Constance E. Cephus

The Medium-Term Findings in Coronary Arteries by Intravascular Ultrasound in Infants and Children After Heart Transplantation

OBJECTIVES The study purposes were to determine 1) whether intravascular ultrasound (IVUS) was more sensitive than angiography for the detection of post-transplant coronary artery disease (PTCAD) in pediatric patients; and 2) whether those transplanted as neonates reacted differently than older patients. BACKGROUND Experience with IVUS for the diagnosis of PTCAD in children is limited. METHODS Patients were divided into two groups: those transplanted as neonates (early group) and those transplanted in infancy or childhood (late group). Morphometric analysis was performed, including maximal intimal thickness (MIT) and intimal index (II). Stanford classification was used to grade lesion severity. Acute rejection and cytomegalovirus (CMV) status were correlated with MIT and II. RESULTS Thirty children were studied (early group, n = 13; late group, n = 17). All segments studied were angiographically normal. Mean MIT and mean II were significantly greater in the late group (0.26 +/- 0.14 vs. 0.13 +/- 0.04 mm, p < 0.001 and 0.11 +/- 0.07 vs. 0.07 +/- 0.03 mm, p = 0.04, respectively). There was a significant correlation between MIT and II in those who had acute rejection in the late group. Patients in the late group who were CMV-positive had a significantly higher MIT compared with those in the late group with negative serology (p = 0.04). CONCLUSIONS Intravascular ultrasound was more sensitive than angiography in detecting PTCAD after pediatric heart transplantation. There is a possible role for acute rejection and CMV in the development of PTCAD.

Incidence and implication of vocal fold paresis following neonatal cardiac surgery.

To study the incidence and implications of vocal fold paresis (VFP) following congenital neonatal cardiac surgery.

Moderate acute rejection detected during annual catheterization in pediatric heart transplant recipients.

BACKGROUND Acute rejection commonly occurs within the first year after heart transplantation, and then decreases in frequency with time. Recently, the long-term utility of endomyocardial biopsy during routine annual catheterization has been questioned. The purpose of this study was to retrospectively review the prevalence of biopsy-proven rejection during routine annual catheterization in our patient population, determine whether biopsies late after transplant are useful, and identify factors that correlate with late unsuspected rejection. METHODS Biopsy results from the annual catheterization were evaluated from 1986 to August 2000. The prevalence of moderate rejection was evaluated and compared with the patients immunosuppressive regimen; the prevalence of late rejection; and how late rejection correlated with recipient age, number of first-year rejections and presence of sub-therapeutic cyclosporine. RESULTS A total of 1108 biopsies were performed in 269 children with a mean follow-up of 5 +/- 3 years (median 5 years, range 1 to 11 years). Three-drug immunosuppressive therapy, including steroids, was used in 93 patients. There was a persistent 8% to 10% prevalence of moderate rejection at up to 10 years post-transplantation. Moderate rejection was more likely in patients: (1). on 3-drug immunosuppressive therapy; (2). with a recipient age >1 year; and (3). with a relatively lower cyclosporine level. CONCLUSIONS These data suggest that continued surveillance of pediatric transplant patients for acute rejection is indicated for long-term follow-up.

Health-related quality of life in children with heart failure as perceived by children and parents.

Advancements in paediatric heart failure management have resulted in improved survival and a focus on long-term outcomes including health-related quality of life. We compared health-related quality of life in children with heart failure with healthy patients, children with chronic conditions, and children with cardiovascular disease. Families (n=63) and children (n=73) aged 2-20 years with heart failure were enrolled and compared with data previously published for healthy patients (n=5480), those with chronic conditions (n=247), and those with cardiovascular disease (n=347). Patients and parents completed the PedsQL 4.0 and the Cardiac 3.0 Module health-related quality-of-life questionnaires. PedsQL scores including Total, Psychosocial Health Summary, and Physical were compared between groups. In general, patients with heart failure had lower scores than the healthy population (p=0.001), and comparable scores with those with chronic conditions. Parents perceived no difference in physical scores for children with heart failure when compared with healthy children, and perceived higher scores for children with heart failure when compared with those with chronic conditions (p⩽0.003). Furthermore, children with heart failure had decremental health-related quality-of-life scores as the American Heart Association stage of heart failure increased, such that patients with stage C heart failure had scores similar to children with severe cardiovascular disease. Children with heart failure reported significantly impaired health-related quality of life compared with healthy children and similar scores compared with children with chronic conditions. Parental perceptions appear to underestimate these impairments. Children with heart failure appear to have progressive impairment of health-related quality of life with advancing stage of heart failure.

Posttransplant recoarctation of the aorta: a twelve year experience

OBJECTIVES This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation. BACKGROUND Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta. METHODS This was a retrospective review of all children (age <18 years) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the most recent follow-up assessment of the aortic arch. RESULTS Two hundred eighty-eight transplants were performed on 279 children (follow-up = 1,075 patient-years; range 0 to 133 months, median 43.7). Thirty-two of 152 patients (21%) who underwent extended aortic arch reconstruction subsequently developed recoarctation. All but one patient developed recoarctation within 2 years after transplant; 87% were hypertensive at presentation. Of 30 patients who underwent intervention for recoarctation (balloon angioplasty [n = 26] and surgical repair of recoarctation [n = 4]), 26 (87%) have remained recurrence-free (follow-up = 133 patient-years; range 8 to 106 months, median 47). CONCLUSIONS The high frequency of recoarctation after cardiac transplantation with extended aortic arch reconstruction mandates serial echocardiographic evaluation of the aortic arch. Patients typically present with systemic hypertension within the first two years after transplantation. Balloon angioplasty is a safe, effective and durable method of treatment.

Transient complete atrioventricular block after placement of a central venous catheter in a neonate.

Objective: We present a case of trauma-induced complete atrioventricular block (transient) after placement of a central venous catheter. Design: Case report. Setting: Neonatal intensive care unit in a tertiary care childrens hospital. Patient: Review of the medical record and clinical course of a single premature infant. Interventions: Removal of central venous catheter, monitoring of the cardiac output exam, and serial monitoring of the arrhythmia profile. Measurements and Main Results: Trauma-induced complete atrioventricular block from placement of the central venous catheter resolved in 9 days, obviating the need for pacemaker placement. Conclusions: Complete atrioventricular block is an infrequent complication of central venous catheter placement and may require several days to resolve.

The effects of lifestyle changes on serum lipid levels in children in a real life setting

OBJECTIVE Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored programs. There programs are difficult to mimic in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients seen in a designated lipid clinic in a real life setting. DESIGN Retrospective review of patients followed for dyslipidemia at the Texas Childrens Hospital Lipid Clinic from May 1, 2012 to May 1, 2015. Patients included were seen more than once, had repeat lipid testing, and abnormal baseline lipid levels. Multivariate analysis using mixed models were performed to compare outcomes in patients who did and did not participate in lifestyle change. RESULTS Of the 268 patients seen within the study period, 174 (56% male, 44% female) met inclusion criteria. Median age was 11 years. Compared to patients who did not make lifestyle changes: patients who made only diet changes demonstrated significant improvement in weight only (slope = -1.55, P-value = .014), and those who made only exercise changes demonstrated significant improvements in serum cholesterol (slope = -22.8, P-value = .017) and non-HDL cholesterol (slope = -28.7, P-value = < .01) levels. Patients who participated in both diet and exercise demonstrated significant improvement in weight (slope = -1.13, P-value = .011), diastolic blood pressure (slope = -1.82, P-value = < .01), and serum lipid levels: LDL (slope = -10.8, P-value = 0.017), HDL (slope = 1.52, P-value = .24), Triglycerides (slope = -0.11, P-value = .033) compared to those who did not make lifestyle changes. CONCLUSIONS Outpatient management of dyslipidemia is difficult. Only patients who participated in both diet and exercise showed significant improvement in outcomes when compared to those who did not make lifestyle changes.

THE EFFECTS OF DIET AND EXERCISE ON SERUM LIPID LEVELS IN CHILDREN FOLLOWED IN A LIPID CLINIC

Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored outpatient settings, but it is difficult to mimic this in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients