• Journal of Chest Surgery
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• v.51 no.3
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• pp.167-171
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• 2018

Background: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half ($1\text\tiny{1/2}$) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. Methods: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or $1\text\tiny{1/2}$ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3-24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. Results: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54-68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62-128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. Conclusion: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or $1\text\tiny{1/2}$ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

• Journal of Chest Surgery
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• v.47 no.3
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• pp.275-279
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• 2014

A 13-year-old girl, who had undergone the total correction of partial atrioventricular septal defect at the age of 4 years, was admitted with severe tricuspid regurgitation in echocardiography. She had received one-and-a-half ventricle repair during follow-up. Her right ventricle showed global akinesia, and the ejection fraction of the left ventricle was 25% with paradoxical interventricular septal motion. We performed right ventricular exclusion adjunct to the Fontan procedure. She is doing well two years after the operation without complications.

• Journal of Chest Surgery
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• v.38 no.6 s.251
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• pp.403-409
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• 2005

Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum, Material and Method: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days $(2\~382\;days)$. The average Z-value of the tricuspid annulus diameter was -3.1$(-5.6\~0.8)$. Thirteen $(36\%)$ patients had right ventricle-to-coronary artery fistulas, and $4(11\%)$ patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months $(3\;months\~2.2\;years)$, Result: Twenty-four patients under-went initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arierial shunt only (Z-value -2.2 vs. -4.8, p=0.000). There were $5(13\%)$ early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in $12(32\%)$ patients, single ventricular repair in $8(21\%)$, and one and a half ventricular repair in $4(l1\%)$ patients. Nine $(24\%)$ patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was $83.2\%$, respectively. Conclusion: Most of the deaths occurred after the initial palliation. Overall long-term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.

• Journal of Chest Surgery
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• v.40 no.7 s.276
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• pp.473-479
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• 2007

Background: The Hetzer procedure for the correction of Ebstein's anomaly has the advantages of technical feasibility and incorporation of the atrialized right ventricle (RV) into the functional RV. Material and Method: We preformed a retrospective review of 11 patients with Ebstein's anomaly and they had undergone a Hetzer procedure between March 2002 and December 2006. Result: The median age at operation was 19.8 years (range: 6 months ${\sim}56$ years). There were 4 males and 7 females. All patients showed severe tricuspid regurgitation (TR) preoperatively, and arterial desaturation (<95%) was present in 3 patients. The original Hetzer technique was employed in 6 patients with the Carpentier type A anomaly. In the remaining 5 patients with the Carpentier type B or C anomalies, valve competence was restored at the level of the displaced tricuspid valve mechanism. Adjunct bidirectional cavo-pulmonary shunt, or one and a half ventricle repair strategy was employed for all the patients. The median follow-up was 8.6 months (range: $0.8{\sim}51.9$ months). There was no early or late death, and the immediate postoperative TR was trivial to mild in 8 patients. The median cardio-thoracic ratios on chest X-ray at the preoperative period and at postoperative 0, 1 and 6 months were 65%, 62%, 55% and 55%, respectively. Conclusion: The original or modified Hetzer procedure for Ebstein's anomaly shows excellent intermediate-term outcomes.