• Journal of Chest Surgery
• /
• v.42 no.5
• /
• pp.639-644
• /
• 2009

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-$\beta$ receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.

• Journal of Chest Surgery
• /
• v.39 no.1 s.258
• /
• pp.12-17
• /
• 2006

Background: Circulatory arrest under deep hypothermia is an important auxiliary means for surgical correction of total anomalous pulmonary venous connection (TAPVC), However, cardiac operations under deep hypothermic circulatory arrest are associated with the risk of post-arrest neurologic abnormalities. The purpose of this study is to evaluate the results of the surgical correction of total anomalous pulmonary venous connection without the total circulatory arrest. Materiai and Method: Between April 2000 and October 2004, hospital records of 10 patients were reviewed retrospectively. Result: The locations for abnormal anatomical connections were supracardiac in 7 cases, cardiac in 1 case, and infracardiac in 2 cases. The mean cardiopulmonary bypass time and aorta cross clamp time were 116.8$\pm$40.7 and 69.5$\pm$24.1 minutes. There was no surgical mortality. Postoperative complications were post-repair pulmonary venous stenosis in 1 case, pneumonia in 1, pneumothorax in 1, wound infection in 1,and diaphragmatic paralysis in 1. All patients without pulmonary venous stenosis were in NYHA class I at mean follow-up of 16.6 months (3$\∼$49 months) Conclusion: We could obtain excellent results by repair without the total circulatory arrest for total anomalous pulmonary venous connection.

• Sleep Medicine and Psychophysiology
• /
• v.4 no.2
• /
• pp.129-139
• /
• 1997

The data collected to date indicate that sleep-related breathing disorders, including sleep-disordered breathing(sleep apnea) and underlying respiratory system diseases, are one of the important risk factors for cardiovascular dysfunction. Sleep-disordered breathing(sleep apnea) is now recognized as one of the leading causes of systemic hypertension, cardiac arrhythmias, coronary heart disease, pulmonary hypertension, right heart failure, and stroke. Sleep may exert a profound effect on breathing in patients with underlying respiratory system disease including bronchopumonary diseases, chest wall abnormalities, central alveolar hypoventilation syndromes or respiratory neuromuscular disorders. Chronic hypoxia and hypercapnia in these patients may accelerate the development of long term cardiovascular complications such as cardiac arrhythmias, pulmonary hypertension, and right heart failure(cor pulmonale). Several recent studies reported that sleep-related breathing disorders are associated with long-term cardiovascular morbidity and mortality. Careful assessment of respiratory and cardiovascular function in these patients is critical. Aggressive and highly effective treatment of sleep-related breathing disorders using tracheostomy, mechanical ventilation, nasal continuous positive airway pressure therapy(nCPAP), intercurrent oxygen therapy or other interventions can reduce the prevalence of cardiovascular dysfunction and the long-term mortality.

• Clinical and Experimental Pediatrics
• /
• v.58 no.10
• /
• pp.392-397
• /
• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of Chest Surgery
• /
• v.49 no.1
• /
• pp.9-14
• /
• 2016

Background: Tetralogy of Fallot (TOF) is a well-recognized congenital heart disease. Despite improvements in the outcomes of surgical repair, the optimal timing of surgery and type of surgical management of patients with TOF remains controversial. The purpose of this study was to assess outcomes following the repair of TOF in infants depending on the surgical procedure used. Methods: This study involved the retrospective review of 120 patients who underwent TOF repair between 2010 and 2013. Patients were divided into three groups depending on the surgical procedure that they underwent. Corrective surgery was done via the transventricular approach (n=40), the transatrial approach (n=40), or a combined atrioventricular approach (n=40). Demographic data and the outcomes of the surgical procedures were compared among the groups. Results: In the atrioventricular group, the incidence of the following complications was found to be significantly lower than in the other groups: complete heart block (p=0.034), right ventricular failure (p=0.027) and mediastinal bleeding (p=0.007). Patients in the atrioventricular group had a better postoperative right ventricular ejection fraction (p=0.001). No statistically significant differences were observed among the three surgical groups in the occurrence of tachycardia, renal failure, and tricuspid incompetence. The one-year survival rates in the three groups were 95%, 90%, and 97.5%, respectively (p=0.395). Conclusion: Combined atrioventricular repair of TOF in infancy can be safely performed, with acceptable surgical risk, a low incidence of reoperation, good ventricular function outcomes, and an excellent survival rate.

• Journal of Chest Surgery
• /
• v.13 no.4
• /
• pp.435-441
• /
• 1980

Ebstein`s anomaly had been amenable to reparative surgery since 1962. However, neither the role of surgery in Ebstein`s anomaly nor the surgical procedure of choice for its correction are clearly defined. Whether or not the atrialized right ventricle, which plays a major role in the functional abnormalities, should be obliterated in all cases remains unsolved. Four cases of Ebstein`s anomaly treated surgically at Seoul National University Hospital were reported. All had closure of the atrial septal defect, obliteration of the atrialized right ventricle by plication, and insertion of a tricuspid bioprosthesis and an epicardial ventricular pacemaker. One patient had a pulmonic valvotomy due to stenotic bicuspid pulmonic valve also. All but one had discharged with a good result.

• Clinical and Experimental Pediatrics
• /
• v.53 no.7
• /
• pp.729-734
• /
• 2010

Congenital anomalies of the kidney and urinary tract (CAKUT) account for more than 50% of abdominal masses found in neonates and involve about 0.5% of all pregnancies. CAKUT has a major role in renal failure, and increasing evidence suggests that certain abnormalities predispose to the development of hypertension and cardiovascular disease in adulthood. To understand the pathogenesis of human renal anomalies, understanding the development of kidney is important. Diverse anomalies of the kidney corresponding to defects at a particular stage of development have been documented recently; however, more research is required to understand the molecular networks underlying kidney development, and such an investigation will provide a clue to the therapeutic intervention for CAKUT.

• Journal of Chest Surgery
• /
• v.22 no.4
• /
• pp.667-671
• /
• 1989

Cor triatriatum is a rare congenital malformation of the heart in which a diaphragm stretches in a transverse plane through the left pulmonary venous chamber, thus creates two subchambers. The proximal chamber connects with the pulmonary veins, and the distal one has left auricle and the mitral valve. A 3 year old boy who had Cor triatriatum underwent surgical excision of the abnormal diaphragm in March, 1989 in Chungnam University Hospital. An obliquely oriented fibromuscular diaphragm divided the left atrium into a proximal chamber which was connected to the pulmonary veins and a distal chamber which had the atrial appendage and the mitral valve. The opening in the diaphragm was 5 mm in diameter. There were no associated abnormalities. The abnormal diaphragm was completely excised. The postoperative result was excellent.

• Journal of Chest Surgery
• /
• v.27 no.11
• /
• pp.973-976
• /
• 1994

Angiomatosis [ or Diffuse Hemangima] is a rare condition in which large segments of the body are involved by proliferating vessels. By now, some authers say that this lesion begins during early intrauterine life when the limb buds form, grow proportionately with the fetus, and consequently affect large areas of the trunk or extremity. The majority of angiomatoses present during childhood or infancy as swelling, induration, or discoloration of the affected area. The patient was 13 year old male and had no specific signs and symptoms except palpable mass on the left lateral chest wall from childhood and a painful tender mass on the posterior chest wall for 5 days before admission. The chest PA showed no abnormalities.The operation was done with the resection of left 10th rib and 9th and 10th intercostal muscles including masses and the ligation of the both intercostal and feeding vessels of the masses. The pathologic result was angiomatosis involving intercostal muscles and rib.

• Journal of Chest Surgery
• /
• v.27 no.11
• /
• pp.965-969
• /
• 1994

Infantile lobar emphysema is a pulmonary hyperinflation state that has the clinical features of an air block syndrome characterized by bronchial cartilaginous abnormalities or unknown origin. Left upper lobe was affected in most of the reported infantile lobar emphysema. Infantile lobar emphyema is divided into two categories. e.g., congenital and acquired. We have experienced a case of left lower lobe involved infantile lobar emphysema which had undergone left pneumonectomy. She had progressive signs of tension accompanied by mediastinal displacement, ventilatory and circulatory failure in infant period. Because of the combined left upper lobe hypoplasia, left pneumonectomy was performed. And there was no cartiliginous abnormality in pathologic finding. This is the first domestic case which was affected in the lower lobe and successful surgical repaired.