Journal of Pediatric Critical Care

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The term univentricular heart encompasses a wide variety ofheart defects that functionally andphysiologically. constitute a single ventricle chamber. The disease variants comprising this spectrum are not amenable to biventricular repair, and require staged palliation culminating in cavopulmonary connection. Broadly, the management hinges on controlling pulmonary blood flow in early infancy, by means of aortopulmonary shunting in pulmonary atresia or stenosis or pulmonary artery banding for excess pulmonary blood flow situations. Concomitant repair of associated anomalies, requires addressal. Neonates and early infancy patients with hypoplastic left heart syndrome(HLHS), undergo staged Norwood procedine, resulting in eventual total cavo pulmonary connection with the right ventricle as the functional systemic chamber. The current review focuses on basics of univentricular physiology, perioperative management and with a brief discussion on the current guidelines for medical management.

Objective:outcome of children treated with Intrapleural alteplase therapy in empyema thoracis Design: Prospective interventional pilot study Setting:Pediatric Critical care and Pulmonology unit at Tertiary care Hospital Subjects: All patients of empyema thoracis from 1 month to 18 years of age admitted from May 2012 to April 2014. Method and intervention:Children were selected for intrapleural alteplase therapy and treated under an IRВ (Institutional Review Board) approved protocol.Alteplase (4 mg) was diluted with 50 ml NS and instilled through Intercostal drain(ICD). Chest tube was kept clamped for 1 hour and then opened. The primary outcomes measured were: clinical improvement, lung expansion, ICD days and hospital stay. Secondary outcomes were 24 hour ICD output and adverse effects. Results:A total of 13 patients were given intrapleural alteplase. Median age was 3 years and ranged from 11 month to 14 years. Clinical and radiologicalimprovement was seen in 84.6% (11/13) cases. Fever subsided within 5 days in 54% with median of 4 days (28?days). Respiratory distress settled in median 5 days (2-7 days). ICD days were 6.63±1.8 (mean±SD) days. ICD days after stalling alteplase were 3.45±l .03 (mean±SD) days. Mean hospital stay wasll.27±4.14 (mean±SD) days in successful cases. Mean hospital stay including failure case was 13±5.75 (mean±SD) days.Average flow per day before alteplase was 64.5±65 ml/day which increased to 194.8± 146.3 ml/day with intrapleural alteplase. (P value 0.006). Persistent fever, distress and lung collapse were seen in 15.4% (2/13) cases and both required surgical intervention. Conclusion: Intrapleural alteplase therapy in empyema is beneficial in reducing effusion volume, clinical symptoms, hospital stay, and the need for surgical mtervention.Further large trials are needed for optimum dose, duration of therapy and clamp timing for intarpleural fibniolytic therapy as well as to define relativecontraindications to use of alteplase therapy.

Transposition of the great arteries (TGA) is a congenital heait defect 111 which the normal anatomic positions of the aorta and pulmonary artery are transposed (ie. the aorta originates from the right ventricle and the pulmonary artery arises from the left ventricle). The association with other cardiac malformations such as ventricular septal defect and left ventricular outflow tract obstruction is frequent and dictates timing and clinical presentation, which consists of cyanosis with or without congestive heait failure. Newborns with TGA/ intact ventricular septum frequently need preoperative stabilization with PGE_r mechanical ventilation and/ or Balloon Atrial Septostomy(BAS). The arterial switch operation is the procedure of choice that restores the aoita and pulmonary artery to their normal anatomicpositions. Intensive support is required to support the left ventricle which faces high resistance systemic circulation postoperatively. The outcome for most of the patients after surgery is good with low incidence of residual defects. Long term follow up is required for concerns regarding neurologic outcome and coronary abnormalities. This review focuses on current standard of care for patients with TGA.

Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. TAPVD can occur in isolation or as an additional component of complex congenital heart disease with univentricular or biventricular physiology. If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. It can present in the neonatal period or infancy depending upon obstruction to the abnormal pulmonary venous drainage. Obstructed TAPVD when presenting in the neonatal period constitutes a significant pre operative challenge and the outcome is often determined by the positive synergy in the transport and referral hospital team. The current review is targeted to stream line perioperative management strategy based on the currently available evidence in literature.

Online since 27^th January 2020