• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.401-408
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• 2008

Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). Results : The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). Conclusion : Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.976-981
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• 2007

Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by echocardiography from January 2002 to December 2003. Of 130 patients, we identified 17 patients with AoRo dilatation, which was defined as ratio of expected AoRo size by standard nomogram (AoRo ratio) >1.5 (dilator group), and 113 TOF controls, with AoRo ratio <1.5 (non-dilator group). Results : Mean indexed AoRo size ($mm/m^2$) in the first echo was $24{\pm}3.2$ in the dilator group and $18{\pm}3.4$ in the non-dilator group (P<0.0001). AoRo rate of change (mm/year) from the first to latest echo study was $1.6{\pm}3.8$ in dilator group and $0.05{\pm}1.6$ in the non dilator group (P=0.0021). Patients from the dilator group showed a higher prevalence of pulmonary atresia (P=0.031) and a history of aortopulmonary shunt before repair (P=0.048), moderate to severe AR (P=0.0065), and increased left ventricular end-diastolic dimensions (P=0.003). Conclusions : A subset of patients late after TOF repair may show progressive dilatation of AoRo. To identify and prevent long-term sequelae in this patient group, regular follow-up and speculation about AoRo after TOF repair is recommended.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.472-480
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• 1998

We reviewed the surgical results of intracardiac lateral tunnel Fontan procedure for the repair of functional single ventricles. Between 1990 and 1996, 104 patients underwent total cavopulmonary anastomosis. Patients' age and body weight averaged 35.9(range 10 to 173) months and 12.8(range 6.5 to 37.8) kg. Preoperative diagnoses included 18 tricuspid atresias and 53 double inlet ventricles with univentricular atrioventricular connection and 33 other complex lesions. Previous palliative operations were performed in 50 of these patients, including 37 systemic to pulmonary artery shunts, 13 pulmonary artery bandings, 15 surgical atrial septectomies, 2 arterial switch procedures, 2 resections of subaortic conus, 2 repairs of total anomalous pulmonary venous connection and 1 Damus-Stansel-Kaye procedure. In 19 patients bidirectional cavopulmonary shunt operation was performed before the Fontan procedure and in 1 patient a Kawashima procedure was required. Preoperative hemodynamics revealed a mean pulmonary artery pressure of 14.6(range 5 to 28) mmHg, a mean pulmonary vascular resistance of 2.2(range 0.4 to 6.9) wood-unit, a mean pulmonary to systemic flow ratio of 0.9(range 0.3 to 3.0), a mean ventricular end-diastolic pressure of 9.0 (range 3.0 to 21.0) mmHg, and a mean arterial oxygen saturation of 76.0(range 45.6 to 88.0)%. The operative procedure consisted of a longitudinal right atriotomy 2cm lateral to the terminal crest up to the right atrial auricle, followed by the creation of a lateral tunnel connecting the orifices of either the superior caval vein or the right atrial auricle to the inferior caval vein, using a Gore-Tex vascular graft with or without a fenestration. Concomitant procedures at the time of Fontan procedure included 22 pulmonary artery angioplasties, 21 atrial septectomies, 4 atrioventricular valve replacements or repairs, 4 corrections of anomalous pulmonary venous connection, and 3 permanent pacemaker implantations. In 31, a fenestration was created, and in 1 an adjustable communication was made in the lateral tunnel pathway. One lateral tunnel conversion was performed in a patient with recurrent intractable tachyarrhythmia 4 years after the initial atriopulmonary connection. Post-extubation hemodynamic data revealed a mean pulmonary artery pressure of 12.7(range 8 to 21) mmHg, a mean ventricular end-diastolic pressure of 7.6(range 4 to 12) mmHg, and a mean room-air arterial oxygen saturation of 89.9(range 68 to 100) %. The follow-up duration was, on average, 27(range 1 to 85) months. Post-Fontan complications included 11 prolonged pleural effusions, 8 arrhythmias, 9 chylothoraces, 5 of damage to the central nervous system, 5 infectious complications, and 4 of acute renal failure. Seven early(6.7%) and 5 late(4.8%) deaths occured. These results proved that the lateral tunnel Fontan procedure provided excellent hemodynamic improvements with acceptable mortality and morbidity for hearts with various types of functional single ventricle.