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Homozygous Familial Hypercholesterolemia: Anesthetic Challenges and Review of Literature.
Ladha, S, Makhija, N, Kiran, U, Aarav, SK
World journal for pediatric & congenital heart surgery. 2020;(4):NP83-NP87
Abstract
Homozygous familial hypercholesterolemia (HoFH; Fredrickson IIa) is a rare autosomal dominant hereditary disease associated with increased low-density lipoprotein cholesterol. We hereby report anesthetic challenges in a rare case of HoFH having severe progressive aortic stenosis, mitral regurgitation, diffuse aortic and carotid vessel involvement, and severe left ventricular dysfunction. The patient underwent a Konno aortoventriculoplasty with mechanical prosthesis along with mitral valve repair.
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Use of Idarucizumab to reverse the anticoagulant effect of dabigatran in cardiac transplant surgery. A multicentric experience in Spain.
Crespo-Leiro, MG, López-Vilella, R, López Granados, A, Mirabet-Pérez, S, Díez-López, C, Barge-Caballero, E, Segovia-Cubero, J, González-Vilchez, F, Rangel-Sousa, D, Blasco-Peiró, T, et al
Clinical transplantation. 2019;(12):e13748
Abstract
BACKGROUND Anticoagulation in heart transplant (HT) recipients increases the risk of hemorrhagic complications, so correct reversal of anticoagulation is needed. Dabigatran, a direct thrombin inhibitor, is increasingly used for anticoagulation in patients with non-valvular atrial fibrillation (NVAF) whose effect can be reversed by idarucizumab. AIM: To present a nationwide experience using idarucizumab for the urgent reversal of dabigatran before HT. METHODS Multicenter observational study in 12 Spanish centers to analyze the clinical outcomes after using idarucizumab before HT surgery. RESULTS Fifty-three patients were included (81.1% male). 7.5% required re-operation in the immediate postoperative period to control bleeding and 66% transfusion of blood products. Median length of stay in the intensive care unit was 6 days and total hospital stay 24 days. 30-day survival was 92.4%. There were four deaths in the first month, all in the first 5 days post-HT. Only in one patient (transplanted due to a congenital heart disease, after sternotomy) who had surgical problems and right ventricular failure post-HT death was associated with bleeding. CONCLUSIONS These results may support the use of dabigatran as an alternative to vitamin K antagonists in patients listed for HT requiring anticoagulation due to NVAF. More studies are needed to reaffirm these observations.
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Early repair of large infant ventricular septal defect despite respiratory syncytial virus-induced respiratory failure with postrepair chylous pericardial effusion requiring pleuropericardial window: a case report and review of the literature.
Sykes, JA, Verma, R, Peshkovsky, C, O'Connor, B, Malik, F, Lubega, J, Malhotra, S, McQueen, D, Mikkilineni, S, Kalyanaraman, M
Pediatric emergency care. 2012;(10):1072-7
Abstract
The surgical correction of congenital cardiac lesions that are complicated by intercurrent respiratory syncytial virus (RSV) pneumonitis has traditionally been deferred for at least 6 to 8 weeks. The presumption is that using cardiopulmonary bypass will increase the risk of postoperative pulmonary complications. We present an infant who developed acute respiratory failure related to RSV pneumonitis and required urgent mechanical ventilation. Cardiac evaluation revealed a large nonrestrictive ventricular septal defect (VSD), aortic arch hypoplasia, normally functioning bicuspid aortic valve, and hemodynamic instability associated with markedly increased pulmonary blood flow. Separation from mechanical ventilation was unsuccessful preoperatively. He underwent VSD repair with cardiopulmonary bypass less than 4 weeks after initial RSV infection. He was extubated successfully within 72 hours of VSD repair. Approximately 6 weeks postoperatively, he developed a circumferential chylous pericardial effusion of unclear etiology--an exceedingly rare complication of VSD repair in early infancy in a non-Down syndrome patient. The chylous effusion was initially managed unsuccessfully with Portogen/Monogen and a percutaneously placed pericardial drain. Two weeks later, he underwent creation of a pleuropericardial window with successful resolution of the chylous effusion. It is of interest to pediatricians to be able to correctly time the repair of congenital heart disease lesions after RSV infection to minimize post-bypass pulmonary complications and yet avoid morbidity from undue delays in repair. In addition, chylopericardium can occur in infants after VSD repair, and dietary modification and catheter drainage may not be adequate.
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Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury.
Kan, CD, Wang, JN, Wu, JM, Yang, YJ
Texas Heart Institute journal. 2007;(1):82-7
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Abstract
Chylopericardium after an intrapericardial procedure is rare, and satisfactory explanations of its possible causes are lacking.Herein, we present 4 cases of chylopericardium that developed after intrapericardial surgery, and we review the literature. Our literature review revealed 29 cases of chylopericardium that complicated intrapericardial operations, to which we added our 4 cases for analysis. The 33 surgical procedures involved repair for congenital heart disease (n=21), valve surgery (n=5), coronary artery bypass grafting (n=6), and other (n=1). Causes were verified in 7 patients: small lymphatic injury in 3 and high venous pressure or venous thrombosis in 4. Of the 26 patients with chylopericardium of unknown origin, 15 had congenital heart disease. Ten of these 15 had chromosomal abnormalities, especially trisomy 21 (Down syndrome); these patients typically had increased lymphatic permeability, which raised the likelihood of chylopericardium. Five revascularizations for coronary artery disease required harvesting of the left internal thoracic artery for reconstruction, incurring a risk of damage to the drainage site of the right efferent lymphatic trunk. In addition, all 26 patients with chylopericardium of unknown origin underwent dissection of the ascending aorta and the main pulmonary artery, near the right efferent lymphatic trunk. Inadvertent injury to the trunk during the dissection would have increased the risk of chylopericardium. Accordingly, even though the overall incidence of chylopericardium during intrapericardial procedures is low, we recommend a meticulous dissection of the ascending aorta from the main pulmonary artery.