The Pediatric Heart Network's Single Ventricle Reconstruction (SVR) trial randomized infants with single right ventricles (RVs) undergoing a Norwood procedure to a modified Blalock-Taussig or ...RV-to-pulmonary artery shunt. This report compares RV parameters in the 2 groups using 3-dimensional echocardiography.
Three-dimensional echocardiography studies were obtained at 10 of 15 SVR centers. Of the 549 subjects, 314 underwent 3-dimensional echocardiography studies at 1 to 4 time points (pre-Norwood, post-Norwood, pre-stage II, and 14 months) for a total of 757 3-dimensional echocardiography studies. Of these, 565 (75%) were acceptable for analysis. RV volume, mass, mass:volume ratio, ejection fraction, and severity of tricuspid regurgitation did not differ by shunt type. RV volumes and mass did not change after the Norwood, but increased from pre-Norwood to pre-stage II (end-diastolic volume milliliters/body surface area BSA(1.3), end-systolic volume milliliters/BSA(1.3), and mass grams/BSA(1.3) mean difference 95% confidence interval=25.0 8.7-41.3, 19.3 8.3-30.4, and 17.9 7.3-28.5, then decreased by 14 months (end-diastolic volume/BSA(1.3), end-systolic volume/BSA(1.3), and mass/BSA(1.3) mean difference 95% confidence interval=-24.4 -35.0 to -13.7, -9.8 -17.9 to -1.7, and -15.3 -22.0 to -8.6. Ejection fraction decreased from pre-Norwood to pre-stage II (mean difference 95% confidence interval=-3.7 -6.9 to -0.5), but did not decrease further by 14 months.
We found no statistically significant differences between study groups in 3-dimensional echocardiography measures of RV size and function, or magnitude of tricuspid regurgitation. Volume unloading was seen after stage II, as expected, but ejection fraction did not improve. This study provides insights into the remodeling of the operated univentricular RV in infancy.
ABSTRACT
Objective. To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia ...with intact ventricular septum.
Design. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome.
Setting. Tertiary‐care pediatric hospital.
Results. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z‐scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs.
Conclusions. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.
Echocardiography has been shown to be highly accurate in infants and children, but its accuracy has not been studied in detail in low birth weight (LBW) infants with structural congenital heart ...disease (CHD). This study was undertaken to examine the diagnostic accuracy of echocardiography in the evaluation of CHD in these infants.
All echocardiograms performed between 1995 and 2000 on infants who had structural CHD and weighed <or=2.5 kg were reviewed (n = 251). Infants who weighed >2.5 kg, matched for type of CHD, were control subjects (n = 319). The results of initial echocardiograms were compared for diagnostic accuracy with consensus diagnoses on the basis of all confirmatory data available. An observer who was blinded to patient group graded a sample of randomly selected echocardiograms (n = 100) for technical quality.
There were 13 major diagnostic errors in the LBW infants (5.2%) and 6 in the control subjects (1.9%). There were 20 minor errors in the LBW group (8.0%) and 21 in the control subjects (6.6%). The technical quality scores of the 2 groups were not significantly different, but a higher proportion of the LBW studies were graded as "poor" or "borderline" quality, compared with the control subjects (40% vs 28%). The majority (54%) of major errors in the LBW infants could potentially be explained by alterations in blood flow secondary to pulmonary hypertension.
Although echocardiography is an accurate imaging modality in premature and LBW infants with structural CHD, the incidence of clinically important diagnostic errors is higher than in infants who weigh >2.5 kg. As surgical and transcatheter interventions are extended to this population, heightened awareness of clinicians to pitfalls of echocardiography in this group of patients is warranted.
Absence of the right superior vena cava (SVC) in visceroatrial situs solitus is rare (0.07% to 0.13% of congenital cardiovascular malformations), and little is known about the type and frequency of ...additional heart defects and arrhythmias. We reviewed previous publications and present 9 new cases. Based on 121 known cases, we found that this anomaly is typically characterized by: (1) persistence of the left SVC draining into the right atrium by way of the coronary sinus, and (2) left-sided azygos vein draining into the left SVC. Less constant features were: (3) additional cardiovascular malformations (46%), and (4) rhythm abnormalities (36%) that usually appeared related to the complications of old age. Since absence of the right SVC is clinically silent, its status should be assessed echocardiographically prior to invasive medical or surgical procedures. This is important to avoid various management difficulties during the following procedures: (1) implantation of a transvenous pacemaker, (2) placement of a pulmonary artery catheter for intraoperative or intensive care unit monitoring without fluoroscopy, (3) systemic venous cannulation for extracorporeal membrane oxygenation, (4) systemic venous cannulation for cardiopulmonary bypass, (5) partial or total cavopulmonary anastomoses; and (6) orthotopic heart transplantation and endomyocardial biopsies.
Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences ...during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41-7.17). Conclusions By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.
Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) ...dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3. Repeated measures linear and logistic regressions were used to determine multivariable predictors of AoR dilation. Receiver operator characteristic curves were used to determine cut-points in AoR dilation predicting referral for aortic surgery. Multivariable analysis showed rapid AoR dilation as defined by change in AoRz/year > 90th percentile was associated with older age, higher sinotubular junction z-score, and atenolol use (
R
2
= 0.01) or by change in AoR diameter (AoRd)/year > 90th percentile with higher sinotubular junction z-score and non-white race (
R
2
= 0.02). Referral for aortic root surgery was associated with higher AoRd, higher ascending aorta z-score, and higher sinotubular junction diameter:ascending aorta diameter ratio (
R
2
= 0.17). Change in AoRz of 0.72 SD units/year had 42% sensitivity and 92% specificity and change in AoRd of 0.34 cm/year had 38% sensitivity and 95% specificity for predicting referral for aortic surgery. In this cohort of young patients with MFS, no new robust predictors of rapid AoR dilation or referral for aortic root surgery were identified. Further investigation may determine whether generalized proximal aortic dilation and effacement of the sinotubular junction will allow for better risk stratification. Rate of AoR dilation cut-points had high specificity, but low sensitivity for predicting referral for aortic surgery, limiting their clinical use.
Clinical Trial Number
ClinicalTrials.gov number, NCT00429364.
Congenital heart disease complicates the management of most accessory pathway-mediated tachycardias and also increases the challenge of radiofrequency ablation. Since 1990, radiofrequency ablation of ...accessory atrioventricular (AV) pathways has been attempted in 10 patients (age range 3.5 months to 30 years) with congenital heart disease: Ebstein's anomaly (n = 5), heterotaxy with AV discordance (n = 3), tetralogy of Fallot (n = 1) and total anomalous pulmonary venous return (n = 1). Eight patients had manifest Wolff-Parkinson-White syndrome and 2 had concealed pathways. Five patients had multiple pathways including 4 of the 5 with Ebstein's anomaly. Of 16 pathways total, 15 were associated with the tricuspid valve including all pathways in the patients with Ebstein's anomaly and heterotaxy. The His bundle area was identified in all patients and involved an anterior AV node in 2 of 3 with heterotaxy. Ablation was performed on the atrial side of the AV ring in all cases. No instance of AV block was encountered. Complete success was achieved in 6 patients including the 3 with heterotaxy. In 2 patients, manifest preexcitation was eliminated and clinical symptoms were greatly modified. The procedure was transiently successful in 1 patient who later had surgical interruption of the accessory pathway during tetralogy of Fallot repair. Ablation was unsuccessful in 1 patient. Thus, the overall success was 80%.
Pulmonary artery atresia with intact ventricular septum is a relatively rare type of congenital heart disease that is typically fatal in early infancy if left untreated. There are two major variants ...of this abnormality. The more common variant is associated with significant hypoplasia of the right ventricle, small and anatomically abnormal tricuspid valve, right ventricular hypertension and abnormal coronary artery development. The less common variant is associated with severe dilation of the right ventricle and right atrium, dysplastic or Ebsteinoid abnormality of the tricuspid valve and severe tricuspid regurgitation. This chapter reviews the anatomy, pathophysiology, treatment and general outcomes for both variants of pulmonary atresia with intact ventricular septum. Detailed description of imaging principles for prenatal and initial postnatal anatomy and physiology are included. In addition, the imaging of intraoperative and post‐operative anatomy and physiology for the different treatment strategies is discussed in detail.
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