One Stage Repair of Berry Syndrome in a Neonate Using an Autologous Arterial Flap / 대한흉부외과학회지

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.

Analysis of the Causes of and Risk Factors for Mortality in the Surgical Repair of Interrupted Aortic Arch / 대한흉부외과학회지

BACKGROUND: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. MATERIAL AND METHOD: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE 1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. RESULT: There were 14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. CONCLUSION: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.

Truncus Arteriosus associated with Interrupted Aortic Arch / 대한흉부외과학회지

A 85-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type A) using one-stage anterior approach without circulatory arrest. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta with regional perfusion and continuity of right ventricle to pulmonary artery was established with Shelhigh(R) pulmonic conduit. The patient experienced left bronchus compression by descending aorta immediately postoperatively, which was improved with positional change and physiotherapy. The patient had reoperation due to stenosis of valved conduit at 13 months later. The patient is currently well under follow-up of 14 months from initial repair.

One-stage Repair of Interrupted Aortic Arch with Ventricular Septal Defect and Valvular Aortic Stenosis: A case report / 대한흉부외과학회지

A male neonate was referred to our hospital with facial cyanosis and tachypnea at 19 days of age. Two-dimensional echocardiography showed type B interrupted aortic arch, posterior malalignment ventricular septal defect and valvular aortic stenosis. A new surgical repair was done with biventricular repair and neo-aortic arch reconstruction. Left ventricular outflow track (LVOT) was consisted of aortic valve and pulmonic valve. Right ventricular outflow (RVOT) track was reconstructed with extracardiac conduit. Postoperative two-dimensional echocardiography showed no stenosis and turbulency flow on LVOT and RVOT.

One-stage Repair of Aortic Arch Anomalies and Intracardiac Defects through Median Sternotomy / 대한흉부외과학회지

BACKGROUND: One-stage repair of aortic arch anomalies and intracardiac defects through median sternotomy has been recently adopted by many institutions since it is known to be safer than the staged repair. The early and midterm results of the one-stage repair of aortic arch anomalies and intracardiac defects were retrospectively evaluated. MATERIAL AND METHOD: 45 patients who underwent one-stage repair of aortic arch anomalies and intracardiac defects performed by one surgeon from January 1996 to July 2003 were included in this retrospective study. The median age of repair was 16 days (range, 3 days~23.7 months) and the mean weight was 3.62+/-1.30 kg. Thirty one (68.9%) had coarctation and 14 (31.1%) had interrupted aortic arch. Associated intracardiac anomalies were VSD in 31 patients (VSD group), TGA or Taussig-Bing anomaly in 10 (TGA group), and others in 4 (ASD in 1, aortopulmonary window 1, truncus arteriosus 1, aortic and mitral stenoses 1, miscellaneous group). The arch obstruction was repaired with end-to-side anastomosis in 23 patients and end-to-end anastomosis in 22. RESULT: Overall postoperative hospital mortality was 22.2% (10/45); 16.1% (5/31) in VSD group, 40% (4/10) in TGA group, and 25% (1/4) in miscellaneous group. There was no mortality in VSD group since 1998, and the mortality in TGA group has remarkably reduced since technical modification for coronary transfer was adopted (75% vs 16.7%). There was no postoperative seizure or other neurological complications. Postoperative aortic restenosis occurred in 5 patients (5/35, 14.3%). Two patients underwent balloon aortoplasty with successful results. There was no reoperation. There was one late death caused by pneumonia 5 months after the operation. Two-year actuarial survival rate including operative death was 72.9%. CONCLUSION: The operative mortality of one-stage repair has been reduced with time and aortic restenosis rate was also acceptable. We concluded that this procedure is a reproducible procedure for aortic arch anomalies associated with intracardiac defects.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

One-stage Repair of Truncus Arteriosus with Interrupted Aortic Arch / 대한흉부외과학회지

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25~50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

One-Stage Repair of Interrupted Aortic Arch and Aortopulmonary Window in a Neonate / 대한흉부외과학회지

The combination of interrupted aortic arch and aortopulmonary window is a rare presentation of congenital heart disease, which requires early diagnosis and surgical treatment. We describe a successful one-stage repair of the anomaly through median sternotomy in a 10-day-old neonate weighing 2.46 kg.

Anatomical Repair of Taussig-Bing Anomaly with Interrupted Aortic Arch and Intramural Left Coronary Artery / 대한흉부외과학회지

We report a case of the successful anatomical correction of the Taussig-Bing anomaly associated with the interrupted aortic arch and intramural left coronary artery for an 38 day-old infant. Aortic arch and neoaortic reconstructions were conducted without any prosthetic or pericardial patch. Intramural left coronary was separated from right one after partial detachment of aortic commissure and both coronary artery buttons were transferred separately to the proximal main pulmonary artery(neo-aorta). Delayed sternal closure was done 3 days after the operation and hospital discharge was delayed for a month because of postoperative pneumonia. Now he is 5 months old and free of symptoms and cardiac drugs.

Common Arterial Trunk: Report of Five Atopsied Cases

The common arterial trunk is a congenital cardiovascular malformation in which one arterial trunk gives origin to the aortic arch, pulmonary and coronary arteries. Other cardiovascular malformations are often associated, such as ventricular septal defect, aortic arch interruption, patent arterial duct and so on. During the early period of life, the persistence of the increased pulmonary arteriolar resistance results in cyanosis. As the pulmonary vascular resistance decreases, the cyanosis disappears but signs of congestive heart failure become the main problems. We report five cases of common arterial trunk that was confirmed by autopsy at Chungnam National University Hospital, Seoul National University Hospital, and Yonsei University Severance Hospital between 1983 and 1995. The ages of these patients at autopsy were 8-28 days and four of them were male. Pulmonary arteries arose as a pulmonary trunk in two cases but three cases showed two arteries arising separately from the posterior wall of the common trunk. The type of ventricular septal defect was juxtatruncal in every case. All five cases had three leaflet truncal valves but three cases showed dysplasia of the leaflets. Interruption of aortic arch was associated in two cases. The cause of death was renal failure in two cases, cardiac failure after corrective surgery in two cases, and pulmonary edema and failure in one case.