Prognostic Risk Analyses for Postcardiotomy Extracorporeal Membrane Oxygenation in Children: A Review of Early and Intermediate Outcomes.

We evaluated the morbidity and mortality of children requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) to determine independent factors affecting early and intermediate outcomes. Between January 2002 and December 2015, 79 instances of ECMO after cardiac surgery in 73 children were retrospectively reviewed. Follow-up was completed in December 2016. Predictive risk analyses were employed concerning weaning of ECMO, hospital discharge, and mortality after discharge. Age and weight were 14.9 ± 25.6 months and 7.0 ± 5.3 kg, respectively. Median support time was 8.3 ± 4.4 days. Sixty-seven (85%) were successfully weaned off ECMO and 48 (61%) survived to hospital discharge. Multi-variate logistic regression analysis identified the first day to obtain negative fluid balance after initiation of support (adjusted odds ratio = 0.42), high serum lactate levels (0.97), and high total bilirubin (0.84) during support as significant independent factors associated with successful separation from ECMO. The first day of negative fluid balance (0.65) after successful decannulation was an independent risk factor for survival to hospital discharge. After hospital discharge, actuarial 1-year, 5-year, and 10-year survival rates were 94%, 78%, and 78%, respectively. Low weight increased the risk of death after hospital discharge by a multi-variate Cox hazard model. High serum lactate, high serum bilirubin, and unable to obtain early negative fluid balance during support impacted mortality of decannulation. Obtaining a late negative fluid balance in post-ECMO were independent risk factors for death after successful weaning. Low weight affected intermediate outcomes.

Effectiveness of cardiac surgery in patients with trisomy 18: a single-institutional experience.

BACKGROUND: Surgical repair for cardiac lesions has rarely been offered to patients with trisomy 18 because of their very short lifespans. We investigated the effectiveness of cardiac surgery in patients with trisomy 18. Patients and methods We performed a retrospective analysis of 20 consecutive patients with trisomy 18 and congenital cardiac anomalies who were evaluated between August, 2003 and July, 2013. All patients developed respiratory or cardiac failure due to excessive pulmonary blood flow. Patients were divided into two subgroups: one treated surgically (surgical group, n=10) and one treated without surgery (conservative group, n=10), primarily to compare the duration of survival between the groups. RESULTS: All the patients in the surgical group underwent cardiac surgery with pulmonary artery banding, including patent ductus arteriosus ligation in nine patients and coarctation repair in one. The duration of survival was significantly longer in the surgical group than in the conservative group (495.4±512.6 versus 93.1±76.2 days, respectively; p=0.03). A Cox proportional hazard model found cardiac surgery to be a significant predictor of survival time (risk ratio of 0.12, 95% confidence interval 0.016-0.63; p=0.01). CONCLUSIONS: Cardiac surgery was effective in prolonging survival by managing high pulmonary blood flow; however, the indication for surgery should be carefully considered on a case-by-case basis, because the risk of sudden death remains even after surgery. Patients' families should be provided with sufficient information to make decisions that will optimise the quality of life for both patients and their families.

Aortic coarctation repair in neonates with intracardiac defects: the importance of preservation of the lesser curvature of the aortic arch.

BACKGROUND AND AIM: The aim of this study was to evaluate the mid-term outcomes of a strategy for repair of coarctation of the aorta (CoA) and hypoplastic aortic arch (HAA) with a modified, extended end-to-end repair that preserves the lesser curvature of the aortic arch in neonates with intracardiac defects. METHODS: We studied 21 neonates who underwent CoA repair and remote intracardiac repair (2000-2013). Fifteen patients had HAA, and six patients had no HAA. Follow-up ranged from 0.4 to 12.8 years (median, 7.5 years), and all patients underwent cardiac catheterization and blood pressure measurement in both the arms and legs. RESULTS: The overall median age at the time of CoA repair was seven days and the median age at the time of intracardiac defect repair was 18.6 months. There were no hospital deaths and one case had recoarctation (4.8%). The overall mean pressure gradient at the latest follow-up was 3.4 ± 5.7 mmHg. Critical deformation of arch geometry was not found. No patient had hypertension or an abnormal arm-leg gradient. There was no difference in the cardiac catheterization data or blood pressure between patients with and without HAA. CONCLUSIONS: A modified, extended end-to-end repair for CoA and HAA resulted in a low rate of recoarctation, no operative mortality, maintenance of a smooth rounded arch, and normal blood pressures in the arms and legs during mid-term follow-up. These results suggest that this technique may be acceptable for repair of CoA and HAA in neonates with intracardiac defects.

Replacement of the extracardiac conduit for infective endocarditis after a fontan operation.

We report the management of infective endocarditis after an extracardiac Fontan operation. Obstruction of the infected conduit and the associated pulmonary arterial embolism led to failure of the Fontan circulation. This was managed by completely removing both the infected conduit and emboli and replacing the conduit.

Anatomical repair of aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery.

Aortopulmonary window (APW) with an anomalous origin of a coronary artery is extremely rare. We report surgical management of a four-week-old infant with the association of a distal type of APW and an anomalous origin of the right coronary artery (RCA) from the pulmonary artery. Complete anatomical correction comprising division of the great arteries and transferring the RCA as an autologous flap to the aortic defect was successfully performed.

An aneurysm at the cannulation site discovered 40 years after cardiac surgery: report of a case.

We report the successful surgical treatment of a pseudoaneurysm of the ascending aorta in a 45-year-old man who underwent surgical closure of a ventricular septal defect at the age of 5. A computed tomography scan ordered for the investigation of a pulmonary mass happened to detect a pseudoaneurysm (20 mm diameter). The pseudoaneurysm protruded anteriorly from the ascending aorta at the previous aortic cannulation site. The ascending aorta, including the lesion, was replaced with a prosthetic graft uneventfully. The cause of the pseudoaneurysm was considered iatrogenic, since he had no prior history of postoperative mediastinitis or blunt chest trauma. A computed tomography scan is a useful means to detect a mass on the ascending aorta following cardiac surgery, and it provides important information to help define a surgical strategy.

Investigation of an appropriate contrast-enhanced CT protocol for young patients following the Fontan operation.

PURPOSE: Children with congenital heart diseases (CHDs) may need to be followed up with contrast-enhanced CT following the Fontan operation because complications such as the occlusion of conduits may occur. The purpose of the present study was to develop an adequate contrast-enhanced CT protocol for children with CHD following the Fontan operation. MATERIALS AND METHODS: Between July 2012 and July 2017, 29 CT examinations for 26 patients aged 2-11 years (median 5 years) with CHD following the Fontan operation were performed using dual-source CT. A non-ionized contrast medium was injected through the dorsum manus vein. Scanning began 60 or 70 s after the start of the injection. The delayed phase was randomly selected to be 60 s in 14 cases and 70 s in 15 cases. We evaluated the enhancement of conduits following the Fontan operation at delayed phases. RESULTS: The CT numbers of conduits at 60 and 70 s were 185 ± 46 and 185 ± 31 HU, respectively (P = 0.97). CONCLUSION: In contrast-enhanced CT for children after the Fontan operation, both of the delayed phases (60 and 70 s) appeared to be adequate for evaluating intraconduit patency.

Translocation of dilated pulmonary artery for relief of bronchial compression associated with ventricular septal defect.

Bronchial compression due to a dilated pulmonary artery is a troublesome problem in the surgical management of infants or children with congenital heart disease. We experienced an infantile case of ventricular septal defect and prolonged respiratory insufficiency caused by right bronchial compression and left pulmonary hypoplasia. Anterior translocation of the dilated right pulmonary artery and intracardiac repair succeeded in relieving the bronchial compression and improving left pulmonary function. We advocate that this procedure is useful for bronchial compression with congenital heart disease and maldevelopment of the lung.

Utility of dual source CT with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to evaluate morphological features of ventricles in children with complex congenital heart defects.

PURPOSE: We evaluated the ability of dual source CT (DSCT) with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to depict the morphological features of ventricles in pediatric patients with congenital heart defects (CHD). MATERIALS AND METHODS: Between July 2013 and April 2015, 78 pediatric patients with CHD (median age 4 months) were examined using DSCT with the Flash Spiral Cardio mode. The types of ventricular abnormalities were ventricular septal defect (VSD) in 42 (the malaligned type in 11, perimembranous type in 23, supracristal type in 2, atrioventricular type in 2, and muscular type in 4), single ventricle (SV) in 11, and congenital corrected transposition of the great arteries (ccTGA) in 4. We evaluated the accuracy of the diagnosis of the VSD type. In cases of SV and ccTGA, we assessed the detectability of the anatomical features of both ventricles for a diagnosis of ventricular situs. RESULTS: DSCT confirmed the diagnoses for all VSDs. The type of defect was precisely diagnosed for all patients. The anatomical features of both ventricles were also depicted and ventricular situs of SV and ccTGA was correctly diagnosed. CONCLUSION: The results suggest that DSCT has the ability to clearly depict the configuration of ventricles.

On the inhibitory action of 29 drugs having side effect gynecomastia on estrogen production.

To examine the influence on aromatase and sulfatase pathways in estrogen pool by drugs reported to cause gynecomastia as the side effect, 29 ethical drugs were incubated with human placental microsomes as an enzyme source. The percent inhibition of drugs on aromatase pathway was obtained by sum of the velocity constants of two products, estrone (E1) and estradiol (E2) from testosterone (T) as the substrate, and that on sulfatase pathway was obtained as the velocity constant of production of E1 from estrone sulfate (E1S). Although several drugs including ketoconazole showed a significant inhibition effect on aromatase pathway at their non-clinical over-dose concentration (100 microM), no influence on the inhibition was observed in any drugs at their approximately therapeutic concentration (1 microM). However, several drugs including spironolactone gave the product ratio (E2/E1) having higher value than that of the control, the result means spironolactone inhibits the conversion of E2 to E1. No inhibitory effect of the drugs tested on estrogen production from E1S (sulfatase pathway) was confirmed. The results suggest the possibility that the tested drugs known to cause gynecomastia have no inhibitory effect essentially on aromatase and sulfatase pathways.

Cardiotrophin-1 is a prophylactic against the development of chronic hypoxic pulmonary hypertension in rats.

BACKGROUND: Cardiotrophin-1 (CT-1) reduces arterial blood pressure by activating nitric oxide synthesis. This study attempted to elucidate the effect of CT-1 on pulmonary arteries of pulmonary hypertensive rats. METHODS: Pulmonary hypertension was induced in rats in a hypoxic chamber containing 10% to 11% oxygen. Rats kept in the hypoxic environment received either recombinant mouse CT-1 at a concentration of 50 micro g/kg (CT-1+hypoxia group, n = 21) or phosphate-buffered saline (hypoxia group, n = 30) once per day. Control rats housed in room air also received either the equivalent concentration of CT-1 (CT-1+normoxia group, n = 18) or phosphate-buffered saline (normoxia group, n = 39). Pulmonary arterial pressure, pulmonary vasorelaxation, and ventricular hypertrophy were measured. RESULTS: The mean pulmonary arterial pressures were as follows (from lowest to highest; p values are relative to the hypoxia group): normoxia group (20.3 +/- 4.0 mm Hg, p < 0.0001), CT-1+normoxia group (21.1 +/- 2.4 mm Hg, p < 0.0001), CT-1+hypoxia group (27.9 +/- 4.1 mm Hg, p = 0.0019), and hypoxia group (33.9 +/- 6.6 mm Hg). The endothelium-dependent vasorelaxation value was largest in the normoxia group (59.5% +/- 17.4%, p < 0.0001), with it decreasing in the other groups in the following order (p values are relative to the hypoxia group): CT-1+normoxia group (52.8% +/- 15.5%, p = 0.0005), CT-1+hypoxia group (42.3% +/- 14.8%, p = 0.0061), and hypoxia group (17.4% +/- 4.8%). Right ventricular hypertrophy was significant only in the hypoxia group. CONCLUSIONS: Our results demonstrate that treatment with CT-1 in a chronic hypoxic pulmonary hypertension model protects the endothelial function of the pulmonary artery; decreases pulmonary arterial pressure; and attenuates right ventricular hypertrophy.

Nitric oxide formation and plasma L-arginine levels in pulmonary hypertensive rats.

The elevation of plasma L-arginine levels stimulates nitric oxide (NO) synthesis, but the underlying mechanisms are not yet understood. We examined the role of physiological changes in pulmonary arteries on endogenous NO production. Male Wistar rats were divided into following groups: (1) control rats receiving normal water orally, (2) ARG rats receiving L-arginine water orally, (3) MCT rats injected with monocrotaline (MCT) on day 0 and receiving normal water orally, and (4) MCT+ARG rats injected with MCT on day 0 and receiving L-arginine water orally. The rats were studied after 23 days of dietary intervention. In MCT+ARG rats, supplemental L-arginine exhibited a significant pulmonary vasodilatory effect, as shown by a decreased pulmonary arterial pressure (PAP) (P<0.001), decreased right ventricular hypertrophy (P<0.01), and improved endothelium-dependent relaxation (P<0.01). Also L-arginine inhibited the elevation of plasma endothelin-1 (P<0.01). Oral L-arginine administration increased plasma L-arginine levels about twofold, but in only MCT+ARG rats (i.e., not in ARG rats) did the urinary nitrate excretion significantly increase (P<0.05), which is an indicator of endogenous NO formation. Oral administration of L-arginine is effective against pulmonary vascular remodeling. The data also suggest that the initial elevation of PAP is important for the induction of endogenous NO synthesis.

Infected pseudoaneurysm following a modified Blalock-Taussig shunt procedure.

A 3-year-old boy with pulmonary atresia with ventricular septal defect, who had undergone placement of a modified Blalock-Taussig shunt, presented with a 1-week history of high fever. Computed tomography showed a pseudoaneurysm at the anastomosis between the right brachiocephalic artery and the graft. After intravenous antibiotic therapy, the pseudoaneurysm and infected graft were resected through a median sternotomy. This report describes successful management of a potentially fatal complication following placement of a modified Blalock-Taussig shunt.

Sivelestat attenuates lung injury in surgery for congenital heart disease with pulmonary hypertension.

BACKGROUND: Pulmonary hypertension associated with congenital heart disease increases the risk of surgery using cardiopulmonary bypass. Sivelestat is a neutrophil elastase inhibitor thought to have a prophylactic effect against lung injury after surgery using bypass. We elucidated that Sivelestat had the protective effect on lung in patients with congenital heart disease and pulmonary hypertension who underwent surgery using bypass. METHODS: This study was a controlled prospective randomized trial and enrolled 13 neonates or infants with ventricular septal defect and pulmonary hypertension. The patients were assigned to either sivelestat with the dose of 0.2 mg/kg per hour (sivelestat group, n = 7) or saline (placebo group, n = 6) from the start of bypass until 6 hours after bypass. Proinflammatory cytokines and adhesion molecules on leukocytes were measured at 10 time points during the above period. Pulmonary function was assessed perioperatively. RESULTS: Compared with the placebo group, the sivelestat group had significantly lower values of alveolar-arterial oxygen tension gradient at 24 hours (p = 0.038) and at 48 hours (p = 0.028) after bypass, and significantly better balance of hydration at 48 hours after bypass (p = 0.012). The sivelestat group also showed significantly lower plasma levels of interleukin-8 immediately after bypass (p = 0.041) and interleukin-10 at 15 minutes after removal of the aortic cross-clamp (p = 0.048), and immediately after bypass (p = 0.037). CONCLUSIONS: Administration of sivelestat during bypass prevented pulmonary damage and activities of proinflammatory cytokines at the cardiac operation in neonates or infants. Our results show that sivelestat may be considered to protect pulmonary function against the injury by bypass.

Surgical management of an aneurysm of the left atrial appendage to prevent potential sequelae.

An aneurysm of the left atrial appendage is an extremely rare anomaly that is commonly associated with supraventricular arrhythmia, compression of the coronary arteries, intracardiac thrombus and pulmonary venous stenosis. This condition may be caused by congenital dysplasia of the musculi pectinati and is usually diagnosed in the second to fourth decades of life. We report the surgical management of an asymptomatic 9-year old girl with this anomaly. She was referred to us because of abnormal chest X-ray findings, and investigation revealed an aneurysm of the left atrial appendage. As this condition may have potentially fatal complications, the aneurysm was completely resected under cardiac arrest with cardiopulmonary bypass to prevent recurrence and thrombus formation. We suggest that resection of an aneurysm of the left atrial appendage under cardiac arrest with cardiopulmonary bypass is a reasonable treatment option to prevent potential complications, particularly in children.

Surgical treatment for infected thrombus in the superior vena cava using an off-pump venoatrial shunt.

We report a septic patient who had an infected thrombus that extended from the right internal jugular vein to the right atrium 1 cm below the superior venocaval junction. The thrombus was successfully removed using an off-pump shunt placed between the innominate vein and the right atrium.

Atrial natriuretic peptide reduces ischemia/reperfusion-induced spinal cord injury in rats by enhancing sensory neuron activation.

We recently demonstrated that calcitonin gene-related peptide (CGRP) released from sensory neurons reduces spinal cord injury (SCI) by inhibiting neutrophil activation through an increase in the endothelial production of prostacyclin (PGI(2)). Carperitide, a synthetic alpha-human atrial natriuretic peptide (ANP), reduces ischemia/reperfusion (I/R)-induced tissue injury. However, its precise therapeutic mechanism(s) remains to be elucidated. In the present study, we examined whether ANP reduces I/R-induced spinal cord injury by enhancing sensory neuron activation using rats. ANP increased CGRP release and cellular cAMP levels in dorsal root ganglion neurons isolated from rats in vitro. The increase in CGRP release induced by ANP was reversed by pretreatment with capsazepine, an inhibitor of vanilloid receptor-1 activation, or with (9S, 10S, 12R)-2,3,9,10,11,12-hexahydro-10-hydroxy-9-methyl-1-oxo-9,12-epoxy-1H-diindolo[1,2,3-fg:3',2',1'-kl]pyrrolo[3,4-i][1,6]-benzodiazocine-10-carboxylic acid hexyl ester (KT5720), an inhibitor of protein kinase A (PKA), suggesting that ANP might increase CGRP release from sensory neurons by activating PKA through an increase in the cellular cAMP level. Spinal cord ischemia was induced in rats using a balloon catheter placed in the aorta. ANP reduced mortality and motor disturbances by inhibiting reduction of the number of motor neurons in animals subjected to SCI. ANP significantly enhanced I/R-induced increases in spinal cord tissue levels of CGRP and 6-keto-prostaglandin F(1alpha). a stable metabolite of PGI(2). ANP inhibited I/R-induced increases in spinal cord tissue levels of tumor necrosis factor and myeloperoxidase. Pretreatment with 4'-chloro-3-methoxycinnamanilide (SB366791), a specific vanilloid receptor-1 antagonist, and indomethacin reversed the effects of ANP. These results strongly suggest that ANP might reduce I/R-induced SCI in rats by inhibiting neutrophil activation through enhancement of sensory neuron activation.

Reconstruction of the right heart in an elderly woman with Ebstein's anomaly and severe heart failure.

We report the case of a 53-year-old woman with Ebstein's anomaly and intractable heart failure who had undergone only tricuspid valve replacement 30 years earlier. She was treated conservatively for 1 month; however, she was placed in the New York Heart Association functional class IV. Therefore, we operated on her with the objective of improving her quality of life and cardiac function. One-and-a-half repair and a second tricuspid valve replacement with right ventriculoplasty were quite effective in ameliorating her critical condition.