Duplex ultrasound abnormalities of the lower limb veins might precede clinical venous reflux signs in post-Fontan adolescents and young adults.

OBJECTIVE: After the Fontan operation (ie, direct anastomosis of the caval veins to the pulmonary arteries, constituting right ventricular bypass circulation), high central venous pressure can lead to peripheral venous stasis and venous valvar insufficiency. We hypothesized that post-Fontan patients are at a higher risk of developing lower extremity venous lesions detectable using duplex ultrasound, even if clinical signs of chronic venous disease might not be evident. METHODS: A total of 87 transplantation-free survivors after the Fontan procedure who reached adolescence or young adulthood (current age, 15-30 years) participated in a leg vein duplex ultrasound study. The median age at the Fontan procedure, median age at the vein study, and median interval between the two were 3.65 years (interquartile range [IQR], 3.1-5.3 years), 21.7 years (IQR, 18.9-24.7 years), and 16.6 years (IQR, 14.9-19.4 years), respectively. Duplex ultrasound scanning was performed using a venous ultrasound imaging system (Logiq P7; GE Healthcare). The patients were categorized according to the presence of venous reflux (VR) in the superficial, deep, or perforating venous systems: no VR, superficial VR (SVR), deep VR (DVR), perforating VR (PVR), and a combination of multiple venous systems. Correlation of the duplex ultrasound-detected venous lesions with clinical severity using the modified CEAP (clinical, etiological, anatomical, pathophysiological) classification was analyzed using Spearman's correlation analysis. RESULTS: Leg pain was reported by 48 of 87 patients (55.2%). The duplex ultrasound findings for the cohort were no VR in 21 patients (24.1%), SVR in 22 (25.3%), isolated PVR in 21 (24.1%), and reflux of multiple venous systems in 23 patients, including SVR and PVR in 19, DVR and PVR in 1, and SVR, PVR, and DVR in 3 patients. Although the patients with advanced venous lesions detected by duplex ultrasound tended to have a higher CEAP clinical class (P < .001), VR of any venous system on duplex ultrasound was present even in patients with a lower CEAP clinical class. The CEAP clinical class was C0 for 66 patients (76%), and VR of any venous system was present on duplex ultrasound in 66 patients (76%). CONCLUSIONS: The prevalence of lower extremity venous lesions detected by duplex ultrasound is strikingly high in post-Fontan adolescents and young adults, and duplex ultrasound-detected venous abnormalities can precede clinical manifestations. Early detection and timely intervention for leg vein problems are mandatory for post-Fontan patients, especially for those considered to have risk factors for developing chronic lower extremity venous disease.

Effects of Lacticaseibacillus casei (Lactobacillus casei) and Saccharomyces cerevisiae mixture on growth performance, hematological parameters, immunological responses, and intestinal microbiome in weaned pigs.

Introduction: This study was conducted to evaluate the effects of Lacticaseibacillus casei (Lactobacillus casei) and Saccharomyces cerevisiae mixture on growth performance, hematological parameters, immunological responses, and gut microbiome in weaned pigs. Methods: A total of 300 crossbred pigs [(Landrace × Yorkshire] × Duroc; 8.87 ± 0.34 kg of average initial body weight [BW]; 4 weeks of age) were divided into two dietary treatments (15 pigs/pen, 10 replicates/treatment) using a randomized complete block design (block = BW): control (CON) and the effective microorganism (MEM). The CON was not treated, while the MEM was treated with the mixture of L. casei (1 × 107 CFU/mL) and S. cerevisiae (1 × 107 CFU/mL) at 3 mL/pig/day for 4 weeks via the drinking water supply. Two feces and one blood sample from the randomly selected pigs in each pen were collected on D1 and D28 after weaning. Pigs were individually weighed, and pen feed intakes were recorded to evaluate pig growth performance. For the gut microbiome analysis, 16S rRNA gene hypervariable regions (V5 to V6) were sequenced using the Illumina MiSeq platform, and Quantitative Insight into Microbial Ecology (QIIME) and Microbiome Helper pipeline were used for 16S rRNA gene sequence analysis. Results and Discussion: The daily weight gain and feed efficiency of MEM were significantly higher than those of CON (p < 0.001). There were no significant differences in hematological parameters and immune responses between CON and MEM. However, MEM had significantly lower Treponema genus, whereas significantly higher Lactobacillus and Roseburia genera compared to CON. Overall, our data showed that L. casei and S. cerevisiae mixture could promote growth performance through the modulation of gut microbiota in pigs. This study will help to understand the correlation between the growth performance and the gut microbiome.

Characteristics of Patients with Surgical Closure of an Atrial Septal Defect during Infancy.

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m2, p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

An educational design and development project for pediatric heart transplant recipients and their families.

PURPOSE: The purpose of this project is to systematically derive and develop educational content for children who have undergone heart transplants and their families to improve the families' and caregivers' ability to manage child health in South Korea. DESIGN AND METHODS: This project developed educational content through the first three steps: "Analysis," "Design," and "Development" of the five steps outlined in the ADDIE (Analysis, Design, Development, Implementation, and Evaluation) model. The educational contents were derived from an analysis of six literature reviews and six studies, seven websites, and interviews with six caregivers of children who have undergone a heart transplant. Based on these analyses, educational items were designed. When a draft of the education contents was developed, the content validity was validated by ten experts. RESULTS: Educational items were designed in nine major categories and 40 sub-categories. All contents were validated with a content validity index of at least 0.8 or higher and included in the education content for pediatric heart transplant families, with recommendations for further modification based on expert advice. PRACTICE IMPLICATIONS: This project is meaningful in that it has developed health management education contents suitable for the situation in South Korea through analyzing previous studies and educational contents of domestic and international medical centers as well as an in-depth interview with caregivers.

Resolution of Postnatal Shock Before Open Heart Surgery in Neonates With Prenatal Diagnoses.

BACKGROUND: The impact of a prenatal diagnosis (PreND) for congenital heart disease on outcomes after neonatal open heart surgery is undetermined. We hypothesized that PreND has a positive impact on surgical outcomes in terms of immediate postnatal intensive care, which may lead to a decreased risk of persistent shock before surgery. METHODS: Among the 949 neonates who underwent open heart surgery between January 2002 and December 2017, 655 patients (69.0%) were diagnosed prenatally (group-PreND) and 294 patients (31.0%) were diagnosed postnatally (group-PostND). Procedural complexity, incidence of postnatal shock (serum lactate >4.0 mmol/L or pH <7.2), hospitalization length of stay, duration of shock, resolution of shock, and in-hospital mortality were compared between the 2 groups. RESULTS: In group-PreND, the procedure-dependent comprehensive Aristotle score (10.8 vs 10.0, P < .001), incidence of extracardiac anomalies (13.0% vs 7.1%, P = .008), heterotaxy syndrome (3.8% vs. 1.0%, P = .021), and postnatal shock (244 of 655 [37.3%] vs 78 of 294 [26.5%], P = .001) were higher than in group-PostND. However, patients in group-PreND were hospitalized earlier after birth (0 day vs 5 days, P < .001), experiencing shorter duration of shock (5.3 hours vs 9.0 hours, P = .01), and, consequently, showing higher incidence of shock resolution (212 of 244 [87%] vs 52 of 78 [67%], P < .001). In-hospital mortality was comparable between the 2 groups (P = .070). CONCLUSIONS: Postnatal shock is more frequently observed in group-PreND. However, prenatal awareness of the disease leads to immediate postnatal initiation of intensive care with shorter exposure to shock, leading to higher probability of shock resolution.

Sexual health knowledge and needs among young adults with congenital heart disease.

Advances in the treatment of congenital heart disease (CHD) have increased life expectancy, entailing medical surveillance for a considerable number of adolescents and young adults with CHD for issues arising in areas such as sexual health. This study aimed to assess the sexual knowledge and the needs for sexual health education among this group. The participants comprised 53 young adult outpatients (27 males, median age: 23 years) who had undergone surgical interventions (median: 3 times) for CHD. The Knowledge related to Safe Sex Practice scale (KSSP), an assessment tool containing 15 questions on sexual knowledge, was administered, and the rates of correct answers for each item and the overall scale were compared with the age and sex of a control group (n = 164). The overall mean KSSP score of the participant group (10.5 ± 1.8) was significantly lower than that of the control group (11.1 ± 1.9, p = .035). The KSSP scores of the participants with low peripheral oxygen saturation (SaO2 < 95%) were significantly lower (9.77 ± 1.85) than those with normal SaO2 (11.06 ± 1.85, p = .009). Regarding sexual health education, the participants reported receiving information about contraception as more important than other areas of sexual health. The rate of incorrect answers was higher for questions regarding natural ways of contraception utilizing infertile periods in the menstrual cycle. Based on an informed understanding of those with CHD, healthcare providers in this field should develop customized sexual health education for adolescents and young adults with CHD and implement customized sexual health education, including effective contraception methods.

Digital and Economic Determinants of Healthcare in the Crisis-Affected Population in Afghanistan: Access to Mobile Phone and Socioeconomic Barriers.

Despite recent progress in Afghanistan's health system from the support of international donors and NGOs, protracted conflicts combined with a series of natural disasters have continued to present substantial health risks. Extreme poverty has still aggravated social determinants of health and financial barriers to healthcare. Little is known about the context-specific factors influencing access to healthcare in the crisis-affected population. Using a subset of data from 'Whole of Afghanistan Assessment (WoAA) 2019', this study analyzed 31,343 households' data, which was collected between 17 July and 19 September 2019 throughout all 34 provinces in Afghanistan. The outcome measured was access to care in the healthcare facility, and multivariable binary logistic regression models were used to identify the specific factors associated with access to healthcare. Of 31,343 households exposed to complex emergencies in Afghanistan, 10,057 (32.1%) could not access healthcare facilities when one was needed in last three months. The access to healthcare was significantly associated with displacement status, economic factors such as employment status or total monthly income, and the distance to healthcare facilities. Significant increase in healthcare access was associated with factors related to communication and access to information, such as awareness of humanitarian assistance availability and mobile phone with a SIM card, while disability in cognitive function, such as memory or concentration, was associated with poorer healthcare access. Our findings indicate that the crisis-affected population remains vulnerable in access to healthcare, despite the recent improvements in health sectors. Digital determinants, such as access to mobile phone, need to be addressed along with the healthcare barriers related to poverty and household vulnerabilities. The innovative humanitarian financing system using mobile communication and cash transfer programs would be considerable for the conflict-affected but digitally connected population in Afghanistan.

Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.

BACKGROUND: The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. METHODS: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before 90 days of age were identified and enrolled in this retrospective study. The cohort was divided into two groups: group-SR, who underwent initial palliation with staged repair (n = 50), and group-PR who underwent primary repair (n = 15). RESULTS: There were three post-palliation in-hospital mortalities, four inter-stage mortalities, and one post-repair in-hospital mortality in group-SR. In group-PR, there was one in-hospital death and one late death. Five-year survival rates were comparable between the two groups (group-SR: 83.6%; group-PR: 86.7%; p = 0.754). During the median follow-up duration of 44.7 months (Inter-quartile range, 19-109 months), 40 post-repair re-interventions (22 in group-SR, 18 in group-PR) were performed in 26 patients (18 in group-SR, 8 in group-PR). On Cox proportional hazards model, primary repair was identified as the only risk factor for decreased time to death/1st post-repair re-intervention (Hazard ratio (HR): 2.3, p = 0.049) and death/2nd post-repair re-intervention (HR 2.91, p = 0.033). CONCLUSIONS: A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.

Factors Influencing Physical Activity in Adolescents with Complex Congenital Heart Disease.

PURPOSE: We aimed to identify factors influencing physical activity in adolescents with complex congenital heart disease. METHODS: We recruited 92 adolescents with complex congenital heart disease from a tertiary medical center in Seoul, measured their levels of physical activity, and identified factors that influenced their physical activity levels using the Global Physical Activity Questionnaire, the New York Heart Association classification, congenital heart disease complexity, the Self-Efficacy Scale, and the Parental Bonding Instrument scale. Stepwise multiple linear regression was used to determine factors influencing physical activity. RESULTS: Total physical activity was higher in males than in females (t=4.46, p<.001). Adolescents who participated in school physical education classes engaged in more physical activity than those who did not (t=6.77, p<.001). Higher self-efficacy (ß=.41, p<.001), male gender (ß=.37, p<.001) and participation in school physical education classes (ß=.19, p=.042) were associated with a higher likelihood of engagement in physical activity. CONCLUSION: It is necessary to develop nursing interventions that enhance self-efficacy in order to promote physical activity in adolescents with complex congenital heart disease. Physical activity should also be promoted in an individualized manner, taking into account gender, disease severity, and parental attitude.

Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries.

BACKGROUND: Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO). METHODS: Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n = 20) or tricuspid atresia (n = 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients. Initial palliative procedures comprised pulmonary artery banding with (n = 4) or without (n = 14) arch repair, bilateral pulmonary artery banding with ductal stenting (n = 5), primary Norwood operation (n = 4), and palliative arterial switch operation (n = 1). Cox proportional hazards model was fitted in 15 patients with initial postnatal echocardiography to identify risk factors for decreased time to SORO. RESULTS: One early and one late death occurred during the median follow-up period of 66 months (10-year survival rate, 93.3%). Various types of SORO were required in 20 of 30 patients (66.7%): Damus-Kaye-Stansel procedure (n = 12), primary Norwood-type palliation (n = 4), palliative arterial switch operation (n = 1), and bulboventricular foramen extension (n = 3). Freedom from SORO at 5 years was 34.5% in all patients (N = 30). Cox regression for the subgroup (n = 15) revealed that arch obstruction (hazard ratio, 20.6; 95% confidence interval, 2.9 to 148.2; p = 0.003) and smaller systemic outflow tract area index at end-systolic phase (hazard ratio, 1.5 at 10 mm2/m2 decrease; 95% confidence interval, 1.0 to 2.1; p = 0.033) were identified as risk factors for decreased time to SORO. CONCLUSIONS: Arch obstruction and a smaller systemic outflow tract area index at end-systolic phase at initial presentation are predictors of subsequent need for SORO in patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries.

The pregnancy experience of Korean mothers with a prenatal fetal diagnosis of congenital heart disease.

BACKGROUND: Prenatal diagnosis of fetal congenital heart disease (CHD) is becoming widely available but there is a lack of understanding on such expectant mothers' experiences during pregnancy. This was the first study to investigate the pregnancy experience of Korean mothers with a prenatal fetal diagnosis of CHD. METHODS: In-depth interviews were conducted with 12 mothers regarding their child's prenatal diagnosis of CHD and the adaptive processes during pregnancy. The data were transcribed and analyzed according to the grounded theory framework. RESULTS: When the diagnosis of fetal CHD was suspected, mothers desperately sought accurate information regarding CHD while hoping in vain for a misdiagnosis. When the definitive diagnosis was made, most pregnant women experienced psychological trauma and pain, framed in the stigma and burden of having an imperfect child. Provision of accurate health advice and emotional support by a multidisciplinary counseling team was crucial at this phase, forming recognition that CHD could be treated. When fetal movements were felt, mothers came to acknowledge the fetus as an independent being, and made their best efforts to protect the fetus from harmful external influences using traditional TaeKyo mindset and practices, which in turn, were helpful in restructuring the meaning of the pregnancy. CONCLUSIONS: Mothers went through a dynamic process of adapting to the unexpected diagnosis of CHD, which was closely linked to being able to believe that their child could be treated. Early counseling with precise information on CHD, continuous provision of clear explanations on prognosis, sufficient emotional support, and well-designed prenatal education programs are the keys to an optimal outcome.

Health condition and familial factors associated with health-related quality of life in adolescents with congenital heart disease: a cross sectional study.

BACKGROUND: The focus of clinical care after the repair of congenital heart disease has shifted from saving life of the patient to the patient's quality of life. The purpose of this study was to examine the health condition and familial factors associated with the health related quality of life of adolescents with congenital heart disease. METHODS: Ninety-eight adolescents aged 13-19 years were collected from a congenital heart clinic from July 22 to August 23, 2013. Perceptions of parental rearing behaviors, health related quality of life of adolescent with congenital heart disease, and general characteristics were measured. We used multiple linear regression analysis to explore factors that are associated with the health related quality of life of adolescents with congenital heart disease. RESULTS: New York heart association class (Adj R2 = .186, p = .000), presence of siblings (Adj R2 = .240, p = .010), and mother's emotional warmth (Adj R2 = .265, p = .043) were significantly associated with the health related quality of life of adolescents with congenital heart disease. CONCLUSIONS: Emotionally warm parental rearing behaviors and the presence of siblings were important familial factors that were positively associated with HRQOL in adolescents with CHD. Therefore, it is important for healthcare providers to develop a greater sensitivity to, and awareness of, the familial influences that may be impacting a subject's HRQOL, as well as the exigencies of the CHD, itself.

School-related adjustment in children and adolescents with CHD.

Advancements in medical and surgical treatment have increased the life expectancy of patients with CHD. Many patients with CHD, however, struggle with the medical, psychosocial, and behavioural challenges as they transition from childhood to adulthood. Specifically, the environmental and lifestyle challenges in school are very important factors that affect children and adolescents with CHD. This study aimed to evaluate school-related adjustments depending on school level and disclosure of disease in children and adolescents with CHD. This was a descriptive and exploratory study with 205 children and adolescents, aged 7-18 years, who were recruited from two congenital heart clinics from 5 January to 27 February, 2015. Data were analysed using the Student's t-test, analysis of variance, and a univariate general linear model. School-related adjustment scores were significantly different according to school level and disclosure of disease (p<0.001) when age, religion, experience being bullied, and parents' educational levels were assigned as covariates. The school-related adjustment score of patients who did not disclose their disease dropped significantly in high school. This indicated that it is important for healthcare providers to plan developmentally appropriate educational transition programmes for middle-school students with CHD in order for students to prepare themselves before entering high school.

Systemic-Pulmonary Shunt Facilitates the Growth of the Pulmonary Valve Annulus in Patients With Tetralogy of Fallot.

BACKGROUND: Transannular patching (TAP) frequently accompanies primary repairs (PRs) in symptomatic neonates with tetralogy of Fallot (TOF). If a systemic-pulmonary shunt (SPS) facilitates the growth of the pulmonary valve annulus (PVA), patients with a marginally small PVA could benefit from a staged repair in terms of lowering the risk of TAP. METHODS: Among 216 infants with TOF who underwent surgical intervention between January 2004 and December 2013, 29 infants underwent SPS with a subsequent repair (SPS group), whereas 187 infants received a PR (PR group). Median age and the Z-score of the PVA (PVA [Z]) at SPS were 32 days and -3.5, respectively. There was one late death and one follow-up loss after SPS, and preservation of the PVA was achieved on repair in 16 patients (16 of 29; 55%). RESULTS: Multiple regression analysis showed that performance of SPS was the only indicator of the increase in the PVA (Z) in the entire cohort (n = 216). On mixed linear regression, the PVA (Z) increased significantly after the placement of an SPS (-3.6 + 0.2*duration in months, p = 0.001), whereas the prerepair changes in the PVA (Z) were not statistically significant in the PR group (p = 0.7), with a significant intergroup difference (p < 0.001). Receiver operating characteristic curve analysis showed that placement of TAP is expected when the preshunt PVA (Z) is smaller than -4.2 (area under the curve: 0.82; 95% confidence interval: 0.62 to 1.00; sensitivity, 100%; specificity, 73%). CONCLUSIONS: SPS facilitates outgrowth of the PVA over somatic growth in patients with TOF. However, preservation of the PVA may not be achieved on staged repair if the initial PVA is too small.

Outcomes of neonatal Ebstein's anomaly without right ventricular forward flow.

OBJECTIVE: In neonates with Ebstein's anomaly and absent right ventricular forward flow, pulmonary valve morphology is normal or abnormal. Although initial postnatal presentations of these 2 conditions are similar, clinical courses and therapeutic strategies for each category differ greatly. METHODS: Among 29 neonates with Ebstein's anomaly without right ventricular forward flow on initial postnatal echocardiography, 16 had a normal pulmonary valve and 13 had an abnormal pulmonary valve. RESULTS: During the postnatal follow-up of the normal pulmonary valve group, right ventricular forward flow commenced approximately 10 days after birth (1-15 days). The ductus arteriosus was surgically ligated in 3 neonates to facilitate right ventricular forward flow. Biventricular or 1 1/2 ventricular physiology was eventually achieved in 14 patients in the normal pulmonary valve group (14/16, 88%) and 2 patients in the abnormal pulmonary valve group (2/13, 15.3%). With respect to the preoperative echocardiographic findings, the normal pulmonary valve group had a significantly larger pulmonary valve annulus (8.2 ± 1.4 mm in the normal pulmonary valve group and 6.4 ± 1.8 mm in the abnormal pulmonary valve group, P = .002) and smaller cardiothoracic ratio (0.79 ± 0.05 in the normal pulmonary valve group and 0.85 ± 0.07 in the abnormal pulmonary valve group, P = .03). Mild to moderate pulmonary regurgitation was present in all patients (16/16, 100%) in the normal pulmonary valve group, but 3 patients (3/13, 23%) in the abnormal pulmonary valve group also had pulmonary regurgitation. On logistic regression analysis, only pulmonary valve annulus size remained as an indicator of a normal pulmonary valve (P = .03). CONCLUSIONS: In patients with Ebstein's anomaly and absent right ventricular forward flow, large pulmonary valve annulus size indicated a normal pulmonary valve. Patients with a normal pulmonary valve showed better survival and had a higher probability of achieving biventricular hemodynamics.

Restoration of Tricuspid Valve Mechanism at the Level of Displaced Septal and Posterior Leaflets in Ebstein's Anomaly.

BACKGROUND: Surgical techniques currently used for the repair of Ebstein's anomaly comprise reconstruction of the tricuspid valve mechanism at the level of the true annulus with or without plication of the atrialized right ventricle. However, performing this procedure for patients with a dysmorphic anterior leaflet (i.e., insufficient leaflet tissue and decreased mobility due to tethering) may necessitate technical modifications. METHODS: A retrospective review was performed of 31 patients (seven males and 24 females, median age at operation 31 years) with Ebstein's anomaly, who underwent tricuspid valve repair between March 2002 and December 2014. The original Hetzer technique (annulus to annulus approximation) was employed for six patients with a well-formed anterior leaflet. In 25 patients, the tricuspid valve mechanism was restored at the displaced septal leaflet by approximating the anterior leaflet attachment in the true annulus to the displaced septal leaflet attachment in the mid-septum. A bidirectional superior cavopulmonary anastomosis was added in 27 of 31 (87%) patients. RESULTS: No early or late death occurred during the median follow-up of 66 months (1-138 months). Immediate postoperative tricuspid regurgitation was trivial to mild in 22 patients, and the median preoperative, immediate postoperative, and last follow-up tricuspid regurgitation jet areas in 21 adult patients were 23.3 cm2, 10.4 cm2, and 7.0 cm2, respectively. Two patients underwent reoperation at 81 and 119 months postoperatively. Five-year freedom from severe tricuspid regurgitation or reoperation was 93.2%. CONCLUSION: Restoration of the tricuspid valve mechanism at the level of displaced septal leaflet leads to excellent long-term outcomes. The addition of the bidirectional superior cavopulmonary anastomosis has contributed to the success of this technique.

Individual Pulmonary Veins Outgrow Somatic Growth After Primary Sutureless Repair for Total Anomalous Pulmonary Venous Drainage.

Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction. TAPVD types were supracardiac in nine, infracardiac in 10, and mixed in two. Utilizing cardiac computed tomography (CT), the maximal diameter of each IPV was measured, and pulmonary vein index (PVI, summation of cross-sectional areas of all four IPVs divided by body surface area) was calculated. There were five early deaths after SR. Among survivors, 10 had both preoperative and postoperative cardiac CT at a 3.6-month median interval. On postoperative cardiac CT, IPVs were patent in all patients except one who developed a left lower pulmonary vein obstruction. There was a 71 ± 48 % postoperative increase in the actual diameter of all four IPVs, and PVI increased significantly from 215 ± 55 to 402 ± 117 mm(2)/m(2) (P value = 0.005). IPVs outgrew somatic growth after primary SR of TAPVD. Primary SR may be a useful measure in TAPVD patients whose IPVs are small.

[Outcomes of home monitoring after palliative cardiac surgery in infants with congenital heart disease].

PURPOSE: Common conditions, such as dehydration or respiratory infection can aggravate hypoxia and are associated with interstage mortality in infants who have undergone palliative surgery for congenital heart diseases. This study was done to evaluate the efficacy of a home monitoring program (HMP) in decreasing infant mortality. METHODS: Since its inception in May 2010, all infants who have undergone palliative surgery have been enrolled in HMP. This study was a prospective observational study and infant outcomes during HMP were compared with those of previous comparison groups. Parents were trained to measure oxygen saturation, body weight and feeding volume and to contact the hospital through the hotline for emergency situations. Telephone counseling was conducted by clinical nurse specialists every week post discharge. RESULTS: Forty-one infants were enrolled in HMP. Nine hundred telephone counseling sessions were conducted. Seventy-three infants required telephone triage with the most common conditions being gastrointestinal (50.7%) and respiratory symptoms (32.9%). With HMP intervention, interstage mortality decreased from 18.6% (8/43) to 9.8% (4/41) (χ²=1.15, p=.283). CONCLUSION: Results indicate that active measures and treatments using the HMP decrease mortality rates, however further investigation is required to identify various factors that contribute to hemodynamic complications during the interstage period.

Long term outcomes of aortic root replacement: 18 years' experience.

BACKGROUND: We reviewed the long-term outcomes of aortic root replacement at Asan Medical Center and investigated the predictors affecting mortality. MATERIALS AND METHODS: A retrospective analysis was performed on 225 consecutive adult patients undergoing aortic root replacement with mechanical conduits (n=169), porcine aortic root prosthesis (n=23), or aortic homografts (n=33) from January 1992 to September 2009. The median follow-up duration was 6.1 years (range, 0 to 18.0 years). RESULTS: The porcine root group was older than the other groups (freestyle 55.9±14.3 years vs. mechanical 46.3±14.6 years, homograft 48.1±14.7 years; p=0.02). The mechanical group had the highest incidence of the Marfan syndrome (mechanical 22%, freestyle 4%, homograft 3%; p=0.01). Surgery performed for infective endocarditis was more frequent in the homograft group (mechanical 10%, freestyle 10%, homograft 40%; p<0.001). The overall 30-day mortality was 5.3% (12/225). Actuarial survival rates in the mechanical, porcine root, and homograft groups were 79.4%, 81.5%, and 83.5% at 5 years and 67%, 61.9%, and 61.1% at 10 years, respectively (p=0.73). By multivariate analysis, preoperative diabetes mellitus, older age, and longer cardiopulmonary bypass time were independent predictors of mortality. Incidence of postoperative complications, including infective endocarditis and thromboembolism were comparable in all of the groups. CONCLUSION: Aortic root replacement can be safely performed with different types of prostheses as the outcome was not affected by the choice of prosthesis. Further studies are required to assess the long-term durability of biological prostheses.