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Bullet embolus is a rare complication of penetrating missile trauma. Removal of the bullet previously required surgery. We report the case of a 14-year-old with an hepatic vein bullet embolus following a gunshot wound to the left buttock. A transjugular approach was used to extract the bullet percutaneously with an Amplatzer gooseneck snare.

Background: Calcineurin inhibitors such as cyclosporine are effective in preventing rejection in recipients of solid organ transplants. Unfortunately, the prolonged use of calcineurin inhibitors may result in progressive renal injury., Methods: We studied the renal function of 15 pediatric heart transplant recipients who were taking calcineurin inhibitors. Their renal function was studied before and after rapamycin was introduced to their immunosuppression regimen. With the introduction of rapamycin, the patients were given a lower dose of calcineurin inhibitors, and the calcineurin inhibitor was discontinued in 5 patients., Results: Renal function improved significantly in the patients by 30 days after these changes in the calcineurin inhibitor dose were instituted. Mean levels of blood urea nitrogen and mean serum creatinine decreased, and mean creatinine clearance increased. Pre-rapamycin, the patients' mean level of blood urea nitrogen was 27.1 +/- 12.4 mg/dl and post-rapamycin they decreased to 18.6 +/- 11.1 mg/dl (p = 0.014). Similarly, creatinine decreased from 1.0 +/- 0.5 mg/dl to 0.8 +/- 0.3 mg/dl (p = 0.019). Their creatinine clearance increased from 88 +/- 28 ml/min/1.73 mol/liter2 to 105 +/- 27 ml/min/1.73 mol/liter2 (p = 0.008). The patients' lipid levels did not change after they were prescribed rapamycin. Biopsy-negative rejection developed in 2 patients., Conclusions: The introduction of rapamycin to the immunosuppressive regimen of patients taking calcineurin inhibitors, with a concomitant reduction of the calcineurin inhibitor dose, may improve renal function within 30 days, without a significant increase in rejection.

Patients with pre-formed antibodies may be at increased risk of rejection after organ transplantation. In this report, we describe the use of rituximab to decrease the percentage of pre-formed antibodies in a pediatric heart transplant recipient.

Background: Aberrant origin (ABO) of a coronary artery (CA) from the contralateral aortic sinus with a subsequent interarterial course is a life-threatening condition. It carries a 28% to 55% risk for a sudden coronary event or death, but there are no reliable screening methods., Objective: We sought to determine whether imaging a cross section of a coronary segment in the anterior aortic wall on the long-axis view may be used as an echocardiographic screening sign for ABO CA., Methods: The echocardiograms of all patients with ABO CA were evaluated for the screening sign and compared with those of age-matched control patients., Results: Between January 1989 and October 2002, we identified 8 patients with ABO CA (median age: 15 years). Of these patients, 4 were symptomatic and 4 were discovered incidentally. The electrocardiogram produced normal findings in 5 of 8 patients, maximal stress test produced normal findings in 5 of 6 patients, and thallium perfusion test produced negative results in 2 of 3 patients. There were 4 patients with ABO in the main left CA, 3 with ABO in the right CA, and 1 with ABO in the circumflex CA branch. The screening sign was readily visible in 7 of the 8 patients (88%), and all 7 of these patients had ABO in a main CA. Only in the ABO in the circumflex CA branch was the screening sign not detected. The 1743 control patients (median age: 14 years) showed normal anterior aortic wall, which was void of any CA segment. In 5 control patients (0.3%, P <.001) the normal right CA was visible on the long-axis view, but was not confused for a false-positive screening sign., Conclusion: We concluded that the proposed screening sign for ABO CA is reliable and easily recognizable, and should prompt a comprehensive assessment of the CA.

The optimal management strategy of the neonate and young infant with native aortic coarctation (AC) is controversial. We reviewed our experience with balloon angioplasty (BA) in neonates and infants 3 months to test our thesis that BA provides successful palliation, defined as avoidance of surgery for 4 weeks along with control of presenting symptoms. We also compared the results of the transumbilical arterial (UA), transfemoral arterial (FA) and transfemoral venous anterograde (FVA) approaches we have used to accomplish BA. During a 6.5-year period ending June 2001, fifty-one neonates and infants 3 months presenting with heart failure, hypertension or both underwent UA (n = 16), FA (n = 26) and FVA (n = 9) balloon coarctation angioplasty. Immediate and follow-up results were evaluated. Acute reduction of peak-to-peak gradients across the coarctation (40 17 mmHg vs. 5 6 mmHg; p < 0.001), increase in diameter of the coarcted segment (2.2 0.5 mm vs. 5.6 0.8 mm; p < 0.001) and improvement in symptomatology occurred following BA. Surgical relief of aortic obstruction was required in 4 infants at 5, 21, 24 and 28 days after the procedure. Effective palliation was thus achieved in the remaining 47 infants (92%). During intermediate-term follow-up, twenty-two infants (50%) developed recoarctation requiring repeat balloon (n = 14) or surgical (n = 8) intervention 2 10 months (median, 3 months) after initial BA. The indication for reintervention was hypertension in all patients. At a median follow-up of 3 years (range, 0.5 5.5 years), blood pressures remained low (98 11 mmHg) with an arm/leg blood pressure gradient of 4 6 mmHg. Comparison of the groups revealed similar effectiveness both immediately and at follow-up. However, femoral artery complications were seen in only the FA group. Based on these data, we conclude that effective palliation is achieved with BA in all 3 groups, femoral artery complications are seen only in the FA group and BA is an excellent alternative to surgical intervention in the management of native AC in neonates and young infants.

The use of color Doppler interrogation significantly improves accuracy in the evaluation of coronary artery anatomy. This was demonstrated in a patient in whom the left coronary artery originated from the posterior and medial aspect of the pulmonary artery, a particularly difficult coronary anomaly to detect by 2-dimensional imaging. In addition to the coronary artery anomaly, this patient also had an arcade mitral valve.

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

Balloon valvuloplasty provides optimal treatment for moderate and severe pulmonary valve stenosis. Dysplastic pulmonary valves may not respond to balloon dilation and frequently require surgical treatment. Balloon angioplasty with or without stenting is the preferred treatment of peripheral pulmonary stenosis.

Recent reports suggest reopening of the patent ductus arteriosus (PDA) after complete occlusion with three-loop Gianturco coils. We hypothesize that five-loop coils may produce a larger thrombus than three-loop coils, which will result in no or less probability of recanalization of PDA during follow-up. This study is designed to test this hypothesis. Follow-up echocardiographic and Doppler data of 30 patients who underwent five-loop coil occlusion of small to medium-sized PDA during a 33-month period ending December 1998 were examined. Thirty patients had no residual shunt on echo Doppler study on the day following the procedure and were followed for 6 to 30 months (median, 12) after coil implantation. At the last follow-up study, none of the patients had a residual shunt and left atrial size decreased. Careful pulsed, continuous wave, and color Doppler interrogation of left/main pulmonary artery junction and proximal descending aorta did not reveal any evidence for obstruction. The follow-up data suggest that complete occlusion of small- to medium-sized PDAs is feasible with five-loop coils without evidence for recanalization at a mean follow-up of 12 months. Much longer (2 to 5 years) follow-up data may be necessary to confirm these observations. We speculate that a greater degree of thrombosis is produced within the ductus by the five-loop coils, which in turn may be responsible for lack of shunt recurrence. We recommend use of five-loop instead of three-loop coils for transcatheter occlusion of small- to medium-sized PDAs.

Coronary artery stenosis (CAS) and coronary ostial stenosis (COS) are potentially life-threatening conditions. The echocardiographic diagnosis of CAS and COS in children has not been described. We report on the transthoracic echocardiography (TTE) findings of CAS and COS in children. Six patients, aged 1 week to 12 years, with clinically confirmed COS (n = 5) and CAS (n = l) were diagnosed by TTE. Their echocardiographic findings were compared with 26 healthy control subjects of a similar age range. Left COS was associated with an aberrant left coronary artery (CA) from the contralateral aortic sinus (n = 2), an intramural left CA with d-transposition of the great vessels (n = l), and supravalvular aortic stenosis (n = l). Right COS was present in a patient with aortic valvular stenosis. Acquired left main CAS was diagnosed in the sixth patient 3 years after orthotopic heart transplantation. Coronary ostial stenosis was recognized when a color flow acceleration signal was present proximal to and extending into the coronary ostium (CO). Coronary artery stenosis was detected when a coarctated color flow stream was present within the stenosed CA segment with turbulent distal flow. These findings were not detected in the control cohort who demonstrated laminar CA and CO flow signals. All patients had increased spectral velocity in the CA distal to the stenosed segment (patients = 50 +/- 5 cm/s, controls = 24 +/- 6 cm/sec; P <.01). Delayed peak diastolic velocity seemed to indicate severe stenosis. We conclude that (1) CO acceleration signals and turbulent coarctated CA flow signals are abnormal findings in TTE coronary Doppler assessment. They indicate COS and CAS, respectively. (2) Knowledge of the normal TTE CA flow velocity patterns is essential so that abnormal velocity signals such as seen with CAS and COS can be recognized and a timely diagnosis made.

It is generally believed that balloon angioplasty of diffuse, long-segment aortic coarctation is not effective. In this report, we describe two neonates with diffuse, long-segment coarctation in association with complex congenital heart defects in whom we were successful in effectively treating coarctation with transumbilical artery balloon angioplasty. Based on this experience, it may be concluded that balloon angioplasty of long-segment coarctation in neonates is feasible and effective, but confirmation in a larger group of patients may be necessary prior to general adoption of this concept.

Collaterals are described between coronary and pulmonary arteries after orthotopic heart transplantation. It is likely the collaterals developed as a result of adhesions in the pericardial space. This is a previously unrecognized complication of heart transplantation with potential clinical implications, as coronary to extracardiac artery collaterals have been shown to cause myocardial ischemia.

Post-transplant lymphoproliferative disease (PTLD) is a well-known complication of solid organ transplantation. While this disorder can often be controlled by decreasing immunosuppression, it is frequently difficult to control the resultant rejection. This case exemplifies this dilemma. To solve this problem, cyclosphosphamide (600 mg/m2) and prednisone (2 mg/kg times 5 days) were given every 3 weeks to simultaneously treat PTLD and provide immunosuppression.

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 +/- 23 versus 24 +/- 6 cm/sec, p = 0.0004), increased diastolic flow (16 +/- 15 versus 6 +/- 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% +/- 14% versus 20% +/- 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m2 (r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight (p < 0.001, r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve.

We report on 4 of 9 sibs with a syndrome of stenosis of the renal arteries and chronic hypertension, variable stenosis or occlusion of cerebral, abdominal and probably coronary arteries due to suspected fibromuscular dysplasia, congenital cardiac abnormalities, brachydactyly and syndactyly of the hands and feet, and increased bone fragility consistent with a mild form of osteogenesis imperfecta. Three affected individuals have had mild to moderate learning disabilities. The parents and the remaining 5 sibs have normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including fibromuscular dysplasia, brachydactyly, syndactyly, and osteogenesis imperfecta, and are autosomal dominant traits in many cases. Explanations for this familial occurrence include autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.

Chronic chest pain is a complaint that frequently prompts referral to pediatric cardiology clinics although very few pediatric patients with this symptom will be found to have cardiac disease. This review will discuss the common non-cardiac and cardiac causes of chest pain. Guidelines on the office management of this group of patients will be presented as well as the indications for referring patients for evaluation and treatment by the subspecialist.

This report describes a 1 day-old infant with valvar pulmonary atresia with intact ventricular septum in whom we were successful in performing transcatheter guidewire perforation and balloon pulmonary valvuloplasty to establish right ventricle-to-pulmonary artery continuity and flow. Also described is implantation of a 4 mm coronary stent into ductus arteriosus in lieu of surgical aortopulmonary shunt to treat pulmonary oligemia and systemic arterial hypoxemia. Details of transcatheter guidewire perforation are presented and it is suggested that this method be used as an alternative to Laser/radio frequency wires, especially in the absence of approval of the latter wires by the regulatory agencies. Stenting of the ductus may be considered an alternative to surgical aortopulmonary shunt. Role of transcatheter technology in the management of selected patients with pulmonary atresia and intact ventricular septum is discussed.

Coil occlusion of patent ductus arteriosus with 5-loop coils was undertaken in 10 patients without coil embolizations, and with 90% immediate occlusion and 100% occlusion at follow-up. We conclude that 5-loop coil occlusion of patent ductus arteriosus is safe and effective.

A survey of 870 different adult blood samples (primarily from patients with non-haematological disorders) found that 269 (31%) had increased proportions (> 25%) and/or absolute numbers (> 1.0 x 10(9)/l) of morphologically-defined large granular lymphocytes (LGL), and/or phenotypically-defined NK-associated (NKa) cells. Of these, 112 were re-analysed at least 6 months after initial presentation and were classified as 'persistent' (92/112) or 'transient' (20/112) according to whether or not the original abnormality was still present. Lymphocyte counts in most patients with persistent abnormalities were within normal limits (18/92) or slightly increased (68/92), with only six having a lymphocytosis exceeding 10.0 x 10(9)/l. With the exception of five persistent LGL expansions in which the granular lymphocytes did not express NKa determinants (designated LGL+NKa-), the remaining 87 cases could be phenotypically grouped according to their primary abnormality as CD8+NKa+ (n = 33), CD4+ NKa+ (n = 14), CD8dim+NKa+ (n = 7) or CD8-NKa+ (n = 33). TCR genotypic studies in 58 patients showed that the 16 patients with rearranged TCR components were restricted to the CD8+NKa+ group and that, in most of these, the CD8+ fraction showed abnormal relative CD16/CD56 expression. Persistent neutropenia (n = 15) also appeared to be associated with primary abnormalities of CD8+NKa+ cells (12/15), with 10 of these additionally showing rearranged TCR genes. In contrast, persistently increased CD8dim+NKa+ and CD8-NKa+ components did not appear to phenotypically differ from their corresponding 'counterparts' in normal bloods or in patients with transient LGL/NKa+ abnormalities. This survey has therefore established that persistent LGL/NKa+ abnormalities are considerably more common than suggested in published work, that a high proportion of patients with expanded CD8+NKa+ components, with quite diverse clinical histories, show evidence of clonal lymphoid populations, and that the clonal nature of such disorders appears to be associated with abnormal NKa phenotypic patterns.

Sixteen patients participated in a Pediatric/Young Adult Cardiac Rehabilitation Program that included exercise training, education about cardiovascular diseases, dietary counseling, and counseling on stress management. Seven patients completed the program, and complete data were available on six. The subjects demonstrated significant changes in their hemodynamics and exercise tolerance after completing the program. Resting blood pressure decreased by 7%, from 119 +/- 12 to 111 +/- 10 mm Hg; peak oxygen consumption increased by 20%, from 31.9 +/- 4.3 mL/kg of body weight per minute to 38.4 +/- 6.0 mL/kg of body weight per minute; and exercise treadmill time increased by 21%, from 8.5 +/- 1.4 to 10.3 +/- 1.0 minutes. No complications occurred during exercise training or testing. Supervised exercise training at moderate intensity is safe and produces significant and beneficial changes in hemodynamics and exercise time in children with cardiac disease.

In a genetic and clinical study in Wales of congenital adrenal hyperplasia due to 21-hydroxylase deficiency, 26 cases were found in the period 1966 to 1977. No one was known to have died from the condition in the period. There were 14 female and 5 male salt-losers, and 5 female and 2 male nonsalt-losers. The discrepancy between the sexes suggests that some cases of congenital adrenal hyperplasia in males are not being detected. This is of concern as salt-losing cases may be dying without a diagnosis being established. The carrier incidence of the condition in females, assuming complete ascertainment, is 1:55. Salt-losers in Wales appear to be more than twice as common as nonsalt-losers. HLA typing of certain affected families confirmed a genetic linkage between the congenital adrenal hyperplasia gene and HLA. This linkage appears to exist in both forms of congenital adrenal hyperplasia and suggests that the genes for salt-losing and nonsalt-losing forms of congenital adrenal hyperplasia are alleles. The linkage between congenital adrenal hyperplasia and HLA provides a potential method for antenatal detection. However, only a few parents would wish to take advantage of this method if it were readily available.

The Coulter S Counter gives a value for mean corpuscular volume which does not necessarily reflect red cell size in vivo. This phenomenon may be used in early diagnosis and hence prevention of the 'hyperosmolar syndrome'.

A simple reproducible in vitro test of anti-human lymphocyte globulin activity is discribed. The method is a "microtitre" complement-fixation test using human platelets as a source of antigen, guinea-pig complement and an amboceptor of sheep red blood cells sensitized by rabbit anti-sheep haemolysin.

Cardiac size and function were studied echocardiographically in 124 children with sickle cell anemia. A group of 78 healthy black children served as control subjects. Sickle cell patients exhibited progressive chamber enlargement and progressively increasing left ventricular mass. Although contractility indices were normal, when the opposing influences of volume overload due to anemia and ventricular dysfunction were separated, abnormalities of systolic time intervals were identified. Left ventricular systolic time interval ratio and left ventricular preejection period were higher in the sickle cell group and became increasingly abnormal with growth, suggesting that left ventricular function deteriorated with time.

The measurement of systolic and diastolic blood pressure during exercise is often difficult to perform due to patient motion and environmental noise. We evaluated an automated system of blood pressure determination in 121 children undergoing cycle ergometer stress testing. The system (Critikon Model 1165) utilized acoustic transduction, with electrocardiogram gating and microprocessor signal filtering. One observer measured the blood pressure values from the same arm as the automated system; this observer was blinded to the readout of the system. The correlation coefficients for systolic blood pressure at rest, during exercise, and during recovery were all greater than 0.91, and improved with the use of a stethophone adapter. This system can eliminate intraobserver variation as well as the necessity for the technician or physician to direct attention away from the electrocardiogram and patient during stress testing.

M-mode echocardiography was performed on 11 normal black subjects and 38 patients with sickle cell anemia while they were at rest to evaluate their left ventricular (LV) systolic and diastolic function. The patients with sickle cell anemia were also evaluated by radionuclide exercise tests and, based on their ejection fraction (EF) response, were separated into 2 groups: a group with a normal EF response to exercise (73 +/- 9%, mean +/- standard deviation) and a group with an abnormal EF response to exercise (53 +/- 9%). Computer-assisted analysis of the M-mode echocardiograms identified abnormalities of diastolic function (impaired left ventricular filling) in patients with sickle cell anemia compared with the normal subjects. The abnormal EF response group had significantly more impaired diastolic function and did less exercise than the normal EF response group. Both groups of patients had a decrease in left ventricular end-diastolic volume during exercise. The patients with sickle cell anemia had abnormalities of systolic and diastolic function on echocardiographic and radionuclide testing. The abnormalities in diastolic and systolic function assumed greater significance at the increased heart rates associated with exercise, accounting for the decrease in left ventricular end-diastolic volume and the abnormal EF response, and contributed to exercise intolerance in patients with sickle cell anemia.

Cardiac performance was studied by radionuclide angiography at rest and during exercise in 22 adolescents with sickle cell (SC) anemia and the results were compared with those in 12 control subjects. At rest, cardiac contractility was normal; cardiac output and end-diastolic volume were increased. At maximal exercise, heart rate, cardiac output response, and work capacity were reduced; the reduction was related to the degree of anemia. Left ventricular end-diastolic volume decreased with exercise most markedly in patients with ischemic exercise electrocardiograms. An abnormal ejection fraction response to exercise occurred in 4 patients; electrocardiographic signs of ischemia developed in all 4, and wall motion abnormalities in 2. Those patients who had electrocardiographic signs of ischemia had a significantly lower heart rate, ejection fraction, and cardiac output response to exercise, and a lower hematocrit level than subjects with normal results on exercise electrocardiography. The increase in cardiac output was not sufficient to maintain a normal level of exercise. The decrease in end-diastolic volume suggests that diastolic function was abnormal during exercise. Cardiac dysfunction was manifested by an abnormal ejection fraction response, wall motion abnormalities, and incomplete left ventricular filling during exercise.

This communication describes a young man with chronic glomerular nephritis whose serum contained cold antibodies directed against both red cells and lymphocytes. A renal biopsy revealed unusual histological features including extensive deposits of IgM and complement. Although abnormal immune responses are not uncommon in chronic renal failure, it is suggested that there may be a causal relationship between the underlying renal lesion and the abnormal antibodies.