Territorial Post-Revascularization Remodeling of Chronic Coronary Syndromes: Cardiac Magnetic Resonance Feature Tracking Study.

BACKGROUND: MRI feature-tracking (MRI-FT) can accurately assess ventricular myocardial deformation and regional function and may be a better predictor of mortality than ejection fraction and infarct extension. However, role of MRI-FT in assessing coronary revascularization is unclear. PURPOSE: To assess coronary revascularization effect on territorial left ventricle (LV) function of chronic coronary syndrome (CCS) patients by MRI-FT. STUDY TYPE: Prospective. SUBJECTS: 50 CCS patients (age: 62.22 ± 8.70 years) scheduled for elective percutaneous coronary intervention (PCI), and 30 healthy controls (age: 35.33 ± 11.57 years). FIELD STRENGTH/SEQUENCE: 1.5T with balanced steady-state free precession cine sequence. ASSESSMENT: Global and segmental peak systolic longitudinal, circumferential, and radial myocardial strains were quantified in both patient and healthy control groups by an experienced operator using dedicated software. Patients were studied both pre-PCI and 6-month post-PCI and LV territorial myocardial strain values were calculated by averaging the segmental values of each revascularized territory. STATISTICAL TESTS: Student's t-test, paired t-test, Mann Whitney test, and Wilcoxon signed ranks test. Significance was judged at the 5% level. RESULTS: Territorial longitudinal strain showed significant 6-month post-PCI improvement in the left anterior descending (LAD) and right coronary artery (RCA) territories, but there was not in the left circumflex (LCX) territory (LAD: mean - 11.41% ± 3.45% pre, -13.01% ± 3.53% post; RCA: mean - 11.11% ± 2.65% pre, -13.25% ± 2.81% post; and LCX: mean - 15.43% ± 3.97% pre, -16.17% ± 4.38% post, P = 0.215). Territorial circumferential strain showed significant post-PCI improvement in all revascularized territories (LAD: mean - 13.73% ± 6.56% pre, -16.98% ± 6.01% post; LCX: mean - 13.23% ± 4.23% pre, -16.34% ± 3.45% post; and RCA: mean - 11.24% ± 3.36% pre, -13.80% ± 3.51% post). Territorial radial strain showed no significant post-PCI improvement (LAD: mean 22.73% ± 12.38% pre, 21.79% ± 11.55% post, P = 0.541; LCX: mean 27.73% ± 7.95% pre, 29.0% ± 7.25% post, P = 0.264; and RCA: mean 36.68% ± 11.10% pre, 31.75% ± 10.95% post, P = 0.208). DATA CONCLUSION: Territorial LV systolic function was significantly improved by coronary revascularization in CCS patients. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 4.

Systematic large-scale assessment of the genetic architecture of left ventricular noncompaction reveals diverse etiologies.

PURPOSE: To characterize the genetic architecture of left ventricular noncompaction (LVNC) and investigate the extent to which it may represent a distinct pathology or a secondary phenotype associated with other cardiac diseases. METHODS: We performed rare variant association analysis with 840 LVNC cases and 125,748 gnomAD population controls, and compared results to similar analyses on dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). RESULTS: We observed substantial genetic overlap indicating that LVNC often represents a phenotypic variation of DCM or HCM. In contrast, truncating variants in MYH7, ACTN2, and PRDM16 were uniquely associated with LVNC and may reflect a distinct LVNC etiology. In particular, MYH7 truncating variants (MYH7tv), generally considered nonpathogenic for cardiomyopathies, were 20-fold enriched in LVNC cases over controls. MYH7tv heterozygotes identified in the UK Biobank and healthy volunteer cohorts also displayed significantly greater noncompaction compared with matched controls. RYR2 exon deletions and HCN4 transmembrane variants were also enriched in LVNC, supporting prior reports of association with arrhythmogenic LVNC phenotypes. CONCLUSION: LVNC is characterized by substantial genetic overlap with DCM/HCM but is also associated with distinct noncompaction and arrhythmia etiologies. These results will enable enhanced application of LVNC genetic testing and help to distinguish pathological from physiological noncompaction.

Multiparametric Rest and Dobutamine Stress Magnetic Resonance in Assessment of Myocardial Viability.

BACKGROUND: MR feature-tracking (FT) is a novel technique that quantitatively calculates myocardial strain and can assess myocardial viability. PURPOSE: To evaluate the feasibility of FT at rest and with low-dose dobutamine (LDD), visual assessment of contractility with LDD and left ventricle (LV) end-diastolic wall thickness (EDWT) in the assessment of viability in ischemic cardiomyopathy (ICM) patients compared to delayed gadolinium enhancement (DGE). STUDY TYPE: Prospective. SUBJECTS: Thirty ICM patients and 30 healthy volunteers. FIELD STRENGTH/SEQUENCES: A 1.5 T with balanced steady-state free precession (bSSFP) cine and phase-sensitive inversion prepared segmented gradient echo sequences. ASSESSMENT: LDD (5 µg/kg/min and 10 µg/kg/min) was administered in the patient group. LV was divided into 16 segments and MR-FT was derived from bSSFP cine images using dedicated software. Viable segments were defined as those with a dobutamine-induced increase in resting MR-FT values >20%, a dobutamine-induced increase in systolic wall thickening ≥2 mm by visual assessment, ≤50% fibrosis on DGE, and resting EDWT ≥5.5 mm. STATISTICAL TESTS: One-way analysis of variance (ANOVA), two-sampled t-test, paired samples t-test, and receiver operating characteristic (ROC) curve analysis. A P value < 0.05 was considered statistically significant. RESULTS: Resting peak global circumferential (Ecc) and radial (Err) strains were significantly impaired in patients compared to controls (-11.7 ± 7.9 vs. -20.1 ± 5.7 and 19.7 ± 13.9 vs. 32.7 ± 15.4, respectively). Segments with no DGE (n = 354) and ≤ 50% (n = 38) DGE showed significant improvement of both Ecc and Err with LDD while segments with >50% DGE (n = 88) showed no improvement. In comparison to viable and nonviable segments identified by reference-standard DGE, the sensitivity, specificity, and diagnostic accuracy of the four methods were: 74%, 92%, and 89%, respectively, for Ecc; 70%, 89%, and 86%, respectively, for Err; 67%, 88%, and 84% for visual assessment; and 39%, 90%, and 80% for EDWT. DATA CONCLUSION: Quantitative assessment of MR-FT, along with EDWT and qualitative visual assessment of myocardial contractility with LDD, are feasible alternative methods for the assessment of myocardial viability with moderate sensitivity and high specificity. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage: 2.

Abnormal drainage of inferior vena cava to left atrium together with a partial abnormal pulmonary venous drainage to right atrium in the presence of atrial septal defect.

The direct abnormal drainage of the inferior vena cava (IVC), while rare, is well-recognized anomaly of systemic venous drainage. It has been reported both in isolation and in association with other cardiac defects. This is a case of an abnormal drainage of IVC into left atrium (LA) together with partial abnormal pulmonary venous drainage to the right atrium (RA) and atrium septal defect (ASD).

Giant left atrial appendage aneurysm compressing the left anterior descending coronary artery.

Left atrial appendage aneurysm (LAAA) is a rare congenital structural heart disease. It is often diagnosed by echocardiography; however, other imaging modalities can add to its diagnosis and its potential effects on the surrounding structures. A 16-year-old boy presented with dyspnea and palpitation. Transthoracic echocardiography showed a large LAAA communicating with the LA through a narrow neck with impaired left ventricular (LV) systolic function. Multidetector cardiac tomography showed that the LAAA is compressing the left anterior descending artery. The LAAA was surgically resected followed by improvement of the LV systolic function.

Utility of 4D Flow mapping in Eisenmenger syndrome with pulmonary atresia.

Management of patients with Eisenmenger syndrome with pulmonary atresia is challenging because of the complexity of the structure-function relationship of the components of the syndrome. Multi-modality imaging including cardiac magnetic resonance (CMR) 4D Flow offers unprecedented opportunities to unravel, at least in part, some of these components, and thus help in the management of these patients. In this study, we describe the use of these integrated methods with particular reference to CMR 4D Flow in a patient with Eisenmenger syndrome and pulmonary atresia and outline both the utility and the limitations. A comprehensive cardiac magnetic resonance (CMR) 4D Flow analysis was performed preoperatively and postoperatively, during peak systole, late systole, early diastole, and late diastole. The focus of the present study was to investigate the pattern of flow and dynamic changes at different levels of the aorta, as well as in the duct and the pulmonary arteries. Preoperatively, a right-handed helix and a vortex were observed in the dilated ascending aorta, and the duct flow was mainly dependent on reverse, upstream flow from the descending aorta, distal to the duct, during diastole, denoting low pulmonary vascular capacitance. Following repair, the flow in the ascending aorta and the descending aorta changed markedly. These changes included both timing and intensity of the right-handed helix, as well as the vortex in the ascending aorta. The significance of the observed changes in flow pattern and their influence on wall structure and function are discussed. Our study demonstrates the extremely powerful potential of CMR 4D Flow in the management of complex congenital anomalies.

Uhl's Anomaly in Adulthood.

Uhl's anomaly is a rare congenital syndrome characterized by the absence of right ventricular myocardium. The widely accepted pathological mechanism is intrauterine myocardial apoptosis. Uhl's syndrome carries a poor prognosis. In rare situations, patients with Uhl's anomaly reach adulthood. We will present a case of a 29-year-old patient with Uhl's syndrome treated at our center, highlighting the diagnostic, surgical, and postoperative challenges in management.

Valvulogenesis of a living, innervated pulmonary root induced by an acellular scaffold.

Heart valve disease is a major cause of mortality and morbidity worldwide with no effective medical therapy and no ideal valve substitute emulating the extremely sophisticated functions of a living heart valve. These functions influence survival and quality of life. This has stimulated extensive attempts at tissue engineering "living" heart valves. These attempts utilised combinations of allogeneic/ autologous cells and biological scaffolds with practical, regulatory, and ethical issues. In situ regeneration depends on scaffolds that attract, house and instruct cells and promote connective tissue formation. We describe a surgical, tissue-engineered, anatomically precise, novel off-the-shelf, acellular, synthetic scaffold inducing a rapid process of morphogenesis involving relevant cell types, extracellular matrix, regulatory elements including nerves and humoral components. This process relies on specific material characteristics, design and "morphodynamism".

Novel concepts and early results of repairing common arterial trunk.

OBJECTIVES: Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the correction of all components of the anomaly, using autologous arterial tissue. This aims to enhance results, availability worldwide, and importantly to avoid the need for repeated reoperations. METHODS: From January 2019 to 4 January 2021, all patients with isolated CAT had repair of the defect using autologous arterial trunk tissue with direct right ventricle (RV) to pulmonary artery (PA) connection. Clinical outcomes, follow-up which included multi-slice computed tomography 3D segmentation and 4D cardiovascular magnetic resonance flow, are presented. RESULTS: Twenty patients were included in the study (median age 4.5 months). There were 2 hospital deaths due to systemic infection and pulmonary hypertensive crisis, respectively. Following discharge all patients remained asymptomatic with no signs of heart failure and improved pattern of growth (median follow-up: 8 months). Early postoperative 3D segmentation showed a conical shaped neo-right ventricular outflow chamber connecting the body of the RV to the main PA through a valveless ostium, and normal crossing of PA and neo-aorta. 4D cardiovascular magnetic resonance pattern of flow showed normal rapid laminar flow through the atrioventricular valves followed by a vortex towards the outflow tracts. There was laminar flow through the neo-aorta and neo-PA with velocity not exceeding 2.5 m/s. The PA regurgitant fraction was 25 ± 5% and was limited to early diastole. CONCLUSIONS: The initial results of utilizing the key concepts, using autologous arterial tissue for the repair of CAT, are encouraging, both clinically and by multimodality imaging.

Pulmonary flow profile and distensibility following acute pulmonary embolism.

OBJECTIVE: Proof of concept study evaluating CMR as screening tool for chronic thromboembolic pulmonary hypertension (CTEPH) in patients treated for acute pulmonary embolism (PE). MATERIALS AND METHODS: Right and left ventricular function of 15 consecutive patients treated for PE and 10 consecutive patients in whom PE was excluded was estimated at baseline by cardiac CT and at 6 months follow-up by CMR. Additionally, during the follow-up visit, pulmonary artery (PA) hemodynamics were studied by CMR and the presence of pulmonary hypertension by echocardiography. RESULTS: CT measured right ventricular ejection fraction (RVEF) was lower in patients with PE compared to patients without PE at time of diagnosis (median 47%, interquartile range 39-53 vs. 55%, 52-58; p = 0.014). After 6 months follow up, the RVEF between patients treated for PE and patients without PE were not statistically significant different (55%, 52-60 versus 54%, 51-57; p = 0.57), as were distensibility index (0.18 ± 0.18 versus 0.25 ± 0.18, p = 0.20), mean velocity (14.1 ± 3.9 cm/s versus 14.0 ± 2.5 cm/s, p = 0.81), peak velocity (86.5 ± 22 cm/s versus 89.6 ± 13 cm/s, p = 0.43) and time to peak PA blood flow velocity (142 ± 49 ms versus 161 ± 29 ms, p = 0.14). One patient was diagnosed with CTEPH and CMR revealed poor right systolic function, decreased PA distensibility and flow velocity, and a systolic notch in the PA flow profile consistent with persistent PA obstruction. CONCLUSION: In this small series, right ventricular performance and PA flow profiles of patients treated for 6 months after PE are equivalent to those parameters in normal patients.

Left Ventricular Remodeling Following Balloon Mitral Valvuloplasty in Rheumatic Mitral Stenosis: Magnetic Resonance Imaging Study.

Background: Rheumatic heart disease affects primarily cardiac valves, it could involve the myocardium either primarily or secondary to heart valve affection. The influence of balloon mitral valvuloplasty (BMV) on left ventricular function has not been sufficiently studied. Aim: To determine the influence of balloon mitral valvuloplasty (BMV) on both global and regional left ventricular (LV) function. Methods: Thirty patients with isolated rheumatic mitral stenosis (MS) were studied. All patients had cardiac magnetic resonance imaging (CMR) before, 6 months and 1 year after successful BMV. LV volumes, ejection fraction (EF), regional and global LV deformation, and LV late gadolinium enhancement were evaluated. Results: At baseline, patients had median EF of 57 (range: 45-69) %, LVEDVI of 74 (44-111) ml/m2 and LVESVI of 31 (14-57) ml/m2 with absence of late gadolinium enhancement in all myocardial segments. Six months following BMV, there was a significant increase in LV peak systolic global longitudinal strain (GLS) (-16.4 vs. -13.8, p < 0.001) and global circumferential strain (GCS) (-17.8 vs. -15.6, p = 0.002). At 1 year, there was a trend towards decrease in LVESVI (29 ml/m2, p = 0.079) with a significant increase in LV EF (62%, p < 0.001). A further significant increase, compared to 6 months follow up studies, was noticed in GLS (-17.9 vs. -16.4, p = 0.008) and GCS (-19.4 vs. -17.8 p = 0.03). Conclusions: Successful BMV is associated with improvement in global and regional LV systolic strain which continues for up to 1 year after the procedure.

A quantitative assessment of aorta root rotation in patients with tetralogy of Fallot evaluated by MSCT.

Lack of conal rotation and conal malseptation is a characteristic anatomical feature for TOF which lead to dextroposed position of aorta and significant RVOT narrowing. The quantitative assessment of these anatomical features using modern cardiac imaging modality has been rarely discussed in the literature. All TOF scanned had in our center from 2013 till 2019 were included. The angle of aortic root rotation was recorded by measuring the angle between a line connecting the midpoint of the non-coronary sinus to the anterior commissure and another line along the interatrial septum. Rotation angles were correlated with proximal main pulmonary artery (MPA) size indexed to BSA. 287 TOF patients were included, 258 patients (91%) had TOF with pulmonary stenosis (TOF-PS) including 138 male (54%), median age 2 years (2 months-40 years), and 29 patients (9%) had TOF with pulmonary atresia (TOF-PA) including 17 male (59%), median age 5 years (1 m-33 years). The whole cases demonstrated clockwise rotation of the aortic root. The mean rotation angle in TOF-PS group was 52.6 ± 20.9° and in TOF-PA group was 64.9 ± 13.9°. Proximal MPA diameter was 11.1 ± 5.9 mm/m2. There was a significant negative correlation between aortic root rotation angle and proximal MPA diameter (r = - 0.262, P = 0.000). The rotation angle of aortic root was significantly higher in TOF-PA compared to TOF-PS (64.9 ± 13.9° vs. 52.6 ± 20.9°, P = 0.001, respectively). MSCT provide a quantitative measurement methodology of conal malseptation and its effect in TOF patients. There is a clockwise rotation angle of the aortic root in TOF patients that correlates negatively with proximal MPA size. TOF-PA have a larger rotation angle of aortic root.

Coronary artery anomalies in tetralogy of Fallot patients evaluated by multi slice computed tomography; myocardial bridge is not a rare finding.

ABSTRACT: Based on coronary angiography and interoperative inspection, anomalous origin of coronary artery crossing the right ventricular outflow tract (RVOT) is common in tetralogy of Fallot (TOF) patients. However, other coronary anomalies may be underestimated due to the overlying myocardium, epicardial fat, or adhesions due to previous palliative surgery. Currently, coronary artery visibility dramatically improved by multislice computed tomography (MSCT). We performed this study to assess the coronary arteries anatomy in TOF patients using MSCT.All TOF patients underwent MSCT examination at our centre from 2013 till 2019 were included. Assessment of the coronary arteries' origin and course were performed. Presence of myocardial bridge were assessed, and indexed RV mass was calculated.318 TOF patients were included, median age 2 years (range 1 month-46 years), 175 males (55%). The abnormal coronary artery origin and course were detected in 20 patients (6%); coronary artery crossed RVOT in 13 patients (65%), 5 patients (25%) had a retro-aortic course and 2 patient (10%) had inter-arterial course. Myocardial bridges of left anterior descending artery or/and right coronary artery were reported in 100 patients (36%), no myocardial bridge of left circumflex was reported. RV mass was 29.0 ±â€Š21.1 g/m2. There was no correlation between RV mass and presence of myocardial bridges.MSCT is a useful imaging modality for detection of coronary arteries anomalies in TOF patients. Coronary artery crossing RVOT is not the only abnormal course and myocardial bridging is not a rare finding. Further studies are needed to demonstrate the clinical significance of these observations.

A New Technique for Shaping the Aortic Sinuses and Conserving Dynamism in the Remodeling Operation.

BACKGROUND: Preserving dynamism and recreating the sinuses in the Dacron graft are thought to be important for optimizing results of aortic valve-conserving operations. METHODS: We describe a novel technique that preserves dynamism and recreates the sinotubular junction. In addition, it tailors 3 sinuses of defined longitudinal and transverse curvatures in a straight Dacron tube during the operation. The technique has been used in 6 patients with varied aortic root pathology. We performed preoperative and postoperative multimodality imaging using computerized image analysis as well as 3-dimensional models. RESULTS: There was no early or midterm death. Upon discharge, patients were clinically well, with echocardiographic evidence of minimal (3 patients) or mild (3 patients) aortic regurgitation. Computed tomography and cardiac magnetic resonance imaging with extensive image analysis of the aortic root size, shape, and function showed partial or complete normalization of these parameters. This included the shape and dynamism of the aortic annulus and the size and shape of the geometric (effective) orifice. The 4-dimensional magnetic resonance imaging pattern of flow in the sinuses and ascending aorta showed favorable vortices in the sinuses, right-handed helical flow, and marked diminution of energy loss in the ascending aorta. CONCLUSIONS: The novel technique described here is simple, practical, and cost-effective because it uses a widely available straight Dacron tube. The technique does not use rigid internal or external support. The early results are encouraging. Larger series with longer follow-up are required.

Pulmonary artery banding in a modified Mustard operation improves biventricular geometry and function.

Patients with transposition of great arteries, with intact interventricular septum (TGA-IVS) and deconditioned left ventricle, represent a considerable challenge in developing countries. The modified Mustard operation was shown to provide a significant improvement for these patients, particularly by enhancing atrial functions and left ventricular filling. Yet, the problems of the systemic right ventricular dysfunction and the resulting secondary tricuspid regurgitation (TR) remain to be of major concern. In addition, the deviation of the interventricular septum towards the left side markedly impairs ventriculo-ventricular interaction and predisposes to dynamic left ventricular outflow tract obstruction (LVOTO). We report that adding a moderately loose pulmonary artery banding to the modified Mustard operation in a case of TGA-IVS results in improvement of biventricular geometry and function, tricuspid and mitral valve functions and disappearance of dynamic LVOTO.

Multi-detector computed tomography in the assessment of tetralogy of Fallot patients: is it a must?

BACKGROUND: Tetralogy of Fallot (TOF) accounts for 10% of all CHD. It classically consists of ventricular septal defect (VSD), aortic overriding, right ventricular outflow tract (RVOT) obstruction, and RV hypertrophy. There are many anatomic variants, associated intracardiac and extracardiac anomalies that must be taken into consideration when imaging and planning the surgical procedure needed. Multi-detector computed tomography (MDCT), with its high spatial and temporal resolution, has a pivotal role in the evaluation of complex anatomical findings in both unrepaired and repaired TOF patients. MAIN BODY: Though MDCT has a limited role in the initial diagnosis of TOF, it is particularly important when there is a question about anatomy of pulmonary arteries (PAs) (whether sizable, hypoplastic, or atretic), presence of major aorto-pulmonary collaterals (MAPCAs) and presence of additional VSDs. Additionally, MDCT is crucial in the diagnosis of different anatomical variants of TOF. TOF patients with absent pulmonary valve classically have hugely dilated PAs which raise an important question about the degree and severity of airways compression. This question can be accurately answered by MDCT. TOF with double-outlet RV (DORV) has variable degrees of aortic override which can be assessed by MDCT. An atrio-ventricular septal defect (AVSD) is seen in about 13% of TOF cases and typically occurs in patients with Down syndrome. MDCT can assess the size and extent of inlet VSD and size of both ventricles (balanced or unbalanced AVSD). Coronary artery anomalies are common and important association. MDCT can identify the presence of a major coronary artery crossing the RVOT, a left anterior descending (LAD) from RCA, or a dual LAD. The clinical importance of these anomalies is its susceptibility to injury during ventriculotomy incision required for TOF repair necessitating changing the usual approach of surgery. Patients with reduced pulmonary blood flow undergo a systemic to pulmonary shunt. MDCT can assess the patency of the shunt, stenotic, or occluded segments. In surgically repaired TOF patients, MDCT can identify the sequalae and long-term complications including residual RVOT obstruction, conduit stenosis, RVOT patch aneurysm, RVH, and aortic root dilatation. CONCLUSION: MDCT is a safe and reliable imaging modality that provides accurate assessment of anatomical variants and associated anomalies of TOF.

Impact of balloon mitral valvuloplasty on left ventricular rotational deformation: Magnetic Resonance Imaging follow up study.

In patients with rheumatic mitral stenosis (MS), some previous studies have investigated the influence of balloon mitral valvuloplasty (BMV) on left ventricular (LV) systolic function. However, the impact of BMV on LV twisting motion in this clinical setting has not been studied before yet. To describe changes in LV torsion in patients with rheumatic MS following BMV. Thirty patients (median age 33 years, 22 women) with isolated severe MS were studied. CMR myocardial tissue tagging was used for assessment of LV rotational deformation. LV torsion was calculated as the twist value (the net difference between apical counterclockwise and basal clockwise rotation) normalized to the length of the ventricle and multiplied by the mean radius at the base and apex. All patients had CMR studies before, 6 months and 1 year after successful BMV. At baseline, patients had a mitral valve area of 0.9 (0.6-1.3) cm2, mean pressure gradient of 12.5 (8-24) mmHg across the valve as measured by transthoracic echocardiography. Median LV ejection fraction (LVEF) estimated by CMR was 57 (range: 45-69) %. A significant improvement in LV base-apex torsion was shown at 6 months (3.3° vs. 2.5°, p < 0.001) with a further improvement at 1 year (4.1° vs. 3.3°, p = 0.05). Similar pattern of change was seen in LV base-mid torsion with a significant increase at 6 months (3.6° vs. 2.3°, p < 0.001) and a further increase at 1 year (4.7° vs. 3.6°, p = 0.007). These changes were associated with a significant increase in LVEF (62% vs. 57%, p < 0.001) at 1 year following BMV. Successful BMV is associated with a significant improvement in LV torsion that is accompanied by a significant improvement in LVEF.

Revival and modification of the Mustard operation.

OBJECTIVE: The neonatal arterial switch operation is currently the procedure of choice for patients with transposition of the great arteries. However, a large number of patients present too late for the arterial switch operation and are best managed with the atrial switch operation. METHODS: We have used the Mustard operation in its original form or following a new modification designed to enhance the atrial functions and filling of the left ventricle in an attempt to improve long-term results. RESULTS: Between July 2013 and November 2018, a total of 101 patients underwent the Mustard operation, 86 with the new modification. The median age at operation was 16 months (6 months to 27 years). A total of 75 patients (74.3%) were male. Median preoperative oxygen saturation was 71%. There were no early deaths and there were 3 late deaths during a median follow-up period of 24.2 months (all in patients with large ventricular septal defect and established pulmonary vascular disease). At the latest follow-up, all patients were in stable sinus rhythm. There were no baffle leaks. Seven patients had asymptomatic narrowing of the superior baffle, and 1 patient required balloon dilatation. Follow-up is 100% complete and includes computed tomography and magnetic resonance imaging at regular intervals (75 patients to date). Computerized analysis of representative subsets showed enhanced rate and pattern of filling of the left ventricle in the modified operation compared with the classic operation. CONCLUSIONS: The use of the Mustard operation, particularly the modified technique should play an important role in treating late-presenting patients with transposition of the great arteries. Improving the pattern of filling of the left ventricle could enhance the long-term results of the Mustard operation.

Delayed improvement of right ventricular diastolic function and regression of right ventricular mass after percutaneous pulmonary valve implantation in patients with congenital heart disease.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) has been introduced as therapy for right ventricular (RV) to pulmonary artery conduit dysfunction in patients with congenital heart disease. It has been shown that RV systolic function improved early after PPVI. The effects of PPVI on RV diastolic function and RV hypertrophy have not yet been studied. PURPOSE: The objective of this study is to assess early and late changes in systolic and diastolic RV function and RV mass after PPVI. MATERIALS AND METHODS: Fourteen patients underwent PPVI (7 male, median age 15 years). Cardiac magnetic resonance imaging was performed before and at 2 time points after PPVI (at 1 and 16 months). Right ventricular volume and systolic and diastolic function as well as RV mass were assessed. RESULTS: At 1 and 16 months after PPVI, the RV mass decreased from 28.6 +/- 2.1 to 25.6 +/- 2.2 g/m(2) (P = .03) and to 22.3 +/- 2.1 g/m(2) (P = .002). E/A volume ratio increased from 1.91 +/- 0.4 to 2.6 +/- 0.4 (not significant [NS]) and to 3.3 +/- 0.4 (P = .01). E/A peak flow ratio increased from 1.34 +/- 0.14 to 1.48 +/- 0.16 (NS) and to 1.73 +/- 0.14 (P = .04). E-wave deceleration time increased from 142 +/- 25 to 160 +/- 27 milliseconds (NS) and to 211 +/- 26 milliseconds (P = .007). At 1 month, RV end-diastolic volume decreased from 124 +/- 8 to 113 +/- 8 mL (P = .01) and RV ejection fraction increased from 36% +/- 2% to 46% +/- 2% (P = .001) without further improvement at 16 months. CONCLUSION: After PPVI, in contrast to rapid improvement of RV systolic function, the improvement of RV diastolic function is delayed. The reduction of RV mass appears to be the underlying mechanism for improvement of RV diastolic function. Long follow-up for patients with PPVI is recommended.

Characterization of size, shape and pattern of flow in the neo-aorta and pulmonary artery in a patient following an innovative technique of repair for truncus arteriosus.

Background. Truncus arteriosus (TA) caries a very poor prognosis. In the absence of early correction, only 12 percent of patients born with this anomaly survive beyond one year. There is no agreement about the best method of surgical correction of this anomaly. We have devised an innovative valveless technique using autologous arterial tissue to repair TA. Objectives. Characterizing the size, shape and pattern of flow in the neo-aorta and pulmonary artery, in a patient following the new technique. Patient and Methods. Cardiac MRI and multislice CT imaging, followed by offline computerized image analysis was used in a patient aged 3 months, within 3 weeks of operating. Results. The size, shape and topology of the neo-aorta and pulmonary artery, approximated that present in normal hearts. The pattern of flow in the reconstructed vessels was laminar, throughout the cardiac cycle with minor acceleration during systole. The pulmonary regurgitation resulting from the absence of a valve occurred during late diastole, and was well tolerated. The size of the right ventricle diminished considerably following operation, and the right ventricular ejection fraction was supernormal. Conclusion. This early study in one patient provides new unique data of the size, shape, topology and pattern of flow in the neo-aorta and pulmonary artery, which appear to approximate normality. The long-term results of this promising operation need to be studied.