Atrioventricular septal defect with an absent or tiny ostium primum defect: a case series of three surgical cases.

Although atrioventricular septal defects are categorized according to the anatomical atrioventricular orifice, the location of the intracardiac shunt in atrioventricular septal defects is important from a surgical perspective. Herein, we report three cases of atrioventricular septal defects with a small or no ostium primum defect. Case 1 (3-month-old girl) was diagnosed preoperatively with a ventricular septal defect, secundum atrial septal defect, and mitral valve cleft. After the operation, the diagnosis was corrected to an atrioventricular septal defect and was repaired completely. Case 2 (9-year-old girl) underwent pulmonary artery banding for a ventricular septal defect with a straddling mitral valve. After the experience with Case 1, we realized similarities between Cases 1 and 2. Therefore, we corrected the diagnosis to atrioventricular septal defect and achieved definitive repair. Based on these experiences, we accurately diagnosed Case 3 (3-month-old boy) with an atrioventricular septal defect. This variant is poorly known; however, proper morphological understanding is necessary to facilitate anatomical repair and prevent postoperative atrioventricular blocks. Some cases of this variant may be diagnosed as a ventricular septal defect with straddling mitral valve and are unable to receive definitive repair. The direction of the cleft, absence of atrioventricular valve offsetting, and trileaflet of the left atrioventricular valve all seem useful for making a diagnosis, and these can be easily confirmed by echocardiography.

Incidentally detected atrioventricular septal defect in an adult.

A 34-year-old woman, a liver transplantation donor for her son, was referred to our hospital for preoperative evaluation. She was diagnosed with an incomplete atrioventricular septal defect (AVSD). She underwent double valve repair and patch closure of the defect. Incidental discovery of an asymptomatic AVSD in an adult is rare.

Surgical repair of ostial obstruction of the coronary artery for quadricuspid aortic valve in an infant.

A congenital quadricuspid aortic valve accompanied by ostial obstruction of the left coronary artery is extremely rare and has not yet been reported in an infant. We herein describe an infant with ostial obstruction of the left coronary artery caused by a small left coronary cusp that formed a pouch. The patient's cardiac function deteriorated due to progressive myocardial ischaemia, and translocation of the ostium of the left coronary artery was performed. Postoperative coronary angiography revealed good filling of the left coronary artery.

Impact of bilateral superior venae cavae on outcome of staged Fontan procedure.

BACKGROUND: The presence of bilateral superior venae cavae may add complexity to the performance of a bidirectional Glenn procedure (BDG). Stagnation of blood flow between the two cavopulmonary anastomoses may increase the risk of thrombosis and impair central pulmonary artery growth. METHODS: Forty patients underwent BDG from January 2004 to April 2011. The cohort was divided into two groups: those receiving bilateral BDG (b-BDG, n = 13) and those receiving unilateral BDG (u-BDG, n = 27). Operative, angiographic, and follow-up data were analyzed retrospectively. RESULTS: None of the patients experienced thrombosis. There was no difference in actuarial survival rate (u-BDG vs b-BDG, 100% vs 92% at 5 years, p = 0.15). On follow-up angiography, no difference in central pulmonary artery index was noted (78.4 ± 45.5 vs 60.4 ± 32.1, p = 0.24). Central pulmonary artery stenosis was detected in 6 patients (4 with u-BDG and 2 with b-BDG), 4 of whom (2 from each group) underwent balloon pulmonary artery plasty before the Fontan procedure. There was no difference in freedom from reintervention for central pulmonary artery stenosis (93% vs 85% at 1 year, p = 0.59). The rate of Fontan completion was comparable between groups, with similar operative variables and satisfactory outcomes. CONCLUSIONS: Bilateral BDG did not increase the risks of thrombosis and central pulmonary artery hypoplasia and can be performed safely without altering the outcome of the Fontan procedure.

[An adult case of tricuspid valve endocarditis with ventricular septal defect; report of a case].

A 50-year-old man was admitted to hospital because of an elevated fever. He had been diagnosed with ventricular septal defect in his childhood, but surgery had not been recommended. An echocardiogram showed vegetations on the tricuspid valve, severe tricuspid regurgitation and perimembranous ventricular septal defect. He was diagnosed with infective endocarditis( IE) and treated with antibiotics and diuretics. Five serious dental caries, which had probably caused IE, were found and extracted before surgery. After 4 weeks of medical treatment, we performed tricuspid valve repair and closed the ventricular septal defect. The postoperative course was uneventful. He has been free from any complication for over 3 years.

[Surgical results of Fontan procedure in hypoplastic left heart syndrome].

BACKGROUND: We reviewed the preoperative hemodynamic factors, surgical results and intermediate outcome of Fontan procedure in hypoplastic left heart syndrome (HLHS). METHOD: From 1990 to 2006, 34 patients with HLHS completed Fontan procedure in our hospital. We compared preoperative hemodynamic factors, surgical results and postoperative course between HLHS group and functional single right ventricle (SRV) group who had undergone Fontan procedure. In addition, influence of the initial palliation [Norwood procedure with modified Blalock-Taussig (BT) shunt, Norwood procedure with right ventricle (RV)-pulmonary artery (PA) conduit and bilateral PA banding] to surgical results and postoperative course was investigated. RESULTS: The PA index was smaller in HLHS group than SRV group but there was no difference in surgical results and intermediate outcome between the 2 groups. Although age at Fontan was older in Norwood with BT shunt, preoperative hemodynamic factors, surgical results and intermediate outcome were similar between the 3 groups. CONCLUSION: Although the PA index in HLHS group was small, the surgical results of Fontan procedure in HLHS group were comparable with those in SRV group. The surgical result of Fontan procedure was satisfactory irrespective of the initial palliative procedure.

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome.

OBJECTIVE: Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. METHODS: Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. RESULTS: Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 +/- 2.6 mmHg, cardiac index of 3.6 +/- 0.8 l/kg/min, and PA index of 194.0 +/- 58.4 mm(2)/m(2); there was no difference between the groups. CONCLUSION: The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity.

Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?

BACKGROUND: Down syndrome is known to affect the natural history of complete atrioventricular septal defect. We analyzed whether Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life. METHODS: Repairs of complete atrioventricular septal defect were performed in 64 infants. Thirty-four infants were associated with Down syndrome, while the other 30 were non-Down patients. RESULTS: Complete follow-up rate was 95% with mean follow-up period of 99+/-47 months (maximum 169 months) in Down patients and 80+/-64 months (maximum 213 months) in non-Down patients. There was one operative death in each group (mortality rate of 2.9% in Down patients and 3.3% in non-Down patients), and three patients died at the late phase (one in Down patients and two in non-Down patients). Five patients underwent re-operation due to postoperative left atrioventricular valve regurgitation (one in Down patients and four in non-Down patients). Freedom from re-operation for left atrioventricular valve regurgitation and actuarial survival rate at 13 years were 96+/-4 and 94+/-4% in Down patients and 85+/-7 and 90+/-5% in non-Down patients (not significantly different). CONCLUSIONS: Down syndrome does not affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life.

Midterm results of mitral valve repair with artificial chordae in children.

OBJECTIVE: We have used artificial chordal replacement with expanded polytetrafluoroethylene sutures for mitral valve repair in children and reported favorable early clinical results. In this article we evaluate the midterm results of mitral valve repair with expanded polytetrafluoroethylene sutures in 39 children. METHODS: From April 1995 through September 2003, mitral valve repair with chordal replacement using expanded polytetrafluoroethylene sutures was performed in 39 patients. In all patients the preoperative grade of mitral regurgitation was moderate or more because of prolapse of the anterior mitral leaflet. The mean age and body weight at the time of the operation were 4.7 +/- 5.3 years (range, 1 month to 17.8 years) and 14.4 +/- 12.2 kg (range, 3.9-54.4 kg), respectively. The number of expanded polytetrafluoroethylene sutures ranged from 1 to 3 (mean, 1.4). The mean follow-up period and body weight at the latest follow-up were 5.0 +/- 2.3 years (range, 1.1-8.5 years) and 25.7 +/- 16.4 kg (range, 6.9-73 kg), respectively. RESULTS: There were no operative or late deaths. Only one patient required mitral valve replacement, which occurred 17 days after repair. Two patients underwent redo mitral valve repair 2 and 5 years after initial repair, respectively. The actuarial freedom from reoperation at 5 and 8 years was 94.8% and 89.5%, respectively. At the latest follow-up, trivial or less mitral regurgitation was observed in 33 (84.6%) patients. CONCLUSIONS: Mitral valve repair with expanded polytetrafluoroethylene sutures in children demonstrated favorable midterm outcome. The procedure is safe and effective, with potential for patients' growth.

The low resistance strategy for the perioperative management of the Norwood procedure.

BACKGROUND: Postoperative course of the Norwood procedure is fragile because of an unstable pulmonary to systemic blood flow ratio caused by fluctuation of systemic and pulmonary vascular resistance. METHODS: Twenty-seven patients with hypoplastic left heart syndrome who underwent the Norwood procedure from June 1998 to February 2002 were managed with the following low-resistance strategy. Intraoperative high-flow and low-resistance cardiopulmonary bypass was achieved with total avoidance of circulatory arrest and a large dose of chlorpromazine. In weaning from the bypass, pulmonary vascular resistance was maximally decreased by inspired oxygen fraction (100%), inhaled nitric oxide (20 ppm), and nitroglycerin (2 to 4 microg/kg/min). Then pulmonary blood flow was determined by adjusting the systemic to pulmonary shunt. Postoperatively, with continuous infusion of chlorpromazine and nitroglycerin as a systemic and pulmonary vasodilator, the inspired oxygen fraction and inhaled nitric oxide were tapered as the arterial oxygen saturation improved. RESULTS: In most patients, inhaled nitrous oxide and inspired oxygen fraction were weaned within 3 days. The postoperative course was stable with minimum changes in circulatory and respiratory status for the survivors. Patients were extubated on a median of 6 postoperative days. Early mortality was 11.1% (3 of 27), and none of the patients died of hemodynamic deterioration. CONCLUSIONS: The low resistance strategy is a simple and useful method for perioperative management of the Norwood procedure, minimizing fluctuation in both pulmonary and systemic vascular resistance and maintaining stable circulatory and respiratory status.