Aggressive Surgical Treatment for Complex Cardiac Anomalies Associated with Right Atrial Isomerism / 대한흉부외과학회지

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The postoperative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.

The Recent Outcomes after Repair of Tetralogy of Fallot Associated with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries / 대한흉부외과학회지

BACKGROUND: Tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) is complex lesion with marked heterogeneity of pulmonary blood supply and arborization anomalies. Patients with TOF with PA and MAPCAs have traditionally required multiple staged unifocalization of pulmonary blood supply before undergoing complete repair. In this report, we describe recent change of strategy and the results in our institution. MATERIAL AND METHOD: We established surgical stratagies: early correction, central mediastinal approach, initial RV-PA conduit interposition, and aggressive intervention. Between July 1998 and August 2004, 23 patients were surgically treated at our institution. We divided them into 3 groups by initial operation method; group I: one stage total correction, group II: RV-PA conduit and unifocalization, group III: RV-PA conduit interposition only. RESULT: Mean ages at initial operation in each group were 13.9+/-16.0 months (group I), 10.4+/-15.6 months (group II), and 7.9+/-7.7 months (group III). True pulmonary arteries were not present in 1 patient and the pulmonary arteries were confluent in 22 patients. The balloon angioplasty was done in average 1.3 times (range: 1~6). There were 4 early deaths relating initial operation, and 1 late death due to incracranial hemorrhage after definitive repair. The operative mortalities of initial procedures in each group were 25.0% (1/4: group I), 20.0% (2/10: group II), and 12.2% (1/9: group III). The causes of operative mortality were hypoxia (2), low cardiac output (1) and sudden cardiac arrest (1). Definitive repair rates in each group were 75% (3/4) in group I, 20% (2/10, fenestration: 2) in group II, and 55.6% (5/9, fenestration: 1) in group III. CONCLUSION: In patients of TOF with PA and MAPCAs, RV-PA connection as a initial procedure could be performed with relatively low risk, and high rate of definitive repair can be obtained in the help of balloon pulmonary angioplasty. One stage RV-PA connection and unifocalization appeared to be successful in selected patients.

Fontan Procedure for Functional Single Ventricle with Major Aortopulmonary Collateral Arteries / 대한흉부외과학회지

Not only morphology of pulmonary artery or pulmonary artery resistance, but major aortopulmonary collateral arteries are risk factors of Fontan procedure. We report a successful Fontan procedure after rehabilitation of pulmonary arteries by unifocalization and systemic to pulmonary shunt in a high risk Fontan candidate with functional single ventricle combined with hypoplastic pulmonary arteries and major aortopulmonary collateral arteries supplying most of the bilateral lung field.

Tetralogy of Fallot Associated with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries: Comparison between the different surgical approaches / 대한흉부외과학회지

BACKGROUND: Patients with tetralogy of Fallot(TOF), pulmonary atresia(PA) and major aortopulmonary collateral arteries(MAPCAs) have been managed by heterogenous surgical strategies. We have taken 5 different surgical approaches to this lesion. In this study, relative advantages and disadvantages of each strategy were discussed by analyzing our surgical results. MATERIAL AND METHOD: Between January 1986 and June 1998, 50 patients aged 1 to 177(39+/-31) months at a various morphologic spectrum of this lesion were treated at our institution. The groups, which were classified according to the initial approaches, consisted of one-stage unifocalization(A-1, n=9), staged unifocalization(A-2, n=11), right ventricle to pulmonary artery connection(RV-PA)(B-1, n=11), one-stage RV-PA plus unifocalization (B-2, n=11), and one-stage definitive repair(C, n=8). Morphologic charateristics, operative mortalities, and probabilities of definitive repair were compared between the groups and the causes of death, complications and the follow-up results were described. RESULT: Mean ages at the first operation were 57+/-18(A-1), 42+/-48(A-2), 18+/-14(B-1), 52+/-55(B-2), and 32+/-34(C) months (p0.05). Nineteen patients had more than 1 dependent MAPCAs. True pulmonary arteries were not present 13 patients and they were confluent in 29. A total of 101 operations were performed. Operative mortalities of initial procedures were 33%(3/9, A-1), 18%(2/11, A-2), 0%(0/11, B-1), 36%(5/11, B-2) and 13%(1/8, C) (p<0.05). Percentages of the definitive repair among the patients of each group were 22%(2/9, A-1), 18%(2/11, A-2). 45%(5/11, B-1), 27%(4/11, B-2), and 100%(8/8, C)(p<0.05). Hypoxic respiratory and cardiac failure(6), hypoxic encephalopathy(2) and sepsis(4) were the major causes of death. Phrenic nerve palsy occured in 5 patients. Thirty-one patients among the survivals have been followed up for a mean duration of 74+/-42(3-145) months. One and five year actuarial survival rates were 73% and 73 %. CONCLUSION: In conclusion, Right ventricle to pulmonary artery connection used in patients with TOF/PA/MAPCA as an inital procedure appeared to be highly successful in enhancing the chance of satisfactory definitive repair without the significant surgical risks. One-stage total repair at an ealier age group could be performed safely with the resonable outcomes. Unifocalization approach, whether it was performed in a single stage or in the multiple stages, resulted in the high operative mortality and the lowest chance of definitive repair, however more tailored selection of the patients and the long follow-up is mandatory to prove the usefulness of this approach.

The Clamshell Incision for the Complete Repair in Pulmonary Atresia with Ventricular Septal Defect , and Major Aortopulmonary Collaterals / 대한흉부외과학회지

A 6-month old girl who had pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals underwent one-stage complete repair with unifocalization through a bilateral thoracosternotomy(clamshell incision). There were no serious postoperative compli cations, and the postoperative echocardio-graphy showed no residual ventricular septal defect or significant pulmonary artery stenosis. In this condition, great surgical variability exists regarding the sources of pulmonary blood flow. Recent clinical work has focused on a one-stage complete repair. The potential advantages of the clamshell incision are apparent in terms of mediastinal approach, postoperative results, and safety.

Midline One-Stage Complete Unifocalization and Repair for Pulmonary Atresia, Ventricular Septal Defect associated with Major Aortopulmonary Collaterals: 1 case report / 대한흉부외과학회지

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a rare and complex lesion in which great morphologic variability exists regarding the sources of pulmonary blood flow. We report a case of this disease with no true central pulmonary arteries in a 9-month-old-boy successfully treated by one-stage complete unifocalization and repair from a midline sternotomy approach.

Staged Total Correction of Complexed Anomalous Origin of Right Pulmonary Artery from the Ascending Aorta: One Case Report / 대한흉부외과학회지

We experienced a case of anomalous origin of right pulmonary artery from the ascending aorta associated with pulmonary atresia, ventricular septal defect, absence of left pulmonary artery and multiple major aortopulmonary collateral artery (MAPCA). At ten month of age, left pulmonary artery creation with unifocalization and right pulmonary artery banding were performed as the first stage, followed by coil embolization of right MAPCA 1 month later, and 1 year later, the total correction was done. After total repair, the patient showed good postoperative course, and excellent angiographic and hemodynamic results at 1 year follow-up study.