Risk Factors for Cardiac Arrest or Mechanical Circulatory Support in Children with Fulminant Myocarditis.

In children with fulminant myocarditis (FM), we sought to describe presenting characteristics and clinical outcomes, and identify risk factors for cardiac arrest and mechanical circulatory support (MCS). A retrospective review of patients with FM admitted at our institution between January 1, 2004, and June 31, 2015, was performed. We compared characteristics and outcomes of FM patients who received cardiopulmonary resuscitation (CPR) and/or were placed on MCS (CPR/MCS group) to those who did not develop these outcomes (Control group). There were 28 patients who met criteria for FM. Median age was 1.2 years (1 day-17 years). Recovery of myocardial function occurred in 13 patients (46%); 6 (21%) had chronic ventricular dysfunction, 6 (21%) underwent heart transplantation, and 3 (11%) died prior to hospital discharge (including one death following heart transplant). Of the 28 FM patients, 13 (46%) developed cardiac arrest (n = 11) and/or received MCS (n = 8). When compared to controls, patients in the CPR/MCS group had a higher peak b-type natriuretic peptide (BNP) levels (p = 0.03) and peak inotropic scores (p = 0.02). No significant differences were found between groups in demographics; chest radiograph, electrocardiogram, or echocardiogram findings; or initial laboratory values including BNP, troponin, C-reactive protein, lactate, and creatinine (p > 0.05 for all). Children with FM are at high risk of cardiovascular collapse leading to the use of CPR or MCS. Aside from peak BNP levels and inotropic scores, the most presenting characteristics were not helpful for predicting these outcomes. FM patients should ideally receive care in centers that provide emergent MCS.

Extracardiac Fontan fenestration using the SafeSept transseptal guidewire and snare-controlled diabolo-shaped covered-stent placement.

Transcatheter Fontan fenestration is a valuable option in situations of prolonged pleural drainage or low cardiac output in patients with failing Fontan circulation. This procedure relies on controlled baffle perforation without separation of the Fontan circuit from the pulmonary venous atrium, and placement of an accurately sized covered stent. We report a novel technique for transcatheter extracardiac Fontan fenestration using the SafeSept transseptal guidewire® and snare-controlled diabolo-shaped covered stent placement.

Blalock-taussig shunt thrombosis prophylaxis in a patient with jacobsen syndrome and thrombocytopenia.

Jacobsen syndrome (JS) is a rare chromosomal anomaly caused by deletions in the distal long arm of chromosome 11. Features of the syndrome include growth and developmental delays, a distinctive facial appearance, and a variety of physical problems including heart defects and bleeding disorders. Congenital heart defects occur in approximately 50% of children with JS. Hypoplastic left heart syndrome (HLHS) has been occasionally reported in association with JS. In such cases, the hematological abnormalities may influence the outcome from single-ventricle palliation through staged surgical reconstruction. Thrombotic obstruction or occlusion of the modified Blalock-Taussig (BT) shunt is a well-documented cause of interstage mortality following the Norwood operation. Although there is no consensus regarding the therapeutic value of antiplatelet therapy during the interstage period following the first stage of palliation, maintenance of shunt patency is critically important. For patients with JS undergoing single-ventricle palliation, decisions regarding antiplatelet therapy during the interstage period may be further complicated by the presence of thrombocytopenia and platelet dysfunction related to JS. We report the case of a patient with HLHS, JS, and thrombocytopenia who underwent the Norwood procedure, and we describe our strategy for prophylaxis against thrombosis of the BT shunt.

Magnetic resonance angiography detection of vascular aneurysms in patients with kawasaki disease and coronary artery aneurysms.

Kawasaki disease is a systemic panvasculitis that causes coronary artery aneurysms in approximately 15% to 25% of untreated patients. Systemic vascular aneurysms may also occur in medium-sized arteries throughout the body and may lead to increased morbidity and mortality in patients with Kawasaki disease. We report a case of diffuse systemic aneurysm formation in a 2-year-old patient with Kawasaki disease with coronary artery aneurysms. Full-body magnetic resonance angiography (MRA) imaging was utilized both in the acute phase and again prior to cardiac catheterization performed at 6 months from the acute illness. The initial MRA detected aneurysmal dilatation of the common and internal iliac arteries bilaterally in the acute phase. Subsequent MRA performed prior to cardiac catheterization 6 months later demonstrated resolution of the iliac artery lesions. Full-body MRA may be useful in screening for associated systemic vascular aneurysms in patients with Kawasaki disease and associated coronary artery aneurysms.