Cerebral abscess following the self-extraction of teeth in patient with Ebstein's anomaly: a case report.

BACKGROUND: Antibiotic prophylaxis before invasive treatments, including dental extractions, is still recommended for patients at high risk of infective endocarditis. However, the risk from self-extraction of teeth in daily life of patients with intellectual disabilities is uncertain. CASE PRESENTATION: A 6-year-old patient with Ebstein's anomaly developed cerebral abscess, which appeared associated with infective endocarditis of dental origin. Two weeks after self-extraction of his deciduous teeth, he began to experience pain in his ear and developed continuous fever, followed by vomiting, facial spasm, and a loss of consciousness. He was admitted into a hospital for 2 months, during which he received intravenously administered antibiotics and a drainage tube in his brain. CONCLUSIONS: Deciduous teeth can be self-extracted before root resorption and natural shedding in patients with intellectual disabilities. When they are at high risk of infective endocarditis and frequently touch mobile deciduous teeth, it seems to be an option to extract the teeth early with antibiotic prophylaxis, rather than to wait natural fall.

Extensibility of autologous pericardium roll conduit in non-confluent pulmonary artery: a case report.

BACKGROUND: There is great discussion about non-confluent pulmonary artery (PA) reconstruction, and several materials have been used. Autologous pericardium is considered feasible for infectious resistance, autoimmune response, extensibility, and growth potential. CASE PRESENTATION: The patient was born at 39 weeks (body mass = 2550 g). He was diagnosed with tetralogy of Fallot, pulmonary atresia, non-confluent PA, and bilateral patent ductus arteriosus. Right and left Blalock-Taussig shunts with patent ductus arteriosus ligations were placed on day 27 and 3 months, respectively. At 19 months (8.8 kg), definitive repair was performed with tricuspid valved conduit concurrent with PA reconstruction using an autologous pericardium roll conduit. The autologous pericardium was treated with glutaraldehyde (autologous pericardium fixed with 0.4% glutaraldehyde for 7 min and rolled as conduit - 12 mm in diameter and 30 mm in length). Following an incision on the visceral side of the PAs before the 1st branch, the autologous pericardial roll conduit was anastomosed. Follow-up angiographies on postoperative months 9 and 57 demonstrated that the PA, including the autologous pericardium roll conduit, had spontaneously enlarged. CONCLUSION: Particularly for non-confluent PA, the patients require increased pulmonary beds at an early age because of hypoplastic PA. While size mismatch between the graft and native PA develops as the child grows, size-adjustable extensibility of the PA graft should be noted.

Neo-aortic insufficiency late after staged reconstruction for hypoplastic left heart syndrome: impact of differences in initial palliative procedures.

The neo-aortic insufficiency in patients with hypoplastic left heart syndrome is an important sequela. We assessed the risks of the neo-aortic valve deterioration by the difference of initial palliations: Group I underwent primary Norwood (Nw) with systemic-to-pulmonary artery shunt (SPS), Group II underwent bilateral pulmonary artery banding (bPAB) and subsequent Nw with SPS (bPAB-Nw/SPS), Group III underwent bPAB and subsequent Nw with bidirectional Glenn (BDG) procedure (bPAB-Nw/BDG). The neo-aortic valve z score changes over time did not reach statistical significance in all groups (p = 0.43 for Group I, 0.20 for Group II, and 0.30 for Group III). The degree of neo-aortic valve insufficiency did not change significantly over time during this period except for Group III (p = 0.34 for Group I, 0.20 for Group II, and 0.02 for Group III). On the other hand, dimensions of the neo-aortic annulus and degrees of neo-aortic insufficiency did not differ significantly among the 3 groups at any pre-determined time. The presence or absence of incision into the sino-tubular junction at Nw did not affect the late neo-aortic valve z score or insufficiency. These data indicate that the difference of initial palliative procedures does not affect late neo-aortic valve insufficiency in Nw survivors. Because valve failure may develop in longer follow-up, further observation should be conducted.

Rapid growth of pulmonary artery after intrapulmonary artery septation.

Intrapulmonary artery septation is employed in patients with unbalanced pulmonary artery growth. A baby girl received a systemic pulmonary shunt in the neonatal period and bilateral Glenn shunts in early infancy. Once the Glenn shunts were established, the right pulmonary artery became hypoplastic. We performed intrapulmonary artery septation involving the Glenn shunts to the left pulmonary artery and the systemic pulmonary shunt to the right pulmonary artery. As early as 3 months after intrapulmonary artery septation, right pulmonary artery growth was observed. Eventually, Fontan circulation was achieved with fenestration.

Pulmonary vein obstruction after primary sutureless pericardial repair of a total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital heart disease. The development of pulmonary venous obstruction is one of the major risk factors for poor outcomes after surgical repair. Sutureless pericardial repair for a total anomalous pulmonary venous connection was introduced to decrease the risk of pulmonary venous obstruction after surgical repair, and favourable outcomes have been reported. Herein, we report the case of an infant with a total anomalous pulmonary venous connection who developed pulmonary venous obstruction after primary sutureless pericardial repair.

Valve-sparing reimplantation for neoaortic root dilatation and regurgitation with an unbalanced cusp after the arterial switch operation.

A 13-year-old male presented with neoaortic root dilatation and severe aortic valve regurgitation 13 years following an arterial switch operation. The valve cusps were unbalanced due to an enlarged non-coronary cusp. A valve-sparing reimplantation with a cusp plication was performed which resulted in a competent valve with trivial regurgitation. Thus, even in an unbalanced cusp, valve-sparing reimplantation can be used for neoaortic root dilatation and valve regurgitation after an arterial switch operation.

Successful staged Fontan completion for a tricuspid atresia patient with left ventricular non-compaction.

We report a case of Fontan completion for a tricuspid atresia (TA) patient with left ventricular non-compaction (LVNC). The patient was diagnosed with TA (Ia) with LVNC by fetal echocardiography. Because the unfavourable prognosis of LVNC was anticipated, Imidapril as well as Carvedilol were administered to improve cardiac function, from the early stages of infancy. Staged Fontan completion with fenestration was successfully achieved with improvement of LV function.

[Impacts of bilateral pulmonary artery banding on the surgical and long-term outcomes in hypoplastic left heart syndrome( HLHS) and its variants].

Although Norwood-type operation is a fundamental procedure for hypoplastic left heart syndrome and its variants, the surgical risk is still high even in the high volume center. Bilateral pulmonary artery (PA) banding( BPAB) is an alternative procedure to avoid the risk in the neonatal period. We clarified the impacts of BPAB on the surgical and long-term outcomes in HLHS and its variants. Consecutive 85 patients( classical HLHS:52, variant:33) who underwent surgical intervention between July, 1993 and December, 2012 were evaluated. Fetal diagnosis was done in 37 patients (44%). Forty-two patients underwent primary Norwood operation[ Gp N, Blalock-Taussig( BT):29, right-ventricle( RV)-PA conduit:13] and the remaining 43 patients underwent BPAB followed by Norwood operation. Among them, 4 patients did not reach Norwood [9%, atrioventricular valve regurgitation (AVVR):1, infection:1, preoperative shock:2). The survived 39 patients were divided into 2 groups;Gp BN (BPAB → Norwood+BT/RV-PA conduit, n=22), Gp BG [BPAB → Norwood+bidirectional Glenn procedure(BDG), n=17]. BPAB for HLHS and its variants is useful in terms of lower mortality. The 2nd stage palliation is so important that modified Norwood procedure with BT shunt or RV-PA conduit contribute to the lower morbidity including neurological outcome. Norwood+BDG strategy might be beneficial for the high risk patients such as with poor cardiac function or low body weight.

Current status of the hybrid approach for the treatment of hypoplastic left heart syndrome.

The hybrid approach for hypoplastic left heart syndrome (HLHS), consisting of bilateral pulmonary artery banding and ductal stenting, has emerged as an alternative to the traditional Norwood approach. This approach defers open heart surgery to beyond the neonatal period, which is believed to reduce postoperative mortality and morbidity and improve neurological development as compared with the conventional approach. However, there have been no scientific studies supporting these hypotheses. Recently, there seems to be a tendency that many centers recommend the hybrid approach as an interim procedure to rescue preoperative high-risk patients. Currently, the decision to adopt the hybrid approach or the Norwood approach seemed to be based on the preference of congenital heart surgeons and cardiologists. Further investigation including a randomized multi-center study would allow a scientific decision as to which approach is more appropriate for the patient with HLHS.

[Re-aortic valve operations in children].

We have performed aortic valve replacement (AVR) with mechanical valve, with/without annular enlargement in pediatric patients with left ventricular outflow tract obstruction (LVOT). Twenty-two patients underwent mechanical AVR between May 1993 and December 2012. The cumulative survival rates by the Kaplan-Meier method were 95% in 5 year, and 95% in 10 year. Long-term result of mechanical AVR with/without annular enlargement in children is excellent. Therefore, it should be the 1st choice of surgical treatment at our institute. In this strategy, we had total 10 re-operation cases. Two cases:Konno operation after performing surgical aortic valvotomy, 6 cases:Konno operation after resection of subaortic stenosis, 1 case:re-valve replacement because of thrombus formation at mechanical valve, and 1 case:re-operation with somatic growth. At the age of operation, 2 years old and over cases had postoperative estimated effective orifice area index (EOAI)>0.85 cm2/m2 in the time of 20 years old. Since some issues concerning anticoagulation-related complications remain, careful observation is mandatory.

Successful staged repair for a rare type of truncus arteriosus with interruption of the aortic arch and abnormal origin of the left coronary artery.

We report a successful staged repair for a quite rare combination of truncus arteriosus (TA), Van Praagh type A4, and abnormal origin of the left coronary artery (CA). Furthermore, the case was complicated by a variant of the chromosomal anomaly in cat-cry syndrome. The presence of interruption of the aortic arch (IAA) and abnormal CA origin has been previously reported to increase mortality. To decrease the risk of bronchomalacia in infants, bilateral pulmonary artery banding (PAB) was performed as the first stage procedure for adjusting the pulmonary flow. Staged repair is a useful strategy for infants with complex TA.

Outcomes of swing-back aortic arch repair in arterial switch and Norwood operations.

The outcomes of the swing-back technique for aortic arch repair during the arterial switch and Norwood operations are not well reported. Between May 2004 and January 2011, we performed this technique during the arterial switch and Norwood operations in 2 and 4 patients, respectively. The median (range) patient age and body weight were 17 (12-147) days and 3.4 (2.2-6.1) kg, respectively. All patients survived the procedures. The median follow-up duration was 4 (1-8) years. One patient showed recoarctation in the early postoperative period, which was successfully repaired by a single-catheter intervention. The latest median pressure gradient across the neoaortic arch was 0 (0-6) mmHg. Neoaortic valve regurgitation was found to be minimal by echocardiography. During the follow-up period, the Fontan operation was performed in all the patients who underwent the Norwood operation. These results suggest that the swing-back technique yields satisfactory mid-term outcomes.

Aortic arch reconstruction for interrupted aortic arch using an aberrant right subclavian artery.

An aberrant right subclavian artery can be used in a variety of ways in complex aortic arch reconstructions, including reconstruction of an interrupted aortic arch. Here, we described the case of a 4-month-old female infant with a type B interrupted aortic arch, who underwent arch reconstruction using an aberrant right subclavian artery.

Aortic arch thrombosis in the neonate.

Aortic arch thrombosis (AAT) of the neonate is rare but life-threatening by fatal compromise associated with thrombotic obstruction of the ascending aorta. We report a neonate with AAT who demonstrated a severe coarctation of the aorta and cerebral hypo-perfusion immediately after birth. Echocardiography confirmed the diagnosis of AAT on the findings of a large thrombus located on the transverse arch and blocking the cervical arterial branches. Low-molecular-weight heparin reduced the size of the thrombus and improved the hemodynamics of coarctation and cerebral perfusion. Echocardiography is a powerful tool to make a diagnosis and to monitor the size and regression of AAT.

Spontaneous dissection of the arterial duct during continuous infusion of prostaglandin E1 in a neonate with aortic arch interruption.

We report a 3-day-old boy with double outlet of the right ventricle and interruption of the aortic arch who developed spontaneous dissection of the arterial duct (DA) despite use of continuous infusion of lipo-prostaglandin E1 (PGE1). Transthoracic echocardiography demonstrated the spontaneous dissecting aneurysm of DA, which was confirmed by histology at the modified Norwood procedure done at age of 18 days. This is the first report of spontaneous dissection of DA in a neonate receiving PGE1, suggesting a new closing mechanism of DA.

[Successful application of cholangioscope as an intravascular endoscope in hybrid operation for a child].

We performed hybrid operation on a 3-year-old boy with thrombosis in the pulmonary arterial conduit which had been implanted concomitantly at the time of Fontan operation. We used a cholangioscope as a substitute of intravascular endoscope. It visualized the organized thrombus and the suture line in the conduit. Hybrid operation was successfully performed based on the detailed findings gained by cholangioscopy.

Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management.

We report a successfully treated case of unilateral absence of a pulmonary artery, associated with an atrial septal defect and chronic lung disease with severe pulmonary hypertension. Because this severe pulmonary hypertension could jeopardize postoperative hemodynamic, the ingenuity of surgical strategy was required. The atrial septal defect was left open as a safety "pop-off" valve, and prosthetic graft was chosen as a reconstructive material to avoid excessive dilatation from exposure to unpredictable postoperative pulmonary hypertension.

Fontan completion after Norwood with bidirectional Glenn shunt: their haemodynamics and pulmonary artery configuration.

OBJECTIVES: We ranked the haemodynamics and pulmonary artery (PA) configurations of Norwood with bidirectional Glenn shunt (NW-G) patients among the other staged Fontan completion (conventional) patients in this study. METHODS: Between August 2001 and April 2010, 91 consecutive patients completed staged Fontan operations. Among them, NW-G was performed in 11. RESULTS: There was no operative or late death except in one case which resulted from a reoperation after Fontan completion. Although the age at bidirectional Glenn (BDG) and Fontan completion was younger in NW-G (4.2 vs. 10.9 months, P < 0.01, and 2.3 vs. 3.3 years, P < 0.01), the interval between BDG and Fontan completion was not significantly different in these groups (23.6 vs. 28.0 months, P = 0.71). On Fontan completion, the extracardiac conduit size was significantly different (16.4 vs. 17.7 mm, P < 0.01). However, the pulmonary artery size (PA index) and the PA pressure were not significantly different (165.4 vs. 205.1 mm(2)/m(2), P = 0.23, and 13.4 vs. 11.0 mmHg, P = 0.08). Particularly in hypoplastic left heart syndrome patients, the PA index was not significantly different between these groups (165.4 vs. 174.9 mm(2)/m(2), P = 0.59). CONCLUSIONS: The NW-G patients could have a subsequent Fontan completion with good results. There was no significant difference with respect to Fontan completion.