Congenitally corrected transposition of the great arteries (CCTGA) in the absence of major cardiac anomalies is thought to have a good outcome, although this has not been well documented. The ...objective of the present study was to compare the characteristics and outcomes of patients with a prenatal diagnosis of CCTGA to the characteristics and outcomes of those diagnosed postnatally. The optimal outcome was defined as intervention-free survival. All patients with CCTGA diagnosed prenatally and postnatally from 1999 to 2006 at 2 tertiary care institutions were reviewed. Patients with a single ventricle, heterotaxy, or valvar atresia were excluded. The differences between groups were assessed using the t test and chi-square test. A total of 54 patients (16 prenatal with 14 live born and 39 postnatal) were included. The patients diagnosed prenatally were diagnosed at a median gestational age of 20 weeks (range 16 to 37). Two deaths in each group were due to heart failure. The intervention-free survival rate for the prenatal and postnatal groups at 1, 6, and 36 months was 79%, 45%, and 30% and 85%, 61%, and 23%, respectively (p = NS). Of 37 patients, 14 (38%) underwent an arterial switch plus atrial baffling so that the morphologic left ventricle supported the systemic circulation, and 6 (16%) underwent repair of associated lesions so the morphologic right ventricle supported the systemic circulation; 4 (11%) of the 37 patients had pacemaker only. Prenatal patients with >1 fetal echocardiogram (12 of 14) did not have progression before birth. In conclusion, CCTGA has a >70% risk of intervention in the first 3 years after birth. The outlook is guarded and has an important effect on prenatal counseling.
The purpose of this study was to evaluate the outcome and risk factors for implant failure in pediatric patients who underwent pulmonary position homograft placement for right ventricular outflow ...tract obstruction compared with conduit placement as a component of the Ross operation. Actuarial 5-year survivals for cryopreserved right ventricle-to-pulmonary artery homografts range from 55% to 94% at all ages. It is not known whether there is a difference in homograft durability when utilized for right ventricular outflow tract obstruction or as part of the Ross operation.
The records of all pediatric patients receiving a right ventricle-to-pulmonary artery homograft from July 1989 through October 2003 were reviewed. Ninety-eight consecutive patients were studied (26 Ross, 72 non-Ross). In addition to Ross versus non-Ross comparisons, other potential risk factors for homograft failure analyzed included age at operation, follow-up time, type of surgery, and homograft type and size.
Ross and non-Ross patients were comparable in age at the time of the operation and follow-up time. Homograft failure rates were 12% and 51% for Ross and non-Ross patients, respectively. Freedom from reintervention was 93% in the Ross and 66% in the non-Ross group at 5 years (
P = .019). On multivariate analysis,
non-Ross operation and age
less than 2 years were significant predictors of homograft failure.
1. Pediatric patients undergoing the Ross operation have longer homograft survival than pediatric patients treated for right ventricular outflow tract obstruction, independent of age. 2. Homografts placed in patients less than 2 years of age have shorter homograft survival.
Exercise training programs can effectively enhance exercise capacity in adults with congenital heart disease, including Fontan patients. However, few studies have explored the impact of exercise ...training exclusively on paediatric Fontan cohorts. This study systematically reviews exercise capacity in paediatric Fontan patients and the impact of training programs on their cardiovascular health.
Medline and Embase were searched for articles published between January 1990 and November 2021. Studies were included in which data could be analyzed discretely for patients who had undergone the Fontan procedure and were ≤20 years old at the time of study. Cardiopulmonary exercise parameters were extracted from all studies, and training protocols were collected from training programs.
The studies demonstrated that Fontan patients exhibit significantly diminished peak exercise capacity relative to healthy peers. We identified 9 training programs that exclusively studied Fontan patients ≤20 years. The programs ranged from 6 weeks to 12 months in duration, with 8 programs incorporating aerobic activity and 1 focused only on inspiratory muscle training. At least 1 measure of maximal or submaximal exercise capacity improved significantly within each program in which statistical analysis was performed, with no reported adverse events. There were 2 additional training programs in which the patients were predominantly (>65%), but not exclusively, Fontan patients.
Overall, the results indicate that exercise training programs can safely and effectively improve at least 1 measure of exercise capacity in paediatric Fontan patients.
Background. Omega-3 fatty acids supplements lower triglyceride (TG) levels in adults; little pediatric information is available. We evaluated their effect in hypertriglyceridemic adolescents. ...Methods. Twenty-five patients aged 10 to 19 years with TG levels 150 to 1000 mg/dL were randomized to 6 months double-blind trial of Lovaza (~3360 mg docosahexaenoic acid + eicosapentaenoic acid per day) versus placebo. Results. Baseline mean TG levels were 227 mg/dL (standard deviation = 49). TG levels declined at 3 months in the Lovaza group by 54 ± 27 mg/dL (mean ± standard error; P = .02) and by 34 ± 26 mg/dL (P = .16) in the placebo group. The difference in TG lowering between groups was not significant (P = .52). There were no between-group differences in endothelial function, blood pressure, body mass index, C-reactive protein, or side effects. Conclusions. High-dose omega-3 fatty acid supplements are well tolerated in adolescents. However, declines in TG levels did not differ significantly from Placebo in this small study.
Coronary artery re-implantation during arterial switch operation in patients with D-looped transposition of the great arteries (D-TGA) can alter coronary arterial flow and increase shear stress, ...leading to local endothelial dysfunction, although prior studies have conflicting results. Endothelial pulse amplitude testing can predict coronary endothelial dysfunction by peripheral arterial testing. This study tested if, compared to healthy controls, patients with D-TGA after arterial switch operation had peripheral endothelial dysfunction. Patient inclusion criteria were (1) D-TGA after neonatal arterial switch operation; (2) age 9–29 years; (3) absence of known cardiovascular risk factors such as hypertension, diabetes, hypercholesterolemia, vascular disease, recurrent vasovagal syncope, and coronary artery disease; and (4) ability to comply with overnight fasting. Exclusion criteria included (1) body mass index ≥85th percentile, (2) use of medications affecting vascular tone, or (3) acute illness. We assessed endothelial function by endothelial pulse amplitude testing and compared the results to our previously published data in healthy controls (
n
= 57). We tested 20 D-TGA patients (16.4 ± 4.8 years old) who have undergone arterial switch operation at a median age of 5 days (0–61 days). Endothelial pulse amplitude testing indices were similar between patients with D-TGA and controls (1.78 ± 0.61 vs. 1.73 ± 0.54,
p
= 0.73).In our study population of children and young adults, there was no evidence of peripheral endothelial dysfunction in patients with D-TGA who have undergone arterial switch operation. Our results support the theory that coronary arterial wall thickening and abnormal vasodilation reported in these patients is a localized phenomenon and not reflective of overall atherosclerotic burden.
Background The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome ...randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Methods and results Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area–adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Conclusions Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome.
Adrenergic receptor (ADR) genotypes are associated with heart failure (HF) and β-blocker response in adults. We assessed the influence of ADR genotypes in children with dilated cardiomyopathy (DCM).
...Ninety-one children with advanced DCM and 44 with stable DCM were genotyped for three ADR genotypes associated with HF risk in adults: α2cdel322-325, β1Arg389, and β2Arg16. Data were analyzed by genotype and β-blocker use. Mean age at enrollment was 8.5 y.
One-year event-free survival was 51% in advanced and 80% in stable DCM. High-risk genotypes were associated with higher left ventricular (LV) filling pressures, higher systemic and pulmonary vascular resistance, greater decline in LV ejection fraction (P < 0.05), and a higher frequency of mechanical circulatory support while awaiting transplant (P = 0.05). While β-blockers did not reduce HF severity in the overall cohort, in the subset with multiple high-risk genotypes, those receiving β-blockers showed better preservation of cardiac function and hemodynamics compared with those not receiving β-blockers (interaction P < 0.05).
Our study identifies genetic risk markers that may help in the identification of patients at risk for developing decompensated HF and who may benefit from early institution of β-blocker therapy before progression to decompensated HF.
Background
No guidelines exist for inpatient postoperative transthoracic echocardiographic (TTE) surveillance in congenital heart disease. We prospectively evaluated indications for postoperative ...TTEs in patients with congenital heart disease to identify areas to improve upon (Phase 1) and then assessed the impact of a simple pilot intervention (Phase 2).
Methods
We included patients with RACHS‐1 (Risk Adjustment for Congenital Heart Surgery) scores of 2 and 3 to keep the cohort homogenous. During Phase 1, we collected data prospectively to identify postoperative TTEs for which there were no new findings and no associated clinical management decisions (“potentially redundant” TTEs). During Phase 2, prior to placement of a TTE order, an “Echo Pause” was performed during rounds to prompt review of prior TTE results and indication for the current order. The number of “potentially redundant” TTEs during Phase 1 vs. Phase 2 was compared.
Results
During Phase 1, 98 postoperative TTEs were performed on 51 patients. Potentially “redundant” TTEs were identified in two main areas: (a) TTEs ordered to evaluate pericardial effusion and (b) TTEs ordered with the indication of “postoperative,” “follow‐up,” or “discharge” in the setting of a prior complete postoperative TTE and no apparent change in clinical status. During Phase 2, 101 TTEs were performed on 63 patients. The number of “potentially redundant” TTEs decreased from 14/98 (14%) to 5/101 (5%) (P = .026).
Conclusion
Our results suggest that the number of “potentially redundant” TTEs during inpatient postoperative surveillance of patients with congenital heart disease can be decreased by a simple intervention during rounds such as an “Echo Pause.”
Background The purpose of this study is to identify independent echocardiographic predictors of mitral stenosis (MS)–related death or intervention in infants. Congenital MS is a rare and ...morphologically heterogeneous lesion with variable prognosis. Among patients diagnosed with MS in early infancy, echocardiographic factors associated with MS-related intervention or death have not been determined. Methods and Results The clinical and echocardiographic data of patients diagnosed with MS at age <6 months by echocardiography between 1986 and 2004 were reviewed. The primary outcome was a composite end point of either mitral valve (MV) intervention (catheter or surgery) or death related to MS. Multiple variables from the initial echocardiogram were analyzed for association with outcomes. Seventy-one patients (median age at diagnosis 63 days) fulfilled the inclusion criteria. Multivariate analysis identified higher initial MV mean inflow gradient ( P = .009) and lower left ventricular (LV) diastolic length Z-score ( P = .006) at presentation as predictors of intervention or death. Among patients with an initial MV inflow gradient < 2 mm Hg, none reached an end point, whereas, among patients with an initial mean gradient ≥ 5.5 mm Hg, the risk of intervention or death was 85%. Among patients with a gradient > 2 and < 5.5 mm Hg, an end point was reached in 38%, and an LV diastolic length Z-score ≤ 0 was predictive of outcome (71% vs 17%, P = .005). Mitral valve morphology was not predictive of outcome. Conclusions In young infants with congenital MS, higher mean MV inflow gradient and shorter LV length, but not MV morphology, are associated with increased risk of MV intervention or MS-related death.
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.
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