Background: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. Material and Method: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. Result: There were .14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. Conclusion: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.
We reviewed our 18-year surgical experience of biventricular repair for double-outlet right ventricle. Material and Method: One hundred twelve consecutive patients (80 males and 32 females) who underwent biventricular repair for double-outlet right ventricle between May 1986 and September 2002 were included. We assessed risk factors for early mortality and reoperation. Reoperation-free survival rate and actual survival rate were analysed. Result: Most common type of ventricular septal defect was subaortic (n=58, 52%) and non-committed type was second most common (n=32, 29%). Four different surgical methods were used: intraventricular baffle repair (n=71 , 63%): right ventricle to pulmonary ariery conduit interposition or REV with left ventricle to aorta baffle repair (n=24, 21 .4%): arierial switch operation with left ventricle to pulmonary artery baffle (n=14, 12.5%): Senning atrial switch operation with left ventricle to pulmonary artery baffle (n=3, 2.7%). Thirty four patients(30%) underwent palliative procedures before definite repair. Twenty three patients (21%) required reoperations. There were 12 (10.7%) early deaths and 4 late deaths. Age younger than 3 months at repair (p=0.003), cardiopulmonary bypass and aortic cross clamp time (p=0.015, p=0.067), type of operation (arterial switch operation) (p <0.001) and type of ventricular septal defect (subpulmonic type) (p=0.002) were revealed as risk factors for early death in univariate analysis, while age under 3 months was the only significant risk factor in multivariate analysis. Patients younger than 1 year of age (p=0.02), pulmonary artery angioplasty at definitive repair (p=0.024), type of ventricular septal defect (non-committed) (p=0.001), type of operation (right ventricle to pulmonary artery conduit interposition and REV operation) (p=0.028, p=0.017) were risk factors for reoperation in univariate analysis but there was no significant risk factor in multivariate analysis. Follow-up was available on 91 survivals with a mean duration of 110.8$\pm$56.4 (2~201) months. 5, 10 and 15 year survival rates were 86.5%, 85% and 85% and reoperation free survival were 85%, 71.5%, 70%. Conclusion: Age under 3 months at repair, subpulmonic ventricular septal defect and arterial switch operation were significant risk factors for early mortality. Patients with non-committed ventricular septal defect and who underwent conduit interposition or REV operation were risk factors for reoperation. With careful attention to chose best timing and surgical approach depending on morphologic characteristics, biventricular repair for double outlet right ventricle can be achieved with good long-term outcome.
Hearing loss is a common congenital disorder that is frequently associated with mutations in the Cx26 gene (GJB2). Recently, the mutation analysis of GJB2 has been used in a newborn screening test for the detection of hearing impairment. Population-based studies should be performed before the application of genetic testing for the identification of deaf newborns. In this study, 8 positions of GJB2 mutations-including 35delG, 167delT, 235delC, V27I, V37I, M34T, E114G, and I203T-were analyzed using PCR-direct sequencing in a total of 437 healthy Korean neonates. DNAs from dried blood spots were extracted using a commercial DNA extraction kit. The PCR-amplified products (783 bps) of the GJB2 gene were detected using 2% agarose gel electrophoresis and subjected to direct sequencing. The sequences were compared with those in the GenBank database by using the BLAST program. In this study, 5 GJB2 mutations -including V27I (79G>A), V37I (109G>A), E114G (341A>G), I203T (608T>C), and 235delC- were found. Of the 437 neonate samples, 301 subjects showed GJB2 mutations (68.9%, 301/437). The V27I mutation was found in 271 subjects and was the most frequent (62.0%, 271/437). The E114G, I203T and V37I mutations were shown in 146, 17 and 14 subjects, respectively. The 235delC mutation was found in 1 subject. The E114G mutation was frequently accompanied by the V27I mutation. V27I/E114G (97.2%, 143/147) was the most common double mutation and 3 subjects had the double mutation V27I/I203T. A triple mutation, V27I/E114G/I203T, was found in 1 subject. In conclusion, PCR-direct sequencing is a convenient tool for the rapid detection of GJB2 mutations and this data might provide information for the genetic counseling of the GJB2 gene.
Park, Jung-Han;Yoon, Sung-Do;Kim, Chang-Youn;Lee, Sung-Kwan
Journal of Preventive Medicine and Public Health
/
v.17
no.1
/
pp.47-55
/
1984
To study the risk factors associated with maternal HBsAg carrier and the effects of maternal HBs antigenemia on the neonatal health, sera of 729 pergnant women admitted to the Keimyung University Hospital for delivery during the period of February 1-May 30. 1982 were tested for HBsAg by RPHA method and for anti-HBs by PHA method. Among them 43 women (5.9%) had HBsAg and 246 women (33.7%) had anti-HBs giving an infection rate of 39.6%. The interview data for 43 HBsAg positive mothers and randomly selected 210 HBsAg negative mothers showed a statistically significant association between acupuncture history and HBsAg positive rate (p<0.005) which suggest that acupuncture might have contributed significantly to the propagation of viral hepatitis in Korea. The living standard of HBsAg positive mothers was generally lower than that of HBsAg negative mothers which supports the hypothesis that environmental factors are associated with viral hepatitis B infection. None of the 43 neonates born to HBsAg positive mothers had HBsAg in their cord blood. Three months after birth, 35 out of 43 infants were retested and only one infant became HBsAg positive. At six months of age, 32 out of 35 infants were retested and none of them were HBsAg positive except the same infant who was positive at three months. Among 20 control infants of HBsAg negative mothers, all of them were HBsAg negative at three and six months follow-up. These findings are not consistent with the supposition that perinatal infection is a main route of viral hepatitis B transmission in south-east Asia including Korea. HBsAg positive mothers had significantly higher rate of premature delivery (27.9%) than HBsAg negative mothers (11.7%) (p<0.05). Also, the low birthweight incidence rate was higher among HBsAg positive mothers (23.3%) than negative mothers (14.1%) but this was not statistically significant (P=0.16). The premature rupture of membrane was more frequent among HBsAg positive mothers (25.5%) than negative mothers (11.1%) (p<0.05). There were no significant differences in the stillbirth rate and incidence of congenital anomalies between HBsAg positive and negative groups. It was not clarified in this study due to small sample size whether higher incidence of premature delivery and premature rupture of membranes among HBsAg positive mothers was due to HBs antigenemia per so or their lower living standard than HBsAg negative mothers.
It is well known that troponin T(below TnT) is present in the myocardial cells and released during myocardial damage, so it`s very specific enzyme to myocardium. Availability of cardiac specific TnT in assessing perioperatively myocardial damage was evaluated from 34 open heart surgery patients. They consisted of 11 ischemic heart, 13 acquired valvular heart and 10 congenital heart cases. Patients were divided into two groups, group A(patients with myocardial damage) and group B(patients without myocardial damage), according to the symptom of chest pain suspecting angina and the ECG findings of ST segment and T wave changes which show myocardial ischemia and injury. Serum TnT levels were measured by enzyme immunoassay method preoperatively, immediately postoperatively, postoperative day 1, day 2, day 3, and day 7. We observed and analyzed the changes of serum TnT levels in two groups and compared the serum TnT levels with CK-MB levels measured at the same time. In group A, serum TnT levels showed 1.37$\pm$0.26$\mu$g/L, 3.16$\pm$0.66$\mu$g/L, 2.39$\pm$0.74$\mu$g/L, 2.49$\pm$0.76$\mu$g/L, and 1.23$\pm$0.60$\mu$g/L, immediate postoperatively, postoperatively day1, day2, day3, and day7, respectively. It was observed there were significant differences compared with those of group B(0.38$\pm$0.04$\mu$g/L, 0.34$\pm$0.05$\mu$g/L, 0.25$\pm$0.03$\mu$g/L, 0.24$\pm$0.04$\mu$g/L, and 0.11$\pm$0.03$\mu$g/L) during identical periods(P<0.01). Serum CK-MB level in group A significantly elevated to 145.04$\pm$35.08 IU/L on the postoperative day 1 compared to group B(31.28$\pm$5.87 IU/L, P<0.05), However, it stiffly decreased from day 2 and returned to preoperative level at day 3. When serum TnT level more than 1.0$\mu$g/L is thought to reflect myocardial damage, serum TnT had 100% of sensitivity and 87% of specificity in diagnosing the postoperative myocardial damage(p<0.01). I conclusion, serum TnT levels increased significantly at very early stage of myocardial damage and persisted much longer period than CK-MB. This suggests that serum TnT has more advantage and availability in assessing the perioperatively myocardial damage than any other tests.
Cardiac transplantation has been the treatment of patients with end-stage heart disease since it was first performed in 1967. In Korea the first case was performed in 1992 and 42 patients underwent heart trans- plantation so far. The purpose of this article is to report short-term result of cardiac transplantation at our center. Between April 1994 and September 1995, 14 patients had undergone orthotopic heart transplantations. There was 12 male and 2 female patients. Mea recipient age was 34 years(range 11 to 54 years) and mean donor age was 28.4 years(16 to 50 years). Mean graft ischemic time was 120.7minutes(80 to 280 minutes). The follow-up period after transplantation was 11 months(3 to 17 months). Recipient diagnosis included dilated cardiomyopathy in 10, ischemic cardiomyopathy in 2, valvular cardiomyopathy in 1, congenital complex heart disease in 1 patient. The preoperative status of the recipients were state I (50%) and ll (50%) by UNOS classification and class 111 (5 patients) and class IV (9) by NYHA functional class. All patients were treated with triple-drug immunosuppression (cyclosporine, azathioprine, steroid) and induction with RATG. The rejection episodes were 5 times in 3 patients during the follow-up. Causes of infection were aspergillosis (2), and hepes zoster (1), CMV pneumonitis (1). Permanent pace- maker was inserted in 1 patient. Currently 9 patients are alive with seven patients in WYHA functional class I and two in class l . The ejection fraction increased from preoperative value of 19.9 $\pm$ 3.4% to postoperative value of 69.0 $\pm$ 5.6%. The causes of death were cellular rejection (1),chronic graft failure due to size-mismatching (1),respirat- oxy insufficiency due to asthma attack (1), subarachnoid hemorrhage (1), and RIO humoral rejection (1).
Purpose: Death is an important problem for physicians and parents in neonatal intensive care unit. This study was intended to evaluate the mortality rate, causes of death, and the change of mortality rate by year for infants admitted to the neonatal intensive care unit. Methods: We retrospectively surveyed the medical records of the infants who were admitted to the neonatal intensive care unit at Asan Medical Center and who died before discharge between 1998 and 2007. Gestational age, birth weight, gender, time to death and the underlying diseases related to the causes of infant deaths and obtained from the medical records and analyzed according to year. Results: A total of 6,289 infants were admitted and 264 infants died during the study period. The overall mortality rate was 4.2%. For very low and extremely low birth weight infants, the mortality rate was 10.6% and 21.4%, respectively. There was no significant change in the mortality rate during the study period. Prematurity related complications and congenital anomalies were the conditions most frequently associated with death in the neonatal intensive care unit. of the infant deaths 37.1% occurred within the first week of life. Conclusion: Even though a remarkable improvement in neonatal intensive care has been achieved in recent years, the overall mortality rate has not changed. To reduce the mortality rate, it is important to control sepsis and prevent premature births. The first postnatal week is a critical period for deaths in the neonatal intensive care unit.
Kim, Hyung-Tae;Sung, Si-Chan;Kim, Si-Ho;Chang, Yun-Hee;Ahn, Hyo-Yeong;Lee, Hyoung-Doo
Journal of Chest Surgery
/
v.44
no.2
/
pp.115-122
/
2011
Background: The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery. Materials and Methods: From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was $13.4{\pm}10.2$ days (4 to 39 days) and mean body weight was $3.48{\pm}0.33$ kg (2.88 to 3.88 kg). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion. Results: There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p>0.1). There was no late death. The mean follow-up duration was $52.1{\pm}43.0$ months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1. Conclusion: The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.
Background: Prosthetic annuloplasty rings are used for the reconstruction of the mitral valve annulus. However, there is controversy over the best ring to use for reconstruction. In this study, we evaluated the long term result of using a Teflon felt strip as an alternative to commercial rings. Material and Method: From 1996 to 2007, we enrolled 47 patients with pure mitral regurgitation greater than grade III. All patients had an ejection fraction of 40% or more. Patients with congenital heart disease or other valvular heart disease were excluded from this study. They were divided into two groups; the commercial ring group (Group CR) and the Teflon felt ring group (Group TF). Result: There was no mortality. The postoperative echocardiography showed no significant change in the overall ejection fraction (from $58.0{\pm}11.2%\;to\;42.8{\pm}8.4%$). There were no significant differences between the two groups with regard to morbidity, the reoperation rate and recurrence of mitral regurgitation greater than grade II. Conclusion: The long term results from this study showed that posterior mitral annuloplasty using Teflon felt had similar results compared to annuloplasty using commercial rings.
Journal of the korean academy of Pediatric Dentistry
/
v.34
no.1
/
pp.140-149
/
2007
Cleft lip and palate are congenital craniofacial malformation. Reconstruction of dental arch in patient with alveolo-palatal clefts is very important, because they have many problems in functions and esthetics. Malnutrition, poor oral hygiene, respiratory infections, speech malfunctions, maxillofacial deformity, and psychological problems may be occured without proper treatment during the long period of management of the cleft lip and palate. So the treatment should be managed with a multidisciplinary approach. Bone grafting is a consequential step in the dental rehabilitation of the cleft lip and palate patient A complete alveolar arch should be achieyed of the teeth to erupt in and to form a stable dentition. And the presence of the cleft complicate the orthodontic treatment. Therefore bone grafting in patients with cleft lip and palate is a widely adopted surgical procedure. Grafted bone stabilizes the alveolar process and allows the canine or incisor to move into the graft site. After the bone grafting, orthodontic closure of the maxillary arch has become a common practice for achieving dental reconstruction without any prosthodontic treatment. Various grafting materials have been used in alveolar clefts. Iliac bone is most widely fovoured, but tibia, rib, cranial bone, mandible have also been used. And according to its time of occurrence, the bone graft may be divided into primary, early secondary, secondary, late secondary. Bone grafting is called secondary when performed later, at the end of the mixed dentition. It is the most accepted procedure and has become part of treatment of protocol A secondary bone graft is performed preferably before the eruption of the permanent canine in order to provide adequate periodontal support for the eruption and preservation of the teeth adjacent to the cleft. In this report, we report here on a patient with unilateral cleft lip and palate, who underwent iliac bone graft. The cleft was fully obliterated by grafted bone in the region of the alveolar process. The presence of bone permitted physiologic tooth movement and the orthodontic movement of adjacent tooth into the former cleft area. Satisfactory arch alignment could be achieved in by subsequent orthodontic treatment.
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