Acute Myocardial Ischemia Following Bee Sting in an Adolescent Male: A Case Report.

BACKGROUND Epinephrine for anaphylactic shock is the standard life-saving treatment in the emergency department. Cardiac symptoms after epinephrine administration in a child with no prior cardiac history are often not suspected. We describe a presentation of diastolic cardiac dysfunction after anaphylaxis from a bee sting in an adolescent male. CASE REPORT A 16-year-old male with no prior history of allergy presented with anaphylaxis following a bee sting. The patient received an inadvertent intravenous rather than intramuscular dose of 1: 1000 epinephrine, leading to myocardial ischemia. Diastolic dysfunction resulting from myocardial ischemia and fluid resuscitation led to development of pulmonary edema. The patient required epinephrine drip for hemodynamic support and BiPAP for respiratory support. CONCLUSIONS This case highlights the risk of giving a rapid intravenous push of epinephrine, which converted an anaphylactic reaction to cardiogenic shock. Anaphylaxis-related coronary ischemia (Kounis) syndrome is another less likely etiology for our patient's presentation.

Quality of life in adult survivors greater than 10 years after pediatric heart transplantation.

BACKGROUND: This study assessed quality of life (QOL) in adult survivors of pediatric heart transplantation who survived > or = 10 years after transplantation. METHODS: Prospective data were collected from heart transplant recipients who were aged > or = 18 years and had survived > or = 10 years after transplantation (transplantation between July 3, 1986, and April 4, 1997). QOL data were collected from patients using the Medical Outcomes Study 36-Item Short Form (SF-36) Health Survey. Clinical data were collected from medical records. Statistical analyses included frequencies and measures of central tendency. RESULTS: Twenty-three patients (65% men, 91% white) completed the study. At the study initiation, they were a mean age of 9.0 +/- 7.1 years at transplantation, and were a mean age of 25.2 +/- 5.5 years (range, 18-34 years) and a mean of 16.2 +/- 3.0 years (range, 11-22 years) post-transplantation. Most were in school or working. Mean patient QOL scores from the SF-36v2 survey were 50.56 +/- 0.5 (range, 27.3-68.9) for physical health and 49.88 +/- 11.72 (range, 23.56-62.84) for mental health, similar to the general United States population. Late complications were frequent, including transplant coronary artery disease, 3; repeat heart transplantation, 2; post-transplantation lymphoproliferative disorder, 6; kidney transplantation, 5; acute late rejection, 5; and arrhythmias, 4. CONCLUSION: This report of QOL in adult survivors of pediatric heart transplantation shows patient perception of physical and mental health is similar to the general population despite serious late complications. A multicenter study is planned to further evaluate QOL in this unique cohort.

Infectious, malignant, and autoimmune complications in pediatric heart transplant recipients.

OBJECTIVE: To review clinical courses of pediatric heart transplant survivors after 5 years from transplantation for infections, lymphoproliferative, and autoimmune diseases. STUDY DESIGN: A total of 71 patients were examined in 2 groups, infant recipients (underwent transplant <1 year of age, n = 38) and older recipients (underwent transplant >1 year, n = 33). All patients received comparable immunosuppression. Calculated occurrence rates were reported as means per 10 years of follow-up with SEs. Differences were examined by using Poisson regression. RESULTS: Infant recipients had significantly higher (P < .001) occurrence rates of severe (mean, 2.04 +/- 0.5) and chronic infections (mean, 4.58 +/- 0.67) compared with older recipients (means, 0.37 +/- 0.19 and 1.87 +/- 0.70, respectively). Types of infections were similar to those in the general population with extremely rare opportunistic infections; however, they were more severe and resistant to treatment. Autoimmune disorders occurred at a frequency comparable with lymphoproliferative diseases and were observed in 7 of 38 infants (18%). Most common were autoimmune cytopenias. CONCLUSIONS: Infant heart transplant recipients who survive in the long term have higher occurrence rates of infections compared with older recipients. Autoimmune disorders are a previously unrecognized morbidity in pediatric heart transplantation.

Regional variation in the measured apparent ultrasonic backscatter of mid-gestational fetal pig hearts.

The goal of this study was to characterize and compare regional backscatter properties of fetal hearts through measurements of the apparent integrated backscatter. Sixteen excised, formalin-fixed fetal pig hearts, representing an estimated 53 to 63 days of gestation, were investigated. Spatially localized measurements of integrated backscatter from these specimens were acquired using a 50 MHz single-element transducer. The apparent integrated backscatter measurements demonstrate different patterns of backscatter from the myocardium of the right ventricle compared with that of the left ventricle. These backscatter measurements appear to be consistent with the anisotropy of the fiber orientation observed in histologic assessment of the same specimens. For each of the 16 hearts, the apparent integrated backscatter from the right ventricular myocardium was larger than that from the left ventricular myocardium, exhibiting mean apparent backscatter values of -35.9 +/- 2.0 dB and -40.1 +/- 1.9 dB (mean +/- standard deviation; n = 16; p < 0.001), respectively. This study suggests that the intrinsic ultrasonic properties of the left and right ventricular myocardium are distinct in fetal pig hearts at mid-gestation.

Infant heart transplantation ten years later--where are they now?

BACKGROUND: Many uncertainties regarding the fate of children undergoing heart transplantation as infants were present when we and others embarked on this program. Although no truly long-term results are available, a significant cohort of children has now reached preteen and early teenage status. We reviewed our group of infants transplanted more than 10 years ago to assess survival and quality of life as they approach their teenage years. METHODS: We retrospectively reviewed the medical records of all infant (younger than 6 months of age) heart transplant recipients, transplanted between 1988 and 1995, to ascertain survival statistics, incidence of complications, and current health status. RESULTS: A total of 42 patients were identified. The majority of these underwent transplantation for hypoplastic left heart syndrome. Eleven patients have died, 4 early and 7 late. The actual survival at 10 years is 76%. Twenty-seven of the 31 long-term survivors attend regular school; 4 are in special education classes owing to developmental delay. Five patients take medication for attention-deficit disorder. Malignancies have been discovered in 5, and 1 died secondary to this. Six patients have significant renal insufficiency, 1 of whom has undergone renal transplantation. One patient has undergone retransplantation for coronary artery disease. One patient required reoperation for supravalvar aortic stenosis. Other general medical problems that are being treated include sleep apnea (n = 1), hypertension (n = 5), and recurrent pneumonias (n = 1). CONCLUSIONS: Although these children require ongoing medical attention, including daily medications and regular follow-up visits, most have a satisfactory quality of life and behave much like normal children.

Sudden arrhythmia death syndrome: importance of the long QT syndrome.

In approximately 5 percent of sudden cardiac deaths, no demonstrable anatomic abnormality is found. Some cases are caused by sudden arrhythmia death syndrome. A prolonged QT interval is a common thread among the various entities associated with sudden arrhythmia death syndrome. A number of drugs are known to cause QT prolongation (e.g., terfenadine), as are hypokalemia, hypomagnesemia, myocarditis, and endocrine and nutritional disorders. Recently, attention has focused on a group of inherited gene mutations in cardiac ion channels that cause long QT syndrome and carry an increased risk for sudden death. Some of the highest rates of inherited long QT syndrome occur in Southeast Asian and Pacific Rim countries. The median age of persons who die of long QT syndrome is 32 years; men are predominately affected. In addition to a prolonged QT interval, which occurs in some but not all persons with long QT syndrome, another characteristic electrocardiographic abnormality is the so-called Brugada sign (an upward deflection of the terminal portion of the QRS complex). Most cardiac events are precipitated by vigorous exercise or emotional stress, but they also can occur during sleep. Torsades de pointes and ventricular fibrillation are the usual fatal arrhythmias. Long QT syndrome should be suspected in patients with recurrent syncope during exertion and those with family histories of sudden, unexpected death. Unfortunately, not all persons with long QT syndrome have premonitory symptoms or identifiable electrocardiographic abnormalities, and they may first present with sudden death. Beta blockers, potassium supplements, and implantable defibrillators have been used for treatment of long QT syndrome. Identifying the specific gene mutation in a given patient with long QT syndrome can help guide prophylactic therapy.