• Journal of Chest Surgery
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• v.42 no.1
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• pp.9-13
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• 2009

Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.793-795
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• 2004

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.547-551
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• 2001

The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

• Journal of the Korean Society for Precision Engineering
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• v.24 no.10
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• pp.123-130
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• 2007

Hypoplastic left heart syndrome is currently the most lethal cardiac malformation of the newborn infant. Survival following a Norwood operation depends on the balance between systemic and pulmonary blood flow, which is highly dependent on the fluid dynamics through the interposition shunt between the two circulations. The purpose of this study is an optimization of the systemic-to-pulmonary artery shunt. In this study, We used computational fluid dynamic(CFD) models to determine the velocity profile in a systemic-to-pulmonary artery shunt and suggested a simplified method of calculating the blood flow in the shunt based on Ultrasound systems. We analyzed the flow characteristic variations and oscillatory shear index(OSI) due to the anastomosis angle and shunt diameter changing. Four different CFD models were constructed with the shunt sizes ranging from 3 to 3.5mm. The angle between the brachiocephalic trunk(BCT) and the shunt were $30^{\circ}$ and $45^{\circ}$, respectively. When the diameter is 3.0 mm, the oscillatory shear index decreased by 1.2% at $30^{\circ}$ as opposed to at $45^{\circ}$. When the diameter is 3.5 mm, it increased by 18% more at $30^{\circ}$ as opposed to at $45^{\circ}$. When the joint angle is $30^{\circ}$ and the diameter is 3.0 mm, the oscillatory shear index decreased by 4.1% in comparison with the 3.5 mm diameter. When the angle is $45^{\circ}$ and the diameter is 3.0 mm, the index increased by 14.6% in comparison with the 3.5 mm diameter.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Journal of Chest Surgery
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• v.29 no.8
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• pp.828-835
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• 1996

From January 1993 to April 1995, 27 neonates (under age of 30 days underwent open heart surgery in the Department of Thoracic and Cardiovascular Surgery, Dong-A Medical Center. Mean age and weight were 12.1 days(2days∼306ays) and 3.29 kg(2.6kg∼4.1 kg) respectively. Cardiac anomalies were simple complete transposition of great arteries(TGA) in 11 neonates, TGA with coarctation of aorta(COA) in 1 , total anomalous pulmonary venous connection(TAPVC) in 5, double inlet right ventricle with TAPVC in 1, interrupted aortic arch(IAA) with ventricular septal defect(VSD) in 3, pulmonary atresia(PA) with intact ventricular septum(IVS) in 3, pulmonary stenosis with IVS in 1, Taussig-Bing anomaly with IAA in 1, and hypoplastic left heart syndrome(HLHS) in 1 . Postoperative complications were myocardial and/or pulmonary edema which caused open sternum in 13 patients(54.2%), acute renal failure( RF) in 10(37.0%), Intractable low cardiac output syndrome (LCOS) including weaning failure from cardiopulmonary bypass in 7(25.9%), bronchopulmonary dysplasia in 1, wound infection in 1, and paroxysmal supraventricular tachycardia in 1. Nine of 13 patients with postoperative open sternum were recovered with delayed sternal closure, and seven of 10 patients survived postoperative ARF with peritoneal dialysis. There were 8 operative deaths(29.6%): 3 in the patients with simple complete TGA, 1 In TCA with COA, 1 in PA with IVS, 1 in Taussig-Bing anomaly with IAA, 1 in DIRV with TAPVC, and 1 in HLHS. One late death occurred after arterial switch operation in simple TGA. The mosts common cause of death was low cardiac output syndrome. Our initial experience of open heart surgery in neonates showed high operative mortality and morbidity, especially in complex anomalies.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.504-509
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• 1999

Background: Heart transplantation is considerated for a selected certain group of complicated congenital heart disease in neonates because corrective surgery is very difficult and has high mortality. Precise planning of transplantation is necessary to adequately fit the donor heart to the recipient. Material and Method: We have performed 4 neonatal pig heart transplantations to test the technical feasibility. Experiment 1: The transplantation was performed using the same technique as the adult heart transplantation. Experiment 2: The transplantation for hypoplastic left heart syndrome was simulated as we reconstructed the whole aortic arch with donor aorta. Experiment 3: The heart transplantation was done with radical pulmonary artery reconstruction. Experiment 4: The experiment was performed for a long term survival. Result: Preoperative planning was very important for adequate fitting. All animals could be weaned from cardiopulmonary bypass, however, two animals died due to bleeding at pulmonary artery and left atrium. Conclusion: We concluded that the neonatal heart transplantation can be applied in some complicated Further using animal model is mandatory.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.504-510
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• 2004

Background: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardio-pulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. Material and Method: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2∼41 days) and median body weight was 2,870 grams (ranges; 900∼3,530 grams). Preoperative diag-noses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. Result: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002∼0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26∼140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7$\pm$7.4 mmHg, 53.7$\pm$11.4 mmHg, and 56.3$\pm$13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3$\pm$7.2, 21.0$\pm$8.4, and 21.2$\pm$8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin admin- istration. Conclusion: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.