Impact of the Grade and Jet-flow Direction of Residual Aortic Regurgitation after Valve-Sparing Root Replacement.

OBJECTIVES: The current study aimed to investigate the impact of the grade and jet direction of residual aortic regurgitation (rAR) after valve-sparing root replacement (VSRR). METHODS: The study enrolled 248 adult patients who underwent VSRR from 1995 to 2021. The patients were divided into groups based on the postoperative rAR. Patients with rAR were further categorized per the rAR grade and jet direction. The primary endpoint was the development of aortic regurgitation ≥ moderate and/or the need for valve replacement during the follow-up, analyzed by a multivariable competing risk analysis. The secondary endpoints included the occurrence of rAR and overall survival. RESULTS: The median age of the patients was 36.5 years, and 79.8% were diagnosed with connective tissue disease. After VSRR, 146 patients did not present with rAR. However, 102 had rAR (77 with minimal central, 18 with minimal eccentric, and 7 with mild). The 5- and 8-year incidence rates of the primary endpoint were 14.6% and 17.9%, respectively. The rAR was a significant risk factor (P=0.001), and eccentricity and mild rAR seemed to play an important role. The risk factors of rAR included dilated root, preoperative moderate regurgitation, and redo sternotomy. The overall survival was influenced only by age. CONCLUSION: rAR after VSRR operation could be a risk factor for AR progression. Minimal central rAR generally has a tolerable clinical course. However, patients with even minimal eccentric AR may develop AR progression, so active surveillance and timely management might be required. Further, early VSRR can help reduce the rAR.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Long-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. CONCLUSIONS: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Successful Modified Nikaidoh Procedure (Pivot Rotation) in a Patient with Double Outlet Right Ventricle and Pulmonary Atresia: Case Report.

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

Long-term extracorporeal membrane oxygenation after severe blunt traumatic lung injury in a child.

Managing acute respiratory distress syndrome (ARDS) after severe blunt traumatic lung injury can be challenging. In cases where patients are refractory to conventional therapy, extracorporeal membrane oxygenation (ECMO) should be considered. In addition, the heparin-coated circuit can reduce hemorrhagic complications in patients with multiple traumas. Although prolonged ECMO may be necessary, excellent outcomes are frequently associated. In this study, we report long-term support with venovenous-ECMO applied in a child with severe blunt trauma in Korea. This 10-year-old and 30 kg male with severe blunt thoracic trauma after a car accident developed severe ARDS a few days later, and ECMO was administered for 33 days. Because of pulmonary hemorrhage during ECMO support, heparin was stopped for 3 days and then restarted. He was weaned from ECMO successfully and has been able to run without difficulty for the 2 years since discharge.

Validation of the Pediatric Index of Mortality 3 in a Single Pediatric Intensive Care Unit in Korea.

To compare mortality rate, the adjustment of case-mix variables is needed. The Pediatric Index of Mortality (PIM) 3 score is a widely used case-mix adjustment system of a pediatric intensive care unit (ICU), but there has been no validation study of it in Korea. We aim to validate the PIM3 in a Korean pediatric ICU, and extend the validation of the score from those aged 0-16 to 0-18 years, as patients aged 16-18 years are admitted to pediatric ICU in Korea. A retrospective cohort study of 1,710 patients was conducted in a tertiary pediatric ICU. To validate the score, the discriminatory power was assessed by calculating the area under the receiver-operating characteristic (ROC) curve, and calibration was evaluated by the Hosmer-Lemeshow goodness-of-fit (GOF) test. The observed mortality rate was 8.47%, and the predicted mortality rate was 6.57%. For patients aged < 18 years, the discrimination was acceptable (c-index = 0.76) and the calibration was good, with a χ² of 9.4 in the GOF test (P = 0.313). The observed mortality rate in the hemato-oncological subgroup was high (18.73%), as compared to the predicted mortality rate (7.13%), and the discrimination was unacceptable (c-index = 0.66). In conclusion, the PIM3 performed well in a Korean pediatric ICU. However, the application of the PIM3 to a hemato-oncological subgroup needs to be cautioned. Further studies on the performance of PIM3 in pediatric patients in adult ICUs and pediatric ICUs of primary and secondary hospitals are needed.

Distinctive clinical features of HPeV-3 infection in 2 neonates with a sepsis-like illness.

We report a human parechovirus-3 (HPeV-3) infection in 2 neonates who had prolonged fever (>5 days) with palmar-plantar erythema. This distinctive rash was observed 4-5 days after fever onset, just before defervescence. Elevated aspartate aminotransferase, lactate dehydrogenase, and ferritin levels were characteristic laboratory findings in the 2 cases, suggesting tissue damage caused by hypercytokinemia. Case 1 was treated with intravenous immunoglobulin, considering the possibility of severe systemic inflammatory responses. The initial ferritin level was 385 ng/mL (range, 0-400 ng/mL); however, the level increased to 2,581 ng/dL on day 5 after fever onset. Case 2 presented with milder clinical symptoms, and the patient recovered spontaneously. HPeV-3 was detected in cerebrospinal fluid and/or blood samples, but no other causative agents were detected. The findings from our cases, in accordance with recent studies, suggest that clinical features such as palmar-plantar erythema and/or hyperferritinemia might be indicators of HPeV-3 infection in neonates with sepsis-like illness. In clinical practice, where virology testing is not easily accessible, clinical features such as palmar-plantar erythema and/or hyperferritinemia might be helpful to diagnose HPeV-3 infection.

A rare type of single coronary artery with right coronary artery originating from the left circumflex artery in a child.

The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG) was normal. Echocardiography showed normal anatomy and normal regional wall motion. When she presented with recurrent chest pain on admission, the ECG showed significant ST-segment elevation in the left precordial leads and inferior leads with ST-segment depression in aVR lead, suggesting myocardial ischemia, and her cardiac enzyme levels were also elevated. We performed coronary angiography that showed a single right coronary artery originating from the left circumflex artery without stenosis. We confirmed the presence of a single coronary artery using coronary computed tomography. In addition, the treadmill test that was performed showed normal results. She was discharged from the hospital without any medications but with a recommendation of a regular follow-up.

Clinical implications of serial serum N-terminal prohormone brain natriuretic peptide levels in the prediction of outcome in children with dilated cardiomyopathy.

N-terminal prohormone brain natriuretic peptide (NT-proBNP) is known to be valuable as a prognostic marker for the management of heart failure. This study aimed to assess the serial changes of serum NT-proBNP levels as a prognostic factor for outcomes of pediatric dilated cardiomyopathy (DC). A retrospective study reviewed echocardiographic measurements and serum NT-proBNP levels obtained at the time of diagnosis, 3 and 6 months after diagnosis, and at the last follow-up in 68 children with DC. They were classified into 3 groups according to the last follow-up of the left ventricular (LV) function: group I (n = 37) with LV fractional shortening (FS) ≥25%, group II (n = 15) with 10% ≤ LVFS < 25%, and group III (n = 16) with LVFS <10% or cardiac death. The median age at diagnosis was 30 months and median duration of follow-up was 45 months (range 8 to 108 months). The origin of DC varied. Serial serum NT-proBNP levels showed statistically significant differences among the 3 groups. Although initial levels of serum NT-proBNP at diagnosis were not different from each other, group III maintained a higher NT-proBNP level throughout the duration of the follow-up. On multivariate Cox analysis, only the NT-proBNP level at 3 months was a significant predictor of an adverse outcome (hazard ratio 2.012, p = 0.028). The percent changes of NT-proBNP from diagnosis were not significant. Patients with DC with a serum NT-proBNP level ≥681 pg/ml at 3 months after diagnosis showed an increased risk of severe LV dysfunction or cardiac death (p <0.001, area under the curve = 0.846). In conclusion, the level of NT-proBNP in serum at 3 months after diagnosis could predict an adverse outcome in pediatric DC and could be used as a guide for long-term treatment plans.

Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center.

BACKGROUND: In spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors. METHODS: From November 1994 until February 2012, all patients that had a functional single ventricle and heterotaxy syndrome who underwent palliative operations at our center were included. Patients with hypoplastic left heart syndrome and operative mortality cases were excluded. The factors that influenced interstage mortality were determined by multivariate Cox analysis. RESULTS: There were 16 patients with interstage mortality (41.0%), much higher than the non-heterotaxy group (vs. 11.3%, P = 0.001, OR = 5.478). The major presumptive causes of death were infection or sepsis (37.5%) and unknown sudden death (31.3%). When we compared the survival group and the mortality group with heterotaxy syndrome, Blalock-Taussig shunt as a 1st palliation is most common for both groups but there were more for the mortality group (81.2% vs. 52.2%), and there were more with bidirectional cavo-pulmonary shunt as a 1st palliation in the survival group (10 patients vs. 2 patients). The existence of pulmonary vein stenosis at initial diagnosis was more common for the mortality group. In multivariate Cox analysis, however, the duration of hospitalization at palliation, the duration of intensive care unit stay after palliation and the existence of pulmonary vein stenosis at diagnosis were significant risk factors. CONCLUSION: Interstage mortality for a functional single ventricle with heterotaxy syndrome is significantly higher than for non-heterotaxy syndrome. Therefore more attention should be given to the prevention of interstage mortality in these patients with risk factors.

A study of the relationship between clinical phenotypes and plasma iduronate-2-sulfatase enzyme activities in Hunter syndrome patients.

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-α-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol·4 hr(-1)·mL(-1). This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.

2009 H1N1 influenza virus infection and necrotizing pneumonia treated with extracorporeal membrane oxygenation.

A 3-year-old girl with acute respiratory distress syndrome due to a H1N1 2009 influenza virus infection was complicated by necrotizing pneumonia was successfully treated with extracorporeal membrane oxygenation (ECMO). This is the first reported case in which a pediatric patient was rescued with ECMO during the H1N1 influenza epidemic in Korea in 2009.