The decision to perform biventricular repair for infants with multiple obstructive or hypoplastic left heart lesions (LHL) and borderline left ventricle (LV) may be controversial. This study sought to assess the mortality and morbidity of patients with LHL after biventricular repair and to determine the growth of the left-sided cardiac structures. Retrospective analysis of 39 consecutive infants with LHL who underwent biventricular repair was performed. The median age at surgery was 7 days (range 1-225 days), and the median follow-up period was 34 months (range 1-177 months). Between diagnosis and the end of the follow-up period, the size of the aortic annulus (z-score -4.1 ± 2.8 vs. -0.1 ± 2.7) and the LV (LV end-diastolic diameter z-score -1.7 ± 2.8 vs. 0.21 ± 1.7) normalized. During the follow-up period, 23 patients required 39 reinterventions (62%) consisting of redo surgery for 21 patients (57%) and catheter-guided reinterventions for 8 patients (22%). At the end of the follow-up period, 25 of 34 patients were doing subjectively well; 10 children (29%) received cardiac medication; 12 (35%) presented with failure to thrive (weight ≤ P3) and 5 (15%) with pulmonary hypertension. The overall mortality rate was 13%. Biventricular repair for patients with multiple LHL results in sufficient growth of the left-sided cardiac structures. Nevertheless, residual or newly developing obstructive lesions and pulmonary hypertension are frequent, causing significant morbidity that requires reintervention.
This report describes an 8-year-old child with acute anthracyclineinduced cardiomyopathy triggered by human herpesvirus 6 and the subsequent implantation of an intracorporeal continuous-flow left ventricular assist device (LVAD) and the process to discharge the child from the hospital. After barely 3 months on mechanical support, the device was explanted after thorough examination. Experiences regarding LVAD removal are limited, and no guidelines for echocardiographic and hemodynamic criteria for LVAD removal in children have been published thus far. We present our institutional algorithm for device selection, surveillance in an ambulatory setting, and testing for myocardial recovery, as well as our criteria for LVAD explantation in children. Pediatrics 2014;134:e894-e899 Drs Cavigelli-Brunner and Schweiger conceptualized and designed the study, drafted the initial manuscript, and were involved in the direct care of the patient described; Dr Knirsch drafted the initial manuscript and supervised and critically reviewed the manuscript; Dr Stiasny reviewed and revised the manuscript and was involved in the direct care of the patient described; Dr Klingel critically reviewed and revised the manuscript; Drs Kretschmar and Hübler supervised and critically reviewed the manuscript; and all authors approved the final manuscript as submitted. Experiences with intracorporeal left ventricular assist devices (LVADs) in children suffering from anthracyclineinduced cardiomyopathy (CMP) with subsequent myocardial recovery and LVAD explantation are rare. 1-3 Protocols for device selection, home discharge, outpatient monitoring, and adequate evaluation for myocardial recovery and decision algorithms for device explantation are missing or have to be generated for this specific patient group.We describe the use of an intracorporeal continuous-flow LVAD in a child with acute heart failure due to anthracycline-induced CMP triggered by human herpesvirus 6 (HHV6), her subsequent discharge home, and successful device explantation after 149 days on support. CASE REPORTAn 8-year-old girl (body weight: 25 kg; body surface area [BSA]: 0.97 m 2 ) was admitted to our hospital due to severe, rapid-onset heart failure. Seven months before, she had been diagnosed with a bone sarcoma of the left tibia and was treated by surgical resection and chemotherapy according to the EURAMOS-1 Trial (The European and American Osteosarcoma Study Group), including methotrexate, cisplatin and doxorubicin(cumulative doses: 450 mg/m 2 ). Ten days after the last chemotherapy course, her general condition worsened, and echocardiography showed a dilated left ventricle, a biventricular reduced contractility with an ejection fraction (EF) of 25%, and a shortening fraction of 15%. Despite maximal inotropic support and mechanical ventilation, the patient' s condition deteriorated, and an LVAD implantation with the use of an LVAD HeartWare Ventricular Assist System (HeartWare Inc, Framingham, MA) was successfully performed (Fig 1).Apart from prolonged medical sup...
Extracorporeal membrane oxygenation (ECMO) is a general term that describes the short-or long-term support of the heart and/or lungs in neonates, children and adults. Due to favorable results and a steady decline in absolute contraindications, its use is increasing worldwide. Indications in children differ from those in adults. The ECMO circuit as well as cannulation strategies also are individualized, considering their implications in children. The aim of this article is to review the clinical indications, different circuits, and cannulation strategies for ECMO. We also present our institutional experience with 92 pediatric ECMO patients (34 neonates, 58 pediatric) with the majority (80%) of veno-arterial placements between 2014 until 2018. We further to also highlight ECMO use in the setting of cardiac arrest [extracorporeal cardiopulmonary resuscitation (CPR) or eCPR].
Evolution of ventricular assist devices (VADs) leading to miniaturization has made intracorporeal implantation in children feasible. Ventricular assist device therapy for anthracycline-induced cardiomyopathy (CMP) in adults has been reported. We report the case of an 8-year-old child (body surface area 0.97 m) presenting with anthracycline-induced CMP being successfully treated with an intracorporeal left ventricular assist device (LVAD) as a bridge to candidacy/recovery. We present our institutional algorithm, which advises intracorporeal LVAD implantation for long-term ventricular assist, in children with a body surface area >0.6 m. Advantages are better mobilization and the possibility to discharge home, leading to enhanced quality of life.
We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.
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