that it is a feasible surgical alternative for such patients.

Thrombocytapheresis: managing essential thrombocythemia in a surgical patient.

A 73-year-old man presented with acute chest pain and shortness of breath suggestive of unstable angina. A detailed investigation revealed essential thrombocythemia and coronary artery pathology. With a baseline platelet count of 2,650×10(3)/µL, coronary artery bypass grafting became nearly impossible. Three therapeutic plateletpheresis procedures successfully lowered the platelet count to 367×10(3)/µL. Thereafter, surgery was performed with no complications. Although a drop and rise in platelet counts were observed postoperatively, the patient could be discharged in stable condition after 14 days. Thus, therapeutic plateletpheresis reduces platelet count rapidly in essential thrombocythemia and relieves patients of acute symptoms.