• Journal of Chest Surgery
• /
• v.26 no.9
• /
• pp.701-704
• /
• 1993

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricle. Our approach was to perform complete repair at one stage. The patient who underwent single-stage repair was discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition.

• Journal of Chest Surgery
• /
• v.20 no.2
• /
• pp.404-410
• /
• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Journal of Chest Surgery
• /
• v.37 no.10
• /
• pp.856-860
• /
• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• Journal of Chest Surgery
• /
• v.34 no.11
• /
• pp.870-874
• /
• 2001

Takayasu arteritis is a chronic non-specific inflammatory arteriopathy that involves primarily the first branches of aortic arch but may also affect the aorta and any of its primary branches. A characteristic trait of the disease is that most of the patients are young females of mainly Asian and South American origin. Recently, we experienced a rare case of Takayasu arteritis which showed a cord-like atresia of abdominal aorta just below renal arteries in 52-year-old woman. We performed a side to side bypass graft from descending thoracic aorta to abdominal aorta just above the aortic bifurcation with a 18mm PTFE(polytetrafluoroethylene) vascular graft. The postoperative course was uneventful. .

• Clinical and Experimental Pediatrics
• /
• v.45 no.2
• /
• pp.267-272
• /
• 2002

Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe human fetal heart in-utero and to diagnose congenital heart disease prenatally over the last 20 years. The diagnosis of HLHS in fetal echocardiography is based on 2-dimensional echocardio -graphic evidence of a diminutive ascending aorta, aortic atresia, mitral atresia or severe stenosis and a hypoplastic left ventricle. Abnormal flow direction through atrial septum or through isthmus greatly aids the diagnosis. This report shows a fetal case who showed hypoplastic left side chambers and retrograde isthmic flow and was diagnosed with hypoplastic left heart syndrome. After birth, although the baby had tachy-dyspnea for the first 3 weeks, she finally recovered without any intervention and showed catch up growth of left side chambers. This case illustrates the extreme difficulty of assessing left ventricle in a fetus.

• Journal of Chest Surgery
• /
• v.34 no.9
• /
• pp.720-723
• /
• 2001

A case of Marfan’s syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan’s syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27mm valved conduit for left coronary artery, and Piehler’s modification for right coronary artery bypass using 6mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan’s syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.

• Journal of Chest Surgery
• /
• v.20 no.4
• /
• pp.659-672
• /
• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Journal of Chest Surgery
• /
• v.25 no.4
• /
• pp.406-411
• /
• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• Journal of Chest Surgery
• /
• v.26 no.3
• /
• pp.180-185
• /
• 1993

From February 1982 to December 1991, 49 neonates and 105 infants in less than 3 months of age underwent open heart surgery in Seoul National University Hospital. There were 98 males and 56 females, and their mean ages were 16 days in neonatal group and 67 days in early infant group. Their body weight and height were less than 3 percentile of normal developmental pattern. In order of decreasing incidence, the corrected conditions included Transposition of great arteries with or without ventricular septal defect [43], isolated ventricular septal defect [34], Total anomalous pulmonary venous return [21], Pulmonary atresia with intact ventricular septum [9] and others [47]. Various corrective or palliative procedures were performed on these patients; Arterial switch operation [36], patch closure for ventricular septal defect [34], Repair of total anomalous pulmonary venous return [21], RVOT reconstruction for congenital anomalies with compromised right ventricular outflow tract [17]. Profound hypothermia and circulatory arrest were used in 94 patients [ 61% ]: 42 patients [ 85.7% ] for neonatal group and 52 patients [ 49.5% ] for early infant group. The durations of circulatory interruption were within the safe margin according to the corresponding body temperature in most cases [ 84% ]. The hospital mortality was 36.4% ; 44.9% in neonatal group and 32.4% in infant group 1 to 3 months of age. The mortality was higher in cyanotic patients [ 46.6% ], in those who underwent palliative procedures [ 57.8% ], in patients whose circulatory arrest time was longer than safe periods [ 60% 0] and in patients who had long periods of cardiopulmonary bypss and aortic crossclamping. In conclusion, there has been increasing incidence of open heart surgery in neonates and early infants in recent years and the technique of deep hypothermia and circulatory arrest was applied in most of these patients, and the mortality was higher in cyanotic neonates who underwent palliative procedures and who had long cardiopulmonary bypass , aortic cross-clamping and circulatory arrest.

• Journal of Chest Surgery
• /
• v.44 no.2
• /
• pp.108-114
• /
• 2011

Background: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. Materials and Methods: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was $79.2{\pm}14.8$ months. Results: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. Conclusion: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.