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1.
Circ J ; 88(5): 652-662, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38325847

ABSTRACT

BACKGROUND: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown.Methods and Results: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021. Among 341 patients, 25 (7.3%) were identified with CACs, and 2 of them died. Among the patients with CACs, congenital coronary anomalies and an interarterial course of the coronary artery were identified in 11 (44%) and 18 (72%) patients, respectively. Interarterial and intramural courses of the coronary artery were associated with a 4.4- and 10.6-fold increased risk of CACs, respectively. Among patients with tetralogy of Fallot and pulmonary atresia, the aortic root was rotated further clockwise in patients with coronary artery compression compared to those without it (mean [±SD] 128.0±19.9° vs. 113.5±23.7°; P=0.024). The cut-off rotation angle of the aorta for predicting the occurrence of coronary artery compression was 133°. CONCLUSIONS: Perioperative coronary artery evaluation and prevention of CACs are required in patients undergoing prosthetic pulmonary valve implantation, particularly in those with coronary artery anomalies or severe clockwise rotation of the aortic root.


Subject(s)
Pulmonary Valve , Humans , Retrospective Studies , Female , Male , Risk Factors , Adult , Pulmonary Valve/surgery , Pulmonary Valve/diagnostic imaging , Heart Valve Prosthesis Implantation/adverse effects , Heart Defects, Congenital/surgery , Coronary Artery Disease/etiology , Coronary Artery Disease/surgery , Coronary Artery Disease/diagnostic imaging , Coronary Angiography , Adolescent , Young Adult , Heart Valve Prosthesis/adverse effects , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessel Anomalies/complications , Incidence , Child , Middle Aged , Tetralogy of Fallot/surgery
2.
Pediatr Cardiol ; 2024 Jan 29.
Article in English | MEDLINE | ID: mdl-38285085

ABSTRACT

Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria in pediatric patients. This retrospective study aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. CT images, echocardiography findings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated. Aortic size on follow-up CT, blood pressure, and left ventricular function and hypertrophy at the last follow-up were analyzed, and possible prognostic factors were examined. Except for one patient, all individuals exhibited a z-score below - 2 at the level corresponding to stenosis. Left ventricular dysfunction occurred more frequently in patients aged < 5 years (p = 0.024). Patients with idiopathic MAS showed a better prognosis in terms of blood pressure and follow-up aortic size (p = 0.051 and 0.048, respectively). CT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS.

3.
J Pediatr ; 262: 113351, 2023 11.
Article in English | MEDLINE | ID: mdl-36806754

ABSTRACT

INTRODUCTION: To investigate cardiovascular characteristics and progressions of hypertrophic cardiomyopathy (HCM) and pulmonary stenosis (PS) and determine whether any genotype-phenotype correlations exist in patients with gene-confirmed RASopathy syndrome. STUDY DESIGN: Eighty patients (male, 55%) confirmed as having RASopathy syndrome by genetic testing at a single tertiary center were enrolled. Subjects' medical and echocardiography records were reviewed and the changes in the z scores of left ventricular wall thickness (LVWT) and the degree of PS over time were examined during follow-up of 5.7 ± 3.1 and 7.5 ± 5.2 years, respectively. RESULTS: The most common RASopathy gene identified was PTPN11 (56%), followed by RAF1 (10%). Eighty-five percent of patients had cardiovascular diseases, wherein 42% had HCM, and 38% PS. Mean maximal LVWT z score on the initial echocardiography (mean age 5.0 ± 6.0 years) was 3.4 ± 1.3 (median 2.8, range 2.1-6.6) in the HCM group. Overall, the maximal LVWT increased with time, especially in the HCM group (z = 3.4 ± 1.3 to 3.7 ± 1.6, P = .008) and RAF1-variant group (z = 3.7 ± 1.7 to 4.6 ± 1.8, P = .031). Five patients newly developed HCM during the study period. Genotype-phenotype correlation was significant for HCM (P = .002); 31% of patients with PTPN11 and 88% with RAF1 variants had HCM. PS did not progress in this study cohort. CONCLUSIONS: In this study, progression of ventricular hypertrophy was seen in a significant number of patients with genotype correlation. Thus, long-term follow up of cardiovascular problems in patients with RASopathy is necessary.


Subject(s)
Cardiomyopathy, Hypertrophic , Pulmonary Valve Stenosis , Humans , Male , Child, Preschool , Child , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/genetics , Genetic Testing , Genotype , Genomics , Pulmonary Valve Stenosis/complications , Pulmonary Valve Stenosis/genetics
4.
Cardiol Young ; 33(11): 2157-2163, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37496425

ABSTRACT

BACKGROUND: To avoid rhythm disturbance, sutures for ventricular septal defect closure have been traditionally placed 2∼5 mm or more away from the edge of the ventricular septal defect. However, the traditional suturing method appears to induce right bundle branch block and tricuspid valve regurgitation after ventricular septal defect closure more than our alternative technique, shallow suturing just at the edge of the ventricular septal defect (shallower bites at the postero-inferior margin). We aimed to verify our clinical experience of perimembranous ventricular septal defect repair. METHODS: The alternative shallow suturing method has been applied since 2003 at our institution. We retrospectively reviewed the clinical data of 556 isolated perimembranous ventricular septal defect patients who underwent surgical closure from 2000 to 2019. We investigated the postoperative occurrence of right bundle branch block or progression of tricuspid regurgitation and analysed risk factors for right bundle branch block and tricuspid regurgitation. RESULTS: Traditional suturing method (Group T) was used in 374 cases (66.8%), and alternative suturing method (Group A) was used in 186 cases (33.2%). The right bundle branch block occurred more frequently in Group T (39.6%) than in Group A (14.9%). In multivariable logistic regression analysis, Group T and patch material were significant risk factors for late right bundle branch block. More patients with progression of tricuspid regurgitation were found in Group T. CONCLUSIONS: Shallow suturing just at the edge of the ventricular septal defect may reduce the rate of right bundle branch block occurrence and tricuspid regurgitation progression without other complications.


Subject(s)
Heart Septal Defects, Ventricular , Tricuspid Valve Insufficiency , Humans , Bundle-Branch Block/etiology , Tricuspid Valve Insufficiency/surgery , Tricuspid Valve Insufficiency/complications , Retrospective Studies , Heart Septal Defects, Ventricular/complications , Sutures/adverse effects
5.
Circ J ; 85(8): 1356-1364, 2021 07 21.
Article in English | MEDLINE | ID: mdl-33980762

ABSTRACT

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.Methods and Results:Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019. Altogether, 99 patients were enrolled (mean age 13.9±4.1 years). The most common underlying disease was a primary electrical disease (56%). An ICD was implanted for primary prevention in 19%. Appropriate shock occurred in 44% of patients at a median of 1.6 years after implantation. There was no significant difference in the appropriate shock rate between patients with primary and secondary prevention indications (32% vs. 48%, respectively). A total of 33 patients (33%) experienced inappropriate shock, which was associated with primary electrical disease and follow-up duration on multivariate analysis. 17% of patients had ICD-related complications. CONCLUSIONS: The utilization rate of ICD for primary prevention was still low in the pediatric population in Korea, but there was a substantial rate of appropriate shock in these patients. Efforts to increase ICD usage to save the lives of high-risk patients and reduce the incidence of inappropriate shock are required.


Subject(s)
Defibrillators, Implantable , Adolescent , Child , Death, Sudden, Cardiac/prevention & control , Follow-Up Studies , Humans , Retrospective Studies , Risk Factors , Treatment Outcome
6.
BMC Pediatr ; 21(1): 25, 2021 01 08.
Article in English | MEDLINE | ID: mdl-33413220

ABSTRACT

BACKGROUND: Because of the absence of a specific diagnostic test and pathognomonic clinical features, physicians must rely on the presence of specific clinical criteria and laboratory data that support the diagnosis of KD. To help clinicians distinguish KD, KDSS, septic shock, and TSS earlier, we suggest differential diagnosis and treatment guideline. METHODS: Medical records of immunocompetent patients who were admitted to the pediatric department with a diagnosis of KDSS, septic shock or TSS (SS group) were retrospectively reviewed. In addition, KD patients were selected by seasonal matching to each case of KDSS patient by date of admission (± 2 weeks). RESULTS: There were 13 patients with KDSS, 35 patients with SS group, and 91 patients with KD. In comparison between KDSS and septic shock group, KDSS group had significantly higher rate of coronary aneurysm incidence, and higher left ventricle dysfunction rate. In comparison between KDSS and TSS, patients with KDSS had a significantly higher erythrocyte sedimentation rate (ESR) and significantly lower creatinine. Receiver operation characteristic curve revealed that the optimal ESR cut off value for determining the KDSS was 56.0 (sensitivity 75.0%, specificity of 100.0%) and the optimal creatinine cut off value for determining the TSS was 0.695 (sensitivity 76.9%, specificity 84.6%). CONCLUSIONS: Clinical symptoms, laboratory finding, echocardiography, and culture studies can be used to differentiate KD, KDSS, septic shock and TSS.


Subject(s)
Mucocutaneous Lymph Node Syndrome , Shock, Septic , Shock , Case-Control Studies , Child , Diagnosis, Differential , Humans , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Retrospective Studies , Shock/diagnosis , Shock/etiology , Shock, Septic/diagnosis , Shock, Septic/etiology
7.
Infection ; 48(5): 783-790, 2020 Oct.
Article in English | MEDLINE | ID: mdl-32474827

ABSTRACT

Abiotrophia defectiva is a rare pathogen of infective endocarditis (IE) but is frequently involved in embolic complication and valvular dysfunction. IE caused by A. defectiva in children is poorly studied. This study reports four cases of A. defectiva IE in children and reviews previously reported five pediatric cases of A. defectiva IE. Most of the patients presented with a subacute course, with prolonged fever or atypical symptoms. Eight patients had embolic complications at presentation. All nine children were treated with combination antimicrobial therapy and six of them received surgical intervention. All patients recovered well without relapse. A. defectiva should be considered in children with infective endocarditis, especially in those with atypical presentations. As complications are frequent and more than half of the patients need surgical treatment, prompt diagnosis along with appropriate treatment is necessary.


Subject(s)
Abiotrophia/physiology , Anti-Bacterial Agents/therapeutic use , Endocarditis, Bacterial/pathology , Gram-Positive Bacterial Infections/pathology , Adolescent , Child , Child, Preschool , Drug Therapy, Combination , Endocarditis, Bacterial/complications , Endocarditis, Bacterial/microbiology , Female , Gram-Positive Bacterial Infections/complications , Gram-Positive Bacterial Infections/microbiology , Humans , Male , Republic of Korea , Treatment Outcome
8.
BMC Cardiovasc Disord ; 20(1): 227, 2020 05 15.
Article in English | MEDLINE | ID: mdl-32414329

ABSTRACT

BACKGROUND: As a result of advances in pediatric care and diagnostic testing, there is a growing population of adults with congenital heart disease (ACHD). The purpose of this study was to better define the epidemiology and changes in the trend of hospitalizations for ACHD in Korean society. METHODS: We reviewed outpatient and inpatient data from 2005 to 2017 to identify patient ≥18 years of age admitted for acute care with a congenital heart disease (CHD) diagnosis in the pediatric cardiology division. We tried to analyze changes of hospitalization trend for ACHD. RESULTS: The ratio of outpatients with ACHD increased 286.5%, from 11.1% (1748/15,682) in 2005 to 31.8% (7795/24,532) in 2017. The number of ACHD hospitalizations increased 360.7%, from 8.9% (37/414) in 2005 to 32.1% (226/705) in 2017. The average patient age increased from 24.3 years in 2005 to 27.4 in 2017. The main diagnosis for admission of ACHD is heart failure, arrhythmia and Fontan-related complications. The annual ICU admission percentage was around 5% and mean length of intensive care unit (ICU) stay was 8.4 ± 14.6 days. Mean personal hospital charges by admission of ACHD increased to around two times from 2005 to 2017. (from $2578.1 to $3697.0). Total annual hospital charges by ACHD markedly increased ten times (from $95,389.7 to $831,834.2). CONCLUSIONS: The number of hospital cares for ACHD dramatically increased more than five times from 2005 to 2017. We need preparations for efficient healthcare for adults with CHD such as a multi-dimensional approach, effective communication, and professional training.


Subject(s)
Cardiology Service, Hospital/trends , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/therapy , Hospitalization/trends , Pediatrics/trends , Survivors , Adolescent , Adult , Cardiology Service, Hospital/economics , Female , Health Expenditures/trends , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/economics , Hospital Charges/trends , Hospital Costs/trends , Hospitalization/economics , Humans , Male , Pediatrics/economics , Retrospective Studies , Seoul/epidemiology , Time Factors , Young Adult
9.
J Korean Med Sci ; 35(16): e107, 2020 Apr 27.
Article in English | MEDLINE | ID: mdl-32329256

ABSTRACT

BACKGROUND: Despite improvements in palliative care for critically ill children, the characteristics of end-of-life care for pediatric patients with advanced heart disease are not well-known. We investigated these characteristics among hospitalized children with advanced heart disease in a tertiary referral center in Korea. METHODS: We retrospectively reviewed the records of 136 patients with advanced heart disease who died in our pediatric department from January 2006 through December 2013. RESULTS: The median age of patients at death was 10.0 months (range 1 day-28.3 years). The median duration of the final hospitalization was 16.5 days (range 1-690 days). Most patients (94.1%) died in the intensive care unit and had received mechanical ventilation (89.7%) and inotropic agents (91.2%) within 24 hours of death. The parents of 74 patients (54.4%) had an end-of-life care discussion with their physician, and the length of stay of these patients in the intensive care unit and in hospital was longer. Of the 90 patients who had been hospitalized for 7 days or more, the parents of 54 patients (60%) had a documented end-of-life care discussion. The time interval from the end-of-life care discussion to death was 3 days or less for 25 patients. CONCLUSION: Children dying of advanced heart disease receive intensive treatment at the end of life. Discussions regarding end-of-life issues are often postponed until immediately prior to death. A pediatric palliative care program must be implemented to improve the quality of death in pediatric patients with heart disease.


Subject(s)
Heart Diseases/pathology , Terminal Care , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Intensive Care Units , Length of Stay , Male , Palliative Care , Parents/psychology , Respiration, Artificial , Retrospective Studies , Severity of Illness Index
10.
Cardiol Young ; 30(4): 571-573, 2020 Apr.
Article in English | MEDLINE | ID: mdl-32090718

ABSTRACT

A 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, suspected based on CT findings. Magnetic resonance lymphangiography revealed chylolymphatic reflux from a tortuously dilated thoracic duct in the mediastinum to the pericardial space, suggesting primary chylopericardium with lymphangiectasia. Pericardial effusion resolved immediately after thoracic duct embolisation at the lower thoracic level. However, pericardial effusion recurred after 5 months, which resolved after additional embolisation of the abnormal lymphatic collateral vessels from the remnant upper thoracic duct. Here, we report an unusual case with chylous massive pericardial effusion diagnosed by magnetic resonance lymphangiography and treated with percutaneous embolisation.


Subject(s)
Chylothorax/diagnosis , Embolization, Therapeutic/methods , Pericardial Effusion/diagnosis , Thoracic Duct , Child, Preschool , Chylothorax/complications , Chylothorax/therapy , Female , Humans , Ligation , Pericardial Effusion/etiology , Pericardial Effusion/therapy
11.
Cytogenet Genome Res ; 158(4): 184-191, 2019.
Article in English | MEDLINE | ID: mdl-31394527

ABSTRACT

Costello syndrome (CS) is a rare genetic disorder characterized by distinctive facial appearance, cardiopulmonary complications, severe growth retardation, skin and skeletal defects, developmental delay, and tumor predisposition. CS is caused by heterozygous de novo mutations in the proto-oncogene HRAS, which is a component of the RAS/mitogen-activated protein kinase pathway. Herein, we reviewed the phenotypic and genetic features of 5 Korean patients who were genetically diagnosed with CS. Atrial tachycardia and polyhydramnios, which are important prenatal features for CS, were observed in 4 and 5 patients, respectively. The distinctive coarse facial appearances of the patients and presence of deep palmoplantar creases supported the clinical diagnosis of CS, which was confirmed by HRAS sequence analysis. Extremely poor postnatal growth was observed in all 5 patients. Further, all patients exhibited cardiac abnormalities; left ventricular hypertrophy and hypertrophic cardiomyopathy were observed in 3 patients. All 5 patients suffered from airway problems; 3 of them required intubation right after birth, and 2 of them received tracheostomy. One patient with a p.Gly12Ser mutation was diagnosed with retroperitoneal rhabdomyosarcoma alveolar type at the age of 5 years. Consistent with previous reports, both patients with p.Gly12Cys mutations died within the first year of life due to cardiopulmonary failure. Our study summarizes the characteristics of these 5 Korean patients with CS and, along with previous studies, provides clues for genotype-phenotype correlation in patients with CS.


Subject(s)
Costello Syndrome/genetics , Phenotype , Child , Child, Preschool , Female , Genotype , Humans , Infant , Infant, Newborn , Male , Proto-Oncogene Mas , Republic of Korea
12.
Pediatr Cardiol ; 40(1): 23-28, 2019 Jan.
Article in English | MEDLINE | ID: mdl-30121863

ABSTRACT

Patients with Eisenmenger syndrome (ES) have a higher mortality rate than patients with simple congenital heart disease (CHD). To determine factors associated with death in the era of advanced pulmonary vasodilator treatment, we analyzed the characteristics of adult ES patients depending on underlying CHD. Simple septal defects and patent ductus arteriosus were classified as simple CHD, and other conditions were classified as complex CHD. Sixty-seven adult ES patients (50.7% women) were reviewed retrospectively. CHD was diagnosed at a median of 10.0 years of age and ES was diagnosed at 18.6 years. Thirteen patients (19.4%) died; the median age was 38.6 years (IQR 32.2-47.8). In a multivariate analysis, patients with SpO2 < 85% had a higher mortality rate than others [hazard ratio (HR) 9.7; 95% confidence interval (CI) 1.002-95.2, p = 0.05]. In simple CHD patients, those with a low platelet count (< 100 × 109/L) or low SpO2 (< 85%) were at a higher risk of death than those without (HR 16.32, 95% CI 1.25-2266.31, p = 0.032; and HR 38.91, 95% CI 3.44-5219.41, p = 0.001, respectively). Advanced pulmonary vasodilators were used more in survivors than in non-survivors (48.1% vs. 15.4%, p = 0.032). Low SpO2 and platelet count were related to mortality in adult ES, especially in those with simple CHD. Therefore, careful attention should be paid to the care of adult ES patients with this tendency; active pulmonary vasodilator treatment should be considered.


Subject(s)
Eisenmenger Complex/physiopathology , Adult , Child , Eisenmenger Complex/classification , Eisenmenger Complex/drug therapy , Eisenmenger Complex/mortality , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Multivariate Analysis , Proportional Hazards Models , Retrospective Studies , Risk Factors , Severity of Illness Index , Thrombocytopenia , Vasodilator Agents/therapeutic use
13.
Pediatr Cardiol ; 40(8): 1584-1590, 2019 Dec.
Article in English | MEDLINE | ID: mdl-31473799

ABSTRACT

Because Fontan circulation does not have a subpulmonary ventricle, the preload is limited. In Fontan circulation with extracardiac conduit, the size of conduit could be an important factor in determining the preload. We compared exercise capacity with each conduit size and tried to search for optimal conduit size in Fontan circulation. We reviewed the medical record of 677 patients with Fontan circulation. Patients who had other type Fontan circulation (Kawashima, atriopulmonary, lateral tunnel), SpO2 < 85%, protein losing enteropathy, results of inappropriate exercise test were excluded. As a result, 150 patients were enrolled and classified according to conduit size. We compared with their exercise capacity and analyzed correlation between exercise capacity and conduit size per body surface area (BSA). 97 Males were included and mean age was 17.5 ± 5.1 years old. In cardiac catheterization, central venous pressure (CVP) was 12.4 ± 2.5 mmHg and pulmonary vascular resistance was 1.2 ± 0.5 wu m2. In cardiopulmonary exercise test, predictive peak VO2 was 59.1 ± 9.7% and VE/VCO2 was 36.2 ± 6.9. In analysis using quadratic model, impacts of gender, age at Fontan operation, ventricular morphology, isomerism, and fenestration on exercise capacity were excluded and conduit size per BSA had a significant curved correlation with predictive peak VO2 and VE/VCO2. Our results showed that patients with about 12.5 mm/m2 conduit per BSA have the best exercise capacity. Patients with larger than smaller-sized conduit were found to be more attenuated in their ability to exercise.


Subject(s)
Exercise Tolerance , Exercise/physiology , Fontan Procedure/adverse effects , Adolescent , Adult , Cardiac Catheterization , Child , Female , Humans , Male , Retrospective Studies , Young Adult
14.
J Pediatr ; 198: 36-45.e3, 2018 07.
Article in English | MEDLINE | ID: mdl-29650415

ABSTRACT

OBJECTIVE: To quantify outcomes of infants (<1 year of age) diagnosed with pulmonary vein stenosis (PVS). STUDY DESIGN: MEDLINE (PubMed), Scopus, and Web of Science were searched through February 1, 2017, with no language restrictions. Publications including infants diagnosed with primary PVS, defined as the absence of preceding intervention(s), were considered. The study was performed according to Meta-analysis of Observational Studies in Epidemiology guidelines, the Systematic Reviews, and Meta-Analysis checklist, and registered prospectively. The quality of selected reports was critically examined. Data extraction was independently performed by multiple observers with outcomes agreed upon a priori. Data were pooled using an inverse variance heterogeneity model with incidence of mortality the primary outcome of interest. RESULTS: Forty-eight studies of 185 infants were included. Studies were highly diverse with regards to the participants, interventions, and outcomes reported. The median (range) age at diagnosis was 5.0 (0.1-11.6) months. Pooled mortality was 58.5% (95% CI 49.8%-67.0%, I2 = 21.4%). We observed greater mortality incidence among infants with 3 or 4 vein stenoses than in those with 1 or 2 vein stenoses (83.3% vs 36.1%; P < .01). We observed greater mortality among infants with bilateral than unilateral disease (78.7% vs 26.0%; P < .01). CONCLUSIONS: Studies of primary PVS during infancy are highly variable in their methodological quality and estimates of clinical outcomes; therefore, estimates of prognosis remain uncertain. Multicenter, interdisciplinary collaborations, including alignment of key outcome measurements, are needed to answer questions beyond the scope of available data.


Subject(s)
Stenosis, Pulmonary Vein/diagnosis , Stenosis, Pulmonary Vein/therapy , Female , Humans , Infant , Infant, Newborn , Male , Outcome Assessment, Health Care , Stenosis, Pulmonary Vein/mortality
15.
Europace ; 20(6): 1043-1049, 2018 06 01.
Article in English | MEDLINE | ID: mdl-29016830

ABSTRACT

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.


Subject(s)
Electrophysiologic Techniques, Cardiac/methods , Fontan Procedure/methods , Heart Defects, Congenital , Punctures/methods , Adolescent , Adult , Blood Circulation , Feasibility Studies , Female , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/physiopathology , Heart Defects, Congenital/surgery , Humans , Male , Outcome Assessment, Health Care , Postoperative Period
16.
Pacing Clin Electrophysiol ; 41(11): 1435-1440, 2018 11.
Article in English | MEDLINE | ID: mdl-30246897

ABSTRACT

BACKGROUND: Patients with repaired congenital heart disease (CHD) can have both depolarization and repolarization abnormalities. A coexisting long QT syndrome (LQTS) may cause electrical instability in these patients; however, diagnosing LQTS is difficult owing to intraventricular conduction delay or paced rhythm after the operation. We report on six patients diagnosed with LQTS after CHD repair. METHODS: We investigated two male and four female patients. Clinical data, electrocardiographic findings, and genetic analysis results were reviewed. RESULTS: The range of patient age at LQTS diagnosis was 1.4-22 years. There were two patients with tetralogy of Fallot, four with septal defect, and one with tricuspid atresia. All patients underwent total correction or a staged operation, without events. The diagnosis of LQTS was made in four asymptomatic patients and two symptomatic patients with recurrent syncope and ventricular fibrillation. During the postoperative follow-up, their median QTc interval and QTc dispersion increased (from 474 and 41 ms preoperatively to 541 and 141 ms postoperatively, respectively; P = 0.043). T-wave notching over three leads was observed in three patients. Genetic analysis showed SCN5A mutation in one, KCNH2 mutation in three, KCNQ1 mutation in one, and no identified mutation in one patient. An implantable cardioverter defibrillator was placed in two patients. CONCLUSION: A coexisting LQTS may confer additional risk for arrhythmia and sudden cardiac death in patients with CHD. Suspicion of LQTS and careful monitoring of the QT interval and T-wave morphology are important during the follow-up of patients with repaired CHD.


Subject(s)
Heart Defects, Congenital/complications , Heart Defects, Congenital/surgery , Long QT Syndrome/diagnosis , Adolescent , Child , Child, Preschool , Electrocardiography , Female , Heart Defects, Congenital/genetics , Humans , Infant , Long QT Syndrome/genetics , Male , Young Adult
17.
Pediatr Cardiol ; 39(8): 1642-1649, 2018 Dec.
Article in English | MEDLINE | ID: mdl-30105463

ABSTRACT

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7 months (range 7-14 months) and the mean body weight was 7.9 ± 2.6 kg (range 4.1-10.5 kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8 mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44 months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.


Subject(s)
Stenosis, Pulmonary Vein/surgery , Stents/adverse effects , Vascular Surgical Procedures/methods , Female , Follow-Up Studies , Humans , Infant , Male , Pulmonary Veins/abnormalities , Pulmonary Veins/surgery , Retrospective Studies , Scimitar Syndrome/surgery , Treatment Outcome
18.
Pediatr Cardiol ; 38(1): 20-26, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27628435

ABSTRACT

To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P < 0.001). Although the dimensional changes in the aorta were not clearly observed in adolescents with TS, the elastic properties of the aorta were significantly decreased in TS patients as compared to control subjects.


Subject(s)
Aorta/pathology , Turner Syndrome/complications , Vascular Stiffness/physiology , Adolescent , Aorta/diagnostic imaging , Blood Pressure/physiology , Child , Echocardiography/methods , Elasticity , Female , Humans , Pulse Wave Analysis/methods
19.
Clin Exp Pediatr ; 66(5): 190-200, 2023 May.
Article in English | MEDLINE | ID: mdl-37070296

ABSTRACT

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection caused worldwide health problems, and coronavirus disease 2019 (COVID-19) was declared a pandemic by the World Health Organization in March 2020. Cardiovascular complications of COVID-19 are not uncommon; among them, arrhythmia is considered a significant risk factor for poor health outcomes in adults. However, data are scarce on the arrhythmia of pediatric patients with SARS-CoV-2 infection, possibly due to their mild symptoms and low incidence of cardiovascular involvement. Multisystem inflammatory syndrome in children reportedly features increased cardiovascular involvement, but arrhythmic complications remain unidentified. Thus, here we review the epidemiology, manifestations, and outcomes of pediatric arrhythmia associated with COVID-19.

20.
Contemp Clin Trials Commun ; 32: 101078, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36762120

ABSTRACT

Background: Several analgesics have been applied under various protocols to control the moderate-to-severe postoperative pain caused by the surgical extraction of an impacted mandibular third molar. However, a consensus on optimal pain management while minimizing side effects is yet to be reached. Methods: This multi-center, prospective, double-blind, randomized controlled trial aims to evaluate the efficacy and safety of sequential multimodal analgesia combined with postoperative zaltoprofen along with multiple preemptive analgesics. A total of 80 participants with bilateral impacted mandibular third molar from two hospitals were randomized into two groups. Two surgical extractions were performed at one-month intervals, and in a crossover design, celecoxib or tramadol/acetaminophen was administered before one extraction and placebo before the other extraction. Following extraction, all subjects took zaltoprofen for 5 days. The outcome measures included pain at specific times, time and intensity of the first pain onset after extraction, need of rescue drugs, and occurrence and frequency of side effects. Conclusions: This ongoing clinical trial was designed to provide evidence regarding a new protocol for effective postoperative pain management of a commonly performed surgical extraction. The results of this study will provide guidance to clinicians regarding the timing and combination of oral analgesics in various oral surgeries performed under local anesthesia. Trial registration: KCT0005450, registered on October 7, 2020.

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