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Background:In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch.Methods:Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock–Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years.Results:There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I.Conclusion:Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Objectives This study aimed to review the clinical outcomes of staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. Methods Six extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation underwent staged cardiac surgery between 2005 and 2017. The median birthweight was 895 g (range 620-990 g), and the median gestational age was 28 weeks (range 23-31 weeks). Clinical outcomes were evaluated, and we focused on pulmonary haemodynamics. Results Pulmonary artery banding or bilateral pulmonary artery banding was performed as the initial palliation at a median age of 23 days with a median body weight of 880 g. Corrective surgery was performed at a median age of 187 days with a median body weight of 3.9 kg. All of the patients successfully underwent corrective surgery and survived to date. Pulmonary hypertension regressed after corrective surgery in all of the patients, except for 1 patient with severe bronchopulmonary dysplasia. Conclusions Acceptable outcomes can be obtained by staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. While early pulmonary artery banding can lead to regression of pulmonary hypertension after corrective surgery, close follow-up is required.

The surgical management of severe truncal valvular dysfunction is still challenging in neonates with persistent truncus arteriosus. This report describes a 14-day-old neonate with severe truncal valve insufficiency successfully undergoing truncal valve repairs, and followed by valve replacement at the age of 4 years. The truncal valve was quadricuspid with two large and two small leaflets, and all leaflets had severe dysplastic and myxomatous changes. We performed leaflet extension and bicuspidization valvuloplasty for this valve. This patient obtained somatic growth for 4 years without heart failure symptoms, and safely underwent prosthetic valve replacement. This technique would be effective for truncal valve dysfunction in neonates as the life-saving and the bridging procedure to valve replacement.

This report describes a 3-year-old infant with post-operative mediastinitis complicated by a contained rupture of the right ventricle. A contained rupture is recognised as the huge pulsating prominence of the anterior chest wall. CT confirmed blood communication between the right ventricular outflow tract and the cavity surrounded by the pectoral major musculocutaneous flap. This is a significant case in which severe adhesion between the right ventricle and the musculocutaneous flap could maintain her stable haemodynamics with a pulsating prominence.

Aim: To determine outcomes of surgical treatment of infants with hypoplastic left heart syndrome (HLHS). Methods: Retrospective analysis of medical records of infants with HLHS. Results: 129 of 206 (63%) infants with HLHS were managed surgically over the period 1983–2004. Survival from all stages of surgical repair was 52 (40%) patients with significantly different (P

Objective: To determine the efficacy of the Glenn procedure in terms of establishing the Fontan procedure.Methods: Surgical results were investigated in 248 patients undergoing either the total or partial right heart bypass. Of these, the conventional and the bidirectional Glenn procedures were employed in 27 and 58 patients, respectively.Results: Conversion to the Fontan circulation was attempted in 32 of 58 patients initially considered unsuitable for the Fontan circulation and undergoing the bidirectional Glenn procedure, with 2 operative deaths. Of the 27 undergoing the conventional Glenn procedure, 13 have undergone conversion with 2 deaths. Actuarial survival rate after the staged Fontan approach did not statistically differ from that after the primary Fontan procedure. Catheterization in 130 patients with the established Fontan circulation showed no significant differences in ejection fraction, end diastolic volume, and end diastolic pressure of the systemic ventricle, as well as in Cardiac Index, between the group of patients treated with the primary Fontan procedure and the group treated with the staged Fontan procedure. When achieving the bidirectional Glenn procedure, the size of the additional channel from the ventricles to the pulmonary arteries was correlated with the postoperative change in pulmonary arterial size, but such additional forward flow produced insufficient ventricular offloading in 9 of 16 patients with significant atrioventricular valvar regurgitation.Conclusion: The staged Fontan strategy has an advantage in patients with complicated circumstances. Whether the primary or the staged Fontan procedure was used, however, did not affect the Fontan circulation itself when once the Fontan circulation was established. The additional forward flow in the bidirectional Glenn plysiology, if employed, should be appropriately adjusted, considering both the advantages and the disadvantages of the option.

We herein reported 2 successful neonates with Ebstein’s anomaly and small pulmonary arteries undergoing Starnes operation preserving the patent ductus arteriosus. Subsequent Blalock-Taussig shunt was carried out 1 or 2 months after the first surgery. One case had already undergone a successful Fontan operation, and the other had a successful bidirectional Glenn shunt so far. This staged Starnes strategy might be a safe and simple choice for neonates with Ebstein’s anomaly and small pulmonary arteries.

絞扼性イレウスにおける腸管循環障害の重症度と腸管切除の必要性を予測することは容易ではない.われわれは過去12年間に当院救急外科に来院した絞扼性イレウス65例を対象とし, 多変量解析を用いてその可能性を検討した.腸管切除群35例と腸管非切除群30例の2群に分け, 各術前因子を比較した.また, 各術前因子から腸管切除長, 腸管切除の必要性を解析した.結果: 体温, 白血球数, 核の左方移動率, Creatinine Phosphokinase値, 血糖値の5因子において, 腸管切除群は非切除群に比べ有意に高値を示した.重回帰分析では腸管切除長は白血球数, 核の左方移動率, CPK値と有意に重相関し, このうちCPK値が最も相関に寄与していた.また, 体温, CPK値, 血糖値の3因子からなる判別式が算出され, その正判別率は90.9%であった.これら術前5因子は絞扼性イレウスにおける腸管切除の必要性を判別し, 術前における腸管循環障害の重症度を推定する指標となることが示唆された.

We report a case of asymptomatic duodenal carcinoid in a 50-year-old male patient. A filling defect was detected on routine upper gastrointestinal X-ray examination, and serious and a sessile polyp of 5 mm in diameter was endoscopically observed. Biopsy revealed typical features of carcinoid. Partial resection of the duodenal was performed. The tumor was confirmed in the mucosa which was type B by Soga's classification. Immunohisto-chemically, numerous secretary granules of gastrin-positive and somatostatin-negative were observed. This patient is doing well with no signs of recurrence 12 months after surgery.

We herein present bidirectional Glenn shunt and concomitant placement of extra-cardiac graft with an advantage of preparation for a later extra-cardiac total cavo-pulmonary connection, which was successfully performed for seven children with a univentricular heart. 2005 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

A male infant weighting 2970 g with total situs inversus, polysplenia, malrotation, duodenal stenosis, and complex cardiac anomalies, was admitted to our hospital. At 4 days of age, he underwent surgery that revealed a blood vessel passing over the duodenum from the mesenterium to the porta hepatis. A loose overbridging duodenoduodenostomy was performed to prevent compression of the vessel. The cardiac anomalies were corrected, and he could eat unrestricted diets. At the age of 1 year and 3 months, a 3-dimensional computed tomographic scan demonstrated that the vessel on the duodenum was the superior mesenteric vein (SMV), and it formed the portal vein with the splenic vein at the porta hepatis. Further, the scan revealed no compression of the SMV at the anastomosis. Doppler ultrasonography revealed a normal portal blood flow of 118.6 mL/min. This report describes the junction between the SMV and the splenic vein in a patient who had the SMV passing over the duodenum from the mesenterium. Correctly, patients previously diagnosed with a preduodenal portal vein could have a preduodenal SMV. The loose overbridging duodenoduodenostomy had advantages not only in passage of the anastomosis but also in maintenance of the portal blood flow for the congenital duodenal obstruction with the preduodenal SMV.

Objective The congenitally bicuspid aortic valve is the most common etiologic factor associated with clinically significant aortic stenosis and/or regurgitation in pediatric patients. Beyond infancy, surgical intervention typically involves valve repair with cusp thinning and commissurotomy or valve replacement, primarily with pulmonary autograft in the current era. An aortic valve repair technique using tricuspidization with cusp extension was introduced in 1999. This study compares the midterm clinical outcome in patients undergoing valve repair by tricuspidization with cusp extension with those receiving a pulmonary autograft (Ross). Methods A retrospective study was performed on all consecutive patients with symptomatic bicuspid aortic valve disease who underwent tricuspidization with cusp extension or a Ross procedure between 1999 and 2005. In both groups, all patients were at least 1 year of age at time of the operation. Results During this period, 21 children (median age 12.6 years, range 2.6–18 years) underwent tricuspidization with cusp extension (TCE group) and 25 children (median age 10.2 years, range 11.5 months–20.1 years) underwent the Ross procedure. Prior balloon valvuloplasty was performed in 5 (24%) of the children in the TCE group and 16 (64%) of the children in the Ross group. Prior surgical commissurotomy was performed in 4 (19%) TCE patients and in 9 (36%) Ross patients. During a median follow-up period of 36.4 months (range 2.5 months–7.4 years), 2 (10%) patients in the TCE group required valve-preserving early revision of the repair, 2 (10%) TCE patients required subsequent aortic valve replacement at 16 and 33 months, 1 (4%) Ross patient required subsequent valve repair at 5 years, and 1 (4%) Ross patient underwent cardiac transplantation at 46 months. At 36 months, the actuarial freedom from reintervention on the aortic valve or autograft was 90% in the TCE group, with 11 patients at risk, and 100% in Ross patients, with 13 patients at risk ( P = .39); the freedom from moderate valve dysfunction or reintervention was 66% for TCE patients and 95% for Ross patients ( P = .07). There were no deaths, and all but 1 Ross patient remain in New York Heart Association class I. Conclusions Reintervention rates in patients undergoing tricuspidization with cusp extension or a primary Ross procedure are similar. Valve performance in the TCE group is satisfactory at midterm follow-up, but the Ross repair appears to provide greater stability of valve function. These results suggest that repair with valve tricuspidization and cusp extension provides reliable palliation of the symptomatic bicuspid aortic valve.

For 5 patients with univentricular heart associated with apico-caval juxtaposition, an extracardiac Fontan procedure was carried out using an artificial graft bridging the vertebra to avoid graft compression by the vertebra and the ventricle. For 2 patients representing nonconfluency between the inferior caval vein and the hepatic vein, a hand-made, shoe-tree graft was used. Postoperatively all patients are doing well without a stenotic venous pathway. This extracardiac operation using an artificial graft bridging the vertebra may be advantageous for univentricular heart associated with apico-caval juxtaposition to prevent a postoperative stenotic venous pathway.

Background To investigate whether the Fontan procedure in an off-pump fashion is less invasive in terms of the systemic inflammatory reaction. Methods Plasma levels of complement and cytokines were measured during and after the Fontan procedure in consecutive 38 patients. Of these, 16 underwent the extracardiac method without use of cardiopulmonary bypass (off-pump group), while the machine was used in the remaining 22 because of intracardiac maneuvers concomitantly needed (CPB group). Results There was no difference, between these two groups, in any value of plasma complement or cytokines after anesthetic induction. Immediately after commencement of the Fontan circulation, however, plasma concentration was significantly lower in the off-pump group for activated complement 3 (C3a), interleukin-6, interleukin-8, and polymorphonuclear elastase. The C3a value was also lower at 2 hours later in the off-pump group than in the CPB group. Furthermore, the values even stayed within the normal ranges, in the off-pump group, for tumor necrosis factor alpha (TNFα) and thrombomodulin. A pulmonary venous oxygen tension divided by an inspired oxygen tension (PpvO 2 /FiO 2 ) ratio immediately after commencement of the Fontan circulation was 528 ± 93 mm Hg (410 to 580 mm Hg) in the off-pump group, and 258 ± 167 mm Hg (86 to 540 mm Hg) in the CPB group ( p = 0.01). Duration of drainage for fluid sequestration was shorter in the former group (6.3 ± 0.7 days versus 13.9 ± 2.5 days, p = 0.02). Conclusions Inflammatory reactions were attenuated when the Fontan procedure was employed in an off-pump fashion compared with the usual procedure on bypass.

We describe herein a successful biventricular repair in a 21-year-old male who had severe hypoplasia of isomeric right appendages. He had previously undergone the conventional Glenn procedure at the age of one and a half years. Although he had grown uneventfully until adolescence, cyanosis as well as fatigue than gradually became worse. When referred to us for further treatment, we deemed a Fontan type procedure to be contraindicated, because of the hypoplastic nature of the right pulmonary artery, and the presence of abundant collateral arteries supplying the right lung. In terms of ventricular morphology, however, because both apical components were present, separated by the hypoplastic septum, biventricular repair seemed feasible. Initially, the Glenn anastomosis was taken down, and systemic-to-pulmonary shunts were constructed via a median sternotomy to both the right and left pulmonary arteries. This was followed by surgical division of the developed collaterals to the right lung via the right thoracotomy. Definitive biventricular repair was then carried out by reconstructing the pulmonary arteries and right ventricular outflow tact, separating and rerouting within the ventricles using a EPTFE patch, and achieving redirection of blood within the atriums using bovine pericardium. Such staged surgical approaches, although extensive, can provide useful options when seeking definitive repair in grown-up patients with complicated malformations.