• Journal of Chest Surgery
• /
• v.35 no.9
• /
• pp.664-667
• /
• 2002

Extracorporeal membrane oxygenation(ECMO) provides stable oxygenation to prevent elevation of pulmonary vascular resistance and bypasses a significant part of cardiac output to the pulmonary vascular bed to reduce pulmonary perfusion pressure. In addition, ECMO prevents right heart failure and low cardiac output by means of ventricular assist and reduction in volume load to right ventricle. As a result, ECMO can be used for the treatment of pulmonary hypertensive crisis after surgery for congenital heart disease, especially when it is refractory to conventional measures. We report a case of postoperative pulmonary hypertensive crisis, developed in a 37-year-old male with patent ductus arteriosus with secondary pulmonary hypertension, which was successfully managed including ECMO.

• Journal of Chest Surgery
• /
• v.32 no.6
• /
• pp.584-587
• /
• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Neonatal Medicine
• /
• v.15 no.2
• /
• pp.200-206
• /
• 2008

Human chromosome 9 is characterized by a high degree of morphologic heteromorphisms, including variation in the size of the heterochromatin. We present a case of a de novo short arm addition of chromosome 9, [46, XY, add(9)(p13)], associated with multiple anomalies, including trigonocephaly, upward slant of the palpebral fissures, patent ductus arteriosus, pulmonary hypertension, hypertrophic cardiomyopathy, umbilical hernia, ambiguous genitalia, and sensorineural hearing and visual loss. This mutation affects the pericentric region of the heterochromatin. This patient exhibited a clinically important breakpoint of the heterochromatic region of chromosome 9 short arm and the associated anomalies.

• Journal of Chest Surgery
• /
• v.14 no.2
• /
• pp.144-152
• /
• 1981

The effect of cardiopulmonary bypass procedures on the blood components were studied through the measurements of several serum enzymes, SGOT, SGPT, CPK and LDH with its isozymes in the patients who had surgery for their heart diseases. They wre 85 patients: 40 Males and 45 Females. Their ages ranged widely between one and fifty-six. They were divided into 5 groups: Group I.patent ductus arteriosus without extracorporeal circulation, Group II-atrial septal defect, Group III-ventricular septal defect, Group IV-tetralogy of Fallot, and Group V-valve replacement. Generally serum enzymes revealed the increased values after surgery and the tendency returning toward preoperative levels. With the high total serum LDH levels seen uniformly after operation which persisted long in patients with replaced valves, the changes of LDH-isozymes were further analyzed. The isozymes, especially LDI and LDs increased their activities postoperatively, and the other fractions varied little. And $LD_1$/$LD_2$ ratios also showed similar rises and falls while the elevated values lasted longer in the patients with cardiac valve replacement. The significant values of LDH-isozymes in predicting the hemolysis occuring after extracorporeal circulation and in following the patients whose valves were replaced have been further discussed with the special stress placed on the $LD_1$/$LD_2$ratios.

• Journal of Chest Surgery
• /
• v.27 no.1
• /
• pp.24-30
• /
• 1994

Eighteen infants with a large ventricular septal defect[VSD] underwent primary surgical repair from January 1986 to December 1992. Operation was done because of failure to thrive, medically intractable heart failure, recurrent pneumonia, increased pulmonary vascular resistance[PVR]. Four patients[22.2%] died in the early postoperative period. Relief of heart failure and normalization of growth and weight gain was evident in all survivor. There was no late postoperative death. The results of primary surgical repair of VSD in infancy are compared with those of palliative pulmonary artery banding[PAB] and of VSD closure after PAB. Twenty-seven patients with isolated VSD or with VSD associated with atrial septal defect, patent ductus arteriosus, or coarctation of the aorta underwent initial palliative PAB. There were 3 early postoperative deaths[11.1%]. Severe elevation of PVR persisted in two patients. Closure of VSD and pulmonary artery debanding was done in twenty patients, with 2 early postoperative deaths[10.0%]. Placement of the PAB too close to the pulmonary annulus necessitated trasannular patching in one patient, but any problem caused by migration of the band was not developed. It is concluded that primary surgical repair of VSD in infancy is reasonable and that PAB is indicated only for those patients less than 6 months old with a complicated defect or in an emergency situation.

• Journal of Chest Surgery
• /
• v.22 no.5
• /
• pp.739-747
• /
• 1989

Between September 1986, and August 1989, eight infants underwent operation for repair of coarctation of the aorta in the first year of life. The patients included 7 males and 1 female ranging in age 19 days and 9 months. Weights ranged from 3.5 Kg to 7 Kg [mean 5 Kg]. All patients had preductal coarctation of the aorta. Each infant had associated cardiac anomalies, including ventricular septal defect [7 infants] and patent ductus arteriosus [5 infants]. All had intractable congestive heart failure, despite aggressive medical therapy. Pressure gradient across the coarctation ranged from 10 mmHg to 60 mmHg. Operative techniques were subclavian flap aortoplasty in five cases, Gore-Tex patch aortoplasty in three cases. In addition to coarctation repair, six infants had concomitant banding of the pulmonary artery. Four infants required ventilator support for several days. There was no operative death. Complications developed in two. One infant had tracheal stenosis after a tracheostomy. Another infant had restenosis of the aorta revealed by cardiac catheterization 30 months after surgery. The pressure gradient was 30 mmHg, necessitating balloon dilatation aortoplasty. Results were satisfactory. During follow up, we performed total correction procedures [patch closure of the ventricular septal defect, infundibulectomy, pulmonary valvotomy and pulmonary artery angioplasty] in one case. Continuing follow-up finds all patients in good condition.

• Archives of Plastic Surgery
• /
• v.46 no.5
• /
• pp.470-474
• /
• 2019

Congenital absent sternum is a rare birth defect that requires early intervention for optimal long-term outcomes. Descriptions of the repair of absent sternum are limited to case reports, and no preferred method for management has been described. Herein, we describe the use of porcine acellular dermal matrix to reconstruct the sternum of an infant with sternal infection following attempted repair using synthetic mesh. The patient was a full-term male with trisomy 21, agenesis of corpus callosum, ventricular septal defect, patent ductus arteriosus, right-sided aortic arch, and congenital absence of sternum with no sternal bars. Following removal of the infected synthetic mesh, negative pressure wound therapy with instillation was used to manage the open wound and provide direct antibiotic therapy. When blood C-reactive protein levels declined to ${\leq}2mg/L$, the sternum was reconstructed using porcine acellular dermal matrix. At 21 months postoperative, the patient demonstrated no respiratory issues. Physical examination and computed tomography imaging identified good approximation of the clavicular heads and sternal cleft and forward curvature of the ribs. This case illustrates the benefits of negative pressure wound therapy and acellular dermal matrix for the reconstruction of absent sternum in the context of infected sternal surgical site previously repaired with synthetic mesh.

• Journal of Chest Surgery
• /
• v.30 no.6
• /
• pp.585-590
• /
• 1997

Between December 1994 and October 1996, 57 premature infants with evidence of a hemodynamically significant PDA associated with cardiopulmonary compromise underwent indomethcin therapy(Group I, n=48) or surgical ligation(Group II, n=9) because of indomethacin failure. The gestational alee(29.6$\pm$ 3.1weeks vs. 28.1 $\pm$ 1.6weeks) and birth weight(1,413 $\pm$ 580gm, ,098 $\pm$ 235gm) showed no significant differences between the two groups. Medical management included fluid restriction, diuretics, and indomethacin therapy(one or two cycles). Surgical libation was done at the neonatal intensive care unit(NICU) without moving the patient to the operation room. There was no complication associated with the operation. There were 9 deaths in Group I(19%, 9/48) and 2 deaths in Group II(22% , 219). The main causes of deaths were persistent bronchopulmonary dysplasia with sepsis(n=8) and intrapulmonary hemorrhage(n=3). The rate of medical treatment failure including death and complication in premature infants whose body weights were less than 1500gm was higher(41%, 15/38) than in premature infants whose body weights were more than 1500gm(16%, 3/19). Early surgical ligation of PDA may be applicable in the premature infant with a large size, low birth weight(<1500 gm), or associated intracardiac anomalies. Perfoming the operation in the NICU may be safe in s ead of moving the patient to the operating room.

• Journal of Chest Surgery
• /
• v.24 no.9
• /
• pp.853-860
• /
• 1991

A clinical study on 139 cases of operated PDA was performed during period from Aug. 1982 to Apr. 1991 at the Dept. of Thoracic and Cardiovascular Surgery of Chonbuk National University Hospital. The following results are obtained. 1. The 35 males and 104 females ranged in age from 6 months to 40 years. [mean 10.2 yrs. ] 2. Chief complaints of the patients were frequent URI in 50%, dyspnea on exertion in 31.2%, palpitation in 11.1%, and no subjective symptoms in 28.78% 3. On auscultation, continuous machinery murmur heard in 79.86% and systolic murmur in 20.14%. 4. Radiologic findings of chest P-A showed increased density of pulmonary vascularity in 80.58%, cardiomegaly in 61.87%, and within normal limit in 19.42% of the patients. 5. The signs of LVH[44.4%], RVH[17.4%], BVH[7.6%] were noted on the EKC. 6. Cardiac catheterizations were performed in 114 patients. The mean Qp/Qs was 2.65 and the mean Pp /Ps was 0.41 and the mean systolic pulmonary artery pressure was 46.6 mmHg. 7. Operative methods were as followed: The 130 cases[93.52%] of ligation and 3 cases[2.16%] of division & suture for PDA were performed through the left posterolateral thoracotomy. And the remained cases were managed under the cardiopulmonary bypass. 8. Operative complications were hoarseness in 8 cases, atelectasis in 6 cases, intraoperative ductal rupture under the left thoracotomy approach 2 cases, recannalization 1 case and others in 3 cases. 9. One patient died due to ductal rupture intraoperatively and the overall mortality was 0.7%.

• Clinical and Experimental Pediatrics
• /
• v.45 no.7
• /
• pp.917-922
• /
• 2002

An unbalanced translocation is frequently the result of inheritance of an unbalanced haploid set from a parent with a balanced translocation. Families in which one parent is a balanced translocation carrier fall into the following classes : Those in which none of the possible abnormal offsprings is viable; Those in which one type of offspring, usually the one with the smaller deletion, is born alive; Those in which two types of abnormal offspring are viable. We report a neonate whose karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),der(20)t(2;20)(q21;p13). She was small for her gestational age and had multiple anomalies such as exophthalmos, corneal opacity, short neck, tongue tie, clinodactyly, atrial septal defect, patent ductus arteriosus and ventriculomegaly. Moreover, her mother's karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),del(16)(q22.1),der(20)t(2;20)(q21;p13) but her father had normal karyotype. The same derivative chrosomes were found between mother and her infant, except for del(16)(q22.1) in her mother and these same unbalanced translocations in a two-generation family are extremely rare.