• Journal of Chest Surgery
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• v.22 no.6
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• pp.944-950
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• 1989

Children with congenital cardiac defects associated with high pulmonary artery pressure may die despite accurate surgery. Postoperative mortality and morbidity have been attributed to acute rises in pulmonary artery pressure and resistance. Acute pulmonary hypertensive crisis is defined as a paroxysmal event in which pulmonary arterial systolic pressure rises to or above systemic levels followed by a rapid fall in systemic pressure and a minor pulmonary hypertensive event is defined as an acute rise in pulmonary arterial pressure to more than 80 % of systemic levels but without a fall in systemic pressure. From Oct. 1988 to Jul. 1989, we experienced 23 patients who showed many pulmonary hypertensive crises after operation in the Department of Thoracic and Cardiovascular Surgery, Seoul National University Children\ulcorner Hospital. Their preoperative PAP/SAPs were 53 to 123 %[mean 93.3%] and diagnoses were VSD[7], TAPVR[5], TGA[4], AVSD[3], MS[1], DORV[1], Truncus arteriosus[1], and AP window[l]. There were 9 deaths among 23 patients and they showed many pulmonary hypertensive crisis episodes during postoperative intensive care, which was managed by sedation, hyperventilation, oxygen, and acidosis correction and which decreased after using tolazoline. In view of our experience, we recommend that pulmonary artery pressure should be monitored in congenital heart defected patient with preoperative pulmonary hypertension to confirm and to manage the pulmonary hypertensive crisis accurately and using tolazoline is helpful in the treatment of pulmonary hypertensive crisis.

• Journal of Chest Surgery
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• v.35 no.9
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• pp.664-667
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• 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
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• v.52 no.4
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• pp.232-235
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• 2019

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.32-40
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• 1990

Surgical correction of congenital cardiac defects in infants and children with an elevated pulmonary arterial pressure or pulmonary vascular resistance carries a significant early postoperative mortality. And accurate assessments of cardiac output is critically important in these patients. From April 1988 through September 1989, serial measurements of cardiac index, ratio of pulmonary-systemic systolic pressure, ratio of pulmonary-systemic resistance, central venous pressure, left atrial pressure, and urine output during the first 48 hours after the cardiac operation were made in 30 congenital cardiac defects associated with pulmonary hypertension. Cardiac index showed significant increase only after 24 hour postoperatively and this low cardiac performance in the early postoperative period should be considered when postoperative management is being planned in the risky patients. There were no variables which showed any significant correlation with cardiac index. In 12 cases[40%], pulmonary hypertensive crisis developed during the 48 hours postoperatively, and they were treated with full sedation, hyperventilation with 100 % 0y and pulmonary vasodilator infusion. In all patient with preoperative pulmonary hypertension, surgical placement of a pulmonary artery catheter is desirable to allow prompt diagnosis of pulmonary hypertensive crisis and to monitor subsequent therapy.

• Kosin Medical Journal
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• v.33 no.2
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• pp.215-222
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• 2018

An incidentaloma is a tumor found incidentally without clinical symptoms or suspicion; the lesion may be adrenal, pituitary, or thyroidal. We report the case of an asymptomatic individual with preoperatively undiagnosed pheochromocytoma (size: 4.86 cm) that was revealed using elective nonadrenal surgical procedures. The patient demonstrated peri- and post-operative hypertensive crisis and tachycardia. Three days after the dramatic onset of symptoms, the patient expired due to pulmonary edema, multiple organ failure, and terminal sepsis, despite administration of extracorporeal membrane oxygenation-assisted cardiopulmonary resuscitation. A left medial kidney mass obtained at autopsy confirmed pheochromocytoma.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1448-1454
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• 1992

Between July, 1990 and July, 1992, 6 male patients of truncus arteriosus, whose age ranged from 2 months to 18 months, underwent total surgical correction without a extracardiac conduit. Their anatomic types were type I in 3, type II in 2 and III, in one by the Collett-Edwards classification. Surgical techniques were similar to the first description by Lecompte except for the fact that distal pulmonary arterial stumps were approximated to ventriculotomy site without Lecompte maneuver in all cases. Also in all cases, mon-ocusps were placed using glutaraldehyde fixed autologous pericardial patch directly in right ventricular outflow tract. Three patients died postoperatively and the causes of death were myocardial failure, pulmonary hypertensive crisis and pulmonary complication due to progressive pulmonary vascualr obstructive disease respectively. The three survivors have been followed up for 6~10 months with good functional results.

• Journal of Chest Surgery
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• v.25 no.6
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• pp.650-660
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• 1992

To elucidate a potential contribution of endotheline-1[ET-1] to the genesis of pulmonary hypertension and postoperative pulmonary hypertensive crisis in the patients with heart disease, we measured plasma levels of the ET-1 during perioperative period of open heart surgery. In addition, we examined changes of ET-1 during perioperative period and correlations between ET-1 levels and hemodynamic variables. 12 patients including 5 acquired heart disease and 7 congenital heart disease patients were selected randomly as a study group, Group A and B, respectively. 6 patients proved not having heart or hemodynamic problem were selected as a control, Group C. 110 blood samples from pulmonary artery[ET-P] and radial artery[ET-S] were taken and assayed by Sep-pak extraction and RIA. ET-1 levels of Group A were ET-P, 3.94$\pm$5.31pg /ml, ET-S, 3.10$\pm$2.90pg/ml[p>0.05], Group B were ET-P, 1.63$\pm$0.62pg/ml, ET-S, 1.99$\pm$2.45pg/ml[p>0.05], Group C were ET-P, 1.97$\pm$2.02pg/ml, ET-S, 1.72$\pm$0.77pg/ml[p>0.05]. There were no statistically significant differences of ET-1 levels among the Group A, B, C[p>0.05]. There was no correlation between pulmonary artery pressure[PAP] and ET-1 level[p>0.05], and ET-1 levels were not increased even in the cases of pulmonary hypertensive criwis or low cardiac output syndrome, whereas significant correlation between ET-S and pulmonary vascular res-istance[Rp] [r=0.36, p<0.05], and negative correlation between ET-S and OS saturation of pulmonary artery[OS-P][r= -0.49, p<0.01] were identified. Another significant finding was peak increase of ET-1 levels in the postoperative period 1 hour[p<0.05] and then gra-dualy decrease through the postoperative period. In conclusion, ET-1 has no correlation with PAP, whereas correlation with Rp, and inverse correlation with OS-P. It is suggested that ET-1 is neither the direct causative substance of pulmonary hypertension nor pulmonary vasospasm but there must be increased production of ET-1 in chronic pulmonary hypertensive state. Counter-regulatory mechanism to ET-1 is speculated during the pulmonary vasospasm.

• Journal of Chest Surgery
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• v.28 no.1
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• pp.11-17
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• 1995

Fourty-three infants underwent intracardiac repairs within the first 12 months of life. Mean age was 7 months and mean weight was 6.7 kg. A quarter of them were below 5 kg. Overall mortality including complex anomalies was 17.9%. Thirty-four infants with a ventricular septal defect[VSD were treated. Three infants[8.8% died in the early postoperative period, but only one[3.7% of the infants without having associated lesions was dead. VSD was repaired either by the incision on the right atrium, pulmonary artery, or right ventricle. Postoperative right bundle branch block[RBBB occurrence were different according to the approach: 30.8 % in pulmonary arteriotomy, 37.5 % in atriotomy, and 42.8 % in ventriculotomy. Surgically induced heart block did not occur in any patient, but 10 had temporary arrhythmia, and 11 patients were under temporary pacing from one day to 4 days without any persisting rhythm disturbances. Pulmonary hypertension was present in 29 infants preoperatively, and seven of them developed postoperative elevation of the pressure. Infants complicated with pulmonary hypertensive crisis were managed successfully. Inotropics were necessary in 55.8 % of the infants and vasodilators in 37.2 %. Platelet transfusion were needed in 10 cases who required 272 ml in average, but one who died from sepsis consumed 5,370cc of platelets. And we discuss complications and causes of deaths.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.123-134
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• 1991

Twenty eight patients had undergone repair of an isolated complete atrioventricular septal defect between April 1986 and September 1990 in Seoul National University Children`s Hospital. The group comprised 13 male and 15 female patients. They ranged in age from 2 months to 8 years[mean 18.6months] and in weight from 3. 4kg to 23kg[mean 9.0$\pm$4.6kg]. They were analysed as Rastelli type A in 17 patients, Rastelli type B in 2 patients, and Rastelli type C in 9 patients. Seven patients had concomitant Down`s syndrome. All patients had large left-to-right shunt[mean pulmonary to systemic flow ratio 3.5 $\pm$2.2 ranging from 0.68 to 10.0] and high pulmonary systolic pressure[mean 74$\pm$18.8mmHg, ranging from 35 to 110]. In 11 patients, one patch technique was used to close the atrial and ventricular septal defect and 16 patients were undergone by two patch technique. We urgently managed only one patient by pulmonary artery banding whose anatomy was Rastelli type C and severe mitral regurgitation was identified. Postoperative complete A - V block was noted in 3 patients, two of whom were dead in operating room due to combined LVOTO and myocardial failure, and one patient with Rastelli type C was undergone by VVI type permanent pacemaker insertion 1wk later after two patch technique, but we had to manage him by modified Konno operation and total correction due to LVOTO and VSD leakage and severe mitral regurgitation 3 years later. Another two reoperation cases due to severe mitral regurgitation after two patch technique were undergone, one of whom we managed by mitral annuloplasty 3 months later but aggravated mitral regurgitation made us to control him by MVR 3 months later. Another one case of VSD leakage and tricuspid regurgitation was managed by total correction but she died of respiratory insufficiency 14 days later. We experienced pulmonary hypertensive crisis in 3 patients, who were dead in two cases comparing with one control case. So operative mortality is 9/27[33.6%], in one patch group of 3/11[29.2%] comparing with two patch group of 6/16[37.5%]. In summary, causes of death were pump weaning failure, myocardial failure and low cardiac output syndrome and pulmonary hypertensive crisis, resp. failure, complete AV block. Mean follow up period is 15.8$\pm$10.7 months[ranging from 3months to 37 months]

• Journal of Chest Surgery
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• v.24 no.2
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• pp.143-152
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• 1991

From 1983, until June, 1990, 10 patients with various type of truncus arteriosus underwent total surgical correction including Rastelli procedure at Seoul National University Hospital. The age at operation ranged from 1 month to 9 years [mean 2.1 years]. Six patients had truncus type I, 3 patients had truncus type II, and one patients had truncus type IIIc. Right ventricular pulmonary artery continuity was established with a porcine valved conduit in 6 patients, mechanical valved conduit in 1 patient, and bovine pericardial conduit in 3 patients. The postoperative right ventricular /left ventricular pressure ratio ranged from 0.4 to 0.71 [mean 0.51${\pm}$0.14]. The lung histology revealed grade II pulmonary obstructive disease even at 4 month of age. Five patients were dead in hospital [50%], and they were less than 2 year of age. One patient, who had severs congestive heart failure preoperatively, died of low output syndrome and the other died of low output syndrome with postoperative bleeding. There were three death, because of a pulmonary hypertensive crisis that might have been prevented. Two of the five survivors had conduit failure over a mean follow up of 42 months [range 1 to 78 months]. Obstructed conduit was removed and a new conduit constructed using the conduit bed as the posterior wall and the patch of bovine pericardium and Dacron as patch the roof of the conduit. One patient died of acute cardiac failure during the operation. Although results in infants less than 2 years old have not been good, current improvement of intra-and postoperative care suggested that prompt repair is indicated for infants with truncus arteriosus.