Ventricular Tachycardia in Neonates: A Report of Two Cases and Review of the Literature

In the case of hemodynamically stable broad complex tachycardia, it is essential to evaluate the causes and to diagnose correctly in the selection of appropriate management and drugs. We report two neonates diagnosed with idiopathic ventricular tachycardia, which is extraordinarily rare. One presented with idiopathic fascicular ventricular tachycardia (right bundle branch block pattern with a superior axis), and the other presented with right ventricular outflow tract ventricular tachycardia (left bundle branch block pattern with an inferior axis). These two forms are representative of benign ventricular tachycardia. No features of cardiovascular shock were observed. While the recommended initial drug treatments are different, the conditions were well controlled by propranolol without the development of any adverse events. There was no recurrence of arrhythmia for several months in the outpatient clinic.

A Neonate Diagnosed with Wolff-Parkinson-White Syndrome Presenting with Cardiogenic Shock

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

Ventricular Tachycardia in Neonates: A Report of Two Cases and Review of the Literature

In the case of hemodynamically stable broad complex tachycardia, it is essential to evaluate the causes and to diagnose correctly in the selection of appropriate management and drugs. We report two neonates diagnosed with idiopathic ventricular tachycardia, which is extraordinarily rare. One presented with idiopathic fascicular ventricular tachycardia (right bundle branch block pattern with a superior axis), and the other presented with right ventricular outflow tract ventricular tachycardia (left bundle branch block pattern with an inferior axis). These two forms are representative of benign ventricular tachycardia. No features of cardiovascular shock were observed. While the recommended initial drug treatments are different, the conditions were well controlled by propranolol without the development of any adverse events. There was no recurrence of arrhythmia for several months in the outpatient clinic.

A Neonate Diagnosed with Wolff-Parkinson-White Syndrome Presenting with Cardiogenic Shock

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

Coronary Artery Vasospasm with Acute Myocarditis in an Adolescent

A 13-year-old boy had a 5-day history of prodromal symptoms of fever and dry cough followed by two episodes of severe anginal chest pain and substantial transient ST-segment elevation. A subsequent evaluation showed that the chest pain was caused by coronary artery vasospasm complicating acute myocarditis. We report a rare case of coronary artery vasospasm in an adolescent with a medical history of Raynaud’s phenomenon who was successfully treated with intravenous immunoglobulin and a calcium channel blocker. This report exemplifies the need for pediatricians to be aware that anginal pain due to coronary artery spasm can, albeit rarely, occur in children as a complication of myocarditis, sometimes as a concomitant feature in patients with Raynaud’s phenomenon; in suspected cases, serial electrocardiography is important to perform.

Coronary Artery Vasospasm with Acute Myocarditis in an Adolescent

A 13-year-old boy had a 5-day history of prodromal symptoms of fever and dry cough followed by two episodes of severe anginal chest pain and substantial transient ST-segment elevation. A subsequent evaluation showed that the chest pain was caused by coronary artery vasospasm complicating acute myocarditis. We report a rare case of coronary artery vasospasm in an adolescent with a medical history of Raynaud’s phenomenon who was successfully treated with intravenous immunoglobulin and a calcium channel blocker. This report exemplifies the need for pediatricians to be aware that anginal pain due to coronary artery spasm can, albeit rarely, occur in children as a complication of myocarditis, sometimes as a concomitant feature in patients with Raynaud’s phenomenon; in suspected cases, serial electrocardiography is important to perform.

Prophylactic Pulmonary Artery Reduction in a Young Female with Severe Pulmonary Hypertension from Complete Atrioventricular Septal Defect

Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

Hypogonadotropic Hypogonadism and Abnormal Olfactory Bulb Development in CHARGE Syndrome with CHD7 Mutation / 대한소아내분비학회지

CHARGE syndrome is a congenital malformation disorder that includes Coloboma, Heart defect, Atresia of the choanae, Retarded growth and development, Genital hypoplasia, and Ear abnormalities. Recently hypogonadotropic hypogonadism and abnormal olfactory bulb development are occasionally described in CHARGE syndrome with chromodomain helicase DNA-binding protein 7 (CHD7) gene mutation. We report the case of Korean female patient with CHARGE syndrome and CHD7 mutation who had hypogonadotropic hypogonadism and abnormal olfactory bulb as manifested by delayed puberty and growth retardation at 13 years of age. She had both optic nerve coloboma, external ear abnormalities and bilateral agenesis of the semicircular canals. She had severe mental retardation and autistic-like behavior. We identified a heterozygous nonsense mutation at exon 20 of the CHD7 gene (c.4601G>A; Trp1534X).

THREE-DIMENSIONAL FINITE ELEMENT ANALYSIS OF STRESS DISTRIBUTION IN ALL-CERAMIC CROWNS WITH VARIOUS FINISH LINE DESIGNS AND INCISAL REDUCTIONS UNDER DIFFERENT LOADING CONDITIONS / 대한치과보철학회지

The purpose of this study was to determine the effect of finish line design, amount of incisal reduction, and loading condition on the stress distribution in anterior all-ceramic crowns. Three-dimensional finite element models of an incisor all-ceramic crown with 3 different finish line designs : 1) shoulder with sharp line angle 2) shoulder with rounder line angle 3) chamfer : and 2 different incisal reductions : 2mm and 4mm were developed. 300 N force with the direction of 45 degree to the long axis of the tooth was applied at 3 different positions : A) incisal 1/3, B) incisal edge, C) cervical 1/5. Stresses developed in ceramic and cement were analyzed using three-dimensional finite element method. The results were as follows : 1. Stresses were concentrated in the margin region, which were primarily compressive in the labil and tensile in the lingual. 2. Stresses were larger in the area near line angle than on the crown surface of the margin region. In case of shoulder with sharp line angle, stresses were highly concentrated in the porcelain near line angle. 3. At the interface between porcelain and cement and at the porcelain above the margin on crown surface, stresses were the highest in chamfer, and decreased in shoulder with sharp line angle and shoulder with rounded line angle, respectively. 4. At the interface between cement and abutment on crown surface, stresses were the highest in shoulder with sharp line angle, and decreased in shoulder with rounded line angle and chamfer, respectively. 5. The amount of incisal reduction had little influence on the stress distribution in all-ceramic crowns. 6. When load was applied at the incisal edge, higher stresses were developed in the margin region and the incisal edge than under the other loading conditions. 7. When load was applied at the cervical 1/5, stresses were very low as a whole.