Thrombosis has long been recognized as a potentially life-threatening complication in children with congenital heart disease (CHD), children with acquired heart disease, and in adults with CHD. High-risk groups include patients with shunt- dependent single ventricles (shunt thrombosis, 8%–12%; 4%

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease A Scientific Statement From the American Heart Association

Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. Although the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low-risk operations, the stage I Norwood procedure remains one of the highest-risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals of >90% for the Norwood procedure. This traditional 3-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomly assigned 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years postrandomization. In addition to modifications to the Norwood procedure, the hybrid procedure, a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a comprehensive stage II, which combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to improve not only short-term survival, but also potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but also into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also in terms of the burden of disease, neurodevelopmental outcomes, psychosocial development, and quality of life.

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions.

Context. Calcium channel blocker poisoning is a common and sometimes life-threatening ingestion. Objective. To evaluate the reported effects of treatments for calcium channel blocker poisoning. The primary outcomes of interest were mortality and hemodynamic parameters. The secondary outcomes included length of stay in hospital, length of stay in intensive care unit, duration of vasopressor use, functional outcomes, and serum calcium channel blocker concentrations. Methods. Medline/Ovid, PubMed, EMBASE, Cochrane Library, TOXLINE, International pharmaceutical abstracts, Google Scholar, and the gray literature up to December 31, 2013 were searched without time restriction to identify all types of studies that examined effects of various treatments for calcium channel blocker poisoning for the outcomes of interest. The search strategy included the following Keywords: [calcium channel blockers OR calcium channel antagonist OR calcium channel blocking agent OR (amlodipine or bencyclane or bepridil or ci...

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Treatment for calcium channel blocker poisoning: A systematic review

Purpose of reviewTo discuss the role of extracorporeal membrane oxygenation (ECMO) in patients with cardiac arrest.Recent findingsReturn to spontaneous circulation dramatically decreases with the duration of cardiopulmonary resuscitation (CPR). In this context, it has been proposed to implement veno

Extracorporeal cardiopulmonary resuscitation.

http://hsrlce.utoronto.ca/wp-content/uploads/2015/03/Factors-Associated-With-Thrombotic-Complications-After-the-Fontan-Procedure.pdf

Factors associated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure.

Combined poisoning with calcium-channel blockers and beta-blockers is usually associated with severe heart failure. This report shows the effectiveness of emergency extracorporeal life support in treating life-threatening simultaneous propranolol and verapamil intoxication. A 15-year-old girl presented in cardiogenic shock after alcohol consumption and a propranolol and verapamil overdose; plasma concentrations: propranolol, 0.53 m/mL; verapamil, 1.06 mg/mL. She was successfully resuscitated with extracorporeal life support. Therapeutic plasma exchange was initiated. Extracorporeal support was discontinued 70 hours later. The patient made a full recovery. Simultaneous verapamil and propranolol overdoses can cause severe hemodynamic compromise and arrest of electrical and mechanical function of the heart. Emergency extracorporeal life support can successfully maintain vital organ blood flow and allows time for drug metabolism, redistribution, and removal. Therapeutic plasma exchange may reduce the time of emergency extracorporeal life support. Emergency extracorporeal life support should be considered early in cases of near-fatal intoxications with cardiodepressive drugs.

Extracorporeal Life Support in Severe Propranolol and Verapamil Intoxication

Objective: The advantageous effect of right ventricle-to-pulmonary artery shunt (RV‐PA) on the early postoperative hemodynamics in the Norwood procedure for hypoplastic left heart syndrome (HLHS) is well known. Numerous controversies still exist with respect to the late consequences of this new palliation method in preparation for the second stage procedure. Methods: Between September 1997 and September 2004, a consecutive series of 78 children with HLHS from a single institution underwent the hemi-Fontan procedure: Group 1 (nZ27) after Blalock‐Taussig shunt (BT), and Group 2 (nZ51) after RV‐PA. Hemodynamic, echocardiographic and clinical perioperative data were analyzed. Results: There were no significant differences in the age and operative weight (Group 1: 6.9G1.04 months, 6.22G0.99 kg; Group 2: 6.57G1.12 months, 6.36G0.86 kg). Children after RV‐PA were characterized by a significantly higher preoperative hematocrit value (PZ0.014), lower aortic and superior vena cava oxygen blood saturation (P!0.001, PZ0.024), severe right ventricle hypertrophy more rarely diagnosed in echocardiography (P!0.004), lower Qp:Qs ratio (PZ0.011), larger right (PZ0.001) and left (PZ0.006) pulmonary artery index and a shorter intensive care unit stay after the hemi-Fontan procedure (PZ0.004). Conclusions: The Norwood procedure with the RV‐PA shunt provides satisfactory late hemodynamics and improves the development of the pulmonary arteries. Children with hypoplastic left heart syndrome subjected to this new method of palliation are good candidates for the hemi-Fontan procedure. Q 2005 Elsevier B.V. All rights reserved.

Right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig shunt in preparation to hemi-Fontan procedure in children with hypoplastic left heart syndrome.

Introduction: The causes of coagulation abnormalities and thromboembolic complications during staged Fontan approach in patients with single ventricle remain unclear. This study was designed to evaluate the coagulation profile in the early postoperative period after hemi-Fontan and Fontan procedures with relationship to liver function and hemodynamic variables. Materials and methods: The prospective study on 43 patients after hemi-Fontan (group 1) and 37 patients after Fontan procedure (group 2) was carried out. Coagulation profile (factor VII, factor VIII, ATIII, fibrinogen, prothrombin), liver function (total serum protein, albumin, AST, ALT, bilirubin), and hemodynamic variables were assessed on postoperative day 1 and 5 and compared to preoperative measures. Results: Factor VIII concentration was significantly higher on first postoperative day in both groups. On postoperative day 5 the concentration of factor VIII was significantly decreased in group 1 whereas constant in group 2. The concentration of factor VII, ATIII, fibrinogen, and prothrombin was significantly decreased on first and increased on fifth postoperative day after both hemi-Fontan and Fontan procedures. The increase in bilirubin concentration was more distinctive after Fontan operation (p = 0.003) with lower AST in this group (p < 0.0001). The single ventricle function, pO2 and central venous pressure had significant influence on factor VIII (p = 0.034), factor VII (p = 0.012), ATIII (p = 0.006), and prothrombin (p = 0.024) concentrations in group 2 with no significant influence in group 1. Conclusions: The distinctive causes of coagulation abnormalities during staged Fontan approach are hemodynamic changes and temporary liver dysfunction. Elevated concentration of factor VIII and significant influence of hemodynamics on coagulation profile could contribute to postoperative thromboembolic complications. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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Coagulation abnormalities and liver function after hemi-Fontan and Fontan procedures - the importance of hemodynamics in the early postoperative period.

We present a case of a patient with a severe form of Ebstein’s anomaly diagnosed in a 36th week of gestation through prenatal echocardiographic evaluation. The emergency surgery consisted of closure of the tricuspid valve and creation of a residual atriventricular communication, enlargement of interatrial communication, closure of the pulmonary artery, and construction of an aortopulmonary shunt. Subsequently, the patient underwent the hemiFontan and Fontan operation. We conclude that prenatal echocardiographic diagnosis is crucial for the survival of children with a severe form of Ebstein’s anomaly, which is a neonatal emergency and requires prompt decision mating to initiate surgical management.

Malgorzata Procelewska

Papers

Extracorporeal Life Support in Severe Propranolol and Verapamil Intoxication

Right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig shunt in preparation to hemi-Fontan procedure in children with hypoplastic left heart syndrome.

Coagulation abnormalities and liver function after hemi-Fontan and Fontan procedures - the importance of hemodynamics in the early postoperative period.

Successful surgical treatment of a neonate with prenatal diagnosis of severe Ebstein's anomaly.