Atrioventricular Area Difference Aids Diastolic Filling in Patients with Repaired Tetralogy of Fallot.

A hydraulic force aids diastolic filling of the left ventricle (LV) and is proportional to the difference in short-axis area between the left ventricle and atrium; the atrioventricular area difference (AVAD). Patients with repaired Tetralogy of Fallot (rToF) and pulmonary regurgitation (PR) have reduced LV filling which could lead to a negative AVAD and a hydraulic force impeding diastolic filling. The aim was to assess AVAD and to determine whether the hydraulic force aids or impedes diastolic filling in patients with rToF and PR, compared to controls. Twelve children with rToF (11.5 [9-13] years), 12 pediatric controls (10.5 [9-13] years), 12 adults with rToF (21.5 [19-27] years) and 12 adult controls (24 [21-29] years) were retrospectively included. Cine short-axis images were acquired using cardiac magnetic resonance imaging. Atrioventricular area difference was calculated as the largest left ventricular short-axis area minus the largest left atrial short-axis area at beginning of diastole and end diastole and indexed to height (AVADi). Children and adults with rToF and PR had higher AVADi (0.3 cm2/m [- 1.3 to 0.8] and - 0.6 [- 1.5 to - 0.2]) at beginning of diastole compared to controls (- 2.7 cm2/m [- 4.9 to - 1.7], p = 0.015) and - 3.3 cm2/m [- 3.8 to - 2.8], p = 0.017). At end diastole AVADi did not differ between patients and controls. Children and adults with rToF and pulmonary regurgitation have an atrioventricular area difference that do not differ from controls and thus a net hydraulic force that contributes to left ventricular diastolic filling, despite a small underfilled left ventricle due to pulmonary regurgitation.

Longitudinal ECG changes in tetralogy of Fallot and association with surgical repair.

Background: ECG abnormalities have been linked to adverse changes in right ventricular (RV) morphology and poor clinical outcomes in repaired Tetralogy of Fallot (rTOF). Our aim was to describe how ECG changes progress in early and intermediate follow-up and whether types of surgical strategy at the time of primary repair affected these changes. Methods: We studied patients with rTOF born 2000-2018 operated at our institution. Seven time points in relation to primary repair, follow-up, and pulmonary valve replacement (PVR) were identified. Patients correct with valve sparing repair (VSR), trans-annular patch (TAP) including with a monocusp valve (TAP + M) and with at least 3 ECGs were included. PQ interval, QRS duration, dispersion, and fragmentation, QTc duration and dispersion, JTc as well as presence of a right bundle branch block (RBBB) were analyzed. Medical records were reviewed for demographic and surgical data. Results: Two hundred nineteen patients with 882 ECGs were analyzed with a median follow-up time of 12.3 years (8.4, 17) with 41 (19%) needing PVR during the study period. QRS duration increased at time of primary repair to discharge from 66 msec (IQR 12) to 129 msec (IQR 27) (p < 0.0001) and at 1- and 6- year follow-up but showed only a modest and temporary decrease after PVR. QTc increased at the time of primary repair as well as prior to PVR. PQ interval showed a small increase at the time of primary repair, was at its highest prior to PVR and decreased with PVR. Type of surgical repair affected mainly QTc and JTc and was consistently longer in the TAP + M group until PVR. In VSR, QTc and JTc were prolonged initially compared to TAP but were similar after 1 year. After PVR, there were no differences in adverse ECG changes between surgical groups. Conclusions: PQ interval and QRS duration best correspond to the assumed volume load whereas the relationship with QTc and JTc is more complex, suggesting that these represent more complex remodeling of the myocardium. Before PVR, QTc and JTc are longer in the TAP + M group which may be due to a longer surgical incision.

Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

BACKGROUND: There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS: Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION: Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

Utility of Fetal Cardiovascular Magnetic Resonance for Prenatal Diagnosis of Complex Congenital Heart Defects.

Importance: Prenatal diagnosis of complex congenital heart defects reduces mortality and morbidity in affected infants. However, fetal echocardiography can be limited by poor acoustic windows, and there is a need for improved diagnostic methods. Objective: To assess the clinical utility of fetal cardiovascular magnetic resonance imaging in cases in which fetal echocardiography could not visualize all relevant anatomy. Design, Setting, and Participants: This cohort study was conducted between January 20, 2017, and June 29, 2020, at Skåne University Hospital (Lund, Sweden), a tertiary center for pediatric cardiology and thoracic surgery. Participants were fetuses referred for fetal cardiovascular magnetic resonance examination by a pediatric cardiologist after an inconclusive echocardiograph. Exposures: Fetal cardiovascular magnetic resonance examination requested by the patient's pediatric cardiologist. Main Outcomes and Measures: Any change in patient management because of diagnostic information gained from fetal cardiovascular magnetic resonance imaging. Results: A total of 31 fetuses underwent cardiovascular magnetic resonance examination at a median gestational age of 36 weeks (range, 31-39 weeks). Overall, fetal cardiovascular magnetic resonance imaging had clinical utility, affecting patient management and/or parental counseling in 26 cases (84%). For aortic arch anatomy including signs of coarctation (20 fetuses), fetal cardiovascular magnetic resonance imaging added diagnostic information in 16 cases (80%). For assessment of univentricular vs biventricular outcome in borderline left ventricle, unbalanced atrioventricular septal defect, and pulmonary atresia with intact ventricular septum (15 fetuses), fetal cardiovascular magnetic resonance imaging visualized intracardiac anatomy and ventricular function, allowing assessment of outcome in 13 cases (87%). In 4 fetuses with hypoplastic left heart syndrome, fetal cardiovascular magnetic resonance imaging helped delivery planning in 3 cases (75%). Finally, fetal cardiovascular magnetic resonance imaging provided valuable information for parental counseling in 21 cases (68%). Conclusions and Relevance: In this cohort study, fetal cardiovascular magnetic resonance imaging added clinically useful information to what was available from echocardiography. These findings suggest that fetal CMR has the potential to affect clinical decision-making in challenging cases of congenital heart defects with inconclusive data from echocardiography. Fetal cardiovascular magnetic resonance imaging showed an association with clinical decision-making, including mode of delivery and early postnatal care, as well as with parental counseling.

[High prenatal detection rates of complex congenital heart defects (CHD)]. / Fler medfödda hjärtfel upptäcks med utökad screening i tre steg.

AIM: To analyze prenatal detection rates of complex CHD after the implementation of an expanded three-tiered screening model at the Skane University Hospitals in Lund and Malmö in 2015.  Methods: Retrospective review of pregnancies screened from January 1, 2015 and being born by June 30, 2018. Complex CHD was defined as needing intervention in the first year of life.  Results: In 27675 screened pregnancies, 51 out of 65 (78 %) cases of complex CHD were detected prenatally. Exclusion of isolated ventricular septal defects yielded detection rates of 93 %. All patients needing surgery within 30 days, potential univentricular hearts and D-transposition of the great arteries were identified, whilst detection rates for tetralogy of Fallot, atrioventricular septal defect and coarctation were about 90 %. CONCLUSION: Our three-tiered model results in high detection rates of complex CHD with optimized resource utilization.

Fetal iGRASP cine CMR assisting in prenatal diagnosis of complicated cardiac malformation with impact on delivery planning.

Limited visualization of the fetal heart and vessels by fetal ultrasound due to suboptimal fetal position, patient habitus and skeletal calcification may lead to missed diagnosis, overdiagnosis and parental uncertainty. Counselling and delivery planning may in those cases also be tentative. The recent fetal cardiac magnetic resonance (CMR) reconstruction method utilizing tiny golden-angle iGRASP (iterative Golden-angle RAdial Sparse Parallel MRI) allows for cine imaging of the fetal heart for use in clinical practice. This case describes an unbalanced common atrioventricular canal where limited ultrasound image quality and visibility of the aortic arch precluded confirming or ruling out presence of a ventricular septal defect. Need of prostaglandins or neonatal intervention was thus uncertain. Cardiovascular magnetic resonance imaging confirmed ultrasound findings and added value by ruling out a significant ventricular septal defect and diagnosing arch hypoplasia. This confirmed the need of patient relocation for delivery at a paediatric cardiothoracic surgery centre and prostaglandins could be initiated before the standard postnatal ultrasound. The applied CMR method can thus improve diagnosis of complicated fetal cardiac malformation and has direct clinical impact.

Longitudinal changes in adolescents with TOF: implications for care.

BACKGROUND: We sought to identify predictors of change in right ventricular function and exercise capacity in adolescents following repair for tetralogy of Fallot. METHODS AND RESULTS: We performed a longitudinal study with serial cardiac magnetic resonance imaging and/or exercise stress tests. Patients with interim intervention on the pulmonary valve were excluded. Paired t-test was used to detect longitudinal changes and multivariable regression models were built to identify predictors of change. Initial and follow up magnetic resonance and exercise stress test studies were available for 65 and 63 subjects, respectively. Age at initial testing was 11.7 ± 2.7 years. Average follow up time was 4.5 ± 1.8 (magnetic resonance) and 4.0 ± 1.6 (exercise test) years. There was a significant increase in right ventricular end diastolic and systolic volume (119 ± 34 to 128 ± 35 ml/m2, P = 0.006; 49 ± 20 to 56 ± 23 ml/m2, P = 0.001, respectively), and a decrease in right ventricular ejection fraction (60 ± 7 to 56 ± 8%, P = 0.001), with no significant change in pulmonary regurgitant fraction or right ventricular cardiac index. Predictors of right ventricular dilation over time included: time elapsed from surgical repair, severity of pulmonary insufficiency and right ventricular dilation at the initial magnetic resonance imaging. Of those, time elapsed from surgical repair had the most significant effect. There was no change in exercise capacity. DISCUSSION: In the adolescent with tetralogy of Fallot, longer time from surgery, more pulmonary insufficiency and greater right ventricular dilation at initial magnetic resonance imaging are associated with progressive right ventricular dilation. These results suggest early monitoring with magnetic resonance imaging might identify those at highest risk for progressive disease.

Longitudinal Validation of the Diastolic to Systolic Time-Velocity Integral Ratio as a Doppler-Derived Measure of Pulmonary Regurgitation in Patients with Repaired Tetralogy of Fallot.

Pulmonary regurgitation (PR) is a common residual lesion and major determinant of outcome following surgical repair for tetralogy of Fallot. We sought to longitudinally study a previously described echocardiographic index as a correlate of PR measured by cardiac magnetic resonance imaging (CMR). We conducted a retrospective longitudinal study of patients with baseline and follow-up echocardiogram and CMR. The baseline studies were obtained as part of a research protocol, while the follow-up studies were performed for clinical purposes. On echocardiogram, the ratio of diastolic and systolic time-velocity integrals (DSTVI) in the main pulmonary artery was calculated. The Wilcoxon matched-pairs signed-rank test was used to test for individual changes in PR on echocardiogram and CMR. A linear regression of pulmonary valve regurgitant fraction (RF) was fit on DSTVI to identify clinically meaningful cut points of DSTVI. Thirty-five subjects were included, age at follow-up 18.3 ± 3.5 years. The follow-up between consecutive CMRs was a median time of 60 months (interquartile range 46-73). There was a moderate correlation between DSTVI and PR measured as RF by CMR (r = 0.62, p = 0.0001). A CMR RF of 20 and 40 % (the boundaries between mild/moderate and moderate/severe PR) corresponded with DSTVI of 0.52 and 0.79 (95 % CI 0.39; 0.66, and 0.69; 89), respectively. There was no significant change in either DSTVI (p = 0.61) or PR (p = 0.89) from baseline to follow-up. This study lends further credence to the DSTVI as an accurate reflection of PR. This index might become helpful in the routine echocardiographic assessment of PR. Further studies are needed to determine whether changes in RF by CMR result in changes in DSTVI.

The modified Ross operation using a Dacron prosthetic vascular jacket does prevent pulmonary autograft dilatation at 4.5-year follow-up.

OBJECTIVE: Following the Ross operation, pulmonary autografts tend to dilate over time. This study researches the fate of the pulmonary autograft - at 4.5 years following the modified Ross operation - with special reference to the impact of the modification on (a) pulmonary autograft dilatation, (b) the neo-aortic root geometry, (c) neo-aortic valve function and (d) the coronary artery reserve. METHODS: A total of 26 patients who underwent the Ross operation were included in this study; of these, 13 consecutive patients underwent a modified Ross operation in which the free-standing autograft root was supported externally by a Dacron vascular prosthetic jacket (DVPJ). These patients were compared to a cohort of 13 matched patients who were operated on using the conventional Ross technique; all patients were followed up prospectively by echocardiography studies. The patients who underwent the modified Ross operation were also subjected to bicycle ergometry. RESULTS: At the 47-month median follow-up, there was no significant increase in the size of the entire neo-aortic root in the patients who underwent the modified Ross operation; in addition, the geometry of the neo-aortic root was also preserved and the left ventricular function had improved significantly, whilst the aortic valve function and excursion remained satisfactory. All patients, with one exception, in the modified Ross operation group exhibited normal exercise capacity. By contrast, there were significant differences in diameters of the aortic root - between the two surgical techniques in favour of the modified Ross technique - following a median follow-up of 23 months in the patients subjected to the conventional Ross operation. CONCLUSIONS: Provision of external support to the entire pulmonary autograft with a DVPJ prevents its dilatation following free-standing pulmonary autograft Ross operation when evaluated at the 4.5-year follow-up. The function and the geometry of the neo-aortic root are not affected negatively by this modification and the patients demonstrated normal exercise capacity.

Acute plateletpheresis and aprotinin reduces the need for blood transfusion following Ross operation.

The effect of acute intraoperative plateletpheresis (25% platelet yield) in combination with intraoperative low-dose aprotinin (2 million units) on blood conservation was investigated in 18 young adult patients undergoing elective Ross operation. The results were compared with a group of 19 similar patients without plateletpheresis (control group). The hematological and coagulation parameters at admission and discharge were statistically similar in both groups. The total blood product transfusion requirements were significantly reduced in the plateletpheresis group compared with the control group (3.2 units and 5.1 units, respectively, P=0.036). The total blood donor exposure was also reduced significantly in the plateletpheresis group compared with the control group (3.2 and 6.9 donors/patient, respectively, P<0.001). The direct costs for the hospital for the plateletpheresis procedure, including costs for all blood products, were similar to those for blood products alone in the control group. In summary, acute plateletpheresis in combination with low-dose aprotinin significantly reduces the blood product transfusions and blood donor exposures following the Ross operation; the treatment is cost-effective.

A modified Ross operation to prevent pulmonary autograft dilatation.

A modification in Ross operation is described in which the free-standing pulmonary autograft root is suspended in a Dacron prosthetic vascular jacket with a view to prevent dilatation of the neo-aortic root. In a group of 13 patients operated consecutively using this technique, there was no significant increase in the diameters of the neo-aortic root after a mean 16-month follow-up. Aortic valve function remained also satisfactory.

CHEK2 1100delC in patients with metachronous cancers of the breast and the colorectum.

BACKGROUND: Development of multiple primary tumors is a hallmark of hereditary cancer. At least 1/10 of breast cancers and colorectal cancers occur because of heredity and recently the cell cycle kinase 2, CHEK2 1100delC allele has been identified at a particularly high frequency in families with hereditary breast and colorectal cancer. METHODS: We utilized the Southern Sweden population-based cancer registry to identify women with double primary breast and colorectal cancer and sequenced tumor material in order to assess the contribution of the CHEK2 1100delC to the development of such metachronous tumors. RESULTS: Among the 75 patients successfully analyzed, 2 (2.5%) carried the CHEK2 1100delC allele. which was not significantly different (p = 0.26) from the 1% (3/300) carriers identified in the control group. CONCLUSION: In summary, our data suggest that the CHEK2 1100delC is not a major cause of double primary breast and colorectal cancer in Sweden, which suggests that this patient group should not routinely be screened for the CHEK2 1100delC variant.