Richard J. Ing

Tetralogy of Fallot: Perioperative Anesthetic Management of Children and Adults

Tetralogy of Fallot (TOF) is a common congenital heart defect in children. Perioperative considerations include preoperative preparation for surgery, intraoperative anesthetic management, and common postoperative issues in the intensive care unit. Surgical debates have shifted away from 2-stage versus single-stage repairs to debates of how surgery to limit pulmonary insufficiency (PI) may have significant long-term impact as the child grows. There are many adult survivors of TOF repair in infancy who now present with a unique set of problems related to PI and right ventricular dysfunction. These adults provide new insights into congenital heart disease (CHD) and how management strategies early in life may have significant implications much later in life. Patients with complex CHD should have lifelong follow-up, so our knowledge will continue to improve, and the best possible care can be provided for these patients.

The Year in Review Anesthesia for Congenital Heart Disease 2014

Congenital cardiac anesthesiology is a rapidly expanding field at both ends of the life spectrum. The care of the unborn child with congenital heart disease is becoming highly specialized in regional centers that offer advanced imaging techniques, coordinated specialist care, and potentially fetal interventions. As more children with congenital heart disease survive to adulthood, patients and their health care providers are facing new challenges. The growing volume of publications reflects this expanding field of congenital cardiac anesthesiology. This year in review article highlights some developing trends in the literature.

Aortoesophageal Fistula and Coarctation of the Aorta in a 15-Year-Old Child

Delayed presentation of coarctation of the aorta can be associated with severe complications. A challenging case of aortoenteric fistula secondary to an aneurysm from coartation of the aorta presented with massive gastrointestinal hemorrhage. The clinical management of this patient and a review of the literature are presented.

Noteworthy Literature in 2015 Anesthesia for Congenital Heart Disease

This article is a review of the literature published during the 12 months of 2015, which is of interest to the congenital cardiac anesthesiologist. While the review is not exhaustive, it identifies 7 themes in the literature for 2015 and cites 78 peer-reviewed publications.

Lack of Association Between Adrenoreceptor Genotype and the Vasoconstriction Response to Dexmedetomidine

An exaggerated vasoconstriction response to dexmedetomidine, an α-2 adrenergic agonist, has been associated with 2 genotypes: a deletion in the α-2B adrenoreceptor gene (ADRA2B deletion) and SNP rs9922316 in the gene for protein kinase C type β (PRKCB). We hypothesized that children with a marked systemic vascular resistance index (SVRI) increase following intravenous dexmedetomidine bolus would carry these genotypes. Following institutional review board approval, DNA samples from 16 children with transplanted hearts who participated in a study in the cardiac catheterization laboratory of hemodynamic responses to dexmedetomidine boluses underwent genotyping by polymerase chain reaction (PCR) amplification and PCR Sanger sequencing for the ADRA2B deletion and for PRKCB rs9922316. A wide range of SVRI (−12% to +76%, median 33%) and mean arterial blood pressure (MAP; −7% to +50%, median 26%) responses to dexmedetomidine was observed. The responses were not significantly different among genotype groups. An association between exaggerated SVRI or MAP responses and either ADRA2B deletion or PRKCB rs9922316 was not observed.

Successful Treatment of Myocardial Infarction in an Infant With Kawasaki Disease

Although early treatment with intravenous immunoglobulin reduces the risk of coronary artery aneurysms, in refractory cases of Kawasaki disease, myocardial infarction can result from thrombosis of coronary artery aneurysms. Early recognition of myocardial infarction from Kawasaki disease myocarditis can reduce morbidity and mortality. This report describes successful treatment of myocardial infarction from coronary thrombosis in an infant with Kawasaki disease using intravenous tissue plasminogen activator and abciximab.

Perioperative Management of Shock in Two Fontan Patients With Plastic Bronchitis

Plastic bronchitis is potentially a life-threatening complication of long-standing surgically palliated single ventricle congenital heart disease. Patients can present with hypoxia requiring urgent bronchoscopy for removal of bronchial casts. Perioperative care for these patients is challenging and anesthesia is associated with significant cardiac risk. As more surgically corrected single ventricle patients survive to adulthood, these patients are expected to present more frequently. This report details the perioperative management of 2 Fontan patients with hypoxia and significant plastic bronchitis disease burden.

Anesthetic Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve

IN SOME OF the earliest descriptions, the incidence of tetralogy of Fallot with absent pulmonary valve (TOF/APV) was estimated to occur in 3% to 6% of patients. It is a rare congenital heart malformation associated with a rudimentary or nearly absent pulmonary valve with or without an obstructive pulmonary valve annulus. The infundibulum orientation and severity of pulmonary valve annular stenosis determine the post-stenotic pulmonary blood flow jet in systole and subsequent regurgitation in diastole. It is thought that this excessive velocity and volume of blood flow throughout the cardiac cycle both lead to marked aneurysmal dilatation in either the right or left pulmonary arteries. In some patients, subsequent central and intrapulmonary compression of the airways from the enlarged pulmonary arteries results in significant respiratory complications soon after birth. In other patients with less severe disease, respiratory complications are not as debilitating, and these patients present later in life. The variable clinical course and subsequent surgical outcome of this disease are determined largely by the difficulties managing the degree of tracheobronchial compression and bronchomalacia in these patients. Respiratory difficulty requiring intubation and ventilation soon after birth has been the most important predictor of poor outcome in these patients. Patients presenting during infancy with respiratory difficulties are more likely to undergo surgery earlier in an effort to avoid the development of long-term airway compression, air trapping, localized lobar emphysema, and pneumothorax. Knowledge of this anatomic variant of TOF/APV is important as it has historically been associated with a much higher mortality than other variants of tetralogy of Fallot. During the 1980s, in approximately 70 infants younger than 1 year of age, the operative mortality was reported to be 35% to 100%. Two and a half decades later, surgical outcomes had improved significantly to greater than 89% survival. One of the reasons for this improvement was the addition of a reduction pulmonary arterioplasty at the time of primary surgical repair. Additionally, mobilization of the pulmonary arteries to the hilum of the lungs and mobilization of the

Cardiac Injury After All-Terrain Vehicle Accidents in 2 Children and a Review of the Literature.

Abstract All-terrain vehicle (ATV) accidents leading to severe morbidity and mortality are common. At our institution, 2 children presented within weeks of each other after ATV accidents. Both children required cardiac valve surgery. The surgical management of these 2 children is discussed, and the literature is reviewed. On initial patient presentation, the diagnosis of a ruptured cardiac valve or ventricular septal defect (VSD) associated with these types of accidents is often delayed. We propose that patients presenting with evidence of high-energy blunt thoracic trauma after an ATV accident should undergo an electrocardiogram, cardiac enzyme assessment, and cardiac echocardiogram as part of the initial work-up to rule out significant myocardial injury.