Development of the Residual Lesion Score for congenital heart surgery: the RAND Delphi methodology.

BACKGROUND AND OBJECTIVE: The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology. METHODS: A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the "final" Residual Lesion Score. RESULTS: The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the "final" Residual Lesion Score. CONCLUSION: Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network's Residual Lesion Score study.

Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association.

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.

Interstage Outcomes in Infants With Single Ventricle Heart Disease Comparing Home Monitoring Technology to Three-Ring Binder Documentation: A Randomized Crossover Study.

BACKGROUND: Interstage outcomes for infants with single ventricle remain suboptimal. We have previously described a tablet PC-based platform Cardiac High Acuity Monitoring Program (CHAMP) for remote monitoring which provides immediate access to data, videos, and instant alerts to our single ventricle care team. METHODS: This study compares traditional three-ring binder monitoring (Binder) to CHAMP using a randomized crossover design to evaluate mortality, resource utilization, and caregiver experience. At discharge, all single ventricle infants were monitored using Binder and randomized to receive CHAMP at either one or two months postdischarge. One month after randomization, caregivers could choose either Binder or CHAMP for the remainder of the interstage period. Caregivers experience was recorded using surveys. RESULTS: Enrollment included 31 single ventricle infants from May 2014 to June 2015. There was no interstage mortality over 4,911 total interstage days (median: 144/patient). Of 73 readmissions, 45 were unplanned. Of the initial 23 unplanned readmissions, 13 were found to have been based on data obtained exclusively through CHAMP (as instant alerts or based on data review) rather than caregiver concerns. Due to concerns regarding patient safety, additional enrollment was stopped. The CHAMP use was associated with significantly fewer unplanned intensive care unit days/100 interstage days, shorter delays in care, lower resource utilization at readmissions, and lower incidence of interstage growth failure and was preferred by a majority of caregivers. CONCLUSIONS: These findings suggest that CHAMP may offer benefits over Binder (improved interstage outcomes, delays in care, and caregiver experience). These findings should be tested across multiple centers in larger populations.

Echocardiographic Detection of Increased Ventricular Diastolic Stiffness in Pediatric Heart Transplant Recipients: A Pilot Study.

BACKGROUND: Pediatric heart transplant recipients are at risk for increased left ventricular (LV) diastolic stiffness. However, the noninvasive evaluation of LV stiffness has remained elusive in this population. The objective of this study was to compare novel echocardiographic measures of LV diastolic stiffness versus gold-standard measures derived from pressure-volume loop (PVL) analysis in pediatric heart transplant recipients. METHODS: Patients undergoing left heart catheterization were prospectively enrolled. PVLs were obtained via conductance. The end-diastolic pressure-volume relationship was obtained via balloon occlusion. The stiffness constant, ß, was calculated. Echocardiographic measures of diastolic function were derived from spectral and tissue Doppler and two-dimensional speckle-tracking. Ventricular volumes were measured using three-dimensional echocardiography. The novel echocardiographic estimates of ventricular stiffness included E:e'/end-diastolic volume (EDV) and E:early diastolic strain rate/EDV. RESULTS: Of 24 children, 18 were heart transplant recipients. Six control patients had hemodynamically insignificant patent ductus arteriosus or coronary fistula. The mean age was 9.1 ± 5.6 years. Median end-diastolic pressure was 9 mm Hg (interquartile range, 8-13 mm Hg). Lateral E:e'/EDV (r = 0.59, P < .01), septal E:e'/EDV (r = 0.57, P < .01), and (E:circumferential early diastolic strain rate)/EDV (r = 0.54, P < .01) correlated with ß. Lateral E:e'/EDV displayed a C statistic of 0.93 in detecting patients with abnormal LV stiffness (ß > 0.015 mL-1). A lateral E:e'/EDV of >0.15 mL-1 had 89% sensitivity and 93% specificity in detecting an abnormal ß. CONCLUSIONS: Echocardiographic estimates of ventricular stiffness may be accurate compared with the gold standard in pediatric heart transplant recipients. The clinical usefulness of these noninvasive measures in assessing LV stiffness merits further study in children.

Medium-term assessment of cardiac function in pediatric cancer survivors. Comparison of different echocardiographic methods, cardiac MRI and cardiac biomarker testing in adolescent cancer survivors.

PURPOSE: To evaluate the feasibility and correlation of 3D echocardiography (echo) and cardiac biomarkers with cardiac MRI, in surveillance of cardiac function for cancer survivors. METHODS: Subjects ≥10 years of age who have survived >2 years after completion of cancer treatment from a single center were enrolled. Cardiac MRI and 3D echo images were obtained on the same day when routine echo was obtained. On the same day, along with annual routine blood test, cardiac biomarkers N-terminal pro-B-type natriuretic peptide levels (NT-proBNP) and troponin-I levels were also measured. RESULTS: Cardiac MRI was feasible in all 50 subjects. Three-dimensional echo and 2D echo images were of poor quality in four subjects. With a median duration of remission of 10 years, there were four subjects with mild LV dysfunction (cardiac MRI LV EF of<53%). None had MRI EF <50%, and nine subjects had LVEF <55%. M-mode echo overestimated EF more than 2D and 3D echo. Two-dimensional and 3D echo methods had much tighter limits of agreement for LV EF. For measurement of LVEF, 3D echo had a lower % error than 2D echo or M-mode echo. One subject had an abnormal troponin-I level and another one had an elevated NT-proBNP. CONCLUSIONS: Three-dimensional echo can be performed in most adolescent cancer survivors, and it correlates well with MRI. Further large-scale research is required in assessing utility of cardiac biomarkers in pediatric cancer survivors.

Implementation of a Quality Improvement Bundle Improves Echocardiographic Imaging after Congenital Heart Surgery in Children.

BACKGROUND: Postoperative echocardiography after congenital heart disease surgery is of prognostic importance, but variable image quality is problematic. We implemented a quality improvement bundle comprising of focused imaging protocols, procedural sedation, and sonographer education to improve the rate of optimal imaging (OI). METHODS: Predischarge echocardiograms were evaluated in 116 children (median age, 0.51 years; range, 0.01-5.6 years) from two centers after tetralogy of Fallot repair, arterial switch operation, and bidirectional Glenn and Fontan procedures. OI rates were compared between the centers before and after the implementation of a quality improvement bundle at center 1, with center 2 serving as the comparator. Echocardiographic images were independently scored by a single reader from each center, blinded to center and time period. For each echocardiographic variable, quality score was assigned as 0 (not imaged or suboptimally imaged) or 1 (optimally imaged); structures were classified as intra- or extracardiac. The rate of OI was calculated for each variable as the percentage of patients assigned a score of 1. RESULTS: Intracardiac structures had higher OI than extracardiac structures (81% vs 57%; adjusted odds ratio [OR], 3.47; P < .01). Center 1 improved overall OI from 48% to 73% (OR, 4.44; P < .01), intracardiac OI from 69% to 85% (OR, 3.53; P = .01), and extracardiac OI from 35% to 67% (OR, 5.16; P < .01). There was no temporal difference for center 2. CONCLUSIONS: After congenital heart disease surgery in children, intracardiac structures are imaged more optimally than extracardiac structures. Focused imaging protocols, patient sedation, and sonographer education can improve OI rates.

Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure.

BACKGROUND: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status. METHODS: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3). Studies were also classified dichotomously (restrictive pattern present or absent). Relationships between DFG and pre-Fontan variables (e.g., ventricular morphology, age at Fontan, history of volume-unloading surgery) and current status (e.g., systolic function, valvar regurgitation, exercise performance) were explored. RESULTS: DFG was calculable in 326 of 546 subjects (60%) (mean age, 11.7 ± 3.3 years). Overall, 32% of patients had grade 0, 9% grade 1, 37% grade 2, and 22% grade 3 diastolic function. Although there was no association between ventricular morphology and DFG, there was an association between ventricular morphology and E', which was lowest in those with right ventricular morphology (P < .001); this association remained significant when using Z scores adjusted for age (P < .001). DFG was associated with achieving maximal effort on exercise testing (P = .004); the majority (64%) of those not achieving maximal effort had DFG 2 or 3. No additional significant associations of DFG with laboratory or clinical measures were identified. CONCLUSIONS: Assessment of diastolic function by current algorithms results in a high percentage of patients with abnormal DFG, but few clinically or statistically significant associations were found. This may imply a lack of impact of abnormal diastolic function on clinical outcomes in this cohort, or it may indicate that the methodology may not be applicable to pediatric patients with functional single ventricles.

Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle.

Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a cloud-based server, with the capacity for instant alerts to be sent to the caregiver team. The cloud-based IT infrastructure lends itself well to being able to be scaled to multiple sites while maintaining strict control over the privacy of each site. All transmitted data are transferred to the electronic medical record daily. The system conforms to recently released Food and Drug Administration regulation that pertains to mobile health technologies and devices. Since this platform was developed in March 2014, 30 patients have been monitored. There have been no interstage deaths. The experience of care providers has been unanimously positive. The addition of video has added to the use of the monitoring program. Of 30 families, 23 expressed a preference for the tablet PC over the notebook, 3 had no preference, and 4 preferred the notebook to the tablet PC.

Impact of Variability in Echocardiographic Interpretation on Assessment of Adequacy of Repair Following Congenital Heart Surgery: A Pilot Study.

Technical Performance Score (TPS) is based largely on the presence and magnitude of residual lesions on postoperative echocardiograms; this score correlates with outcomes following repair of congenital heart defects. We evaluated reader variability for echocardiographic components of TPS for complete repair of tetralogy of Fallot (TOF) and arterial switch operation (ASO) in two centers and measured its effect on TPS. Postoperative echocardiograms were evaluated in 67 children (39 TOF and 28 ASO). Two readers (one per center) interpreted each echocardiogram. Reader variability in image quality assessments and measurements was compared using weighted kappa (κ), percent agreement, and intra-class correlation. TPS class (1 optimal-no residua, 2 adequate-minor residua, 3 inadequate-major residua) was assigned for each echocardiographic review by an independent investigator. The effect of reader interpretation variability on TPS classification was measured. There was strong agreement for TPS between the two readers (κ = 0.88). The readers were concordant for TPS classes for 57 children (85%) and discordant for classes 2 (minor residua) versus 3 (major residua) in six (9%). Coronary arteries and branch pulmonary arteries were frequently suboptimally visualized. Although inter-reader agreement for TPS was strong, inter-reader variation in echocardiographic interpretations had a small, but important effect on TPS for TOF and ASO, particularly for the distinction between minor and major residua. Further studies of generalizability and reproducibility of TPS and refinement of scoring modules may be needed before it can be used as a tool to assess pediatric cardiac surgical performance and outcomes.

Changes in speckle tracking echocardiography measures of ventricular function after percutaneous implantation of the Edwards SAPIEN transcatheter heart valve in the pulmonary position.

BACKGROUND: Patients with free pulmonary regurgitation or mixed pulmonary stenosis and regurgitation and severely dilated right ventricles (RV) show little improvement in ventricular function after pulmonary valve replacement when assessed by traditional echocardiographic markers. We evaluated changes in right and left ventricular (LV) function using speckle tracking echocardiography in patients after SAPIEN transcatheter pulmonary valve (TPV) placement. METHODS: Echocardiograms were evaluated at baseline, discharge, 1 and 6 months after TPV placement in 24 patients from 4 centers. Speckle tracking measures of function included peak longitudinal strain, strain rate, and early diastolic strain rate. RV fractional area change, tricuspid annular plane systolic excursion, and left ventricular LV ejection fraction were assessed. Routine Doppler and tissue Doppler velocities were measured. RESULTS: At baseline, all patients demonstrated moderate to severe pulmonary regurgitation; this improved following TPV placement. No significant changes were detected in conventional measures of RV or LV function at 6 months. RV longitudinal strain (-16.9% vs. -19.6%, P < 0.01), strain rate (-0.87 s(-1) vs. -1.16 s(-1) , P = 0.01), and LV longitudinal strain (-16.2% vs. -18.2%, P = 0.01) improved between baseline and 6 month follow-up. RV early diastolic strain rate, LV longitudinal strain rate and early diastolic strain rate showed no change. CONCLUSION: Improvements in RV longitudinal strain, strain rate, and LV longitudinal strain are seen at 6 months post-TPV. Diastolic function does not appear to change at 6 months. Speckle tracking echocardiography may be more sensitive than traditional measures in detecting changes in systolic function after TPV implantation.

Percutaneous implantation of the Edwards SAPIEN transcatheter heart valve for conduit failure in the pulmonary position: early phase 1 results from an international multicenter clinical trial.

OBJECTIVES: The purpose of this study was to evaluate the safety and effectiveness of the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position in patients with moderate to severe pulmonary regurgitation with or without stenosis. BACKGROUND: Transcatheter pulmonary valve replacement is evolving, but to date, experience has been limited to the Melody valve (Medtronic Inc., Minneapolis, Minnesota). METHODS: Eligible patients with dysfunctional right ventricle-to-pulmonary artery conduits were screened if body weight was ≥35 kg and the in situ conduit diameter was ≥16 mm and ≤24 mm. Standardized implantation and follow-up protocols were used. RESULTS: Thirty-six patients from 4 centers were recruited between April 2008 and May 2010. Mean body weight was 73.4 ± 22.9 kg. Successful valve deployment was achieved in 33 of 34 attempts (97.1%). Valve migration occurred in 3 patients, with 2 requiring surgical retrieval; however, 1 patient underwent successful perventricular valve implantation. Further intraprocedure complications included pulmonary hemorrhage (n = 2), ventricular fibrillation (n = 1), and stent migration (n = 1). Pullback gradient across the conduit decreased from 26.8 ± 18.4 mm Hg to 11.7 ± 8.0 mm Hg (p < 0.001). The right ventricular/aortic pressure ratio decreased from 0.6 ± 0.2 to 0.4 ± 0.1 (p < 0.001). Peak Doppler gradient across the right ventricular outflow tract decreased from 41.9 ± 27.9 mm Hg to 19.1 ± 13.3 mm Hg (p < 0.001). At 6-month follow-up, all patients were alive. The number of patients with New York Heart Association functional class I increased from 5 at baseline to 27 at follow-up. Pulmonary regurgitation was ≤2+ in 97% of patients. Freedom from reintervention was 97% with 1 patient undergoing elective placement of a second valve due to conduit-induced distortion of the initial implant. CONCLUSIONS: Transcatheter pulmonary valve replacement using the Edwards SAPIEN transcatheter heart valve is safe and effective in patients with dysfunctional right ventricle-to-pulmonary artery conduits.

Internal right ventricular band for multiple ventricular septal defects in a neonate undergoing arterial switch and aortic arch repair.

A neonate presented with d-transposition of the great arteries, aortic arch hypoplasia, aortic coarctation, and multiple ventricular septal defects. During the arterial switch procedure and the aortic arch repair, a fenestrated Gore-Tex disk (W.L. Gore & Assoc, Flagstaff, AZ) was sewn into the right ventricular outflow tract to restrict pulmonary blood flow. The internal right ventricular band successfully controlled the pulmonary blood flow, maintaining a systemic oxygen saturation of 88% to 92%, and allowing growth from 3.5 to 10.5 kg. At 8 months of age, the internal band in the patient was removed, and the ventricular septal defects were successfully closed.

Comparison of Norwood shunt types: do the outcomes differ 6 years later?

BACKGROUND: A modification to the Norwood procedure involving a right ventricle-to-pulmonary artery (RV-PA) shunt may improve early postoperative outcomes. Concerns remain about the effect of the right ventriculotomy required with this shunt on long-term ventricular function. METHODS: Between January 2000 and April 2005, 76 patients underwent the Norwood procedure, 35 with a modified Blalock-Taussig shunt (mBTS) and 41 with a RV-PA shunt. Patients were monitored until death or September 1, 2009, with an average follow-up of 6.8 years. Cardiac catheterization, echocardiograms, perioperative Fontan courses, and need for cardiac transplantation were compared between groups. RESULTS: Cumulative survival was 63% (22 of 35) in the mBTS group vs 78% (32 of 41) in the RV-PA group (p = 0.14). Pre-Fontan echocardiography revealed poorer ventricular function in RV-PA patients (p = 0.03). Cardiac transplantation was required in 6 of 32 (19%) patients with a prior RV-PA shunt vs 1 of 23 (4%) in the mBTS group (p = 0.06). This results in an almost identical cumulative transplant-free survival between groups; 60% (21 of 35) in the mBTS group and 63% (26 of 41) in the RV-PA group (p = 0.95). CONCLUSIONS: Neither shunt offers a clear survival advantage through an average follow-up of 6.8 years. The RV-PA shunt results in impaired late ventricular function that may result in an increased need for cardiac transplantation.

Initial experience with a miniaturized multiplane transesophageal probe in small infants undergoing cardiac operations.

PURPOSE: There has been reluctance to use intraoperative transesophageal echocardiography (TEE) in small infants. We assessed the utility and safety of a new miniaturized multiplane micro-TEE probe in small infants undergoing cardiac operations. DESCRIPTION: Hemodynamic and ventilation variables were prospectively recorded before and after micro-TEE insertion and removal in infants weighing 5 kg or less undergoing cardiac operations. EVALUATION: The study included 42 patients with a mean weight of 3.6 +/- 0.9 kg (range, 1.7 to 5 kg). All probe insertions were successful. There were no complications or clinically significant changes in hemodynamic or ventilation variables. Information provided by TEE resulted in surgical revision in 6 of the 42 patients. CONCLUSIONS: The micro-TEE provides high quality, useful diagnostic images without hemodynamic or ventilation compromise in small infants undergoing cardiac operations. This advance is important with the growing trend towards complete repair of complex structural heart disease in small infants.

Universal screening for extracardiac abnormalities in neonates with congenital heart disease.

Extracardiac or genetic abnormalities (EGA) represent a factor in the morbidity of patients with congenital heart disease. We evaluated the way neonates with CHD are screened at our institution and determined the yield for the screening tests. We reviewed the charts of 223 neonates with structural CHD. Subjects were categorized into 6 groups: univentricular, left-sided obstructive lesions, right-sided obstructive lesions, septal defects, conotruncal defects (CTD), and other. We reviewed which patients underwent cranial ultrasonogram (CUS), abdominal ultrasonogram (AUS), and/or genetic studies (GS) as well as their results. There was a high prevalence of EGA in each group by CUS (32% to 42%), AUS (32% to 69%), and GS (10% to 60%). There was considerable variability in the proportion within each group that underwent screening tests, and the consistency of screening often was not congruent with the likelihood of abnormal results. Approximately 50% of our patients had >/=1 EGA identified, resulting in a cost-yield ratio of $4,508/patient with EGA. Screening for EGA at our institution is not uniform and is often at odds with the prevalence of such patients. Given the high prevalence of EGA, we advocate for a universal screening program for neonates with CHD using cranial/abdominal ultrasonography and genetic testing.

Survival after bidirectional cavopulmonary anastomosis: analysis of preoperative risk factors.

OBJECTIVE: Prognostic factors for survival after bidirectional cavopulmonary anastomosis for functionally single ventricle are not well defined. We analyzed preoperative hemodynamic and echocardiographic data to determine risk factors for death or transplantation at least 1 year after bidirectional cavopulmonary anastomosis. METHODS: Data for all patients who underwent bidirectional cavopulmonary anastomosis before 5 years of age at our institution from September 1995 through June 2005 were analyzed. Available preoperative echocardiograms and catheterizations were reviewed. Survivors were compared with those who died or underwent transplantation. Bivariable associations between demographic and clinical risk factors and survival status (alive without transplantation vs dead or transplanted) were assessed with Wilcoxon rank sum test and chi2 or Fisher exact tests. Survival functions were constructed with Kaplan-Meier estimates, and event times compared between subgroups with log-rank tests. Cox proportional hazard modeling was used for multivariable modeling of risk of death or transplantation. RESULTS: One hundred sixty-seven patients underwent bidirectional cavopulmonary anastomosis with hemi-Fontan (n = 62) or bidirectional Glenn (n = 105) operations. Three patients died before discharge, 11 died later, and 1 has undergone transplantation. Freedom from death or transplantation after bidirectional cavopulmonary anastomosis was 96% at 1 year and 89% at 5 years. Multivariable analysis of preoperative variables showed atrioventricular valve regurgitation to be an independent risk factor for death or transplantation (hazard ratio 2.8, 95% confidence interval 1.1-7.1, P = .02). CONCLUSION: Although survival after bidirectional cavopulmonary anastomosis is high, preoperative atrioventricular valve regurgitation is an important risk factor for death or transplantation.

Does a ventriculotomy have deleterious effects following palliation in the Norwood procedure using a shunt placed from the right ventricle to the pulmonary arteries?

INTRODUCTION: A recent modification to the Norwood procedure involving a shunt placed directly from the right ventricle to the pulmonary arteries may improve postoperative haemodynamics. Concerns remain, however, about the potential problems produced by the required ventriculotomy. METHODS: We compared 76 patients with hypoplastic left heart syndrome who underwent the Norwood procedure, 35 receiving a modified Blalock-Taussig shunt and the remaining 41 a shunt placed directly from the right ventricle to the pulmonary arteries. We reviewed their subsequent progress through the second stage of palliation. A single observer graded right ventricular function, and the severity of tricuspid regurgitation, based on blinded review of the most recent echocardiograms prior to the second stage of palliation. RESULTS: At the time of catheterization prior to the second stage, patients with a shunt placed from the right ventricle to the pulmonary arteries, rather than a modified Blalock-Taussig shunt, had higher arterial diastolic blood pressure, at 44 versus 40 millimetres of mercury, p equal to 0.02, lower ventricular end diastolic pressures, at 8 versus 11 millimetres of mercury, p equal to 0.0002, and larger pulmonary arteries as judged using the Nakata index, at 270 versus 188 millimetres squared per metres squared, p equal to 0.009. There was no difference in qualitative ventricular systolic function or tricuspid regurgitation between groups. No differences were found between groups during the hospitalization following the second stage of palliation. A trend towards improved survival to the second stage was seen following the construction of a shunt from the right ventricle to the pulmonary arteries. CONCLUSIONS: Construction of a shunt from the right ventricle to the pulmonary arteries is associated with lower right ventricular end diastolic pressures, larger pulmonary arterial size, and higher systemic arterial diastolic pressures. No apparent deleterious effects of the right ventriculotomy were observed in terms of qualitative ventricular systolic function or tricuspid regurgitation.

Early experience with real-time three-dimensional echocardiographic guidance of right ventricular biopsy in children.

BACKGROUND: Right ventricular endomyocardial biopsy is the gold standard for detecting active myocardial inflammation in cardiomyopathy as well as rejection after cardiac transplantation. This procedure has historically required the exclusive use of fluoroscopic guidance to guide catheter, sheath, and bioptome manipulation. The current study evaluates the feasibility and utility of real-time transthoracic three-dimensional echocardiography (3DE) to guide right ventricular endomyocardial biopsies in children. METHODS: From July 2003 to April 2004, we utilized real-time 3DE in 28 consecutive cardiac catheterizations in children aged 18 months to 16 years who were undergoing endomyocardial biopsy. A commercially available 3DE scanner (Philips Sonos 7500) equipped with a 2-4 MHz 3D matrix array transthoracic probe was utilized in all cases. RESULTS: A total of 123 endomyocardial biopsy samples were obtained in nine patients (BSA 0.85 m(2)+/- 0.33 m(2)). Of these 123 samples, 99 (80%) were obtained with the use of real-time transthoracic 3DE. There were no complications, including no new tricuspid valve leaflet flail or pericardial effusion. 3DE proved to be a reliable noninvasive modality to properly direct the bioptome to the desired site of biopsy within the right ventricle. As familiarity with this technique increased, the need for fluoroscopic guidance of bioptome manipulation in the right ventricle was minimized. CONCLUSIONS: The use of real-time transthoracic 3DE in endomyocardial right ventricular biopsies in children is both feasible and safe. Further study to determine the impact of real-time 3DE guidance on fluoroscopy and case times for endomyocardial biopsies is warranted.

Current issues and perspectives in hypoplasia of the left heart.

Hypoplastic left heart syndrome is a rare but serious form of congenital cardiac disease, characterized by underdevelopment of the components of the left heart, rendering the left ventricle non-functional. Its aetiology is largely unknown, but there is certainly a genetic component. Prenatal diagnosis nowadays uncovers about half of cases. Postnatal options for treatment include comfort care, 3-stage palliative surgery, or cardiac transplantation. In this review, we discuss the morphology, possible pathogenetic mechanisms, clinical management, and perspectives of prenatal intervention based on work in animal models.