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Background: Long-term enlargement of the aortic arch after aortic arch reconstruction in hypoplastic left heart syndrome is not well described., Methods: Aortic arch measurements for 50 patients with hypoplastic left heart syndrome who achieved Fontan completion were converted to Pediatric Heart Network z-scores. Dimensions were assessed using linear mixed models, and differences among time points were evaluated with F-tests. Sub-analysis was conducted comparing Norwood (n = 36) with hybrid (n = 14) strategies., Results: Median time to last imaging was 6.4 (interquartile range, 3.5-11.3) years. Before intervention, the main pulmonary artery was dilated, whereas the ascending aorta, transverse arch, and isthmus were hypoplastic. With aortic arch reconstruction, there were expected increases in all arch z-scores. The aortic arch continued to dilate after aortic arch reconstruction, reaching peak values at 7 months (neo-aortic complex: z = 6.9 [5.6-8.0]) or 12 months after stage I (ascending aorta: z = 6.1 [2.9-8.3]; transverse arch: z = 4.7 [3.0-5.9]). After peak values, there was a gradual decline in z-scores with most components still at least mildly dilated at 16 years (neo-aortic complex: z = 3.2 [3.1-3.9], ascending aorta: z = 3.9 [3.3-4.2]; transverse arch: z = 3.1 [2.5-3.7]) with abrupt caliber change at the isthmus: z = -0.8 (-1.1 to -0.3). Norwood and hybrid strategies showed similar enlargement profiles after 7 months of age., Conclusions: Neo-aortic root and aortic arch in hypoplastic left heart syndrome are enlarged early after aortic arch reconstruction and continue to enlarge out of proportion to normal controls until 12 months of age, with gradual decline in enlargement up to adolescence. Further work should focus on modifiable surgical factors that may prove important to optimize arch growth and geometry., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Decision-making in fetal cardiology is fraught with ethical issues yet education in bioethics for trainees is limited or nonexistent. In this innovation report, we describe the development of a fetal cardiology bioethics curriculum designed to address this gap. The curriculum was developed to supplement the core curriculum for cardiology fellows and fetal cardiology subspecialty trainees. The series combines didactic and interactive teaching modalities and contains 5 key components: (1) introduction to bioethics and its role in fetal cardiology, (2) counseling and pathways for compassionate terminal care, (3) case vignette-based ethical analysis and discussion cases, (4) fetal counseling considerations for shared decision-making and recommendations, (5) facilitated communications role play. The curriculum was refined using session evaluations from end users. This report describes the innovative curriculum as a starting point for further incorporation and study of bioethical education in pediatric cardiology and fetal training programs., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Abstract: Knowledge regarding the long-term consequences of pulmonary embolism (PE) in children is limited. This cohort study describes the long-term outcomes of PE in children who were followed-up at a single-center institution using a local protocol that included clinical evaluation, chest imaging, echocardiography, pulmonary function tests, and cardiopulmonary exercise tests at follow-up, starting 3 to 6 months after acute PE. Children objectively diagnosed with PE at age 0 to 18 years, who had ≥6 months of follow-up were included. Study outcomes consisted of PE resolution, PE recurrence, death, and functional outcomes (dyspnea, impaired pulmonary or cardiac function, impaired aerobic capacity, and post-PE syndrome). The frequency of outcomes was compared between patients with/without underlying conditions. In total, 150 patients were included; median age at PE was 16 years (25th-75th percentile, 14-17 years); 61% had underlying conditions. PE did not resolve in 29%, recurrence happened in 9%, and death in 5%. One-third of patients had at least 1 documented abnormal functional finding at follow-up (ventilatory impairments, 31%; impaired aerobic capacity, 31%; dyspnea, 26%; and abnormal diffusing capacity of the lungs to carbon monoxide, 22%). Most abnormalities were transient. When alternative explanations for the impairments were considered, the frequency of post-PE syndrome was lower, ranging between 0.7% and 8.5%. Patients with underlying conditions had significantly higher recurrence, more pulmonary function and ventilatory impairments, and poorer exercise capacity. Exercise intolerance was, in turn, most frequently because of deconditioning than to respiratory or cardiac limitation, highlighting the importance of physical activity promotion in children with PE., (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)

Background: The relationship between genotype and phenotypical vascular and cardiac properties in paediatric Loeys-Dietz syndrome (LDS) patients are not well characterized. This study explores the phenotypical differences in aortic properties and cardiac structural and functional parameters between paediatric LDS patients with TGFBR1 and TGFBR2 mutations., Methods: We included 32 LDS patients with either TGFBR1 (n = 17) or TGFBR2 (n = 15) mutations. Echocardiographic data included aortic dimensions, distensibility, strain, and stiffness at the level of the annulus, sinuses of Valsalva, sinotubular junction, ascending aorta, and descending aorta. Parameters for left ventricular size and function were also recorded., Results: Demographics were similar between the groups. Patients with TGFBR2 were more likely to have undergone aortic surgery (47% vs 12%, P  = 0.057) and use angiotensin receptor blockers (93% vs 47%, P  = 0.015). Aortic z scores were significantly larger in the TGFBR2 group at the level of the aortic valve annulus ( P  = 0.007), sinuses of Valsalva ( P  = 0.001), sinotubular junction ( P  = 0.001), and ascending aorta ( P  = 0.054). Patients with TGFBR2 also had significantly lower aortic distensibility and strain coupled with higher stiffness index at the level of the annulus, sinotubular junction, and ascending aorta. Parameters for the descending aorta, cardiac morphology, and cardiac function were similar between the groups., Conclusions: Paediatric LDS patients with TGFBR2 present with more severe cardiovascular phenotypes than patients with TGFBR1 with larger aortic dimensions and increased aortic stiffness. Our findings suggest that genotypes should be taken into consideration in the clinical management of paediatric LDS patients., (© 2024 The Authors.)

Echocardiography has evolved the first-line imaging for diagnosis and management of pediatric and congenital heart disease all over the world. While it recognized as essential component of pediatric cardiac care delivery, organization of pediatric echocardiography services is very heterogeneous across the world, mainly related to significant differences in material and human resources in heterogeneous health care systems. In this paper, we focus on the role of pediatric sonographers, defined as expert technicians in pediatric echocardiography. While in some services sonographers are an essential part of the organizational structure, other laboratories operate only with physicians trained in echocardiography. The impact of sonographers on clinical, academic and financial performance will be discussed. Two organizational models (with and without sonographers) will be compared, and the advantages and disadvantages of each model will be evaluated. Different models of care provision are possible and decisions on organizational models need to be adjusted to the demands and available resources., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Arbic, Venet, Iriart, Dragulescu, Thambo, Friedberg, Guerra, Morgan, Mertens and Villemain.)

Introduction: Antibody-mediated complete atrioventricular block (CAVB) is considered irreversible. We sought to examine the effects of transplacental steroids on fetal AV conduction., Methods: Fifty-nine fetuses diagnosed with CAVB at our center from 1996 to 2018 were reviewed. Routine dexamethasone administration to birth was used to limit cardiac inflammatory damage. Restoration of fetal AV conduction was classified as "unexpected" treatment response., Results: CAVB resolved in 5/29 (17%) fetuses first treated ≤24-week gestation with 8 mg/day of dexamethasone, when compared with 0/30 (0%) when treatment was initiated later and/or at a starting dose of 4 mg/day (odds ratio 13.69; 95% confidence interval 0.72-260.13; p = 0.024). Treatment response was also associated with a faster ventricular rate at diagnosis (median [range]: 80 [60-97] beats per minute [bpm] vs. 58 [38-92] bpm; p = 0.0036). CAVB reappeared in all 5 responders either prenatally (n = 1) or postnatally before (n = 3) or after (n = 1) the first year of life. When compared with infants with treatment-resistant CAVB (median follow-up 10.3 years), responders (median follow-up 12.3 years) required postnatal pacing less frequent (2/5 [40%] vs. 45/49 [92%]; p = 0.013)., Conclusions: In a subgroup of CAVB fetuses, dexamethasone transiently restored AV conduction. This was associated with a lower rate of postnatal pacing when compared with nonresponders., (© 2021 The Author(s) Published by S. Karger AG, Basel.)

Digital reality is an emerging platform for three-dimensional representation of medical imaging data. In this technical innovation paper, the authors evaluated the accuracy and utility of mixed-reality technology in the morphological evaluation of complex congenital heart disease. The authors converted CT datasets of 12 heart specimens with different subtypes of double-outlet right ventricle to stereoscopic images and interrogated them using a mixed-reality system. The morphological features identified on the stereoscopic models were compared with findings at macroscopic examination of the actual heart specimens. The results showed that the mixed-reality system provided highly accurate stereoscopic display of spatially complex congenital cardiac lesions, with interactive features that might enhance 3-D understanding of morphology. Additionally, the authors found that high-resolution digital reproduction of cardiac specimens using clinical CT scanners is feasible for preservation and educational purposes.

Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patients with moderate-size right atria remain controversial. Evidence in support of medical management and timing of prophylactic surgery is reviewed. We propose the use of three echocardiographic indices to help identify inappropriate atrial growth and facilitate surgical decision-making.

Background: The purpose of the study was to evaluate the association between fetal echocardiographic measurements and the need for intervention (primary coarctation repair, staged coarctation repair, or catheter intervention) in prenatally diagnosed coarctation of the aorta., Methods: A single-centre retrospective cohort study (2005-2015) of 107 fetuses diagnosed with suspected coarctation of the aorta in the setting of an apex-forming left ventricle and antegrade flow across the mitral and aortic valves., Results: Median gestational age at diagnosis was 32 weeks (interquartile range, 23-35 weeks). Fifty-six (52%) did not require any neonatal intervention, 51 patients (48%) underwent a biventricular repair. In univariable analysis, an increase in ascending aorta (AAo) peak Doppler flow velocity (odds ratio [OR], 1.40 [95% confidence interval [CI], 1.05-1.91] per 20 cm/s; P = 0.03) was associated with intervention. No intervention was associated with larger isthmus size (OR, 0.23; P < 0.001), transverse arch diameter (OR, 0.23; P < 0.001), and aortic (OR, 0.72; P = 0.02), mitral (OR, 0.58; P = 0.001), and AAo (OR, 0.53; P < 0.001) z-scores. In multivariable analysis, higher peak AAo Doppler (OR, 2.51 [95% CI, 1.54-4.58] per 20 cm/s; P = 0.001) and younger gestational age at diagnosis (OR, 0.81 [95% CI, 0.70-0.93] per week; P = 0.005) were associated with intervention, whereas a higher AAo z-score (OR, 0.65 [95% CI, 0.43-0.94] per z; P = 0.029) and transverse arch dimension (OR, 0.44 [95% CI, 0.18-0.97]; P = 0.05) decreased the risk of intervention., Conclusions: In prenatally suspected coarctation, the variables associated with intervention comprised smaller AAo and transverse arch size, earlier gestational age at diagnosis, and the additional finding of a higher peak AAo Doppler., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

The increasing prevalence of overweight or obese children and adolescents is a significant global health concern. Although the effect of obesity on cardiovascular function has been investigated, little is known on the impact of associated obstructive sleep apnea (OSA) in obese youth. The aim of the present study was to investigate the influence of OSA on cardiovascular functional parameters in obese youth. This is a prospective single-center observational cross-sectional study. Forty-four obese patients and 44 age- and gender-matched control subjects were included. All patients underwent polysomnography and cardiovascular assessment including functional echocardiography and carotid-femoral pulse wave velocity (PWV). Obese patients had higher left ventricular (LV) mass/height 2.7 , preserved LV systolic parameters, differences in LV diastolic parameters, and increased PWV and systolic blood pressure at rest compared with control group. In obese youth, 14 of 44 (32%) had OSA. There was no correlation between obesity and the apnea-hypopnea index (AHI). LV mass/height 2.7 significantly correlated with body mass index z-score (r = 0.648, p <0.001) whereas PWV correlated with AHI (r = 0.352, p = 0.038). In obese patients, body mass index z-score was an independent predictor for LV mass/height 2.7 (r = 0.61, p <0.001) and AHI was an independent predictor for higher PWV (r = 0.352, p = 0.038). In conclusion, both obesity and OSA influence cardiovascular performance in obese youth. Although obesity is associated with increased LV mass and reduced LV diastolic function, OSA is associated with changes in arterial stiffness., (Copyright © 2018. Published by Elsevier Inc.)

The aim of this study was to evaluate the association of in utero exposure to highly active antiretroviral therapy (HAART) with left ventricular (LV) function and structure in HIV-exposed uninfected (HEU) children. A prospective, multisite cohort study in HEU children was conducted by the Pediatric HIV/AIDS Cohort Study (PHACS). Echocardiographic measures of LV systolic and diastolic function and cardiac structure were obtained from HEU subjects aged ≥6 years enrolled in the PHACS Surveillance Monitoring of ART Toxicities study. Echocardiographic Z-scores were calculated using normative data from an established reference cohort. We used adjusted linear regression models to compare Z-scores for echocardiographic measures from HEU children exposed in utero to HAART with those exposed to non-HAART, adjusting for demographic and maternal health characteristics. One hundred seventy-four HEU subjects with echocardiograms and maternal ARV information were included (mean age 10.9 years; 48% male, 56% black non-Hispanic). Among 156 HEU youth with any ARV exposure, we observed no differences in Z-scores for LV systolic function measures between youth exposed in utero to HAART (39%) and HAART-unexposed youth in either unadjusted or adjusted models. In adjusted models, those exposed to HAART had significantly lower mitral late diastolic inflow velocities (adjusted mean Z-score = 0.00 vs. 0.52, p = .04) and significantly higher adjusted mean LV mass-to-volume ratio Z-scores (adjusted mean Z-score = 0.47 vs. 0.11, p = .03) than HAART-unexposed youth. Uninfected children with perinatal exposure to HAART had no difference in LV systolic function. However, small but significant differences in LV diastolic function and cardiac structure were observed, suggesting that continued monitoring for cardiac outcomes is warranted in this population.

Background: Left ventricular (LV) dysfunction is the major reason for poor outcomes in patients with congenital complete atrioventricular block (CCAVB) and pacemaker. Long-term pacing has been associated with LV mechanical dyssynchrony. However, the relationship of dyssynchrony and LV dysfunction is not clear., Objective: We sought to evaluate the prevalence of LV dyssynchrony by real time three-dimensional echocardiography (RT3DE) in patients with CCAVB and its association with LV dysfunction. In addition, we evaluated the agreement between RT3DE and tissue Doppler imaging (TDI) for detecting LV dyssynchrony., Method: We studied 50 patients [median age 20 years old (5 months to 62 years), 68% women] with CCAVB and pacemaker who underwent complete two-dimensional echocardiography and RT3DE. LV dyssynchrony was considered if the systolic dyssynchrony index (SDI) was ≥ 5%. Intraventricular mechanical delay was defined by TDI when differences in electromechanical activation between LV walls were > 65 msec., Results: LV systolic dysfunction was present in 16 patients (32%) by two-dimensional and in 20 patients (40%) by RT3DE. There was a good correlation between LV ejection fraction by two-dimensional and RT3DE (r = 0.75; P < 0.001). Fourteen (28%) patients had intraventricular dyssynchrony by TDI, while 12 (24%) had intraventricular dyssynchrony by RT3DE. There was a good agreement between LV dyssynchrony by TDI and RT3DE (Kappa = 0.735; P < 0.001). There was a negative correlation between LV ejection fraction and SDI obtained by RT3DE (r = -0.58; P < 0.001) CONCLUSIONS: In patients with CCAVB and long-term pacing, LV dyssynchrony occurred in one-third of patients and was related to LV dysfunction. There was a good correlation between dyssynchrony obtained by RT3DE and TDI., (© 2015, Wiley Periodicals, Inc.)

Objective: To describe patent ductus arteriosus (PDA) stenting regardless of ductal morphology in high risk patients with hypoplast physiology undergoing hybrid stage I palliation (PDA stenting and placement of bilateral pulmonary artery bands)., Background: Hybrid palliation is an accepted alternative for patients with hypoplastic left heart syndrome. Patients weighing less than 2.5 kg, history of prematurity, intracranial hemorrhage, and chromosomal abnormality belong to a high-risk group who otherwise might not be ideal candidates for traditional surgical repair (Norwood Operation)., Methods: Between May 2005 and February 2013, a series of 13 high-risk patients with hypoplast physiology with varying types of ductal morphology underwent PDA stenting as part of hybrid stage I palliation., Results: Three major types of ductal morphology were identified: (1) short and semi horizontal, (2) long and semi horizontal, and (3) tortuous. All patients underwent successful PDA stenting. One patient developed proximal coarctation from inadequate coverage of the pulmonary end of the PDA and was successfully treated with a balloon expandable stent 69 days after the initial procedure., Conclusions: Mid- to long-term follow-up indicates that PDA stents remain widely patent regardless of ductal morphology until comprehensive stage II repair., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Frequent causes of premature ductal closure include spontaneous idiopathic closure in utero and maternal use of nonsteroidal anti-inflammatory drugs late in pregnancy., Case: We describe a case of a preterm infant born to a mother treated with lithium throughout pregnancy who presented with right-sided cardiac enlargement at 18 weeks' gestation. Immediately following delivery, echocardiography demonstrated a small closing patent arterial duct., Conclusion: We recommend that serial fetal echocardiography with emphasis on Doppler interrogation of the patent arterial duct be performed whenever a pregnant woman is taking lithium. The interrogation of the patent arterial duct is particularly important if right-sided chamber enlargement is noted at fetal sonography as this finding can be an early manifestation of premature ductal constriction.

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band. Emergency cardiac catheterization confirmed thrombosis of the right pulmonary artery at the site of the band. Direct infusion of tissue plasminogen activator successfully lysed the thrombus.

Traumatic ventricular septal defect is an uncommon complication of blunt chest trauma. We report a case of ventricular septal avulsion, associated with traumatic ventricular septal defect and the associated management concerns., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction., Methods: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period., Results: Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31)., Conclusion: Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-term follow-up., (2010 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

We present the case of a 2.5 year-old child with unbalanced atrioventricular septal defect due to a small left ventricle (LV) (mitral annulus of 10mm and a 0.4 ratio in relation to the tricuspid annulus, LVDD: 17 mm, LV Vd2: 15 ml/m(2) and LV/RV long-axis ratio of 0.71); he had a favorable outcome after biventricular surgical repair. Normal LV development was observed three months after the operation (mitral annulus of 22 mm, with a 0.84 ratio in relation to the tricuspid annulus, and LVDD of 30 mm). Current parameters for utilization of the hypoplastic ventricle are discussed.

Although a new protocol of dobutamine stress echocardiography with the early injection of atropine (EA-DSE) has been demonstrated to be useful in reducing adverse effects and increasing the number of effective tests and to have similar accuracy for detecting coronary artery disease (CAD) compared with conventional protocols, no data exist regarding its ability to predict long-term events. The aim of this study was to determine the prognostic value of EA-DSE and the effects of the long-term use of beta blockers on it. A retrospective evaluation of 844 patients who underwent EA-DSE for known or suspected CAD was performed; 309 (37%) were receiving beta blockers. During a median follow-up period of 24 months, 102 events (12%) occurred. On univariate analysis, predictors of events were the ejection fraction (p <0.001), male gender (p <0.001), previous myocardial infarction (p <0.001), angiotensin-converting enzyme inhibitor therapy (p = 0.021), calcium channel blocker therapy (p = 0.034), and abnormal results on EA-DSE (p <0.001). On multivariate analysis, the independent predictors of events were male gender (relative risk [RR] 1.78, 95% confidence interval [CI] 1.13 to 2.81, p = 0.013) and abnormal results on EA-DSE (RR 4.45, 95% CI 2.84 to 7.01, p <0.0001). Normal results on EA-DSE with beta blockers were associated with a nonsignificant higher incidence of events than normal results on EA-DSE without beta blockers (RR 1.29, 95% CI 0.58 to 2.87, p = 0.54). Abnormal results on EA-DSE with beta blockers had an RR of 4.97 (95% CI 2.79 to 8.87, p <0.001) compared with normal results, while abnormal results on EA-DSE without beta blockers had an RR of 5.96 (95% CI 3.41 to 10.44, p <0.001) for events, with no difference between groups (p = 0.36). In conclusion, the detection of fixed or inducible wall motion abnormalities during EA-DSE was an independent predictor of long-term events in patients with known or suspected CAD. The prognostic value of EA-DSE was not affected by the long-term use of beta blockers.

Purpose: We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini adjustable banding system., Description: Through a mid-sternotomy, a 5-day-old neonate underwent bilateral pulmonary artery banding using this new system, combined with placement of a main pulmonary artery to the innominate artery shunt., Evaluation: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75% to 85% range. On day 48 of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. Afterward, seven additional percutaneous adjustments of the banding system were necessary. The Norwood operation and the bidirectional Glenn shunt were carried out on the day 106 of life. The bands were removed with no pulmonary artery distortion., Conclusions: The clinical use of this innovative pulmonary artery banding system was feasible, safe, and effective. This allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Aim: The aim of this study was to determine the influence of gender on chronotropic and hemodynamic response during dobutamine stress echocardiography with early injection of atropine (EA-DSE)., Methods: From January 2000 to June 2003, we retrospectively evaluated patients who underwent EA-DSE for known or suspected coronary artery disease. We studied 494 patients, 243 men and 251 women, who were not under beta-blocker or calcium channel-blocker therapy (Group A) and 326 patients, 157 men and 169 women, using these medications (Group B). Differences on chronotropic and hemodynamic responses during EA-DSE were assessed by gender in the two groups. Predictors of need for higher doses of dobutamine were determined by multivariate analysis., Results: In Group A, higher proportion of women achieved test end points at the stage of 20 mcg/kg per minute. Dobutamine and atropine doses were lower in women than in men (29 +/- 5 versus 31 +/- 6 mcg/kg per minute; P = 0.001 and 0.61 +/- 0.32 versus 0.78 +/- 0.43 mg; P = 0.006). Cardiac chronotropism and systolic blood pressure response patterns differed by gender. In Group B, there was no difference in the proportion of patients who achieved test end points at each stage of dobutamine. The independent predictors of the need for higher doses of dobutamine in Group A were gender [Odds Ratio (OR) = 1.99, 95% Confidence Interval (CI) = 1.19-3.32; P = 0.008], age (OR = 0.91,CI = 0.89-0.93; P < 0.001), and baseline heart rate (OR = 0.95,CI = 0.93-0.98; P < 0.001). Independent predictors in the total population were age (OR = 0.92,CI = 0.90-0.94; P < 0.001), baseline heart rate (OR = 0.95,CI = 0.93-0.97; P < 0.001), and beta-blocker therapy (OR = 0.42,CI = 0.18-1.51; P = 0.04)., Conclusion: Gender has influence on heart rate and blood pressure response to the EA-DSE in patients without use of drugs with negative chronotropic effects.

We sought to compare the feasibility and accuracy of myocardial blood flow reserve (MBFR) measured by quantitative real-time myocardial contrast echocardiography with those of coronary flow velocity reserve (CFVR) obtained by transthoracic Doppler echocardiography for detecting left anterior descending coronary artery (LAD) stenosis. We studied 71 patients who underwent adenosine stress contrast echocardiography, transthoracic Doppler echocardiography, and quantitative coronary angiography within 1 month. An index of myocardial blood flow (A x beta) was determined by quantification of peak plateau acoustic intensity (A) and microbubble replenishment velocity (beta) by contrast echocardiography. Feasibilities of qualitative analysis of myocardial perfusion, and CFVR and MBFR measurements were 98%, 83%, and 94%, respectively. Patients with LAD stenosis had lower CFVR (1.1 +/- 0.4 vs 2.7 +/- 0.8, P < .001), MBFR (1.2 +/- 0.5 vs 2.5 +/- 0.8, P < .001), and beta reserve (1.1 +/- 0.5 vs 2.4 +/- 0.6, P < .001) than those without lesion. Sensitivities, specificities, and accuracies for detecting LAD stenosis were 64%, 93%, and 80% for qualitative analysis of myocardial perfusion; 92%, 94%, and 93% for CFVR; 84%, 87%, and 86% for MBFR; and 80%, 97%, and 89% for beta reserve. In this selected study population, CFVR was the best index for detecting LAD stenosis (odds ratio = 1.78, 95% confidence interval = 1.28-2.47).

Objective: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow., Method: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt., Results: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75%-85% range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries., Conclusions: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Objectives: The purpose of this study was to determine whether 3-dimensional echocardiography (3DE) provides additional information regarding the mechanisms and sites of left atrioventricular valve regurgitation in atrioventricular septal defect compared with transesophageal 2-dimensional echocardiography (2DE)., Methods: Eleven patients with a median age of 5.4 years (2.9-11.6 years) and a median weight of 16.8 kg (13.7-38.3 kg) with an atrioventricular septal defect underwent simultaneous transesophageal 2DE and 3DE before operation., Results: The 2DE-3DE agreement for the assessment of the superior and mural leaflet size was 72.7%. The 2DE-3DE agreement for coaptation failure, a residual or primary cleft, and commissural abnormalities as a mechanism of regurgitation were 72.7%, 63.6%, and 36.4%, respectively. For jet sites the 2DE-3DE agreement was 63.6% for a commissural and central location., Conclusion: Three-dimensional echocardiography provides new and superior data regarding the mechanisms and sites of left atrioventricular valve regurgitation in atrioventricular septal defect.

Background: We evaluated our experience with aortic valve cusp extension techniques to identify predictors of successful intraoperative repair and subsequent durability., Methods: Twenty-two children (ages 5-18 years) underwent aortic cusp extension with autologous pericardium between 1999 and 2005. Sixteen children had previous surgical or percutaneous intervention. Ten children had bicuspid aortic valves. Cusp extensions were performed on 1 cusp in 3 patients, 2 cusps in 3, and 3 cusps in 16. Serial echocardiographic measures (n = 81) were obtained during a 5-year period and underwent blinded review. Longitudinal trajectories of ventricular and aortic valve function were modeled using mixed linear regression analysis., Results: There was no hospital or late mortality. Five-year freedom from valve replacement was 75%. Comparison of preoperative and post-repair echocardiograms demonstrated reductions in aortic insufficiency (decreased in jet-width/aortic valve diameter ratio from 0.39 +/- 0.12 to 0.22 +/- 0.11; p < 0.0001), aortic stenosis (decreased in peak aortic valve gradient from 41 +/- 25 mm Hg to 29 +/- 15 mm Hg; p = 0.04), and left ventricular end-diastolic dimensions Z-score (decreased from 1.39 +/- 0.38 to 1.16 +/- 0.34; p < 0.001). During the follow-up period, post-repair jet-width and aortic valve diameter increased nonlinearly (p < 0.001). Patients with postoperative peak aortic gradients greater than 30 mm Hg had progression of aortic stenosis, whereas those with lesser postoperative peak gradients tended to regress during follow-up (p < 0.001). The decrement in Z-score of the left ventricular end-diastolic dimensions remained stable during the follow-up period., Conclusions: Aortic valve cusp extension can result in acceptable hemodynamic results with stabilization of left ventricular geometry. However, residual lesions are common and progression and regression of these lesions can be predicted based on echocardiographic data.

Background: Tricuspid regurgitation (TR) has a negative impact on outcome in hypoplastic left heart syndrome (HLHS). There is a paucity of data assessing the mechanisms of TR. Lateral forces from the left ventricle play an important role in normal tricuspid valve (TV) function. This study evaluates the role of real-time 3-dimensional echocardiography in the assessment of the TV annulus and subvalvular apparatus in HLHS., Methods: In all, 31 real-time 3-dimensional echocardiographic studies from 26 patients (1 day-17 years old) with classic HLHS were included. In all, 7 studies were before and 8 were after first-stage palliation; 8 were postcavopulmonary shunt and 8 were post-Fontan. Only patients with severe left ventricular attenuation were included. Studies were subdivided into two groups: mild TR (N = 20) and severe TR (STR) (N = 11). Data from 17 control subjects were used for comparison. TV annular area, bending angle (saddle shape), and position of anterior papillary muscle were analyzed., Results: All annular segments moved in equally during systole in mild TR, whereas septal-lateral diameter contraction was significantly diminished in STR. TV annular area was larger in HLHS (mild TR, 1265 +/- 325; STR, 1767 +/- 450 mm2/m2) than in control subjects (762 +/- 85) (P < .001). However, area change was reduced only in STR (12.4 +/- 4.6% vs control subjects 20.4 +/- 8.3%, P < .05). Annular shape was flat during systole in HLHS. Anterior papillary muscle was laterally displaced in STR., Conclusions: TV annular dynamics were different in patients with HLHS as a result of lack of interaction form left ventricle. Annular function, especially in septal-lateral direction, was further impaired in STR.

We describe a case of giant aneurysm of right atrium in a child with a previous diagnosis of Ebstein's anomaly of tricuspid valve. Three-dimensional real-time echocardiography provided more precise anatomic detail of the tricuspid valve, right atrium, and right ventricle than did its 2-dimensional counterpart.

Objective: We have previously reported a limited but favorable experience with a novel sutureless technique for surgical management of postoperative pulmonary vein stenosis occurring after repair of total anomalous pulmonary venous drainage. Because this technique requires integrity of the retrocardiac space for hemostasis, extension of the technique to the primary repair of pulmonary vein anomalies requires evaluation. This analysis reviews our experience with the sutureless technique in patients with postrepair pulmonary vein stenosis, as well as our extension of the technique into primary repair of pulmonary vein anomalies., Methods: Retrospective univariable-multivariable analysis of all pulmonary vein stenosis procedures and sutureless pulmonary vein procedures over a 20-year period was performed. Cox proportional hazards modeling was used to identify variables associated with freedom from reoperation or death., Results: Sixty patients underwent 73 procedures, with pulmonary vein stenosis present in 65 procedures. The sutureless technique was used in 40 procedures. Freedom from reoperation or death at 5 years after the initial procedure was 49%. Unadjusted freedom from reoperation or death was greater with the sutureless technique for patients with postrepair pulmonary vein stenosis ( P = .04). By using multivariable analysis, a higher pulmonary vein stenosis score was associated with greater risk of reoperation or death. After adjustment, the sutureless repair was associated with a nonsignificant trend toward greater freedom from reoperation or death ( P = .12). Despite the absence of retrocardiac adhesions, operative mortality was not increased with the sutureless technique ( P = .64). Techniques to control bleeding (intrapleural hilar reapproximation) and improve exposure (inferior vena cava division) were identified., Conclusion: The sutureless technique for postrepair pulmonary vein stenosis is associated with encouraging midterm results. Extension of the indications for the technique to primary repair appears safe with the development of simple intraoperative maneuvers.

Subaortic left brachiocephalic, or innominate, vein is an uncommon finding in congenital heart disease, usually associated with obstruction of the right ventricular outflow tract. We describe our experience with 14 patients in whom the lesion was identified echocardiographically, 12 of them with right ventricular obstruction, one with totally anomalous pulmonary venous connection in the absence of obstruction to the right ventricular outflow tract, and the final one with a normal heart. A precise diagnosis of this venous anomaly is of great importance, since it needs to be differentiated from a central pulmonary artery, a pulmonary venous confluence, or an ascending vertical vein in totally anomalous pulmonary venous connection. In patients referred for surgery without catheterization, an incorrect echocardiographic diagnosis could lead to disastrous surgical results.