Predictive Scoring System for Spontaneous Closure of Infant Ventricular Septal Defect: The P-VSD Score.

Ventricular septal defect (VSD) is a common congenital heart disease. However, consensus on the utility of echocardiography in predicting spontaneous closure (SC) of VSD remains lacking. This study aimed to identify and validate significant predictors of SC through a predictive scoring system. This retrospective study included medical records of 712 echocardiography instances performed on 304 patients diagnosed with VSD from 2016 to 2020 in their first year of life. A novel scoring system for predicting the SC of VSD was developed and validated using another dataset from different hospitals. Of the 304 patients, 215 (70.7%) had perimembranous (PM) VSDs and 89 had muscular (29.3%) VSDs. The median follow-up periods were 36.2 (interquartile range [IQR], 13-59) months and 13.7 9 (IQR, 5-37.4) days for PM and muscular VSDs, respectively. The overall SC rate during follow-up was 29.3%. Pulmonary hypertension (HTN), concomitant left ventricle (LV)-right atrium (RA) shunt, VSD size to aortic valve (AV) annulus size ratio, and left ventricular end-diastolic dimension (LVEDD) z-score were significant risk factors affecting SC of VSD. The "P-VSD" score, a new scoring system, demonstrated an area under the curve for predictability of 0.769. Pulmonary HTN, concomitant LV-RA shunt, LVEDD z-score, and VSD size-to-AV annulus size ratio at diagnosis were significantly associated with non-SC VSD after infancy. The P-VSD score can predict the SC of VSD in clinical settings and simplify the identification and appropriate management of high-risk patients.

Effects of Familial Relationships on School Adjustment of Adolescents and Young Adults With Congenital Heart Disease.

BACKGROUND: As more than 85% of patients with congenital heart disease (CHD) have grown to adulthood through improvement in treatment and surgery, the difficulties they experience are expanding into areas related to daily life. Accordingly, adjustment to school in adolescents and young adults (AYAs) with CHD is of increasing interest and is influenced by familial factors. OBJECTIVE: This was a cross-sectional descriptive study to examine the effects of parental positive emotional expressiveness and sibling relationships on school adjustment of AYAs with CHD. METHODS: In this study, a self-reported questionnaire survey was used to collect the data. The participants were 104 AYAs with CHD aged 13 to 21 years who were attending school and had siblings. RESULTS: Maternal positive emotional expressiveness ( r = 0.584, P < .01), paternal positive emotional expressiveness ( r = 0.584, P < .01), and sibling warmth/closeness ( r = 0.478, P < .01) were significantly correlated with school adjustment. However, the results of multiple regression analysis showed that only maternal positive emotional expressiveness (ß = 0.459, P < .05) and sibling warmth/closeness (ß = 0.236, P < .05) were significantly associated with school adjustment. CONCLUSIONS: Adolescents and young adults with CHD who reported higher maternal positive emotional expressiveness and sibling warmth/closeness exhibited better school adjustment. Findings suggest that intervention programs to increase parental positive expressiveness and enhance warmth/closeness of sibling relationships may contribute to improving school adjustment.

Successful Heart Transplant in Dilated Cardiomyopathy Associated With Alström Syndrome: A Case Report.

Alström syndrome is a rare, multisystemic genetic disorder, and dilated cardiomyopathy occurs in approximately two-thirds of patients with this condition. Because of donor organ shortage and unfavorable prognosis of multiple organ dysfunction, heart transplant is not the most desirable therapeutic option for patients with dilated cardiomyopathy with Alström syndrome. However, eliminating heart dysfunction elements at an appropriate time itself plays a pivotal role in preventing or even reversing other organ failures. Herein, we report the case of a 17-year-old boy who underwent successful isolated heart transplant despite severe liver dysfunction.

Heart Transplantation in a Patient with Complex Congenital Heart Disease, Physiologic Single Lung, and Severe Pulmonary Hypertension.

Here we report a case of successful heart transplantation (HT) in a patient with high risk on HT due to her complex congenital heart disease and its consequent complications; physiologic single lung and severe pulmonary arterial hypertension. HT was successfully performed in our patient using meticulous perioperative management techniques, such as control of collateral vessels, appropriate donor selection, pulmonary vasodilator therapy, post-transplant extracorporeal membranous oxygenation support, and atrial septostomy for right ventricular unloading.

Assessment of Device Neoendothelialization With Cardiac Computed Tomography Angiography After Transcatheter Closure of Atrial Septal Defect.

BACKGROUND: Although the transcatheter closure of atrial septal defect was established as the treatment of choice several decades ago, the process of device neoendothelialization (NE) in humans is not well understood. We aimed to measure the extent of device NE using cardiac computed tomography angiography and analyze its risk factors. METHODS: Between January 2005 and February 2021, we retrospectively reviewed 164 devices of 112 patients on cardiac computed tomography angiography. We investigated device shape, contrast opacification within the device that differentiated device NE, and device-related thrombosis or vegetation. Risk factor analysis for major adverse cardiovascular events and incomplete NE according to the postprocedural period was performed. RESULTS: Seventy patients (62.5%) were women, with a median (range) age at the time of device closure of 44.5 (0.6-79.2) years. The mean (±SD) defect size was 16.6 (±7.8) mm, and patients were followed for 35.9±33.9 months. After 6 months of device implantation, 35% of the devices (42/120) had incomplete NE. The intensity of intradevice opacification shifted from complete to partial or nonopacification over time (P<0.001), and a similar pattern was observed in the shunt flow (P<0.001). The bulkiness of devices also decreased in proportion to the postprocedural period (P<0.001). Risk analysis revealed device diameter (hazard ratio, 1.18 [95% CI, 1.04-1.27]; P<0.001) as the only significant factor of incomplete NE and major adverse events. CONCLUSIONS: Incomplete NE of atrial septal defect devices was identified on cardiac computed tomography angiography in significant numbers after 6 months of the procedure. The device diameter was related to incomplete NE and major adverse events. Further prospective and multicenter studies are warranted to validate this new assessment of device NE.

Intracardiac Echocardiogram: Feasibility, Efficacy, and Safety for Guidance of Transcatheter Multiple Atrial Septal Defects Closure.

We aimed to determine the feasibility, efficacy, success, and safety of intracardiac echocardiography (ICE) in transcatheter multiple atrial septal defect (ASD) closure. Of 185 patients with multiple ASDs who underwent transcatheter closure, 140 (76%) patients who weighed <30kg with a narrow distance between defects or in whom single device closure was anticipated were guided by ICE and 45 patients were guided by three-dimensional (3D) transesophageal echocardiography (TEE) with or without ICE. Patients in the ICE group were relatively younger and weighed less than those in the 3D TEE group (p < 0.0001). The ratio of the distance between defects >7 mm was high, and more cases required ≥2 devices in the 3D TEE group than those in the ICE group (p < 0.0001). All patients in the 3D TEE group and seven patients (5%) in the ICE group were operated on under general anesthesia (p < 0.0001). The fluoroscopic time was shorter in the ICE group (13.98 ± 6.24 min vs. 24.86 ± 16.47 min, p = 0.0005). No difference in the complete closure rate and complications was observed. ICE-guided transcatheter and 3D TEE were feasible, safe, and effective in successful multiple ASD device closures, especially for young children and patients at high risk under general anesthesia.

Cardiovascular events and safety outcomes associated with remdesivir using a World Health Organization international pharmacovigilance database.

On October 2020, the US Food and Drug Administration (FDA) approved remdesivir as the first drug for the treatment of coronavirus disease 2019 (COVID-19), increasing remdesivir prescriptions worldwide. However, potential cardiovascular (CV) toxicities associated with remdesivir remain unknown. We aimed to characterize the CV adverse drug reactions (ADRs) associated with remdesivir using VigiBase, an individual case safety report database of the World Health Organization (WHO). Disproportionality analyses of CV-ADRs associated with remdesivir were performed using reported odds ratios and information components. We conducted in vitro experiments using cardiomyocytes derived from human pluripotent stem cell cardiomyocytes (hPSC-CMs) to confirm cardiotoxicity of remdesivir. To distinguish drug-induced CV-ADRs from COVID-19 effects, we restricted analyses to patients with COVID-19 and found that, after adjusting for multiple confounders, cardiac arrest (adjusted odds ratio [aOR]: 1.88, 95% confidence interval [CI]: 1.08-3.29), bradycardia (aOR: 2.09, 95% CI: 1.24-3.53), and hypotension (aOR: 1.67, 95% CI: 1.03-2.73) were associated with remdesivir. In vitro data demonstrated that remdesivir reduced the cell viability of hPSC-CMs in time- and dose-dependent manners. Physicians should be aware of potential CV consequences following remdesivir use and implement adequate CV monitoring to maintain a tolerable safety margin.

Using Etomidate in a Two-month-old Infant with Cushing Syndrome due to Adrenocortical Carcinoma

Cushing syndrome (CS) is a rare disease caused by hypercortisolemia. Although surgical treatment is the first-line treatment in CS, the appropriate medication for the patient's condition should be selected when medical treatment is needed. Etomidate is an adrenal-blocking drug used to treat CS and the most suitable for severe hypercortisolemia and adrenocortical carcinoma (ACC), due to cardiovascular stability and an anti-tumorigenic effect. However, its use and safe recommended dosage in infants with CS is unreported. Here we describe the case of a 2-month-old girl treated with etomidate for CS caused by ACC. Even though radical mass excision was performed, severe hypercortisolemia persisted, resulting from metastatic lesions in the liver, and medical treatment was considered. The etomidate doses, no bolus dose and infusion rate of 0.03 mg/kg/hour, may be an appropriate dose for severe hypercortisolemia in infants. This case will help determine future treatment strategies for similar cases in infants.

Sibling Relationships of Adolescents with Congenital Heart Disease.

Adolescents with congenital heart disease (CHD) continuously need family support because of their repeated follow ups, treatments, and complications. However, sibling relationships have not been well studied among adolescents with CHD. The purpose of the present study was to explore the relationships between adolescents with CHD and their siblings, and to examine these relationships according to birth order and age. Adolescents aged from 13 to 21 years who had been diagnosed with CHD and had siblings were included as participants. The Sibling Relationship Questionnaire (SRQ) was used. The SRQ consists of four factors: warmth/closeness, conflict, relative power/status, and rivalry. A univariate general linear model was conducted to identify the sibling relationship factors according to birth order and sibling ages. The score for relative power/status of participants who were the eldest sibling was higher than that of younger siblings. The score for rivalry increased as sibling age increased. Therefore, healthcare providers need to investigate sibling relationships and to explain the importance of self-identity and power balance between adolescents with CHD and their siblings to parents.

Early Outcomes of Percutaneous Pulmonary Valve Implantation with Pulsta and Melody Valves: The First Report from Korea.

Percutaneous pulmonary valve implantation (PPVI) is used to treat pulmonary stenosis (PS) or pulmonary regurgitation (PR). We described our experience with PPVI, specifically valve-in-valve transcatheter pulmonary valve replacement using the Melody valve and novel self-expandable systems using the Pulsta valve. We reviewed data from 42 patients undergoing PPVI. Twenty-nine patients had Melody valves in mostly bioprosthetic valves, valved conduits, and homografts in the pulmonary position. Following Melody valve implantation, the peak right ventricle-to-pulmonary artery gradient decreased from 51.3 ± 11.5 to 16.7 ± 3.3 mmHg and right ventricular systolic pressure fell from 70.0 ± 16.8 to 41.3 ± 17.8 mmHg. Thirteen patients with native right ventricular outflow tract (RVOT) lesions and homograft underwent PPVI with the new self-expandable Pulsta valve-a nitinol wire stent mounted with a trileaflet porcine pericardial valve. Following Pulsta valve implantation, cardiac magnetic resonance imaging showed a decreased PR fraction and that the right ventricular end-diastolic volume index decreased from 166.1 ± 11.9 to 123.6 ± 12.4 mL/m2. There were no mortality, severe procedural morbidity, or valve-related complications. At the mean 14.2 month (4-57 months) follow-up, no patients had more than mild PR. PPVI using Melody and Pulsta valves was first shown to provide excellent early outcomes without serious adverse event in most patients with RVOT dysfunction in Korea.

The First Pediatric Heart Transplantation Bridged by a Durable Left Ventricular Assist Device in Korea.

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.

Evaluation of Flow Pattern in the Ascending Aorta in Patients with Repaired Tetralogy of Fallot Using Four-Dimensional Flow Magnetic Resonance Imaging.

OBJECTIVE: To evaluate flow pattern characteristics in the ascending aorta (AA) with four-dimensional (4D)-flow MRI and to determine predictors of aortic dilatation late after tetralogy of Fallot (TOF) repair. MATERIALS AND METHODS: This study included 44 patients with repaired TOF (25 males and 19 females; mean age, 28.9 ± 8.4 years) and 11 volunteers (10 males and 1 female, mean age, 33.7 ± 8.8 years) who had undergone 4D-flow MRI. The aortic diameters, velocity, wall shear stress (WSS), flow jet angle (FJA), and flow displacement (FD) at the level of the sinotubular junction (STJ) and mid-AA were compared between the repaired TOF and volunteer groups. The hemodynamic and clinical parameters were also compared between the aortic dilatation and non-dilatation subgroups in the repaired TOF group. RESULTS: The diameters of the sinus of Valsalva, STJ, and AA were significantly higher in the repaired TOF group than in the volunteer group (p = 0.002, p < 0.001, and p = 0.013, respectively). The FJAs at the STJ and AA were significantly greater in the repaired TOF group (p < 0.001 and p = 0.003, respectively), while velocities and WSS parameters were significantly lower. FD showed no statistically significant difference (p = 0.817). In subgroup analysis, age at TOF repair was significantly higher (p = 0.039) and FJA at the level of the AA significantly greater (p = 0.003) and mean WSS were significantly lower (p = 0.039) in the aortic dilatation group. FD were higher in the aortic dilatation group without statistical significance (p = 0.217). CONCLUSION: Patients with repaired TOF have an increased FJA, dilated AA, and secondarily decreased WSS. In addition to known risk factors, flow eccentricity may affect aortic dilatation in patients with repaired TOF.

Procedural, Early and Long-term Outcomes after Percutaneous Closure of Atrial Septal Defect: Comparison between Large and Very Large Atrial Septal Defect Groups.

BACKGROUND: This study aimed to compare procedural, early and long-term outcome of device closure of atrial septal defect (ASD) between large ASD patients and very large ASD patients. METHODS: We carried a retrospective study of adult large ASD (defined as ≥25 mm) treated by percutaneous closure using Amplatzer septal occluder during 12-year period (May 2003-February 2015) at a single tertiary center. A total of 269 patients were divided into 2 groups, according to the pre-procedural maximal ASD diameter; 25 mm≤ASD<35mm, group 1 (n=216) and 35 mm≤ASD, group 2 (n=53). We compared procedural parameters, early complications and long-term follow-up results between 2 groups. RESULTS: The need of modified implantation techniques (MITs) was higher group 2 (23.6% vs. 37.7%, p=0.034). Procedural success rate was considerably high in both groups (99.1% in group 1 vs. 100% in group 2, p=0.620). Major complications were occurred in 4 (1.5%) patients (1.4% vs. 1.9%, p=0.804). Minor complication rate was not different between 2 groups. During long-term follow-up (47.2±32.0 months, range, 6.0-135.5), there was one major complication (0.4%) of stroke. Most common long-term minor event was migraine headache (3.9%) followed by arrhythmias (1.9%) without statistical difference between 2 groups. CONCLUSIONS: Although MIT was more frequently required in very large ASD groups, the procedural, early and long-term outcomes after percutaneous ASD closure were similar in both groups. This suggested that percutaneous ASD closure for very large ASD could be considered a good treatment option.

Early to Mid-Term Follow-Up Outcomes of Percutaneous Closure of Atrial Septal Defects Using Recent Generation Devices: a Single-Center Experience.

BACKGROUND AND OBJECTIVES: This study aimed to describe our early to mid-term experience with transcatheter atrial septal defect (ASD) closure using the Occlutech Figulla® Flex II device (FSO), Gore® Cardioform septal occluder (GSO), and Amplatzer® septal occluder (ASO) after they were first approved in Korea in 2014, and to compare the three aforementioned kinds of ASD closure devices. METHODS: Between September 2014 and August 2016, 267 patients underwent transcatheter ASD closure in our institution. Baseline characteristics, hemodynamic features, comorbidities, and procedural success and complication rates were analyzed retrospectively. The unpaired Student t-test or variance analysis was used in the statistical analysis. RESULTS: The FSO was most commonly used (n=152, 56.9%), followed by the ASO (n=98, 36.7%) and GSO (n=17, 6.4%). Baseline characteristics and hemodynamic features were similar between the devices, except that the defect size and pulmonary flow-to-systemic flow ratio were lower in the GSO group than in the other groups. Overall, the procedural success rate remained at 100%, and major complication rate was <1%. No late complication occurred during the follow-up. CONCLUSIONS: The FSO and GSO are feasible, safe options for use in transcatheter ASD closure, and they are comparable to the ASO.

Impact of Flow Differentials According to Cardiac and Respiratory Cycles on Three Types of Fontan Operation.

Few hemodynamic comparison studies on various types of Fontan operation have been reported. The objective of this study was to perform hemodynamic comparisons for flow size and volume in three types of Fontan operation: atriopulmonary connection (APC), lateral tunnel (LT), and extracardiac conduit (ECC). Forty patients with Fontan operation (8 with APC Fontan, 22 with LT Fontan, and 10 with ECC Fontan) were enrolled. Velocity time integral (VTI) and average peak velocity (APV) were assessed according to cardiac and respiratory cycles in SVC, IVC, hepatic vein, conduit, LPA, and RPA using direct intravenous Doppler echocardiography. During each cardiac cycle in APC, VTI and APV between inspiration and expiration did not show significant differences in SVC, IVC, HV, LPA, or RPA. During each cardiac cycle in LT and ECC, VTI and APV between inspiration and expiration showed significant differences in all native vessels. The gap between S and D wave in APC was the highest, followed by that in LT. It was the lowest in ECC regardless of inspiration or expiration. Hepatic reverse VTI and APV in APC showed significant decreases compared to those in VC and PA during inspiration and expiration. Flow size and volume in APC were more influenced by cardiac cycle. Those in LT were moderately influenced by both respiratory cycle and cardiac cycle while those in ECC were more influenced by respiratory cycle. APC Fontan has hemodynamic inefficiency with prominent reverse flow. However, total cavopulmonary connection (TCPC) Fontan has more hemodynamic efficiency without prominent reverse flows.

A stepwise approach to conduit puncture for electrophysiological procedures in patients with Fontan circulation.

Aims: In patients with Fontan circulation, the conduit may be punctured for electrophysiological procedures. We evaluated the feasibility and safety of a stepwise approach to conduit puncture in adults who have undergone Fontan operation. Methods and results: We included 13 consecutive patients with lateral tunnel or extracardiac conduit Fontan circulation [median age (interquartile range), 24.0 (16.0-25.0) years; seven men] who had undergone electrophysiological procedures. We performed a stepwise approach to conduit puncture: 1st, Brockenbrough needle; 2nd, Brockenbrough needle with snare; 3rd, extra-steep Brockenbrough needle with/without snare; 4th radiofrequency transseptal needle with/without snare; 5th, wiring through the puncture; 6th, conduit dilation with angioplasty balloon; 7th, non-compliant or cutting balloon; and 8th, Inoue dilator. In 12 patients, conduit puncture was successful. In two, one, and two patients with a lateral tunnel made of the pericardium or right atrial wall, conduit puncture was performed by steps 1st, 2nd, and 4th, respectively. In one, three, two, and one patient with the Goretex lateral tunnel or extracardiac conduit, conduit puncture was performed by steps 1st, 6th, 7th, and 8th, respectively. Puncture time was significantly longer in patients with Goretex conduits than with pericardial conduits [62.0 (50.0-120.0) and 11.5 (10.0-14.8) min, respectively; P < 0.001]. A snare was necessary in patients with angles ≤ 35° between the conduit wall and vertical line. Conclusion: A stepwise conduit puncture approach is feasible and safe in patients with lateral tunnel and extracardiac conduit Fontan circulation. Goretex conduit puncture was more difficult than pericardial conduit puncture.

Detection of mechanical complications related to the potential risk of sudden cardiac death in patients with pulmonary arterial hypertension by computed tomography.

BACKGROUND: We explored the value of cardiac computed tomography (CT) for the detection and prediction of mechanical complications related to the risk of sudden cardiac death (SCD) in pulmonary arterial hypertension (PAH) patients. METHODS: PAH patients (n=60, mean age 47±15, 31.7% male) with pulmonary artery (PA) enlargement (≥40mm) by echocardiography were studied with cardiac CT. Complications explored were the presence of left main coronary artery (LM) compression, airway compression, PA dissection and PA thrombosis in relation to diameters of main PA (MPA) which were measured in (1) axial plane (MPAAx) and (2) LM oblique view (MPALMobq). RESULTS: Mechanical complications were found in 21 patients (35.0%): LM compression in 20 patients; airway compression in 3 patients; and PA thrombosis in 4 patients. Patients with complications had more dilated MPALMobq than patients without complication (59.4±13.0mm vs. 42.4±7.0mm, p<0.001). The area under the receiver operating characteristic curve for MPALMobq was 0.889 (95% confidence interval: 0.795 to 0.983, p<0.001) with the highest discriminating sensitivity and specificity being 90.5% and 69.2%, respectively at MPALMobq of 45mm. MPAAx failed to predict the presence of mechanical complications (p>0.05). CONCLUSION: MPALMobq≥45mm was significantly associated with the presence of mechanical complications of PAH. Evaluation with CT should be considered in PAH patients with dilated MPA.

Retrospective Multicenter Study of Respiratory Syncytial Virus Prophylaxis in Korean Children with Congenital Heart Diseases.

BACKGROUND AND OBJECTIVES: We conducted a review of current data on respiratory syncytial virus (RSV) prophylaxis with palivizumab, in Korean children with congenital heart diseases (CHD). In 2009, the Korean guideline for RSV prophylaxis had established up to five shots monthly per RSV season, only for children <1 year of age with hemodynamic significance CHD (HS-CHD). SUBJECTS AND METHODS: During the RSV seasons in 2009-2015, we performed a retrospective review of data for 466 infants with CHD, examined at six centers in Korea. RESULTS: Infants received an average of 3.7±1.9 (range, 1-10) injections during the RSV season. Fifty-seven HS-CHD patients (12.2%) were hospitalized with breakthrough RSV bronchiolitis, with a recurrence in three patients, one year after the initial check-up. Among patients with simple CHD, only five (1.1%) patients received one additional dose postoperatively, as per the limitations set by the Korean guideline. Among the 30 deaths (6.4%), five (1.1%) were attributed to RSV infection; three to simple CHD, one to Tetralogy of Fallot, and one to hypertrophic cardiomyopathy (HCM). Of the three HCM patients that exceeded guidelines for RSV prophylaxis, two (66.6%) were hospitalized, and one died of RSV infection (33.3%). CONCLUSION: In accordance to the Korean guideline, minimal injections of palivizumab were administered to patients having HS-CHD