Costs, mortality, and hospital usage in relation to prenatal diagnosis in d-transposition of the great arteries.

BACKGROUND: The impact of prenatal diagnosis of d-transposition of the great arteries (dTGA) on health-care usage is largely unknown. We evaluated a population-based cohort to assess costs, mortality and inpatient encounters by whether dTGA was prenatally diagnosed or not. METHODS: The dTGA cases (born 1997-2011) identified at the Utah Birth Defect Network, which includes data on timing of diagnosis, were linked to statewide inpatient discharge data. We excluded preterm cases or cases with additional major heart defects. We evaluated hospitalizations and costs for infants (first year of life) and mothers (10 months before birth) using multivariable models adjusted for demographic and clinical risk factors. RESULTS: Of 119 cases, 14 (12%) were prenatally diagnosed. Birth weight, surgical complexity and extracardiac defects/syndromes were similar between groups. Of 7 deaths (6%), two occurred pre-intervention in postnatally diagnosed infants. Prenatal diagnosis was associated with more in-hospital days (estimate 13 additional days, p = 0.03) and higher mean costs for mothers ($4,141 vs $12,148) and infants (90,419 vs $49,576). Prenatal diagnosis independently predicted higher adjusted costs for the overall cohort ($22,570, p = 0.045). After excluding deaths, total costs were no longer significantly different. CONCLUSION: Mothers of prenatally diagnosed infants with dTGA had higher inpatient costs compared with those postnatally diagnosed. Costs trended higher for their infants, although were not significantly different. Linkage of population-based surveillance systems and outcome databases can be a powerful tool to further explore the complex relationship of prenatal diagnosis to costs and outcomes in other types of congenital heart diseases. Birth Defects Research 109:262-270, 2017. © 2017 Wiley Periodicals, Inc.

Tricuspid annular plane systolic excursion for the assessment of ventricular function in adults operated on with mustard procedure for complete transposition of the great arteries.

BACKGROUND: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients. METHODS: Patients were recruited from those evaluated at the adult congenital clinic of our department between 2010 and 2012. All patients who had an echocardiographic assessment within 6 months of their MRI examination were selected. Patients clinically unstable, not in sinus rhythm, with a prosthetic systemic atrioventricular valve, permanent pacemaker, or more than moderate systemic atrioventricular valve regurgitation were excluded. RESULTS: Eighteen Mustard-operated patients aged 22 ± 3.7 years were studied. The mean values of TAPSE and RVEF were 13.22 ± 1.7 mm and 49.7 ± 6%, respectively. TAPSE and RVEF were normal in 1 (5.5%) and 10 (55.5%) patients, respectively. Seventeen (94.4%) patients showed reduced TAPSE (12.9 ± 1.3 mm): RVEF was reduced in eight (47%) of these subjects, and normal in nine (53%). In patients with normal RVEF, both the MRI-RV end-diastolic and the MRI-RV end-systolic volumes were significantly lower than in patients with reduced RVEF. There were no other statistically significant differences between these patients. No correlation was found between TAPSE and both the MRI-RV end-diastolic and the end-systolic volumes. Globally, agreement between TAPSE and RVEF was slight (K = 0.09 ± 0.089). CONCLUSIONS: Our results indicate that in these patients TAPSE is not a useful measure of RV function.

Predicting functional capacity in patients with a systemic right ventricle: subjective patient self-assessment is better than B-type natriuretic peptide levels and right ventricular systolic function.

BACKGROUND: Many adults with transposition of the great arteries have an anatomic right ventricle functioning as the systemic ventricle and are known to develop congestive heart failure, premature cardiac death, and need for cardiac transplantation. Predictors of poor clinical outcome and functional status in patients with left ventricular failure do not always apply to these patients. We aimed to identify predictors of poor functional status in those patients with a systemic right ventricle. METHODS: We performed a prospective study of 51 adults with transposition of the great arteries and systemic right ventricles. Demographic, clinical, laboratory, and imaging data were collected, and patients completed a Minnesota Living with Heart Failure Questionnaire (MLHFQ). Comparisons were made between those patients with d-type transposition of the great arteries (dTGA) who have undergone prior atrial switch and those with congenitally corrected transposition (ccTGA). A correlation analysis was performed to identify predictors of poor functional status, as determined by a 6-minute walk distance test. RESULTS: Median age was 30 years (range 19-65). Median B-type natriuretic peptide was 48 pg/mL (range 16-406). There were 27 patients (53%) with moderate-severe right ventricular dysfunction and 10 (20%) with moderate-severe tricuspid valve regurgitation. The median MLHFQ score was 9 (range 0-78) and 6-minute walk test was 510 m (range 231-703). Forty-one patients had a diagnosis of dTGA atrial switch and 11 patients had ccTGA. Patients with ccTGA were significantly older (40 vs. 28 years, P =.004) and had more tricuspid valve regurgitation (P =.02). Despite this, their MLHFQ scores were significantly lower (2.5 vs. 17, P =.04) and they walked further (635 vs. 504 m, P =.02). Predictors of a short 6-minute walk distance included short stature (P =.009) and dTGA (P =.002). The patient's self-assessment of poor health, as measured by an increased New York Heart Association class (P =.003) and a decreased MLHFQ score (P >.0001) also correlated. B-type natriuretic peptide levels, right ventricular dysfunction, severity of tricuspid valve regurgitation, need for pacemaker, and clinical signs of heart failure did not correlate with exercise tolerance. CONCLUSIONS: Traditional parameters used to predict outcomes in patients with left ventricular failure are not predictive in patients with a systemic right ventricle. Instead, patient's self-assessment of functional status did correlate with objective functional status.

Assessment of right ventricular volumes and function using cardiovascular magnetic resonance cine imaging after atrial redirection surgery for complete transposition of the great arteries.

Cardiovascular magnetic resonance (CMR) imaging is the reference standard for measurement of right ventricular (RV) volumes and function. To date, no study has compared methods of data acquisition and analysis by CMR for adults with a systemic RV. Our objective was to evaluate RV size and function using axial and short axis views in adults post atrial switch (Mustard) surgery. A total of 34 adults (20 male, mean age at CMR 32 ± 6 years) were identified at our centre. Volumes, RV end-diastolic (EDV) and end-systolic (ESV) were measured in short axis and axial orientations by two independent experienced readers, blinded to clinical and CMR data. Intra and interobserver measurements in each view were compared using Bland-Altman plots and intraclass correlation coefficients (ICC). Although mean volumes were larger in the axial as compared with the short axis view [RVEDV 247 ± 67 vs. 233 ± 54 ml (p = 0.002) and RVESV 148 ± 54 vs. 136 ± 50 ml (p = 0.001)], mean RV ejection fractions (EF) were similar [41 ± 9 % vs. 43 ± 12 % (p = 0.13)]. Bland-Altman plots demonstrated better agreement for axial measures of RVEDV and right ventricular ejection fraction (RVEF) within and between observers. Similarly, ICC values were stronger for axial as compared with short axis volumes and function-intraobserver RVEDV 0.99 (0.98-0.99) versus 0.96 (0.92-0.98) and RVEF 0.96 (0.93-0.98) versus 0.90 (0.82-0.95); interobserver RVEDV 0.97 (0.94-0.98) versus 0.90 (0.73-0.95) and RVEF 0.85 (0.53-0.94) versus 0.82 (0.67-0.90). Axially derived measurements of RV volumes and function have better agreement and reproducibility as compared with short axis values; whereas axial volumes tend to be larger, RVEF is not significantly different between the two methods.

Dynamic evolution of practice guidelines: analysis of deviations from assessment and management plans.

OBJECTIVE: A Standardized Clinical Assessment and Management Plan (SCAMP) standardizes the care of patients with a predefined diagnosis while actively inviting and collecting data on clinician deviations (DEVs) from its recommendations. For 3 different pediatric cardiac diagnoses managed by SCAMPs, we determined the frequency of, types of, and reasons for DEVs, which are considered to be a valuable source of information and innovation. METHODS: DEVs were collected as part of SCAMP implementation. DEVs were reviewed by the SCAMP committee chairperson and by a separate protocol deviation committee; they were characterized as either justifiable (J), possibly justifiable (PJ), or not justifiable (NJ). RESULTS: We analyzed 415 patients, 484 clinic encounters, and 216 DEVs. Eighty-six (39.8%) of the DEVs were J, 21 (9.7%) were PJ, and 109 (50.4%) were NJ. The percentage of NJ DEVs relative to the number of opportunities for DEV was 4.1%. J and PJ DEVs were mostly due to management of unrelated conditions (11% overall) or special circumstances (22% overall). NJ DEVs primarily involved follow-up intervals (66%) and deleted tests (24%). The reason for deviating from SCAMP recommendations was not given for 31% of DEVs, even though such information was requested. CONCLUSIONS: The overall low rate of NJ DEV suggests that practitioners generally accept SCAMP recommendations, but improved capture of practitioners' reasons for deviating from those recommendations is needed. This analysis revealed multiple opportunities for improving patient care, suggesting that this process can be useful in both promulgating sound practice and evolving improved approaches to patient management.

Resource utilization after introduction of a standardized clinical assessment and management plan.

INTRODUCTION: A Standardized Clinical Assessment and Management Plan (SCAMP) is a novel quality improvement initiative that standardizes the assessment and management of all patients who carry a predefined diagnosis. Based on periodic review of systemically collected data the SCAMP is designed to be modified to improve its own algorithm. One of the objectives of a SCAMP is to identify and reduce resource utilization and patient care costs. METHODS: We retrospectively reviewed resource utilization in the first 93 arterial switch operation (ASO) SCAMP patients and 186 age-matched control ASO patients. We compared diagnostic and laboratory testing obtained at the initial SCAMP clinic visit and control patient visits. To evaluate the effect of the SCAMP over time, the number of clinic visits per patient year and echocardiograms per patient year in historical control ASO patients were compared to the projected rates for ASO SCAMP participants. RESULTS: Cardiac magnetic resonance imaging (MRI), stress echocardiogram, and lipid profile utilization were higher in the initial SCAMP clinic visit group than in age-matched control patients. Total echocardiogram and lung scan usage were similar. Chest X-ray and exercise stress testing were obtained less in SCAMP patients. ASO SCAMP patients are projected to have 0.5 clinic visits and 0.5 echocardiograms per year. Historical control patients had more clinic visits (1.2 vs. 0.5 visits/patient year, P<.01) and a higher echocardiogram rate (0.92 vs. 0.5 echocardiograms/patient year, P<.01) CONCLUSION: Implementation of a SCAMP may initially lead to increased resource utilization, but over time resource utilization is projected to decrease.

A simplified approach for assessment of intracardiac baffles and extracardiac conduits in congenital heart surgery with two- and three-dimensional magnetic resonance imaging.

BACKGROUND: Intracardiac baffles and extracardiac conduits have been used in the reconstructive surgery of a broad spectrum of congenital cardiac malformations. Periodic evaluation of these structures may not lend itself readily to echocardiographic and angiographic imaging. The purpose of the study was to describe the experience of our institution with the use of magnetic resonance imaging (MRI) in evaluating conduits and baffles and to describe the simplified approach we developed to image these structures, which allows for grouping individual lesions into broad categories. METHODS AND RESULTS: We retrospectively reviewed our MRI experience in visualizing these structures from 1989-1996. One hundred thirty-nine patients underwent MRI to visualize 144 structures (116 baffles, 28 conduits). The 116 baffles included 86 Fontan, 16 Mustard, 6 Senning, 6 left ventricle to aorta, 1 right ventricle to aorta, and 1 pulmonary vein to left atrium baffle. The 28 conduits included 15 right ventricle to pulmonary artery, 4 left ventricular apical to aorta, 2 left ventricle to pulmonary artery, 3 aorta to aorta, 2 inferior vena cava to left atrium conduits, and 2 aortic root replacements. Of the 3 aortic-aortic conduits, 1 was in conjoined twins. Both inferior vena cava-left atrial conduits were in a Baffes procedure. An infectious mass missed by echocardiography in a right ventricle to pulmonary artery conduit was visualized by MRI. With multiplanar reconstruction, contiguous images were stacked atop each other and resliced to define the salient points of the anatomy. Three-dimensional reconstruction further added to this delineation. All structures were visualized successfully, and an assessment of obstruction was made. Multiple examples of conduit and baffle narrowing were diagnosed by spin echo and cine MRI and were subsequently confirmed by catheterization and surgical inspection. CONCLUSION: MRI, with multiplanar and 3-dimensional reconstruction, is useful in examining the variety of baffles and conduits used in congenital heart surgery. MRI can add to the care of patients whose echocardiographic windows or whose angiographically overlapping structures do not allow adequate delineation of conduits and baffles.

Therapeutic assessment of adult patients with isolated corrected transposition of the great arteries.

Corrected transposition of the great arteries is a rare congenital heart disease, affecting 1% of children with cardiac malformation. Patients with transposition of the great arteries and without associated cardiovascular anomalies are very infrequent and may remain undiagnosed until adult life, because they usually are asymptomatic until the fourth or fifth decades. At this time, most symptoms occur in close connection with deterioration in systemic (right) ventricle performance and with an increase in left atrial pressure. In this report, we describe two new adult cases of isolated, corrected transposition of the great arteries, offering several considerations on their clinical profile and therapeutic assessment.

[Assessment with magnetic resonance tomography of anatomy and ventricular function after Mustard correction of transposition of the great arteries]. / Magnetresonanztomographische Beurteilung von Anatomie und Ventrikelfunktion nach Mustard-Korrektur bei Transposition der grossen Arterien.

In order to evaluate postoperative sequelae and ventricular function after Mustard-operation in patients with transposition of the great arteries (TGA), 30 patients were assessed by magnetic resonance imaging in EKG-triggered spin-echo (SE) and gradient-echo (GE) technique. Twenty-three patients, aged 4.7 to 15.8 years, had transposition of the great arteries with intact ventricular septum with or without left-ventricular outflow tract obstruction (TGA+IVS +/- SPS). Seven patients aged 9.5 to 21.7 years had transposition of the great arteries with ventricular septal defect (TGA+VSD). Five patients showed a residual baffle leak, one had a pulmonary venous obstruction, five an obstruction at the caval veins, 13 a left-ventricular outflow tract obstruction, and 14 a tricuspid regurgitation. Right-ventricular enddiastolic volume in patients with TGA+VSD (77.0 +/- 25.5 ml/m2) was significantly higher than in patients with TGA+IVS +/- SPS (61.2 +/- 12.0 ml/m2). In TGA+VSD right-ventricular ejection fraction (47.6 +/- 13.0%) was significantly lower than in patients with TGA+IVS +/- SPS (56.7 +/- 10.7%). The ratio of muscle masses of right to left ventricle was 1.8:1 in patients with TGA+IVS +/- SPS and 2.5:1 in patients with TGA+VSD. In conclusion, after Mustard-operation in patients with transposition of the great arteries (TGA) magnetic resonance imaging provides a comprehensive and noninvasive assessment of postoperative sequelae, residuae, and ventricular function and will, therefore, become the method of choice for postoperative evaluation.

Assessment of bedside umbilical vein balloon septostomy using two-dimensional echocardiographic guidance in transposition of great arteries.

The effectiveness of "bedside" balloon atrial septostomy via the umbilical vein using 2-dimensional echocardiography was compared to the traditional femoral vein approach using fluoroscopy in a series of neonates with transposition of great arteries from March, 1984 to April, 1987. There were 7 neonates who had balloon septostomy performed at the "bedside" (Group I) compared to 13 who had the procedure performed in the catheterization laboratory (Group II). Group II consisted of 7 newborns who had elective femoral vein catheterization under fluoroscopy (Group IIA) and 6 who failed "bedside" umbilical vein balloon septostomy and subsequently had the femoral vein approach under fluoroscopy (Group IIB). Results showed that adequacy of balloon septostomy was not related to the approach used, with 4 of 7 in Group I and 9 of 13 in Group II with an adequate atrial tear and clinical response. The Delay time to septostomy (i.e. time elapsed from initial assessment to commencement of balloon septostomy) and Procedure time (i.e. time taken to complete the balloon septostomy) was significantly shorter for Group I (mean time = 0.7 hours and 0.26 hours respectively) compared with Group IIA (mean time = 2.6 hours and 1.8 hours) and Group IIB (mean time = 2.4 hours and 1.4 hours). Of note, there was no significant increase in Delay time between Group IIA and IIB.(ABSTRACT TRUNCATED AT 250 WORDS)

Assessment of right ventricular performance by pulsed Doppler echocardiography in patients after intraatrial repair of aortopulmonary transposition in infancy or childhood.

The purpose of this study was to determine whether measurement of aortic blood flow velocity and acceleration by pulsed Doppler ultrasound can be used to assess the global performance of the systemic right ventricle noninvasively in young patients who have undergone intraatrial repair of aortopulmonary transposition. The effect of age at surgery on right ventricular performance in these patients was also studied. Pulsed Doppler velocity profiles of aortic blood flow were analyzed at a mean age of 5.5 years in 24 patients who had undergone intraatrial repair of aortopulmonary transposition either in early infancy (mean age 3.4 +/- 1.3 weeks) or later (mean age 8.5 +/- 6.5 months). Velocity and acceleration variables in these patients were compared with the same variables in 24 age-matched normal subjects and with the performance of their own right ventricle as assessed by two-dimensional echocardiographic measurement of ejection fraction. The 12 patients who underwent early repair had a higher ejection fraction than did the 12 who underwent later repair (mean +/- SD 0.60 +/- 0.07 versus 0.42 +/- 0.10; p less than 0.001). Aortic flow velocity was similar in all patients. Aortic acceleration was normal in patients after early intraatrial repair of aortopulmonary transposition (20.8 +/- 2.3 m/s2), but was abnormally slow in patients after late repair (11.1 +/- 1.8 m/s2; p less than 0.001), thus suggesting that the latter group had diminished right ventricular performance. Concomitantly, acceleration time and ratio of acceleration time to ejection time were increased in patients after late repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrioventricular and ventriculoarterial discordance (corrected transposition of the great arteries). Wide-angle two-dimensional echocardiographic assessment of ventricular morphology.

Wide-angle two-dimensional echocardiography was used to establish criteria for recognition of ventricular situs and ventricular morphology in 27 patients (ages 2 month to 58 years) with atrioventricular amd ventriculoarterial discordance (corrected transposition of the great arteries). The morphologic left ventricle was recognized by the presence of an atrioventricular valve that was attached more superiorly along the ventricular septum than the other atrioventricular valve, that had a fish-mouth (bicommissural) appearance in diastole, and that had two discrete papillary muscles; by a ventricle with a finely trabecular pattern and ellipsoid shape; and by the presence of direct continuity between the atrioventricular and semilunar valves. The morphologic right ventricle was recognized by the presence of an atrioventricular valve that was attached more inferiorly along the ventricular septum, that had a triangular (tricommissural) appearance, and that had multiple irregular papillary muscles; by a ventricle with a coarsely trabecular pattern and a triangular or crescentic shape, and by the presence of muscular atrioventricular-semilunar valve discontinuity. Using these criteria, ventricular situs and morphology could be reliably determined by noninvasive two-dimensional echocardiography. In addition, two-dimensional echocardiography allowed the recognition of frequently associated anomalies. In this series, two-dimensional echocardiography demonstrated Ebsteins's malformation of the left atrioventricular valve in four patients and a straddling atrioventricular valve in eight (one right-sided, seven left-sided.

Ultrasonic assessment of transposition of the great vessels.

Echocardiography can provide important diagnostic information that identifies the transposition complexes, evaluates relative pressure levels in the pulmonic and systemic circuits, describes subpulmonic obstruction, and identifies some associated congenital anomalies. Following the Mustard procedure, baffle dysfunction may be recognized, and the function of the procine heterograft used in the Rastelli procedure studied. Echocardiography can also demonstrate other congenital cardiac defects that may mimic transposition of the great vessels or reveal normal cardiac and great vessel anatomy and function that can reliably rule out the transposition complexes.