• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제23권1호
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• pp.31-35
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• 2012

Noonan syndrome is characterized by short stature, typical facial dysmorphology, and congenital heart defects. The main facial features of Noonan syndrome are hypertelorism with down-slanting palpebral fissures, ptosis, and low-set posteriorly-rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency, and lymphatic dysplasias. The patient is a 10-year-old boy. He had experienced repeated febrile convulsions. He had typical facial features, a short stature, chest deformity, cryptorchidism, vesicoureteral reflux, and mental retardation. His language and motor development were delayed. When he went to school, it was difficult for him to pay attention, follow directions, and organize tasks. He also displayed behavior such as squirming, leaving his seat in class, and running around inappropriately. Clinical observation is important for the diagnosis, so we report a patient who was diagnosed with Noonan syndrome, mental retardation, and attention-deficit hyperactivity disorder.

• Journal of Chest Surgery
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• 제18권2호
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• pp.174-181
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• 1985

From Jun. 1980 to Mar. 1984, 33 cases of atrial septal defect, secundum type, operated at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Keimyung University, were analyzed retrospectively. They were 24.3% of all congenital heart diseases operated in the same period. Among the 33 cases, 17 cases were males and 16 cases were females. Their ages were ranged from 4 to 42 years and cases of over 15 years of age were 60.6%. Main symptoms at admission were exertional dyspnea [69.7%], palpitation [63.6%] and frequent upper respiratory infections [51.5%], but 2 cases [6.1%] were asymptomatic. All 33 cases were operated under the direct vision with cardiopulmonary bypass. All cases were secundum type defect of atrial septal defect and single defect were in 29 cases [90.6%], and oval type defect were in 31 cases [96.9%]. In 7 cases [21.9%], other lesions of cardiovascular system were associated, and the most common lesion was pulmonic valvular stenosis [4 cases; 12.5%]. The defects were closed directly in 27 cases and in 6 cases with Dacron patch. Postoperative complications were occurred in 8 cases [24.2%], and they were pleural effusion, congestive heart failure, and alopecia mainly. One case died due to air embolism postoperatively and operative mortality was 3.1%.

• Journal of Chest Surgery
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• 제30권10호
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• pp.986-990
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• 1997

23례의 환자에서 1989년 12월부터 1996년 12월까지 우전측방흉부절개술(RALT; right anterolateral thorncotomy)을 이용하여 선천성 심장병을 수술하였다. 22례에서 심방중격결손증(단순난원공형 13례, 하대정맥부 위로 확대된 저위형 쩨, 후방확대형 쎄, 정맥동형 1례, 일차공형 떼)이었고, 떼에서 심실중격결손증이었다. 우전측방흉부절개술(RALI)과 관련된 수술중 사망률이나 후기이환율은 없었다. 우전측방흉부절개술(RALI)은 선택된 환자에서(특히 여자) 정중흉골절개술에 비해 안전하고 효과적인 방법으로 생각되며, 추적관찰시 미용의 측면에서 결과는 매우 좋았다.

• Journal of Chest Surgery
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• 제49권2호
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• pp.112-114
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• 2016

The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or $r{\acute{e}}paration$ $\grave{a}$ $l^{\prime}{\acute{e}}tage$ ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.

• 대한신경집중치료학회지
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• 제11권2호
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• pp.124-128
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• 2018

Background: Secundum atrial septal defect (ASD) is a common congenital heart defect in adults. Patients with ASDs at high risk of cardiovascular complications undergo either surgical repair or percutaneous device closure. Case Report: We report the case of an 85-year-old male with unusual recurrent cerebral infarctions. The patient has undergone repair of secundum ASD 12 years ago. Evaluation by transesophageal echocardiography revealed a mobile mass at the patch repair site in the left atrium. The mass was surgically removed due to recurrent stroke during the anticoagulation. Conclusion: This case emphasizes the importance of regular cardiac checkup and the need to consider cardioembolic source as being part of the etiology of stroke recurrence, even if the event occurs many years after intracardiac shunt closures.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.232-237
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• 2012

Purpose: With feasibility in the diagnoses of congenital heart disease (CHD) in the antenatal period, we suspect changes have occurred in its incidence. No data have been reported about the current incidence of simple forms of CHD in Korea. We have attempted to assess the recent incidence and characteristics of CHD in the neonatal care unit of a secondary referral medical center. Methods: Medical records of 497 neonatal care unit patients who underwent echocardiography in the past 5 years were reviewed. Preterm infants with patent ductus arteriosus and other transient, minimal lesions were excluded from this study. Results: Although the number of inpatients remained stable, the incidence of simple forms of CHD showed a gradual decrease over the 5-year study period; a markedly low incidence of complex forms was seen as well. CHD was observed in 3.7% full-term and 6.8% pre-term infants. CHD was observed in 152 infants weighing >2,500 g (3.5% of corresponding birth weight infants); 65 weighing 1,000 to 2,500 g (9.3%); and 6 weighing <1,000 g (8.0%). The incidence of CHD was higher in the pre-term group and the low birth weight group than in each corresponding subgroup (P<0.001); however, the incidence of complex CHD in full-term neonates was high. The number of patients with extracardiac structural anomalies has also shown a gradual decrease every year for the past 5 years. Conclusion: Findings from our study suggest that the recent incidence and disease pattern of CHD might have changed for both complex and simple forms of CHD in Korea.

• Journal of Chest Surgery
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• 제33권5호
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• pp.419-421
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• 2000

Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

• Journal of Chest Surgery
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• 제48권5호
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• pp.351-354
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• 2015

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.

• Journal of Chest Surgery
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• 제50권3호
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• pp.207-210
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• 2017

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

• Clinical and Experimental Pediatrics
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• 제59권1호
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• pp.40-42
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• 2016

Lynch syndrome is the most common inherited colon cancer syndrome. Patients with Lynch syndrome develop a range of cancers including colorectal cancer (CRC) and carry a mutation on one of the mismatched repair (MMR) genes. Although CRC usually occurs after the fourth decade in patients with Lynch syndrome harboring a heterozygous MMR gene mutation, it can occur in children with Lynch syndrome who have a compound heterozygous or homozygous MMR gene mutation. We report a case of CRC in a 13-year-old patient with Lynch syndrome and congenital heart disease. This patient had a heterozygous mutation in MLH1 (an MMR gene), but no compound MMR gene defects, and a K-RAS somatic mutation in the cancer cells.