Objective Liver fibrosis has emerged as an important long-term complication of the Fontan operation. We aimed to describe liver histology at autopsy in patients who had undergone the Fontan operation ...and to determine whether patient variables are associated with the degree of fibrosis. Methods A review was performed of all patients with a history of the Fontan operation who died and underwent autopsy at our institution from 1980 to 2009. Autopsy liver slides were evaluated independently by 2 pathologists. Results Twenty-two patients were studied. The median interval between Fontan and death was 20 days (range, 1 day–17.5 years). Portal fibrosis was observed in 20 (91%) patients and sinusoidal fibrosis was observed in 17 (77%) patients. Using simple linear regression, time from the Fontan operation was significantly associated with the degree of portal fibrosis on Ishak ( P = .03) and modified Scheuer fibrosis ( P = .02) scales. Significant portal fibrosis was observed in 8 (57%) of the 14 patients who died 30 days or less after the Fontan operation. In these 14 patients, severity of portal fibrosis was associated with length of hospitalization after pre-Fontan cardiac operations ( P = .03) and pre-Fontan mean right atrial pressure ( P = .04). Conclusions At autopsy, hepatic fibrosis was commonly observed in patients who had undergone the Fontan operation. Portal fibrosis has been previously unrecognized in this population. Significant portal fibrosis occurred in most who died soon after the Fontan procedure and was associated with pre-Fontan morbidity. Hepatic disease in the single-ventricle population is multifactorial and may begin before the Fontan operation.
Objectives We explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis. ...Background Left ventricular (LV) dysfunction, mitral regurgitation (MR), and aortic root dilation occur early after diagnosis; their associations with coronary artery dilation and inflammatory markers have not been well-described. Methods Centrally interpreted echocardiograms were obtained at KD diagnosis and 1 and 5 weeks after diagnosis on 198 subjects in the National Institutes of Health-sponsored Pediatric Heart Network KD pulsed steroid trial. Regression models were constructed to investigate the relationships among early LV dysfunction, MR, and aortic root dilation with coronary artery dilation and laboratory inflammatory markers. Results At diagnosis, LV systolic dysfunction was present in 20% of subjects and was associated with coronary artery dilation, seen in 29% (p = 0.004). Although LV dysfunction improved rapidly, LV dysfunction at diagnosis predicted greater odds of coronary artery dilation at 1 and 5 weeks after diagnosis (5-week odds ratio: 2.7, 95% confidence interval: 1.2 to 6.3). At diagnosis, MR was present in 27% of subjects and aortic root dilation was present in 8%; each was associated with larger coronary artery size at diagnosis. Left ventricular dysfunction was associated with higher erythrocyte sedimentation rate and, at diagnosis only, lower serum albumin; MR was associated with higher erythrocyte sedimentation rate and lower albumin at all times. Aortic root size had little association with inflammatory markers. Conclusions Noncoronary cardiac abnormalities are associated with coronary artery dilation and laboratory evidence of inflammation in the first 5 weeks after KD, suggesting a shared inflammatory mechanism. (Trial of Pulse Steroid Therapy in Kawasaki Disease A Trial Conducted by the Pediatric Heart Network; NCT00132080 )
Progressive hepatic fibrosis is common after the Fontan operation, but little is known about its onset. We sought to determine whether hepatic abnormalities before the Fontan operation, and whether ...further abnormalities can be identified soon afterward.
Patients undergoing the Fontan operation at our institution were prospectively enrolled and underwent hepatic ultrasound with Doppler and serum testing immediately before and 3 to 6 months after the operation.
Thirty patients were enrolled at a median age of 3.1 years (range, 2.2 to 8.1 years). Extracardiac Fontan was performed in 67%. Three patients (10%) had abnormal hepatic echotexture before the operation. At the post-Fontan study, mean liver length increased (9.9 versus 10.9 cm; p < 0.0001), and mean hepatic artery end-diastolic velocity decreased (18.8 versus 14.5 cm/s; p = 0.03). One patient showed new, abnormal hepatic echotexture after surgery. Mean aspartate aminotransferase (56.7 versus 60.7 U/L; p = 0.04), alanine transaminase (18.9 versus 33.9 U/L; p = 0.0002), and γ-glutamyl transferase (18.7 versus 46.1 U/L; p = 0.002) increased at the post-Fontan assessment compared with the preoperative values. By linear regression, hospital length of stay and duration with chest tube after Fontan operation were both significantly associated with an increase in γ-glutamyl transferase (p < 0.001 for both) and alanine transaminase (p = 0.008, p = 0.048).
In this cohort, hepatic abnormalities were observed in some patients before the Fontan operation and new abnormalities developed soon afterward. These findings suggest that liver insult may occur before or soon after the Fontan operation.
Objectives We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes. Methods The ...demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006. Results The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect closure (10%). The incidence of residual septal defects and the degree of left atrioventricular valve regurgitation (LAVVR) did not differ by repair type. The median interval of intensive care stay were 4 days, ventilation use 2 days, and total hospitalization 8 days. All were independent of the presence of trisomy 21 (80% of the cohort). The in-hospital mortality rate was 2.5% (3/120). The overall 6-month mortality rate was 4% (5/120). The presence of associated anomalies and younger age at surgery were independently associated with a longer hospital stay. The age at repair was not associated with residual ventricular septal defect or moderate or greater LAVVR at 6 months. Moderate or greater LAVVR occurred in 22% at 6 months, and the strongest predictor for this was moderate or greater LAVVR at 1 month (odds ratio, 6.9; 95% confidence interval, 2.2–21.7; P < .001). Conclusions The outcomes after repair of complete atrioventricular septal defect did not differ by repair type or the presence of trisomy 21. An earlier age at surgery was associated with increased resource use but had no association with the incidence of residual ventricular septal defect or significant LAVVR.
The Relationship Between Neo-Aortic Root Dilation, Insufficiency, and Reintervention Following the Ross Procedure in Infants, Children, and Young Adults Sara K. Pasquali, Meryl S. Cohen, David Shera, ...Gil Wernovsky, Thomas L. Spray, Bradley S. Marino We evaluated neo-aortic root size, neo-aortic insufficiency (AI), and reintervention in 74 patients at median 4.7 years (range 3 months to 9.3 years) after the Ross procedure. Neo-aortic root size increased significantly out of proportion to somatic growth (z-score increase of 0.75/years p < 0.0001), but was not associated with significant neo-AI or reintervention. At 6 years after the Ross, freedom from neo-aortic sinus z-score >4 was only 3%, freedom from ≥ moderate neo-AI was 60%, and freedom from neo-aortic reintervention was 88%. Prior aortic valve replacement and VSD repair were associated with neo-AI and reintervention after the Ross procedure.
The Pediatric Heart Network is conducting a large international randomized trial to compare aortic root growth and other cardiovascular outcomes in 608 subjects with Marfan syndrome randomized to ...receive atenolol or losartan for 3 years. The authors report here the echocardiographic methods and baseline echocardiographic characteristics of the randomized subjects, describe the interobserver agreement of aortic measurements, and identify factors influencing agreement.
Individuals aged 6 months to 25 years who met the original Ghent criteria and had body surface area-adjusted maximum aortic root diameter (ROOTmax) Z scores > 3 were eligible for inclusion. The primary outcome measure for the trial is the change over time in ROOTmaxZ score. A detailed echocardiographic protocol was established and implemented across 22 centers, with an extensive training and quality review process.
Interobserver agreement for the aortic measurements was excellent, with intraclass correlation coefficients ranging from 0.921 to 0.989. Lower interobserver percentage error in ROOTmax measurements was independently associated (model R(2) = 0.15) with better image quality (P = .002) and later study reading date (P < .001). Echocardiographic characteristics of the randomized subjects did not differ by treatment arm. Subjects with ROOTmaxZ scores ≥ 4.5 (36%) were more likely to have mitral valve prolapse and dilation of the main pulmonary artery and left ventricle, but there were no differences in aortic regurgitation, aortic stiffness indices, mitral regurgitation, or left ventricular function compared with subjects with ROOTmaxZ scores < 4.5.
The echocardiographic methodology, training, and quality review process resulted in a robust evaluation of aortic root dimensions, with excellent reproducibility.
Hot Topics in Tetralogy of Fallot Villafañe, Juan, MD; Feinstein, Jeffrey A., MD; Jenkins, Kathy J., MD, MPH ...
Journal of the American College of Cardiology,
12/2013, Letnik:
62, Številka:
23
Journal Article
Recenzirano
Odprti dostop
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore “hot topics” to highlight areas of emerging science for clinicians and scientists in moving toward a better ...understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.
Background Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome. Methods We analyzed data from the National ...Heart, Lung, and Blood Institute Pediatric Heart Network cross-sectional study of Fontan patients. Groups were defined by height ( z -score <−1.5 or ≥−1.5) and body mass index (body mass index BMI z -score <−1.5 or −1.5 to 1.5 or ≥1.5). Associations of anthropometric measures with measurements from clinical testing (exercise, echocardiography, magnetic resonance imaging) were determined adjusting for demographics, anatomy, and pre-Fontan status. Relationships between anthropometric measures and functional health status (FHS) were assessed using the Child Health Questionnaire. Results Mean age of the cohort (n = 544) was 11.9 ± 3.4 years. Lower height- z patients (n = 124, 23%) were more likely to have pre-Fontan atrioventricular valve regurgitation ( P = .029), as well as orthopedic and developmental problems (both P < .001). Lower height- z patients also had lower physical and psychosocial FHS summary scores (both P < .01). Higher BMI- z patients (n = 45, 8%) and lower BMI- z patients (n = 53, 10%) did not have worse FHS compared to midrange BMI- z patients (n = 446, 82%). However, higher BMI- z patients had higher ventricular mass-to-volume ratio ( P = .03) and lower % predicted maximum work ( P = .004) compared to midrange and lower BMI- z patients. Conclusions Abnormal anthropometry is common in Fontan patients. Shorter stature is associated with poorer FHS and non-cardiac problems. Increased adiposity is associated with more ventricular hypertrophy and poorer exercise performance, which may have significant long-term implications in this at-risk population.
Objectives To determine the cardiovascular effects of obesity on patients with tetralogy of Fallot (TOF) repair. Study design Ventricular performance measures were compared between obese (body mass ...index BMI ≥95%), overweight (85% ≤BMI <95%), and normal weight subjects (BMI <85%) in a retrospective review of patients with TOF who underwent cardiac magnetic resonance from 2005-2010. Significance was P < .05. Results Of 260 consecutive patients with TOF, 32 were obese (12.3%), 48 were overweight (18.5%), and 180 were normal weight (69.2%). Biventricular mass was increased in obese compared with normal weight patients with right ventricular mass more affected than left ventricular mass. Obese patients demonstrated decreased biventricular end-diastolic volume (EDV) and stroke volume (SV) when indexed to body surface area (BSA) with an increased heart rate when compared with normal weight patients; cardiac index, ejection fraction, and pulmonary regurgitation fraction were similar. When indexed to ideal BSA, biventricular EDV and SV were similar. EDV and SV for overweight patients were nearly identical to normal weight patients with ventricular mass in between the other 2 groups. Conclusions Approximately 12% of patients after TOF repair referred for cardiac magnetic resonance in a tertiary referral center are obese with increased biventricular mass. Obese patients and normal weight patients have similar cardiac indices, however, when indexed to actual BSA, obese patients demonstrate decreased EDV and SV with increased heart rate and similar cardiac indices. When indexed to ideal BSA, no differences in biventricular volumes were noted.
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