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Progressive aortic root dilation and stiffening have been reported in patients with tetralogy of Fallot (TOF), even after repair. However, the implications of such aortic dilation and stiffening have not been fully assessed. A stiff aorta has a negative effect on the coronary circulation. 15 patients with TOF after repair were enrolled in this study. Using a left ventriculogram and pressure waveform recorded by a pressure sensor-mounted catheter, we analyzed the diameter and distensibility of the ascending and descending aorta. We also calculated the subendocardial viability ratio (SEVR), which measures the cardiac blood supply-workload balance. These values were compared with those in one-to-one age-matched controls. Moreover, the correlation between the diameter ratio (aortic diameter of the patient/aortic diameter of the matched control subject) and the distensibility ratio (distensibility of the patient/distensibility of the matched control subject) was analyzed. The mean age of patients was 3.9 ± 1.6 years. In patients with TOF, the indexed diameter was larger (0.20 ± 0.02 vs. 0.15 ± 0.02 mm/cm, p < 0.0001) and the distensibility was lower (5.1 ± 2.2 vs. 7.7 ± 2.0 cm2 dynes−1 10−6, p = 0.0009) in the ascending aorta than in control subjects. There was no difference in the SEVR between patients and control subjects. The distensibility ratio was significantly negatively correlated with the indexed diameter ratio in the ascending aorta (r2 = 0.35, p = 0.019). Regardless of the aortic stiffening, the cardiac blood supply-workload balance was maintained. We speculate that the aortic root dilation might be a mechanism to functionally complement aortic stiffness in patients with TOF. [ABSTRACT FROM AUTHOR]

In a fetus with suspected heterotaxy syndrome, a decreased/absent baseline variability of fetal heart rate pattern developed at gestational week 36+5 and continued for 5 days until birth at gestational week 37+2, while repeat biophysical profile scorings with ultrasound were consistently unremarkable. This neonate weighing 2404 g with Apgar scores of 7 (1-min) and 8 (5-min) and umbilical arterial cord blood pH of 7.28 with base deficit of 3.9mmol/L, showed a heart rate of 120 b.p.m. for 3 h after birth, but subsequently developed sinus bradycardia (84 b.p.m.) unresponsive to crying. Isoproterenol initiated 9 h after birth was effective in the increase of heart rate to 120 b.p.m. in this neonate. Brain magnetic resonance imaging at 16 days of age was unremarkable. The decreased/absent baseline variability of fetal heart rate pattern was speculated to have been caused by sinus node dysfunction, and not by reduced fetal oxygenation in this case. [ABSTRACT FROM AUTHOR]

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by impaired left ventricle growth and diastolic dysfunction. We hypothesized that the patients with TAPVC reduced blood flow into the left heart prenatally could affect left atrium (LA) not just growth but function. We compared the age-related changes in LA deformation using two-dimensional speckle-tracking echocardiography (2DSTE) in Patients with TAPVC. Method: This single-center, retrospective cohort study was conducted on consecutive isolated TAPVC patients who underwent neonatal surgery between January 1, 2009 and January 1, 2022. The LA datasets in TAPVC patients were analyzed before surgery (n = 28) and follow-ups at 1−2 (n = 24) and 5−7 years of age (n = 13) and compared with those of age-matched healthy controls (January 2009−2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) strains. LA pressure was evaluated by periodic follow-up catheterization after repair. Results: Compared to the controls, the TAPVC patients had significantly smaller LA maximum volume preoperatively, and with age, the LA maximal volumes reached normal levels, while the LA minimal volumes were larger. All 2DSTE-determined LA strains showed significant reductions at all time points in the TAPVC group compared to those in the control (median εR, εCD, and εCT; before surgery: 17.0% vs. 26.0%, 12.9% vs. 15.9%, and 6.3% vs. 10.4%; follow-up at 1−2 years: 30.0% vs. 45.7%, 23.2% vs. 29.6%, and 6.1% vs. 16.3%; follow-up at 5−7 years: 31.2% vs. 43.1%, 25.0% vs. 31.2%, and 5.2% vs. 10.8%, respectively; p < 0.05). Only εCT did not represented a significant change over time even though after correction of blood flow (median εCT: 6.0% → 5.9%). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. Conclusion: This study showed that nevertheless maximum volume of LA was recovered within the normal range, reduced LA strains, especially contractile function lasted from birth even after repair in Patients with TAPVC. [ABSTRACT FROM AUTHOR]

Biplane Area-Length (AL) method by left ventriculography (LVG) has been widely adopted as a standard method to estimate left ventricular volume. However, we have experienced difficulties in adopting the value by AL method for the children with Tetralogy of Fallot (TOF) due to the discrepancy among volumetric modalities. This study validated some limitations of AL method, considering the basic principles of its formulation. A single center retrospective cohort study was conducted for 1 year. The confirmed 22 cases with repaired TOF at our hospital were enrolled. The clinical characteristics, some cardiac MRI analyses, and all the cardiac catheterization studies were collected. Angiographic data were compared with historic cohorts of Kawasaki disease without any coronary artery lesions by using AL method. Cardiac MRI analyses of ten TOF patients were additionally available. LVG studies showed that the length of the long axis on anteroposterior view (AP) was not equal to that on lateral view (LT) due to anatomically apical elevation in TOF, followed by a significant difference found in the sagittal lengths of the LV long axis between AP and LT (P = 0.003). Because the difference critically affected the formula depending on biplane AL method, the calculated LVEDV of TOF group appeared overestimated, compared with the control group (TOF vs control group: 119.5% ± 6.3% vs 96.4 ± 3.5% of Normal, P = 0.006). Available cardiac MRI analyses of some patients in TOF group revealed 55% increase of LVEDV by AL method (angiocardiography 116 ± 7.0 vs CMR 75 ± 3.7 ml/m2, P = 0.0025). A pitfall exists when applying biplane AL method to measure LV volume especially for TOF patients, because the long axis on AP view is not always identical to that on LT view. [ABSTRACT FROM AUTHOR]

It is well known that the reflected pressure wave in small children returns earlier than that in adolescent. The reason of early return of the reflected pressure wave in infancy is their height. The short distance between heart and reflection point makes the reflected pressure wave returning to the heart earlier. In adult, the early return (during systole) of the reflected pressure wave means disadvantage to cardiac blood supply-workload balance. The purpose of this study was to clarify whether the early return of the reflected pressure wave in small children impairs the cardiac blood supply-workload balance. This study enrolled 37 small left-to-right shunt patients with normal aortic circulation below 15 years of age. The aortic pressure waveform was recorded using a pressure sensor mounted catheter, and augmentation index and subendocardial viability ratio were calculated. The age of patients was 6.1 ± 3.2 years. The augmentation index was 8.7 ± 14.3 % and the index had a negative correlation with patients' age ( r = −0.6243, p < 0.0001). The subendocardial viability ratio, which means the cardiac blood supply-workload balance, was 0.92 ± 0.14 and the index had a positive relationship with patients' age ( r = 0.6435, p < 0.0001). The cardiac blood supply-workload balance gradually improves from infancy to young adulthood. One of the causes of the unfavorable cardiac blood supply-workload balance in infancy would be the accelerated aortic pressure wave reflection due to their short height. [ABSTRACT FROM AUTHOR]

Quantitative assessment of the right-to-left ratio of pulmonary blood flow distribution is important for determining the clinical indications for treating pulmonary arterial branch stenosis. A novel theory was recently proposed that can be used to quantitatively assess the right-to-left ratio on conventional X-ray angiography images. In the proposal, further developments were indicated, especially automated calculation. In this study, a new automated algorithm was developed. In the X-ray image, regions of interest were set in right and left lung, and time-signal intensity curves were measured. The new automated algorithm is applied to determine the optimal time window for the analysis of the time-signal intensity curve and to calculate the slope of the curve in the optimized time window. The right-to-left ratios in seven consecutive patients calculated by the new automated algorithm were compared to those calculated by lung perfusion scintigraphy. The ratios were in good agreement with linear regression with a slope of 1.27 and a Pearson correlation coefficient of 0.95. The processing time was less than 10 s, which is one-eighth of the manual processing time. The new automated algorithm is accurate, stable, and fast enough for clinical use in the real world. [ABSTRACT FROM AUTHOR]

Objective: Quantitative assessment of pulmonary blood flow distribution is important when determining the clinical indications for treating pulmonary arterial branch stenosis. Lung perfusion scintigraphy is currently the gold standard for quantitative blood flow measurement. However, it is expensive, cannot provide a real-time assessment, requires additional sedation, and exposes the patient to ionizing radiation. The aim of this study was to investigate the feasibility of a novel technology for measuring pulmonary blood flow distribution in each lung by conventional X-ray pulmonary angiography and to compare its performance to that of lung perfusion scintigraphy. Methods: Contrast-enhanced X-ray pulmonary angiography images were acquired at a frame rate of 30 frames per second. The baseline mask image, obtained before contrast agent injection, was subtracted from subsequent, consecutive images. The time-signal intensity curves of two regions of interest, established at each lung field, were obtained on a frame-to-frame basis. The net increase in signal intensity within each region at the torrent period during the second cardiac cycle before contrast agent enhancement over the total lung field was measured, and the right-to-left ratio of the signal intensity was calculated. The right-to-left ratio obtained with this approach was compared to that obtained with scintigraphy. Agreement of the right-to-left ratio between X-ray angiography and lung scintigraphy measurements was assessed using linear fitting with the Pearson correlation coefficient. Result: The calculation of the right-to-left ratio of pulmonary blood flow by our kinetic model was feasible for seven children as a pilot study. The right-to-left ratio of pulmonary blood flow distribution calculated from pulmonary angiography was in good agreement with that of lung perfusion scintigraphy, with a Pearson correlation coefficient of 0.91 and a slope of linear fit of 1.2 (p<0.005). Conclusion: The novel diagnostic technology using X-ray pulmonary angiography from our kinetic model can feasibly quantify the right-to-left ratio of pulmonary blood flow distribution. This technology may serve as a substitute for lung perfusion scintigraphy, which is quite beneficial for small children susceptible to radiation exposure. [ABSTRACT FROM AUTHOR]

Elevated right atrial (RA) pressure and progressive RA dilation are thought to play pivotal roles in the development of late complications after atriopulmonary connection (APC) Fontan surgery. However, no clear cut-off value for RA pressure or RA volume has been determined for stratifying the risk of developing Fontan complications. We hypothesized that RA tension, which incorporates information about both RA pressure and volume, might help predict the risk of developing complications. We retrospectively studied 51 consecutive APC Fontan patients (median postoperative period 14 years). RA tension was computed from the RA pressure and RA radius, which was calculated from RA volume measured by RA angiography. The correlation between the cardiac catheterization hemodynamic data and the complications of APC Fontan was investigated. Of the 51 patients, 28 had complications, including liver fibrosis ( n = 28), arrhythmia ( n = 8), protein-losing enteropathy ( n = 1), and RA thrombosis ( n = 1). Among the hemodynamic data, RA volume and RA tension, but not RA pressure, were significantly higher in patients with complications than in those without ( P = 0.004 and P = 0.001, respectively). The cut-off level for RA tension to predict Fontan complications was 26,131 dyne/cm by receiver operating characteristic curve (area under the curve 0.79, sensitivity 71.4%, and specificity 73.9%). The present study demonstrated the significance of RA tension rather than high venous pressure for the development of Fontan complications. Amid the uncertainty about clinical outcomes, the present results, subject to further validation, may contribute to the indications for Fontan conversion. [ABSTRACT FROM AUTHOR]

Aim This retrospective study was performed to determine the frequency of malformed infants born at a tertiary center in Hokkaido, Japan. The accuracy of prenatal diagnosis rates was also investigated. Methods An observational study was performed using data of 1509 and 1743 newborn infants at a single center during two study periods, 2005-2009 (first) and 2010-2014 (second), respectively. Cases including minor anomalies (accessory auricle, nevus and fistula auris congenita) were not included. Results In total, 274 and 569 malformations were identified in 191 and 337 newborn infants in the first and second study periods, respectively. The number of malformed infants increased significantly over time (13% [191/1509] vs 19% [337/1743], respectively; P < 0.001), mainly as a result of an increase in cases of congenital heart disease (CHD), from 59 to 141 (31% [59/191] vs 42% [141/337] of all malformed infants in the first and second periods, respectively). The overall accurate prenatal diagnosis rate improved over time from 47% (128/274) to 58% (329/569) because of significant improvements in accurate prenatal diagnosis of CHD subtypes (23% [16/70] vs 65% [151/232] in the first and second periods, respectively, P < 0.0001). Conclusions The frequency of malformed newborns was higher in the tertiary center than in the general population. The increased number of cases with prenatal suspicion and diagnosis of CHD contributed to the increased frequency of malformed infants during the study period. [ABSTRACT FROM AUTHOR]