• Journal of Chest Surgery
• /
• v.40 no.1 s.270
• /
• pp.8-16
• /
• 2007

Background: We performed a prospective clinical study to evaluate the ultrastructural integrity of the myocardium after using Histidine-Tryptophan-Ketoglutarate (HTK) solution in comparison with blood cardioplegic solution during congenital heart surgery. Material and Method: Twenty two patients with acyanotic heart disease, who were scheduled for elective open heart surgery, were randomized into two groups. The HTK Group (n=11) received HTK cardioplegic solution; the blood group (n=11) received conventional blood cardioplegic solution during surgery. The preoperative diagnoses included ventricular septal defect (n=9) and atrial septal defect (n=2) in each group. A small biopsy specimen was taken from the right ventricle's myocardium, and this was processed for ultrastructural examination at the end of 30 minutes of reperfusion. Semiquantitative electron microscopy was carried out 'blindly' in 4 areas per specimen and in 5 test fields per area by 'random systematic sampling' and 'point and intersection counting'. The morphology of the mitochondrial membrane and cristae were then scored. The interstitial edema of the myocardium was also graded. Result: The semiquantitative score of the mitochondrial morphology was $19.65{\pm}4.75$ in the blood group and $25.25{\pm}5.85$ in the HTK group (p=0.03). 6 patients (54.5%) in the blood group and 3 patients (27.3%) in the HTK group were grade 3 or more for the interstitial edema of the myocardium. Conclusion: The ultrastructural integrity was preserved even better with HTK solution than with conventional blood cardioplegic solution.

• Journal of Veterinary Clinics
• /
• v.31 no.2
• /
• pp.112-116
• /
• 2014

A 2-month-old intact male Pitbull terrier (weighing 1.01 kg) were referred with primary complaints of severe abdominal distension, exercise intolerance, retarded growth, yellow nasal discharge and anorexia. Diagnostic imaging studies found enlarged right atrium partitioned by abnormal membrane, severe pulmonic systolic jets (5.66 m/s of peak velocity) and right-to-left blood shunt at the inter-atrial septum. Based on clinical and diagnostic findings, the case was diagnosed as cor triatriatum dexter complicated with severe pulmonic stenosis and right-to-left shunted patent foramen ovale. Either surgical or interventional therapy has not been attempted, because of unstable patient's condition. Using blood transfusion, oxygen supply and cardiac medications (i.e., sildenafil, spironolactone, enalapril), the dog was recovered.

• Clinical and Experimental Pediatrics
• /
• v.52 no.7
• /
• pp.832-836
• /
• 2009

Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients diagnosed with plastic bronchitis accompanied with a different underlying disease, which were treated with inhaled corticosteroid and low-dose oral clarithromycin.

• Journal of Korean Academy of Nursing Administration
• /
• v.21 no.3
• /
• pp.243-253
• /
• 2015

Purpose: The purpose of this study was to identify the impact of practical communication strategies (PCS) on the reduction of AEs (Adverse Events) in pediatric cardiac ICU (PCICU). Methods: Intra-operative findings and care plans were documented and shared between staff members on a daily basis from the day of operation to the day of general ward transfer. Incidence of AEs was investigated in all patients who were admitted to the PCICU and was compared with incidence of AEs one year after establishment of PCS. Results: The study population consisted of 216 patients in pre-PCS group and 156 patients in post-PCS group. Incidence of readmission decreased from 6.0% (13/216) in pre-PCS group to 0.6% (1/156) in post-PCS group (${\chi}^2=7.23$, p=.010). Incidence of other major complications decreased from 4.2% (9/216) to 0.6% (${\chi}^2=6.66$, p=.012). Minor AEs such as intervention omission, order error, and protocol misunderstanding were reduced from 23.3 cases per 100 patient-days to 7.5 cases per 100 patient-days (${\chi}^2=20.31$, p<.001). Conclusion: Handover protocol is an effective strategy to reduce AEs for critically ill patients after pediatric cardiac surgery. Efforts to develop effective communication strategies should be continued and outcome research about communication strategies for patient safety should be further studied.

• Journal of Chest Surgery
• /
• v.37 no.10
• /
• pp.856-860
• /
• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• Clinical and Experimental Pediatrics
• /
• v.59 no.sup1
• /
• pp.14-18
• /
• 2016

Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes. A 7-year-and 6-month-old boy experienced afebrile generalized seizure at the age of 5 years and 3 months. He had recurrent febrile seizures since 12 months of age and showed severe global developmental delay, remarkable hypotonia, short stature, and dysmorphic features such as microcephaly; small, low-set ears; dark, straight eyebrows; deep-set eyes; flat nasal bridge; midface hypoplasia; and a small, pointed chin. Previous diagnostic work-up, including conventional chromosomal analysis, revealed no definite causes. However, array-comparative genomic hybridization analysis revealed 1p36 deletion syndrome with a 9.15-Mb copy loss of the 1p36.33-1p36.22 region, and fluorescence in situ hybridization analysis (FISH) confirmed this diagnosis. This case highlights the need to consider detailed chromosomal study for patients with delayed development and epilepsy. Furthermore, 1p36 deletion syndrome should be considered for patients presenting seizure and moderate-to-severe developmental delay, particularly if the patient exhibits dysmorphic features, short stature, and hypotonia.

• Clinical and Experimental Pediatrics
• /
• v.54 no.6
• /
• pp.267-271
• /
• 2011

Distal duplication, or trisomy 15q, is an extremely rare chromosomal disorder characterized by prenatal and postnatal overgrowth, mental retardation, and craniofacial malformations. Additional abnormalities typically include an unusually short neck, malformations of the fingers and toes, scoliosis and skeletal malformations, genital abnormalities, particularly in affected males, and, in some cases, cardiac defects. The range and severity of symptoms and physical findings may vary from case to case, depending upon the length and location of the duplicated portion of chromosome 15q. Most reported cases of duplication of the long arm of chromosome 15 frequently have more than one segmental imbalance resulting from unbalanced translocations involving chromosome 15 and deletions in another chromosome, as well as other structural chromosomal abnormalities. We report a female newborn with a de novo duplication, 15q24- q26.3, showing intrauterine overgrowth, a narrow asymmetric face with down-slanting palpebral fissures, a large, prominent nose, and micrognathia, arachnodactyly, camptodactyly, congenital heart disease, hydronephrosis, and hydroureter. Chromosomal analysis showed a 46,XX,inv(9)(p12q13),dup(15)(q24q26.3). Array comparative genomic hybridization analysis revealed a gain of 42 clones on 15q24-q26.3. This case represents the only reported patient with a de novo 15q24-q26.3 duplication that did not result from an unbalanced translocation and did not have a concomitant monosomic component in Korea.

• Journal of Chest Surgery
• /
• v.30 no.4
• /
• pp.373-377
• /
• 1997

Between January, 1991 and May 1995, mitral valve repair was undertaken on 32 patients under 15 years for congenital mitral regurgitation. Mean age was 24.0$\pm$26.1 months(range 3 months to 15 years), 16 patients were male and 16 patients were female associated cardiac anomalies were found in 26 patients (81%), and ventricular septal defects were noted in 18 patients(56%). In regards to pathologic findings, there were annular dilatation(n:7), leaflet prolapse(n=18), cleft leaflet(n=5) and restricted valve motion (n=2). The method of repair consisted of annuloplasty(Modif ed Devega type) in 14, repair of redundunt leaflet in 6, closure of cleft in 5, triangular resection in 2 and splitting of papillary muscle in one. There was no operative mortality and two late deaths occurred as a result of heart failure and sepsis. Tro patients required replacement of the mitral valve after 3 months and 7 months respectivehy because of recurrent mitral regurgitation. Actuarial survival was 92.5% at 46 months and actuarial freedom from reoperation was 95% at 12 months and 92.5% at 46 months. Actuarial freedom from valve repair failure was 68% at 12 months and 61.8% at 46 months. Although valve repair failure rate was high, we believe that mitral valve repair should be of rcrred to children because of low mortality and low reoperation rate.

• Journal of Genetic Medicine
• /
• v.7 no.1
• /
• pp.37-44
• /
• 2010

Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.

• Journal of Chest Surgery
• /
• v.37 no.6
• /
• pp.504-510
• /
• 2004

Background: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardio-pulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. Material and Method: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2∼41 days) and median body weight was 2,870 grams (ranges; 900∼3,530 grams). Preoperative diag-noses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. Result: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002∼0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26∼140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7$\pm$7.4 mmHg, 53.7$\pm$11.4 mmHg, and 56.3$\pm$13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3$\pm$7.2, 21.0$\pm$8.4, and 21.2$\pm$8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin admin- istration. Conclusion: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.