• Journal of Chest Surgery
• /
• v.9 no.1
• /
• pp.1-9
• /
• 1976

The present. study reports four cases of congenital heart diseases, who received open heart surgery by the Sarn's Heart-Lung-Machine in the department of Thoracic Surgery, Hanyang University Hospital during the period between July 1975 and May 1976. The Heart-Lung-Machine consisted of the Sarn's five head roller pump motor system (model 5000), heat exchanger, bubble trap, the Rygg-Kyvsgaard oxygenator, and monitors. The priming of pump oxygenator was carried out by the hemodilution method using Hartman's solution and whole blood. Of the four cases of the heart diseases, three whose body weight were below 30kg, received the partial hemodilution priming and the remaining one whose body weight was 52kg received the total hemodilution priming with Hartman's solution alone. The rate of hemodilution was in the average of 60.5ml/kg. Extracorporeal circulation was performed at the perfusion flow rate of the average 94.0ml/kg/min, and at the moderate hypothermia between 35'5"C and 30'5"C of the rectal temperature. In the total cardiopulmonary bypass, arterial blood pressure was anged between 30 mmHg and 85 mmHg, generally maintaining over 60 mmHg and venous pressure was measured between 4 and $23cmH_2O$, generally maintaining below $10cmH_2O$. The first case: The patient, a nine year old girl having the symptoms and physical signs typical to cardiac anomaly was definitely diagnosed as isolated pulmonary stenosis through the cardiac catheterization. There was, however, no cyanosis, no pathological finding by X-ray and E.C.G. tracings. The valvulotomy was performed through the arteriotomy of pulmouary artery under the total cardiopulmonary bypass. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The second case: A 12 year old boy with congenital heart anomaly was positively identified as having ventricular septal defect through the cardiac catheterization. As in the case with the first case, the patient exhibited the symptoms and physical signs typical to cardiac anomaly, but no pathological abnormality by X-ray and E.C.G. tracings. The septal defect was localized on atrioventricular canal and was 2 by 10 mm in size. The septal defect was closed by direct simple sutures under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle and pulmonary artery were decreased satisfactory. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The third case: The patient, a 19 year old girl had been experienced the clinical symptoms typical to cardiac anomaly for 16 years. The pink tetralogy of Fallot was definitey diagnosed through the cardiac catheterization. The patient was placed on an ablolute bed rest prior to the operation because of severe exertional dyspnea, fatigability, and frequent syncopal attacks. However, she exhibited very slight cyanosis. Positive findings were noted on E.C.G. tracings and blood picture, but no evidence of pathological abnormality on X-ray was observed. All of the four surgical approaches such as Teflon patch closure (3 by 4cm in size) of ventricular septal defect, myocardial resection of right ventricular outflow tract, valvulotomy of pulmonary valvular stenosis, and pericardial patch closing of ventriculotomy wound were performed in 95 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The fourth case: The patient, a 7 1/4 year old girl had the symptoms of cardiac anomaly for only three years prior to the operation. She was positively identified as having acyanotic tetralogy of Fallot by open heart surgery. The patient showed positive findings by X-ray and E.C.G. tracings, but exhibited no cyanosis and normal blood picture. All of the three surgical approaches, such a myocardial resection of hypertrophic sight ventricular outflow tract, direct suture closing of ventricular septal defect and pericardial patch closing of ventriculotomy wound were carried out in 110 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and the symptoms disappeared.

• Journal of Chest Surgery
• /
• v.38 no.4 s.249
• /
• pp.271-276
• /
• 2005

We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. Material and Method: From August 1989 to June 2001, thirty three adult patients, 18 females and 15 males, underwent total correction for ToF. Their age ranged from 15 years to 54 years (median: 34). Patients were divided into 2 groups by preoperative $SaO_2$ (arterial oxygen saturation): group I$(n=cyanotic,\;SaO_2\;\geq94\%)$ and group II $(acyanotic,\; SaO_2\geq95%)$. Preoperative median hemoglobin level was higher in group I compared to group II (17.5 g/dl vs 15 g/dl). Postoperative follow-up duration ranged from 1 to 94 months (670 patient-month, median: 14 months), and 63 two-dimensional echocardiographic examinations were done during this period. Result: There were no early or late mortality. With regard to RVOT (right ventricular outflow tract) reconstruction, trans-annular patch and RV-PA extracardiac conduit were used in 7 and 3 patients respectively, and all of them belonged to group I. In group I, cardiopulmonary bypass time, aortic cross-clamping time, ICU day, hospital day were significantly longer than in group II, and postoperative inotropic support was significantly greater than in group II. There was no ventricular arrhythmia in both groups, and one patient in group I suffered from atrial arrhythmia, which was resolved spontaneously after tricuspid and pulmonary valve replacement. During follow-up periods, functional class, residual RVOT stenosis and pulmonary regurgitation, tricuspid regurgitation, occurrence of ventricular and atrial arrhythmias were comparable between two groups. Conclusion: In adult ToF patients with severe preoperative cyanosis, more aggressive RVOT reconstruction and careful postoperative care are mandatory. However intermediate-term outcome of this group of patients is comparable to the patients with minimal or no preoperative cyanosis.

• Clinical and Experimental Pediatrics
• /
• v.49 no.6
• /
• pp.635-642
• /
• 2006

Purpose : The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. Methods : We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group : PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group : MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group : TAP). Results : In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. Conclusion : Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.

• Journal of Chest Surgery
• /
• v.35 no.5
• /
• pp.350-355
• /
• 2002

Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

• Journal of Chest Surgery
• /
• v.34 no.8
• /
• pp.597-603
• /
• 2001

Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.42 no.1
• /
• pp.75-79
• /
• 2015

Pulmonary arterial hypertension (PAH) is a common complication of Congenital heart defects (CHD) with left-to-right shunts, and PAH with increased pulmonary vascular resistance (PVR) is associated with considerable morbidity and mortality. General anesthesia (GA) can be life-threatening in patients with PAH, because the positive pressure ventilation during GA increases pulmonary arterial pressure and decreases pulmonary blood flow. This may also lead to hypoxia. Therefore, spontaneous ventilation may be safer than positive pressure ventilation in patients with PAH. A five-year-old male child, weighing 11 kg, with medical history showing a total correction of Tetralogy of Fallot (TOF) in 2009 and ongoing treatment with hypertension (HTN) medicine since 2007, visited the Dankook University Dental Hospital. He had multiple dental caries, and the treatment was completed under sevoflurane insufflation sedation via nasal cannula. The patient remained sedated throughout the operation while maintaining normal vital signs and spontaneous respiration. In conclusion, sevoflurane insufflation sedation may be a safer alternative to GA for the dental treatment of patients with PAH.

• Journal of Chest Surgery
• /
• v.38 no.8 s.253
• /
• pp.583-588
• /
• 2005

Congenital tracheal stenosis can be a life-threatening disease, especially in cases involving the long-segment of the trachea. When patients are symptomatic immediately after birth or develop an accompanying complex cardiac anomaly, surgical repair can be a considerable challenge. We experienced a tracheoplasty in one early infant weighing 2.6 kg and one neonate who had ventilator dependency from long-segment congenital tracheal stenosis and congenital cardiac anomaly. One early infant, who had diffuse stenosis of distal trachea after ventricular septal defect closure, underwent resection and extended end to end anastomosis. One neonate who had diffuse stenosis of proximal trachea with tetralogy of Fallot (TOF), underwent slide tracheoplasty with total correction for TOF Postoperative chest computed tomography showed widely patent trachea. Both infants are now well without symptoms.

• Clinical and Experimental Pediatrics
• /
• v.45 no.2
• /
• pp.208-213
• /
• 2002

Purpose : The major cause of cardiac dysfunction, after open heart surgery for congenital heart disease, is perioperative myocardial injury. Cardiac troponin I is found only within the myocardial cell, so it can be used as a biochemical marker of the myocardial injury. We performed this study to evaluate the worth of cardiac troponin I as a biochemical marker reflecting the extent of perioperative myocardial injury and recovery. Methods : Thirty-four patients who had undergone elective open heart surgery of congenital heart disease(CHD) from April to July 2001 were enrolled in this study. We measured types of CHD, serial cardiac troponin I(baseline 1 day before operation, postoperative day 1, 2, 3, 7), duration of cardiopulmonary bypass(CPB), aortic cross clamping(ACC), intubation and postoperative hospital stay. Results : Compared with the baseline before operation, there was a significant, increase of cardiac troponin I on the postoperative day 1 and a significant gradual decrease on the day 2, 3, 7. The levels of cardiac troponin I were the highest in the transposition of great artery(TGA) repair on the postoperative day 1 and high in the tetralogy of Fallot(TOF), atioventricular septal defect (AVSD), ventricular septal defect(VSD) and atrial septal defect(ASD) repair with decreasing sequence. The longer duration of CPB, ACC and intubation, the higher of cardiac troponin I, but there were no significant correlations between cardiac troponin I levels and duration of hospital stay. Conclusion : Because there was significant increases or decreases of cardiac troponin I according to the perioperative time and types of the congenital heart disease, it is a worthy biochemical marker which reflects the extent of perioperative myocardial injury and recovery after open heart surgery.

• Journal of Genetic Medicine
• /
• v.10 no.2
• /
• pp.99-103
• /
• 2013

Purpose: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. Materials and methods: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. Results: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. Conclusion: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.

• Pediatric Infection and Vaccine
• /
• v.23 no.3
• /
• pp.209-216
• /
• 2016

Purpose: This study aimed to evaluate and compare the characteristics of infective endocarditis (IE) between children and adults with congenital heart disease (CHD) at a single tertiary care center. Methods: In this retrospective medical record review, we extracted the demographic characteristics, diagnostic variables, and outcomes of patients diagnosed with IE and CHD between 2000 and 2016. Results: We identified a total of 14 pediatric patients (nine male; median age at diagnosis, 3 years). Of the 14 patients, six had a history of previous open heart surgery, while four had undergone tetralogy of Fallot repair, with transannular patch or Rastelli procedure. Among the 10 children with positive blood cultures, the most common isolated organism was Staphylococcus spp. (8/10, 80%). Eleven adult patients had IE and CHD. Among the adult patients, only four were diagnosed with CHD before IE, and ventricular septal defect was the most common CHD. The most common isolated organism was Streptococcus spp. (6/11, 55%). Compared with adult patients, pediatric patients had a higher incidence of previously diagnosed CHD (P=0.001), with Staphylococcus spp. as the causative organism (P=0.027). The median duration between the onset of symptoms and diagnosis of IE was 9 days in children and 42 days in adults (P=0.012). Conclusions: Significant differences with regard to the diagnosis and progress of IE were observed between children and adults. Age-adjusted and systematic reassessment may be necessary for the diagnosis and management of IE.