Orthotopic heart transplantation in a child with hereditary spherocytosis.

Hereditary spherocytosis (HS) is a genetic, frequently familial hemolytic blood disease that presents with varying degrees of hemolytic anemia, splenomegaly, and jaundice.The disease arises as a result of defects in any of a number of proteins responsible for maintaining the shape and flexibility of the red blood cell, resulting in an osmotically fragile and characteristically spherical red blood cell. Theoretically, cardiopulmonary bypass can exacerbate hemolysis and subsequent renal dysfunction.There are few reports of open heart surgery for patients with HS and none for orthotopic heart transplantation.We report a 6-year-old boy with HS who underwent orthotopic heart transplantation.

Anesthetic vaporizer mount malfunction resulting in oxygenation failure after initiating cardiopulmonary bypass: specific recommendations for the pre-bypass checklist.

Modern technologic advances in medicine have allowed commonly used machines to perform safely with very low risk and a high degree of success. To detect or prevent potential malfunctions, professionals routinely perform pre-use checks for equipment such as anesthesia machines and cardiopulmonary bypass (CPB) machines. These machine checklists are not only critical for a safe operation but also have large impacts on outcomes. For example, when malfunctions are encountered that could have potential negative ramifications or adverse outcomes, multi-approach strategies should be used to identify rectifiable causes and find solutions that are practical. This information can be used to promulgate safe practice guidelines. This case report identifies a machine-based contributing factor to precipitous hypoxia on initiation of bypass in one of our patients. After a detailed approach to identify preventable root causes, we made simple additions to our pre-bypass checklist and recommend these changes to other institutions.

Superior vena cava perforation in a child from a lawnmower projectile.

Lawnmowers are one of the most frequent causes of mutilating injuries to children. The majority of accidents are caused by negligence of the operator. Most injuries of this type are caused by direct contact with the spinning blade and, less frequently, by projectiles propelled by the blade. Such projectiles usually produce bruises, but can penetrate skin and soft tissues. This report presents a 6-year-old child who suffered a small, outwardly insignificant puncture wound of the chest from a lawnmower-propelled projectile who presented with fever and chest pain the following day. The diagnostic work up and treatment of this deceptively life-threatening wound are discussed.

High-dose argatroban for heparin-induced thrombocytopenia in a child using a ventricular assist device.

A 10-year-old boy who was receiving support from a ventricular assist device (VAD) experienced heparin-induced thrombocytopenia that was successfully treated with high-dose argatroban infusion to attain therapeutic activated partial thromboplastin time in spite of high serum argatroban levels. This case also highlights bolus argatroban dosing for VAD change in the setting of persistent ventricular fibrillation.

Preliminary single center North American experience with the Berlin Heart pediatric EXCOR device.

For children requiring mechanical circulatory support as a bridge to cardiac transplantation in North America, options previously were limited to extracorporeal membrane oxygenation (ECMO) or centrifugal pump ventricular assist, both of which were suitable for only very short term application and were associated with significant complications and limitations. The Berlin Heart EXCOR ventricular assist device (VAD) was recently introduced into practice in North America to address this deficiency. We report a preliminary single center experience with the EXCOR in 17 children, 13 who received only a left-sided pump and four who required biventricular support. Before EXCOR placement, six patients were on ECMO, and one was on a centrifugal VAD. Eleven children were bridged to transplantation, one was bridged to recovery, and one remains on support. Three children died during support and one died after explantation. There was one late death nearly 2 years after transplant. Complications included stroke in seven patients, two of which were ultimately fatal. Five patients required re-operations for bleeding or evacuation of hematoma. Despite a disappointing rate of neurologic morbidity, our preliminary experience with the EXCOR has been very encouraging.