Korean Circulation Journal最新文献

Background and objectives: The growth trajectories of children diagnosed with congenital heart disease (CHD) remain unclear when analyzed through population-based data. This study examined the risk of poor growth outcomes in preschool-aged children with CHD.

Methods: This study included 325,930 children born between 2007 and 2014, of whom 65,186 were diagnosed with CHD (1,026 with complex, 2,073 with moderate, and 62,087 with simple CHD). Growth Z-scores (height, weight, head circumference, and body mass index) were measured through the National Health Screening Program for Infants and Children, which is conducted annually for children aged six months to six years. Participants were observed until death or December 31, 2020, whichever occurred first.

Results: Overall, there were 1,390 deaths in the CHD group, with a cumulative survival rate of 97.9%. Growth deficits were most pronounced in the complex CHD group, followed by the moderate and simple CHD groups, as well as the normal control groups (short stature at 4-6 years of age: 10.7% vs. 8.9% vs. 5.9% vs. 2.4%, p<0.001). Growth curves illustrated that patients with complex CHD experience persistent height and weight impairments throughout the preschool period. The β estimate (standard error) of height was -0.432 standard deviation score (SDS) (0.041) for the complex CHD group, -0.343 SDS (0.028) for the moderate CHD group, and -0.200 SDS (0.005) for the simple CHD group.

Conclusions: Children with CHD showed poor growth outcomes throughout the preschool period. We highlight the importance of longitudinal growth surveillance for high-risk children with CHD during this period to mitigate the development of health issues.

Background and objectives: Despite advances in the outcomes of arterial switch operation (ASO), long-term complications such as neo-aortic regurgitation (AR), neo-aortic root dilatation (ARD), neo-pulmonary stenosis (PS), and coronary artery-related events (CARE) pose challenges. We aimed to evaluate the long-term outcomes of ASO following a consistent surgical strategy adopted since 2003.

Methods: We retrospectively analyzed 133 patients who underwent ASO between January 2003 and June 2021. Consistent surgical strategies included the trap-door method for coronary artery transfer, preservation of the neo-sinotubular junction, and extensive mobilization of pulmonary artery branches. Clinical outcomes and risk factors for adverse events were assessed.

Results: Among the 133 patients, 64 had transposition of the great arteries (TGA) with intact ventricular septum (IVS), 52 had TGA with ventricular septal defect (VSD), and 17 had Taussig-Bing anomaly (TBA). Operative mortality was 5.2% (n=7), with no late mortality over a median follow-up of 7.1 years. Freedom from reoperation and the composite of reoperation and re-intervention rates at 15 years were 80.7% and 74.0%, respectively. Freedom from significant neo-AR (≥ mild-to-moderate), neo-ARD (z-score ≥3.0), neo-PS (peak velocity >3.0 m/s or requiring re-intervention), and CARE at 15 years was 91.7%, 37.1%, 77.7%, and 92.3%, respectively. Complex TGA morphological subtypes (TBA > TGA-VSD > TGA-IVS) had higher neo-ARD (log-rank p<0.001) and composite of reoperation and re-intervention rates (log-rank p=0.021).

Conclusions: Our surgical outcomes of ASO following the standardized strategy were favorable, despite concerns regarding long-term adverse events. Complex TGA morphological subtypes demonstrated higher neo-ARD and composite of reoperation and re-intervention rates.

Background and objectives: Quantitative flow ratio (QFR) is a method for estimating fractional flow reserve (FFR) without the use of an invasive pressure wire or hyperemic agent. However, the reasons for variation in QFR accuracy across studies and the factors associated with its accuracy remain unclear. The aim of this study was to investigate the diagnostic performance of QFR under different clinical and analysis conditions.

Methods: This multicenter trial prospectively enrolled patients undergoing coronary angiography with an indication for invasive FFR. The composite score for the QFR analysis factors was calculated based on the presence or absence of the angiographic factor, system factor, lesion factor, and subjective difficulty factor. The diagnostic performance of the QFR was assessed for each composite score using FFR ≤0.80 as the reference.

Results: A total of 285 vessels from 239 patients were analyzed. The median FFR and QFR values were 0.83 (interquartile range [IQR], 0.78-0.88) and 0.83 (IQR, 0.76-0.89), respectively. Using FFR ≤0.80 as a reference, QFR showed an overall diagnostic accuracy of 81.4%. Higher composite scores were associated with lower diagnostic performance of QFR in predicting FFR ≤0.80 (p-for-trend=0.010). The diagnostic accuracy of QFR ranged from 94.1% in vessels with low composite scores to 73.7% in those with high composite scores.

Conclusions: The diagnostic accuracy of QFR decreases with increasing lesion complexity, system factors, lower angiographic image quality, and analysis difficulty. These findings suggest that specific lesion, system, and imaging-related factors can significantly impact the reliability of QFR in clinical practice.

Trial registration: ClinicalTrials.gov Identifier: NCT06305572.

Background and objectives: The Fontan operation is a significant advancement in the palliative treatment of patients with single ventricular physiology. However, pregnancy in women with Fontan circulation presents increased risks for both the mother and fetus, with limited data available on maternal and fetal outcomes. This study reports maternal and fetal outcomes of pregnancies in women with Fontan circulation from a single tertiary center.

Methods: This retrospective study included all pregnant patients with Fontan physiology treated at Seoul National University Hospital from June 1986 to September 2023. The cases reviewed encompassed miscarriages, premature births, and full-term deliveries.

Results: Eight patients underwent 15 pregnancies. The mean age at first pregnancy was 26.7 years. There were 7 live births (46.7%) and 8 miscarriages (53.3%), including 1 therapeutic miscarriage. The average age at first delivery was 28 years and the mean gestational age was 36^±2.5 weeks. Out of 7 live births, 2 (28.6%) were premature births. For delivery, 6 (85.7%) underwent cesarean section. All pregnancies were monitored with echocardiography. Aspirin was administered as an antithrombotic medication in 5 patients (62.5%), and 2 patients (25%) was on warfarin prior to pregnancy. Two patients (28.6%) experienced postpartum cardiac complications (transient heart failure and thromboembolism, respectively), which were successfully managed.

Conclusions: We observed a 46.7% success rate of live births among pregnant women with Fontan circulation. Although 2 pregnancies had cardiac complications, there was no severe morbidity and mortality. Thus, pregnancy and planned delivery are feasible in selected women with Fontan circulation under close monitoring.