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Objective: The Ross operation is an excellent surgical option for young children, regardless of aortic pathology type. However, failure rates are concerning during the second postoperative decade. We sought to determine the predictors of long-term outcomes after Ross and Ross-Konno operation performed in childhood., Methods: We performed 34 Ross and 9 Ross-Konno operations in pediatric patients (age < 15 years) from 1996 to 2016, and retrospectively evaluated the long-term results., Results: The postoperative follow-up period was 13.5 ± 3.9 years. In the Ross group, there were one inpatient death and one death after discharge. There were no inpatient deaths in the Ross-Konno group. There were 6 reoperations in the Ross group and 1 in the Ross-Konno group for left ventricular outflow tract (LVOT). Cumulative survival rates were 96.8% and 100% in the Ross and Ross-Konno groups, respectively. The reoperation free rate for LVOT /RVOT (right ventricular outflow tract) were 98.6/85.5% and 91.9/63.4% in the 5th and 10th years of follow-up, respectively. Patients who underwent the operations at age > 8.6 years had higher risks of reoperation for LVOT. Aortic annulus measurements > 24 mm or aorta/pulmonary artery diameter (Ao/PA) ratios > 1.2 conferred higher risks of reoperation for LVOT., Conclusions: Long-term outcomes after Ross and Ross-Konno operations in children were satisfactory. However, new-onset aortic regurgitation was progressive and reoperation was needed in some children. Age, aortic annulus diameter, and Ao/PA ratio may be able to predict of long-term outcomes after Ross and Ross-Konno operations.

Background: This study aimed to evaluate Fontan circulation which was observed over 30 years., Methods: Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2-32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6)., Results: Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL -1 , and Holter monitoring revealed sinus rhythm in 10 patients. New York Heart Association class was I in 12 patients, II in 6, and III in 2., Conclusions: For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important., (© The Author(s) 2016.)

If the heart is malpositioned with apicocaval juxtaposition (ACJ), what constitutes the ideal course for the conduit pathway of a total cavopulmonary connection must be considered. When the conduit is positioned between the inferior vena cava and the same side of the pulmonary artery behind the ventricle, potential stenosis of the conduit due to compression by the ventricle or obstruction of the pulmonary vein due to compression by the conduit must be recognized. We reported two cases of ACJ in which a straight conduit pathway behind the ventricle was accomplished. Comprehensive dissection of the heart, especially the posterior side of the ventricle, to make a wide opening into the thoracic cavity is needed to obtain enough space behind the ventricle. Postoperative catheter studies performed 6 months after the operations showed no obstruction or deformity of the conduit or the pulmonary veins, and the mean pulmonary artery pressure measured 9 mmHg in both patients.

Two children, aged 1 and 14 years with methicillin-resistant Staphylococcus aureus mediastinitis after pediatric open-heart surgery, were fitted with a vacuum-assisted closure system. Complete healing was achieved in both cases, and primary wound closure could be carried out without an omental flap after 6 and 16 days.

We experienced a conversion case from total cavopulmonary shunt (TCPS) to the Fontan circulation with an 11-year interval in the left isomerism and an interruption of the inferior caval vein. Marked cyanosis (pulse oximetry measuring 74%) progressed 8 years after TCPS because of the prominent development of bilateral pulmonary arteriovenous fistula (PAVF), which was diminished after the Fontan conversion, and recent pulse oximetry measured 95% 22 months after the conversion. We believe that this report is a case with the longest interval from TCPS to Fontan in which PAVF was diminished and marked cyanosis was improved.

In the present report we describe a neonate with the common arterial trunk who underwent reconstruction of the right ventricular outflow tract with a downsized value allograft. Our experience and results suggest that reducing the size of the allograft is a promising surgical option for young infants in whom reconstruction of the nonregurgitant pulmonary circulatory system is absolutely needed for a smooth postoperative recovery.

We have performed 174 pediatric open-heart operations using a lower midline skin incision and a minimal sternotomy approach since 1996. There was no death or major complication caused by this approach, and the resulting scar in each patient is difficult to be seen under an ordinary undershirt. The technique of a lower mid-line skin incision and minimal sternotomy approach is a safe, reliable and cosmetically advantageous method for a pediatric cardiac operation. We have applied an autologous blood predonation protocol in pediatric open-heart surgery since 1996. The study included 103 children weighing 7.5 kg or more. 8 ml/kg of blood was taken twice before operation. 97 patients (94.2%) needed no homologous blood transfusion during cardiopulmonary bypass. We consider our protocol of autologous blood predonation to be safe and effective for avoiding homologous blood transfusion in pediatric patients. Twelve children with patent ductus arteriosus (PDA) were successfully treated by thoracoscopic surgery under transesophageal echocardiography, by which interruption of the ductal flow was confirmed. Video-assisted endoscopic surgery can be safely applied to pediatric patients with PDA. This technique may be an effective addition to the staged management of more complex forms of congenital heart defects. To minimize deleterious postoperative influences of cardiopulmonary bypass on the pulmonary circulation, the right heart bypass operation was achieved without use of cardiopulmonary bypass. Four patients in whom no intracardiac procedure was needed, have undergone this operative maneuver using a temporary bypass. This operative procedure is especially recommended for pulmonary atresia with intact ventricular septum with sinusoidal communication when decompression of right ventricular pressure might cause coronary steel and ischemia.

We experienced 2 infants in whom octreotide acetate was effective on intractable chylothorax after surgery for congenital heart diseases. They were 8- and 5-month-old. They were diagnosed as having corrected transposition of the great arteries (TGA) and tetralogy of Fallot respectively, and underwent bidirectional Glenn anastomosis and right modified Blalock Taussig shunt. Chylothorax was revealed on the 11th and the 1st postoperative day, and was not improved by any conventional therapy in either case. Then octreotide acetate was infused continuously with 0.1-0.6 micorg/kg/hour for 24 and 7 days. Chylothorax disappeared completely without any complications such as disturbance of blood sugar level or growth retardation. Octreotide acetate was effective and safe even in infants in intractable chylothorax after surgery for congenital heart diseases, as long as used for short period.

Thirteen cases of functional single ventricle who had undergone bidirectional Glenn procedure were divided into 2 groups according to presence (5) or absence (8) of additional pulmonary blood flow. Additional flow was preserved in cases with relatively small pulmonary artery index (PA index), and their sources were antegrade pulmonary blood flow (2), and Blalock-Taussig (BT) shunt (3). In the control group, PA index was reduced to about 70% of the preoperative value, while in the additional group, pulmonary artery growth was recognized without significant elevation of mean pulmonary artery pressure. However, atrioventricular valve regurgitation progressed and systemic ventricular volume did not decrease after Glenn in the additional group. Therefore special consideration for the timing of Fontan procedure is mandatory.

Background: Plasma pheresis and reinfusion of platelet-rich plasma has not been shown to reduce blood loss in cardiac patients. Recently, freshly prepared autologous platelet concentrates (PC) can be made from patient's blood and has a higher concentration than conventional platelet rich plasma. In this study, the effects of autologous PC reinfusion were examined after open heart surgery in patients with congenital heart disease., Methods: Eight patients with noncyanotic congenital heart disease, who underwent open heart surgery and reinfusion of autologous PC, were classified as the PC group. Eight other patients with noncyanotic congenital heart disease, who underwent only open heart surgery, were defined as the control group. Ages ranged from 2 to 24 years and were not significantly different between the two groups (9.3 +/- 5.1 years in the PC group and 12.6 +/- 7.9 years in the control group, p = 0.33). In the PC group, blood was collected from the femoral vein through a 6F catheter introducer; 9 to 20 U (13.0 +/- 5.4 U, 0.42 +/- 0.22 U/kg) of autologous PC were prepared and were reinfused after protamine administration. The time course of platelet counts was examined until postoperative day 7. Aggregation responses to adenosine diphosphate; (4 micromol/L and 8 micromol/L), collagen (1 micromol/L and 5 micromol/L), and epinephrine (5 micromol/L and 10 micromol/L) were evaluated after induction of anesthesia (individual references), after protamine administration, at the end of the operation; these responses were shown as recovery ratios., Results: Blood loss during surgery in the PC group was significantly less than in the control group (4.8 +/- 3.0 mL/kg versus 7.8 +/- 1.7 mL/kg, p = 0.044). Similarly blood loss on postoperative day 1 in the PC group was significantly less than in the control group (3.6 +/- 1.2 mL/kg versus 7.2 +/- 3.1 mL/kg, p = 0.013). The platelet counts in the PC group were larger than those in the control group until postoperative day 5, after reinfusion of prepared autologous PC. The recovery ratios of the aggregation responses to adenosine diphosphate, collagen, and epinephrine after protamine administration were not significantly different between the two groups. However, recovery in the PC group after reinfusion of the prepared autologous PC was greater than in the control group., Conclusions: Reinfusion of the freshly prepared autologous PC was followed by good aggregation responses and low blood loss in patients with noncyanotic congenital heart disease after open heart surgery. This procedure may be useful in pediatric open heart surgery without blood transfusion or with little administration of homologous blood products.

Superior-inferior ventricles are a rare cardiac malformation characterized by the two ventricles lying one above the other instead of side by side. Consequently, the interventricular septum that separates such ventricles is horizontal, and anomalies of the atrioventricular valves and the ventriculoarterial relations are almost always present. This complex anomaly is difficult to manage with an operation, so few cases have been reported. We describe a successful experience in which we performed a double switch operation, consisting of the Senning and Jatene procedures, for this rare malformation accompanied by double-outlet right ventricle [S,L,L]. This is the first report we have been able to locate of a double switch operation for superior-inferior ventricles.

A 7-year-old boy underwent ventricular and atrial septal defect patch closures, pulmonary valvulotomy, left pulmonary artery dilatation, and bicaval Glenn shunt, which maintained good patency between the superior vena cava and right atrium, because of a reduced right ventricular end-diastolic volume (RVEDV). The RVEDV had increased at 15 years of age; therefore, we performed an anatomic biventricular correction by removing the bicaval Glenn shunt. We found that anatomic biventricular correction becomes possible if RVEDV increases postoperatively.

The surgical results for congenital cardiovascular anomalies in neonates between August 1987 and July 1997 were reviewed. Two hundred thirty-four neonates underwent the corrective surgery with the use of cardiopulmonary bypass (CPB) for the cardiac anomalies including transposition of the great arteries (d-TGA) (157 patients), total anomalous pulmonary venous connection (TAPVC) (44 patients), cardiac defects with the aortic arch anomalies (11 patients), and others (22 patients), with an early mortality rate of 12%. The survival rates through the arterial switch operation for d-TGA and correction for TAPVC in 30 days were satisfactory (92% and 89%, respectively). The early mortality rate of palliative surgery done without CPB in 115 neonates due to aortic arch anomalies, pulmonary outflow tract obstruction, or pulmonary hypertension was low (6%). In contrast with these results, the outcome of palliative surgery using CPB for hypoplastic left heart syndrome, the aortic arch anomalies with subaortic stenosis, or TAPVC in asplenia hearts was poor, with the surgical mortality of 80%.

Background: The endothelium-derived vasoconstrictor endothelin-1 (ET-1) may be involved in pulmonary hypertension (PH), but production of the endothelium-derived vasodilator nitric oxide (NO) after cardiopulmonary bypass (CPB) in congenital heart disease is unclear., Methods: Twenty patients (age, 4 months to 12 years) were divided into three groups: severe PH (mean pulmonary-to-systemic arterial pressure ratio > 0.5) and high pulmonary flow (n = 8), mild PH (mean pulmonary-to-systemic arterial pressure ratio < 0.35) and high pulmonary flow (n = 6), and no PH and low pulmonary flow (n = 6). The mean pulmonary-to-systemic arterial pressure ratio was calculated and blood samples were taken, and NO3-, an NO metabolite, was measured., Results: Levels of ET-1 in the group with severe PH and high pulmonary flow were higher than in the other groups until 6 hours after CPB, and NO3- was not changed significantly in the group with severe PH and high pulmonary flow and or the group with mild PH and high pulmonary flow during CPB. Endothelin-1 in the group with no PH and low pulmonary flow was higher than in the group with mild PH and high pulmonary flow after CPB, and NO3- in the group with no PH and low pulmonary flow significantly decreased after CPB. A positive correlation was obtained between mean pulmonary-to-systemic arterial pressure ratio and ET-1 (r = 0.742 before CPB; r = 0.689 after CPB)., Conclusions: Imbalance between increased ET-1 and constant NO after CPB in the group with severe PH and high pulmonary flow could contribute to dominant effects of ET-1, which may injure the lung. The increased ET-1 and the decreased NO after CPB in the group with no PH and low pulmonary flow may induce a mechanism of protective vasoconstriction against an acute increase in pulmonary flow.