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Andersons pediatric cardiology 1525

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cases with relapse of tuberculosis or multidrug resistance, a combination of second-line antitubercular drugs for a longer duration is recommended There is no consensus about the use of corticosteroids in tubercular pericarditis.81 In a recent randomized controlled trial in adults, steroid therapy did not show any beneficial effect on the composite endpoints of death, tamponade, or pericardial constriction Although less number of patients developed pericardial constriction with the use of steroids, there was an increased risk of cancer in patients with HIV.82 The recommended dose of oral prednisolone is 1 to 2 mg/kg per day It should be continued for 10 to 12 weeks with slow tapering after 4 weeks Colchicine therapy is not useful.83 Children with large effusion benefit from pericardiocentesis Despite adequate treatment, a significant number of children develop pericardial constriction and worldwide tubercular pericarditis remains one of the leading cause of pericardial constriction.3,4,78 As in bacterial pericarditis, intrapericardial fibrinolysis is shown to be effective in reducing pericardial constriction.73 However, it is not routinely recommended as the evidence is limited In cases requiring pericardiectomy, it should be deferred for at least 6 weeks of initiating antitubercular therapy, allowing time for the surgical cleavage plane to develop.79,84,85 HIV-Related Pericarditis Pericardial effusions are common and are seen in up to 25% children with HIV infection Cardiac tamponade is rare In an immunocompromised state, these patients are at higher risk of developing parasitic and fungal infections of the pericardium The presence of HIV is a risk factor for developing tuberculous pericarditis.3,78,79 Renal Failure Renal failure accounts for approximately 8% of pericardial effusion in children.71,86 It is more common in patients with concurrent connective tissue disorders These patients present as either uremic pericarditis, dialysis-associated pericarditis, or as pericardial constriction.4 ECG abnormalities are absent in the majority The pericardial fluid is serous and responds quickly to dialysis.4,86 Pericardiocentesis should be performed in those with progressive increase in effusion and hemodynamic compromise Pericardiectomy is reserved for rare cases with pericardial constriction.4 Hypothyroidism Pericardial effusion is common in severe hypothyroidism.71 Owing to slow accumulation of fluid, tamponade is uncommon Unlike other causes of pericardial effusion, patients may present with bradycardia Most effusions resolve gradually after initiation of thyroid hormone replacement therapy and pericardiocentesis is generally not required Pericardial fluid, if aspirated, contains elevated protein and mucopolysaccharides.71 High cholesterol content sometimes give it a characteristic gold paint appearance.87 Neoplastic Disease Primary tumors of the pericardium are rare The majority of patients have metastatic tumors.88 Neoplasm-related pericardial effusion is more frequent in developed countries and may account for up to one-third of patients requiring pericardial drainage.86 Primary tumors include lymphoma, mesothelioma, teratoma, and angiosarcoma Common metastatic tumors are Hodgkin disease, non-Hodgkin lymphoma, leukemia, malignant melanoma, Wilms tumor, neuroblastoma, and other HIV-related malignancies Intrapericardial cisplatin has been shown to be effective in preventing recurrence of neoplastic pericardial effusion in adults.88 Postpericardiotomy Syndrome Pleural and pericardial inflammation following cardiac surgery result in postpericardiotomy syndrome It is suspected if any two of the following are present: fever beyond first postoperative week without infection; pleuritic chest pain; pericardial rub; new or worsening pleural effusion; and new or worsening pericardial effusion Pericardial effusion typically presents 1 to 2 weeks after surgery and peaks around the tenth postoperative day, though recurrences months later are not uncommon.78,89–92 The exact mechanism of postpericardiotomy syndrome is not known and is hypothesized to be an autoimmune reaction.93,94 Handling of the pericardium during cardiac surgery also contributes to the inflammation It is more common in older children than in infants and toddlers, possibly related to their robust immunologic response.95 Postpericardiotomy syndrome is reported in up to 30% of patients following cardiac surgery However, recent reports have shown much lower prevalence In a recent review of 1.4 million cardiac surgeries in patients aged less than 18 years, pericardial effusion was seen in 1.1% Heart transplant, systemic-topulmonary shunt, and atrial septal defect were independent risk factors for its development in children.95 It is usually self-limiting and the majority of patients respond to NSAIDs or steroids Patients with symptomatic or recurrent effusion require pericardiocentesis or pericardiectomy.78 Chylopericardium Chylous pericardial effusion is typically seen following thoracic duct injury during cardiac surgery and is usually associated with chylous pleural effusion.71,96,97 Chylopericardium can also occur in patients with mediastinal masses obstructing lymphatic drainage or following radiation therapy The pericardial fluid is milky colored and has elevated levels of triglycerides and proteins Initial management includes a low-fat or medium-chain triglyceride diet Some children may require total parenteral nutrition Octreotide, a longacting somatostatin analog, has also been shown to be effective.98,99 Children with persistent chylous effusion require thoracic duct ligation or pericardioperitoneal shunt.78

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