• Neonatal Medicine
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• v.18 no.1
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• pp.158-162
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• 2011

Preterm infants with oligohydramnios after preterm premature rupture of membranes can present with severe respiratory distress immediately after birth, and the most common cause is pulmonary hypoplasia. Unlike infants with pulmonary hypoplasia, some cases have shown dramatic improvement with aggressive ventilatory support during the initial 1-2 days of distress: those patients have been defined as having dry lung syndrome. It is assumed that oligohydramnios leads to functional pulmonary hypoplasia by compression of the fetal lungs: some of the improvement in dry lung syndrome may thus have resulted from inflation of compressed lung tissue and increase of lung compliance. We report two incidences of dry lung syndrome that were treated successfully with high inflation pressure and inhaled nitric oxide (NO): these are the first dry lung syndrome cases to be reported in Korean infants.

• Journal of Chest Surgery
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• v.24 no.7
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• pp.719-724
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• 1991

A case of pulmonary atresia with intact ventricular septum was presented in a 10-month-old cyanotic female patient, which was congenitally rare. Infant with pulmonary atresia and intact ventricular septum usually require urgent surgical intervention. Angiogram showed the pulmonary atresia at the level of the pulmonary valve, the hypoplasia of tricuspid valve and atrial septal defect without patent ductus arteriosus. We performed the pericardial patch graft on the right ventricular outflow tract and pulmonary artery after ventriculotomy using pacemaker wire as electrical saw and main pulmonary arteriotomy and then modified Waterston shunt from the ascending aorta to patch on the right ventricular outflow tract without extracorporeal circulation. Patient was postoperatively good condition.

• Journal of Chest Surgery
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• v.23 no.5
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• pp.953-961
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• 1990

Congenitally corrected transposition of the great arteries is a rare congenital heart anomaly, in isolation, has no hemodynamic consequences. It is usually associated with one or more of a variety of intracardiac lesions, ventricular septal defect, valvular or subvalvular pulmonary stenosis, and deformity of the systemic atrioventricular valve with insufficiency. This report describes a successful two stage operation for congenitally corrected transposition, [SLL] type, with ventricular septal defect, pulmonary atresia, persistent ductus arteriosus, and atrial septal defect. A 9 years old patient underwent modified Blalock-Taussig operation because of severe pulmonary hypoplasia. 2 years later a corrective operation, direct closure of ASD and PDA, VSD closure with Dacron patch, Enlargement of left pulmonary artery with pericardial patch and Relief of ROTO with Rastelli procedure could be successfully performed without complication.

• Journal of Chest Surgery
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• v.25 no.1
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• pp.23-31
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• 1992

The ideal approach in the staged management of patients with pulmonary atresia has been a challenging problem and the result has not been always satisfactory. We reviewed our early result of initial palliative surgeries in fifteen cases of pulmonary atresia with interventricular communication Included are eight cases of simple pulmonary atresia with ventricular septal defect and seven cases of pulmonary atresia associated with other complex cardiac anomalies. The ages of the patients were less than one year except one. The morphology of pulmonary vasculature was highly variable and showed unfavorable conditions in most cases. Pulmonary artery was nonconfluent in two. Two-thirds of all cases showed significant problems such as juxtaductal stenosis or diffuse hypoplasia. The ductus arteriosus usually narrowed at its pulmonic end. Initial palliation was done by modified Blalock-Taussig shunt in six, central shunt with or without pulmonary angioplasty in five, right ventricular outflow tract [RVOT] reconstruction in three and direct connection of nonconfluent pulmonary arteries with bilateral cav-opulmonary shunt in one patient. There were 3 hospital deaths. Two of them underwent simultaneous repair of the associated anomaly of TAPVR. Among the six patients who received modified Blalock-Taussig shunt, three needed early second palliative procedure by central shunt, RVOT patch reconstruction and pulmonary angioplasty in each case, All patients who received central shunt showed marked clinical improvement. Among the twelve patients who survived the palliative procedures, two patients underwent total correction 13 months and 18 months after initial palliation respectively. We think that the choice of palliative procedure must be individualized according to the morphology of the pulmonary arteries. More experience and long term follow-up data are necessary to meet this challenging problem.

• Journal of Chest Surgery
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• v.21 no.1
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• pp.175-183
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• 1988

Congenital diaphragmatic hernia [CDH] is a surgical emergency in the newborn infant because it causes severe cardiorespiratory distress. Congenital diaphragmatic eventration [CDE] may also produce severe cardiorespiratory distress in the newborn infant. CDH is an anatomically simple defect that can be easily repaired by reduction of the displaced viscera from the pleural cavity and closure of the diaphragmatic defect. But these infants mortality has not been reduced and still remains very high. The barrier to survival is pulmonary parenchymal and vascular hypoplasia as well as the complex syndrome of persistent fetal circulation. Between May, 1985 and Oct, 1987, 4 neonates with CDH and 1 neonate with CDE were seen in respiratory distress within 12 hrs of birth at St. Francisco general hospital. Each had severe acidosis and hypoxia. And was transferred from a local clinic. They were surgically repaired within 24 hrs of birth. Three neonates lived and two died. Two of the three neonates with CDH operated in the first 6 hrs died. The remaining two [one with CDH, the other with CDE] operated between 6hrs and 24 hrs lived. One case of mortality was combined with bilateral pulmonary hypoplasia and contralateral pneumothorax. The other one case of mortality was combined with complex syndrome of persistent fetal circulation after honeymoon period.

• Clinical and Experimental Pediatrics
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• v.55 no.6
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• pp.212-214
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• 2012

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1124-1136
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• 1988

Massive hemoptysis, usually rapid flooding of tracheobronchial tree and asphyxia, is associated with high mortality. We have controlled massive hemoptysis in two cases with use of bronchial artery angiography & selective bronchial artery embolization with Gelfoam particle. One case was inoperable case that was confirmed as TOF c severe pulmonary artery hypoplasia with massive hemoptysis due to hypertrophied bronchial artery and its collaterals. Another case was congenital ASD with pulmonary Aspergillosis, postop. empyema and BPF associated with massive bleeding due to erosion of hypervascular bronchial artery. We experienced dramatic improvement of general condition and cessation of massive hemoptysis for above two cases. No other problems and complication were noted during postop. hospitalization and follow-up period.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.215-224
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• 1979

Corrected transposition of great arteries associated with dextrocardia [I.D.D.] is a very rare congenital cardiac malformation in which the atria and ventricles are in discordant relation, as are the ventricles and great arteries, but the aorta is to the right of the pulmonary artery. A 13 year old male patient who complained cyanosis and dyspnea on exertion for 8 years, was diagnosed as congenitally corrected transposition of great arteries associated with dextrocardia [I.D.D.], large ventricular septal defect, patent foramen ovale and pulmonary stenosis. He was operated on Nov. 22 1978. The ventricular septal defect was closed with Teflon felt and the pulmonary hypoplasia was corrected with Rastelli operation successfully. On 34th postoperative days, complete heart block was occurred and permanent epicardial pacemaker was implanted with good result.

• Tuberculosis and Respiratory Diseases
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• v.43 no.6
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• pp.1042-1047
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• 1996

The Swyer-James syndrome is a relatively uncommon disease entity presented with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. The main finding is a hyperlucent lung with small hilar shadows on the chest X-ray. Pulmonary angiography is the standard method for diagnosis. We report a case of the Swyer-James syndrome with a brief review of literature.

• Journal of Chest Surgery
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• v.26 no.8
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• pp.591-594
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• 1993

To clarify the apparent hypoplasia of the descending aorta in infants with atrioventricular septal defect[AVSD] patients, we reviewed the catheterization data and angiograms of 34 consecutive patients with AVSD less than 1 year of age who underwent repair at our institution since 1985. We compared them to 10 patients with Atrial Septal Defect[ASD] and 10 patients with Ventricular Septal Defect[VSD] who were matched for age, size and Qp/Qs. The Descending Aorta Index [DAI] of the AVSD group was not different from the VSD or ASD groups, [147.9$\pm$ 34.8 mm2/m2 versus 158.6$\pm$ 31.5 mm2/m2 and 153.2$\pm$ 43.1 mm2/m2].However, the Pulmonary Artery Index [PAI] of the AVSD group was significantly larger than the other groups [684.3$\pm$ 170.7 mm2/m2 versus 454.1$\pm$ 109.1 mm2/m2 and 534.9$\pm$ 148.4 mm2/m2][p<0.05], as was the ratio of PAI/DAI in the AVSD group [4.99$\pm$ 1.77 versus 2.89$\pm$ 0.81 and 3.6$\pm$ 0.92][p<0.05]. Despite similar Qp/Qs ratios, both the mean PA pressure and the Rp/Rs in the AVSD group was higher than the VSD and ASD groups: 43.1$\pm$ 15.6 mmHg versus 29$\pm$ 11.6 mmHg and 24$\pm$ 18.1 mmHg [p<0.05], and 0.27$\pm$ 0.22 versus 0.14$\pm$ 0.03 and 0.11$\pm$ 0.05 [p<0.05] respectively. The apparent hypoplasia of the descending aorta in infants with AVSD is an illusion created by the abnormally large pulmonary arteries, which are significantly larger than in patients with ASDs or VSDs.