Search

Supplemental Digital Content is available in the text., OBJECTIVES: To demonstrate the efficacy, safety, and pharmacokinetics of dexmedetomidine as a potential sedative for pediatric surgery patients in the ICU. DESIGN: Phase 3, multicenter, open-label study. SETTING: This study included 61 patients at 13 tertiary hospitals in Japan. PATIENTS: Pediatric patients (≥ 45 wk corrected gestational age to < 17 yr) undergoing intensive care treatment with mechanical ventilation requiring greater than 6 hours estimated duration of sedation following elective cardiac surgery. INTERVENTIONS: Dexmedetomidine was IV administered without a loading dose at age-specific dose regimens 0.2–1.4 (< 6 yr) and 0.2–1.0 µg/kg/hr (≥ 6 yr). The primary endpoint was the percentage of patients who did not require a rescue sedative (midazolam) infusion during mechanical ventilation or for the first 24 hours of a greater than 24 hours ventilation following the commencement of dexmedetomidine administration. MEASUREMENTS AND MAIN RESULTS: Overall, 47 of the 61 patients (77.0%) did not require rescue midazolam. Adverse events were reported in 53 patients (86.9%). Frequently observed adverse events were hypotension (47.5%), bradycardia (31.1%), and respiratory depression (26.2%). Most of these adverse events were mild, a few moderate, and none severe. Although serious adverse events occurred in four patients, including one cardiac tamponade resulting in the withdrawal of dexmedetomidine, none of the adverse events resulted in mortality or were directly related to dexmedetomidine. The plasma dexmedetomidine concentration generally reached the target concentration of 0.3–1.25 ng/mL at 1–2 hours prior to completion of administration or immediately prior to the commencement of tapering. CONCLUSIONS: The age-specific dose regimens of dexmedetomidine without an initial loading dose achieved an adequate sedation level during mechanical ventilation and caused no clinically significant adverse events in the intensive care pediatric patients. These effects were achieved within the therapeutic range of dexmedetomidine plasma concentration and were accompanied by minimal effects on hemodynamics and respiration.

OBJECTIVES Air leakage after lung resection is a common morbidity that may lengthen hospital stay. Applying sealant to a lesion is an effective prophylaxis in clinical practice. This study aimed to examine the effect of a combination of a bioabsorbable polyglycolic acid (PGA) fabric and fibrin glue (FG) on air sealing by measuring the in vitro mechanical strength and degradation of the fabric, and in vivo histological changes after implantation. METHODS A defect was created in the canine left upper lung lobe, and then filled with a fibrinogen solution and covered with a PGA sheet spray-coated with fibrinogen and thrombin. After 1 and 4 weeks, air leakage from the lesion was examined in vivo under airway pressure. Tissue samples were harvested for histological assessment. RESULTS The mechanical strength of the PGA fabric remained at 80–90% of the baseline level for 1 week in phosphate-buffered saline, and then rapidly decreased to zero thereafter. Air leakage from the lung defect was prevented by the combination of PGA fabric and FG at 1 and 4 weeks. Histological examinations showed that PGA bundles persisted with a non-specific inflammatory response for 2 weeks and then gradually broke into sparse yarns surrounded by collagen fibres and capillaries by 8 weeks. The lung defect was filled with FG at 1 week and by granulation tissue thereafter. CONCLUSIONS These results provide evidence for the efficacy of a combination of PGA fabric and FG for the prevention of air leakage in the critical period after lung surgery.

Background:Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery.Methods:We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital.Results:We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure.Conclusion:Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

The safety and efficacy of landiolol have not been fully elucidated in pediatric patients. This study aimed to clarify the safety and efficacy of landiolol in a pediatric cohort. We retrospectively assessed the clinical features of 21 pediatric patients who were administered landiolol at our hospital. We also investigated the rates of sinus rhythm conversion and heart rate response. The median patient age was 7 months (interquartile range 1–13 months). The etiology of tachyarrhythmia was junctional ectopic tachycardia in 10 patients (47.6%), atrial tachycardia in 10 patients (47.6%), and ventricular tachycardia in 1 patient (4.8%). Of the 21 children, 18 (85.7%) had congenital heart defects, including 14 (77.8%) in whom a landiolol infusion was performed perioperatively. The landiolol infusion was effective in 18 pediatric patients (85.7%), as measured by the conversion to sinus rhythm or a reduced heart rate. Atrial tachycardia in the perioperative period was terminated in all patients. Of 7 patients with tachyarrhythmias unrelated to the perioperative period, landiolol was effective in 5. No adverse effects were reported in any patient. Landiolol infusion is effective and safe in pediatric patients with tachyarrhythmia of various etiologies, especially those with atrial tachyarrhythmia during the perioperative period.

Objectives Materials used in paediatric cardiac surgery have drawbacks of deterioration, calcification and pseudointimal proliferation resulting in haemodynamic disturbance. The aim of this study was to investigate whether these drawbacks can be overcome by in situ tissue regeneration using a newly developed synthetic hybrid fabric (SHF). Methods The SHF is an expandable, warp-knitted fabric composed of a combination of biodegradable [poly-l-lactic acid (PLLA)] and non-biodegradable (polyethylene terephthalate) yarns. The fabric is coated with cross-linked gelatin. Mechanical properties of the SHF were compared with those of 2 commercial products: expanded polytetrafluoroethylene sheet and glutaraldehyde-treated bovine pericardium. An oval-shaped defect created in the canine descending aorta or inferior vena cava was filled with the SHF patch. After 2 weeks and 1, 3, 6 and 12 (or 24 in the inferior vena cava) months, the patch was removed for histological examination and evaluation of the remaining PLLA. Results The SHF exhibited satisfactory tensile and suture retention strength for surgical implantation similar to or better than the 2 commercial products. Tissue regeneration was induced with multilayered smooth muscle cells and collagen fibres on both sides of the patch, along with a mature endothelial layer and tissue connections containing vasa vasorum across the patch in the aorta and inferior vena cava. Inflammatory reactions were minimal, and no calcium deposition occurred. The molecular weight of PLLA was reduced to half at 12 months after implantation. Conclusions The SHF may solve the drawbacks of the existing products. Further studies of the expandability of the SHF patch after degradation of PLLA are warranted.

In the original publication of the article, the title was incorrectly published.

Quadricuspid aortic valve is a rare congenital disease. We experienced 3 surgical cases of quadricuspid aortic valve. Patient 1 was a 72-year-old man who was noted to have a quadricuspid aortic valve associated with aortic regurgitation and an ascending aortic aneurysm(51 mm in diameter). He underwent replacement of the aortic valve and the ascending aorta. Patient 2 was a 71-year-old man with severe aortic stenosis, regurgitation, and coronary triple vessel disease. He underwent aortic valve replacement and coronary artery bypass grafting. Preoperative echocardiography revealed no abnormalities in the number of valve leaflets, but quadricuspid aortic valve was identified during surgery. Patient 3 was a 79-year-old man with severe aortic regurgitation, who underwent aortic valve replacement. In all patients, the 4 valve cusps were approximately of the same size. Multi-detector computed tomography is useful for evaluation of valve morphology. Indication of prophylactic ascending aorta replacement in patients with aortic dilatation requires further study.

A 77-year-old man, who had been under medical treatment for myasthenia gravis without thymoma, was diagnosed with aortic arch aneurysm. He underwent total aortic arch replacement and total resection of the thymus through median sternotomy. His symptoms relating to myasthenia gravis dramatically disappeared after the surgery. The serum anti-acetyl chorine receptor antibody decreased from 2.7 to 0.7 nmol/l (N0.2) with the reduction of oral predonisolone from 12.5 to 5 mg/day at 4 years after the surgery. The concomitant operations significantly improved his quality of life.

The surgical sheets that are currently used for congenital cardiovascular surgery have several drawbacks, including material deterioration, calcification, and pseudo-intimal proliferation resulting in hemodynamic disturbance. The aim of this study was to evaluate a newly developed sheet made from a combination of silk fibroin (SF) and a synthetic polymer, thermoplastic polyurethane (TPU), for surgical use. The hybrid SF/TPU sheet was a non-woven fabric with nanofibers that was made using the electrospinning method. The mechanical properties of the SF/TPU sheet were characterized. To determine its biocompatibility, part of the wall of the canine descending aorta was replaced with a SF/TPU sheet as a patch. The patches were removed after 3 months and a histological examination was performed. The flexibility, water permeability, and suture retention strength of the SF/TPU sheet were excellent and equivalent to those of existing sheets. The SF/TPU sheet had excellent handling properties and fit well into the vascular wall without needle hole bleeding. The histological examination revealed that the intimal tissue was restored well over the intraluminal surface of the explanted SF/TPU sheet, the absence of calcium deposition, and minimal inflammatory reaction, without signs of degradation. The SF/TPU sheet had excellent mechanical properties and tissue biocompatibility. These favorable features and possible biodegradability of the SF portion warrant a long-term follow-up study.

The Blalock–Taussig shunt (BTS) operation is a cornerstone as initial palliative surgery for congenital heart disease with severely reduced pulmonary blood flow (PBF). The ideal PBF provided by BTS is crucial for an uneventful postoperative course, since excess PBF results in acute distress of the systemic circulation and insufficient PBF requires another BTS surgery. Therefore, the goal of this study was to develop a simple device to control the shunt graft flow percutaneously using a constrictor balloon connected to a subcutaneous port. The device consists of a cylindrical balloon and an anti-bending structure extension connected to the balloon center. A PTFE vascular graft wrapped by the device was connected to a simulated closed circuit to measure the relationship between pressure and blood flow while changing the inner volume of the balloon. In a beagle model of replacement of the right carotid artery, blood flow velocity was measured in the carotid artery after saline injection into the balloon. The blood flow velocity before and after balloon inflation was compared immediately after implantation of the device and at 3 months after implantation. The device provided good flow control by inflating and deflating the balloon ex vivo and in vivo for up to 3 months in a canine model with a small graft wrapped with the device. The simple device developed in this study may enable regulation of PBF through a small vascular graft and help to prevent severe morbidity and mortality in the clinical setting of BTS.

We describe a case of successful treatment of mycotic pseudoaneurysm of the transverse aortic arch in a male infant. The aneurysm was resected and the defect was repaired using a patch made from a rifampicin-impregnated Dacron graft.

We report the case of a newborn baby with an unguarded mitral orifice associated with asplenia syndrome, double-outlet right ventricle, dysplastic tricuspid valve, and pulmonary stenosis. This case was accompanied by severe tricuspid regurgitation and severe right ventricular hypertrophy. The patient had a fatal clinical course due to severe hypoxia and congestive heart failure. Unguarded mitral orifice is a rare disease in which there has been no previous report of lethal clinical course during the neonatal period. Prior reports stated that unguarded mitral orifice was a new constellation of defects and that its etiology and embryology could be classified in the same category because of similar associated malformations of double-outlet right ventricle and pulmonary stenosis or atresia. However, the present case was diagnosed on autopsy as also having asplenia syndrome. Therefore, it is possible that the genetic etiology of unguarded mitral orifice in this case was different from cases of non-heterotaxy. Learning objective: Unguarded mitral orifice is a rare disease that might be associated with asplenia syndrome and dysplastic tricuspid valve. If unguarded mitral orifice is associated with such defects, the clinical course can be fatal. Therefore, when this diagnosis is recognized, the physician should explain the possibility of neonatal death and plan the treatment of such a case to include grief therapy for the family.>

In response to requests from the sites of radiation emergency medicine, we developed a prototype of a computer system for assessing radioactive contaminants remaining in a wound or on the skin surface. This system implements numerical calibration of detectors measuring the contamination using a Monte Carlo simulation-based radiation transport code (MCNPX), coupled to a numerical phantom modeling the contaminants and the surrounding tissue. With the help of functional graphical user interfaces (GUIs) provided by the system, a user can perform desired simulations without complicated procedures to prepare input files for MCNPX. Benchmark calculations of MCNPX were conducted to verify the simulations and adjust detection-related parameter values not sufficiently provided by suppliers. The developed system should aid in making better assessments of the radiological contamination.

Sildenafil is a strong pulmonary vasodilator that increases the intracellular cyclic guanosine monophosphate concentration through inhibition of phosphodiesterase-5. We assessed the benefit of oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in paediatric patients.Sildenafil was administered at a starting dose of 0.5 mg kg(-1) following admission to the intensive care unit. With careful monitoring of haemodynamics, the dose was increased stepwise by 0.5 mg kg(-1) every 4-6 h up to a maximum of 2 mg kg(-1). After successful weaning from a ventilator and from other vasodilators, sildenafil was gradually discontinued over the next 5-7 days.A retrospective review of medical records showed an age distribution of1 month (n=26),or = 1-6 months (n=36),or = 6-12 months (n=19), 1-3 years (n=8), 4-9 years (n=9) and10 years (n=2) at the time of surgery. The surgeries were performed for ventricular septal defect closure (n=17), arterial switch (n=30), truncus arteriosus repair (n=10), complete atrioventricular septal defect repair (n=12), total anomalous venous drainage repair (n=9), and other open-heart surgery (n=22). The aforementioned concomitant inhaled nitrous oxide treatment was performed in 66 patients. Pulmonary arterial pressure decreased in 28, was unchanged in five and elevated in one patient out of the total of 34 cases for which data from continuous pressure monitoring were available. Bosentan was added in three cases with persistent symptoms due to pulmonary hypertension despite sildenafil treatment. After sildenafil administration, modest oxygen desaturation occurred in seven cases, but no 'rebound' pulmonary hypertension occurred. There were no significant adverse events during sildenafil treatment.Our results suggest that oral sildenafil is a safe and effective alternate for persistent pulmonary hypertension following congenital heart surgery in children.

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease-free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.

We describe a case of successful treatment of mycotic pseudoaneurysm of the transverse aortic arch in a male infant. The aneurysm was resected and the defect was repaired using a patch made from a rifampicin-impregnated Dacron graft.

Objective To evaluate structure-oriented left ventricular volume reduction surgery (LVVRS). The purpose of this study was to report the early and mid-term results of left volume reduction surgery for dilated cardiomyopathy (DCM). Methods We performed LVVRS on 29 patients with DCM. The age of the patient ranged from 8 to 73 years (mean 58 +/- 18 years). There were 19 male patients (63%). Twenty-three patients were ischemic, 5 were non-ischemic, and 1 had salcoidosis. Twenty-three patients were in New York Heart Association class III or IV. Fourteen patients underwent the Dor operation, 11 underwent a septal anterior ventricular exclusion operation, and 6 underwent a modified Batista operation. Fifteen patients underwent mitral annuloplasty and 2 patients had mitral valve replacement. All patients were divided into two groups, a Dor group (n = 14) and non-Dor group (n = 15). Postoperative early results and mid-term survival rate were compared between the two groups. Results Hospital mortality was 13.8% (4/29). The causes of death were low-output syndrome (n = 3) and septic shock (n = 1). Survival rate was 80% at 1 year and 72% at 3 years. Two-year survival rate of Dor and non-Dor groups were 69.8% and 93.8%, respectively (p = 0.099). Conclusions Early and mid-term results of LVVRS were satisfied, and the non-Dor operation tended to be superior in mid-term survival to the Dor operation. Long-term follow-up is warranted.

Background—Tumor necrosis factor (TNF) is initially synthesized as a 26-kDa transmembrane protein that is enzymatically cleaved by TNF-α converting enzyme (TACE) to generate a 17-kDa form of “secreted” TNF. Whereas the effects of secreted TNF in the heart have been characterized extensively, the effects of transmembrane TNF in the heart are unknown.Methods and Results—We generated lines of transgenic mice with cardiac-restricted overexpression of a noncleavable, transmembrane form of TNF. We next treated a previously generated transgenic line of mice with cardiac-restricted expression of cleavable TNF (referred to as MHCsTNF mice) with a TACE inhibitor (DPC-IDR1) to determine whether TACE inhibition would prevent the transition from concentric hypertrophy to left ventricular (LV) dilation that occurs in this line of transgenic mice. Two of the founder lines did not have a demonstrable phenotype (M-41 and M-45), whereas a third line developed a concentric hypertrophic cardiac phenotype (M-48). Characterization of the M-48 line at 6 weeks of age showed that this line developed concentric hypertrophy, with an increase in myocyte cross-sectional area and reexpression of the fetal gene program. Four weeks of TACE inhibition abrogated the LV dilation in the MHCsTNF mice and resulted in an increase in LV wall thickness and increased myocyte cross-sectional area, thus mimicking the effects observed in the mice with noncleavable, transmembrane TNF.Conclusions—These studies show that transmembrane TNF is biologically active and provokes a concentric hypertrophic cardiac phenotype, thus suggesting that posttranslational processing (ie, secretion) of TNF is responsible for the dilated cardiomyopathic phenotype in mice with targeted, cardiac-restricted overexpression of TNF.

Measurement of left ventricular (LV) function is often overlooked in murine studies, which have been used to analyze the effects of genetic manipulation on cardiac phenotype. The goal of this study was to address the effects of changes in LV contractility on indexes of contractility in mice. LV function was assessed in vivo in closed-chest mice by echocardiography and by LV catheterization using a conductance pressure-volume (P-V) catheter with three different interventions that alter contractility by 1) atrial pacing to increase inotropy by augmentation of the force-frequency relation (modest increment of inotropy), 2) dobutamine to maximize inotropy, and 3) esmolol infusion to decrease contractility. Load-independent parameters derived from P-V relations, such as slope of end-systolic P-V relations (ESPVR) and slope of the first maximal pressure derivative over time (dP/d t max)-end-diastolic volume relation (dP/d t-EDV), and standard echocardiographic parameters were measured. The dP/d t-EDV changed the most among parameters after atrial pacing and dobutamine infusion (percent change, 162.8 ± 95.9% and 271.0 ± 44.0%, respectively). ESPVR was the most affected by a decrease in LV contractility during esmolol infusion (percent change, −49.8 ± 8.3%). However, fractional shortening failed to detect changes in contractility during atrial pacing and esmolol infusion and its percent change was

Background—The molecular mechanisms responsible for sepsis-induced myocardial dysfunction remain undefined. CD14 mediates the inflammatory response to lipopolysaccharide (LPS) in various organs including the heart. In this study we investigated the role of CD14 in LPS-induced myocardial dysfunction in vivo.Methods and Results—Wild-type and CD14-deficient (CD14-D) mice were challenged withEscherichia coliLPS. Myocardial tumor necrosis factor, interleukin-1β (IL-1β), and NOS2 induction was measured before and 6 hours after LPS challenge. Echocardiographic parameters of left ventricular function were measured before and 6 hours after LPS administration. LPS challenge induced a significant increase in myocardial tumor necrosis factor and IL-1β mRNA and protein expression in wild-type mice. In contrast, mRNA and protein levels for TNF and IL-1β were significantly blunted in CD14-D mice. An increase in NOS2 protein was noted within 6 hours of LPS provocation only in the hearts of wild-type mice. This was associated with an increase in ventricular cGMP levels. Activation of nuclear factor-κB was observed within 30 minutes of LPS in the hearts of wild-type mice but not in CD14-D mice. In wild-type mice, LPS significantly decreased left ventricular fractional shortening, velocity of circumferential shortening, and dP/dtmax. LPS-treated CD14-D mice maintained normal cardiac function.Conclusions—These results suggest that CD14 is important in mediating the proinflammatory response induced by LPS in the heart and that CD14 is necessary for the development of left ventricular dysfunction during LPS-induced shock in vivo.

ObjectivesThe goal of this study was to determine the therapeutic efficacy of angiotensin-converting enzyme (ACE) inhibitors and beta-adrenergic receptor blockers in experimental chronic mitral regurgitation (MR), gaining knowledge using methods difficult to apply in humans.BackgroundBoth ACE inhibitors and beta-blockers are cornerstones in the treatment of human congestive heart failure. However, the roles of these treatments for chronic MR is unclear.MethodsMitral regurgitation was created in 11 closed-chest dogs. Three months after the creation, the ACE inhibitor lisinopril 20 mg was given orally daily. After three months of lisinopril therapy, the beta-blocker atenolol was added to lisinopril for another three months. Atenolol was begun at a dose of 12.5 mg daily and increased gradually to 100 mg daily. Hemodynamics and left ventricular (LV) function were assessed throughout the study.ResultsRegurgitant fraction was consistently >50% over the course of this study. Pulmonary capillary wedge pressure and LV end-diastolic pressure were significantly increased after three months of MR and decreased during both lisinopril and the combined therapy in which it was not different from baseline. Left ventricular contractility measured by the end-systolic stiffness constant was depressed from 3.66 ± 0.20 to 2.65 ± 0.12 (p < 0.05) at three months of MR and rose insignificantly after lisinopril treatment (2.99 ± 0.17). When atenolol was added, it rose significantly and returned to normal (3.50 ± 0.22, p < 0.05).ConclusionsAlthough lisinopril significantly reduced preload, its effect on LV contractility was insignificant in experimental MR. Conversely, atenolol, when added to lisinopril, achieved maximum hemodynamic benefit and also restored LV contractility.

Our current understanding of the pathogenesis of sepsis suggests that bacteria as well as bacterial-derived products activate an uncontrolled network of host-derived mediators such as proinflammatory cytokines (ie, tumor necrosis factor [TNF] and interleukin [IL]-1β), which can ultimately lead to cardiovascular collapse and death. Despite the potentially important role that TNF and IL-1β may play in producing cardiac dysfunction in human septic shock, little is known with regard to the basic biochemical mechanism(s) by which bacterial pathogens induce their expression in the heart. A major advance in understanding the early events that are downstream from bacterial-mediated signaling has been the identification of Toll-like receptors (TLRs). TLR-mediated signaling is known to activate the transcription factor nuclear factor-κB and to upregulate TNF expression. It has recently been shown that the heart expresses TLRs, raising the possibility that these receptors may be responsible for mediating the deleterious effects of bacterial pathogens on cardiac function. In this review, we will discuss the emerging role for TLRs in the pathogenesis of the cardiovascular collapse that occurs during sepsis.

A 42-year-old woman was admitted with chest pain. Coronary angiography did not reveal any significant stenosis, but left ventriculography showed akinesis and ballooning of the apex with a hyperkinetic basal segment indicating Takotsubo cardiomyopathy. Cerebral embolism occurred after one and a half years because of a left ventricular thrombus. The apical akinesis had worsened to a left ventricular aneurysm (maximum diameter 43 mm). The left ventricle was reconstructed to avoid repeated thrombus formation and cerebral infarction despite anticoagulant therapy. A pathological assessment revealed a fibrotic myocardium, but the cause of the cardiac aneurysm remained unknown. Although the outcome of Takotsubo cardiomyopathy is relatively good, careful observation and appropriate treatment are needed considering the possibility of aggravation.

Hemolysis caused by cardiopulmonary bypass causes renal dysfunction and other organ failure presumably by superoxide production catalyzed by iron derived from free hemoglobin (f-Hb). It might also impair cardiac function by the same mechanism, especially in the ischemia-reperfusion period and in neonates where serum antioxidant activity is lower than adults.We evaluated effects of f-Hb on cardiac function with or without ischemia and reperfusion using a newborn (7 days old) rabbit crystalloid-perfused Langendorff model. After baseline measurements, the hearts were divided into the following four groups (8 hearts per group): (1) those perfused with regular Krebs-Henseleit bicarbonate buffer, (2) those perfused 30 minutes with KH buffer containing 1 mg/mL of f-Hb obtained from osmotic hemolysis, (3) those subjected to 180 minutes of cold global ischemia with infusion of crystalloid cardioplegia and reperfused with Krebs-Henseleit buffer, and (4) those subjected to the same ischemia and reperfused with Krebs-Henseleit buffer containing 1 mg/mL of f-Hb. The left ventricular function (using conductance catheter and isovolumic balloon) and coronary flow were measured.Free hemoglobin significantly impaired not only left ventricular function but also coronary flow even without ischemia (p0.05). When ischemia and reperfusion were involved, the group reperfused with f-Hb showed the worst left ventricular function and coronary flow among the groups.This study shows that f-Hb directly impaired cardiac function and coronary flow in neonatal hearts especially in ischemia and reperfusion.

右冠動脈-左房瘻に合併した多発巨大冠動脈瘤の1例を経験した．症例は35歳，女性．24歳時，第1子妊娠中に心エコーで心嚢内の嚢腫様病変を初めて指摘されたが放置していた．今回，第2子出産後， X 線造影 CT で右冠動脈入口部より起始する最大径85mmの冠動脈瘤およびそれより右房の横洞面を通り左房に向けて併存する最大径40mmの2つの冠動脈瘤を指摘された．心拡大を呈し，瘤径が大きく破裂の危険があるため，手術適応と判断された．体外循環，心停止下に冠動脈瘤切除，術中に判明した瘻孔の閉鎖および冠動脈#3への冠動脈バイパス術を施行した．術後経過は良好であり，独歩退院となった．

We report a case of an 8-year-old girl with fulminant myocarditis successfully treated with percutaneous cardiopulmonary support (PCPS). She was first taken to our hospital for treatment of suspected infective enterocolitis since her main symptoms were fever, vomiting and diarrhea. On day 2 after admission, her ECG showed wide QRS and echocardiography demonstrated severe hypokinesis. She was transferred to the ICU with suspected acute myocarditis. On admission to the ICU, circulatory collapse was not detected. ECG showed severe bradycardia and ventricular fibrillation after intubation. Cardiopulmonary resuscitation was performed immediately for 50 minutes prior to initiation of PCPS. She was treated intensively with catecholamines, plasma exchange, continuous hemodiafiltration, high-dose gamma-globulin, and high dose methylprednisolone. Hypothermia therapy was also performed. She was weaned from PCPS on day 6 after initiation of PCPS. The patient was finally discharged from the hospital without any neurological complications on day 68 after weaning from PCPS. The proportion of patients in whom cardiopulmonary resuscitation was performed or having ventricular tachycardia or fibrillation were higher in non-survivors than in survivors.

We report a case of successful reoperation for pulmonary autograft root dilatation causing severe regurgitation after a Ross-Konno procedure in an infant. The procedure consisted of reduction of the circumference of the sinotubular junction and the autograft annulus. This technique is an effective alternative to prolong the transition period after a Ross procedure prior to other definitive surgeries, such as valve-sparing aortic root replacement or prosthetic valve replacement, which are preferably avoided at this young age.

Left ventricular noncompaction (LVNC) represents arrest of the normal myocardial compaction process and results in the persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses. LVNC can be classified into 2 forms: isolated LVNC in the absence of other cardiac anomalies and non-isolated LVNC associated with congenital heart disease. The clinical presentation and the natural history of LVNC are highly variable, ranging from no symptoms to congestive heart failure, arrhythmias, and systemic thromboemboli. LVNC is genetically heterogeneous and can be inherited as an autosomal dominant or X-linked recessive disorder. It is also linked to mutations in several genes, encoding the sarcomeric proteins, such as myosin heavy chain 7 (MYH7). MYH7 encodes the β-myosin heavy chain, expressed in the cardiac muscle. The operative indication for patients with non-isolated LVNC is unclear. Here, we report the first successful case of surgical repair of a ventricular septal defect (VSD) in an infant with non-isolated LVNC associated with a novel MYH7 mutation. This mutation leads to the substitution of 7 amino acid residues (671-677) in the actin-binding region of the protein. After the VSD operation, the patient's congestive heart failure and pulmonary hypertension improved. His condition has remained stable for 18 months with pharmacotherapy comprising diuretics, an angiotensin converting enzyme inhibitor, and a β-blocker. Although the postsurgical observational period was short, the findings indicate that LVNC mutation analyses may facilitate surgical decisions and help predict clinical courses.

We experienced 2 patients who died early after emergency cardiac operation because of malignant diseases. In one case, we operated on a 67-year-old woman for infective endocarditis and performed aortic valve replacement, but she died of terminal pancreatic cancer on the 26 postoperative day. In the other case, a 53-year-old woman underwent emergency operation for cardiac tumor of the left atrium. However, the tumor was diagnosed as cardiac sarcoma during the operation, and it could not be completely resected. The sarcoma recurred after 2 months and she passed away. An emergency cardiac operation without enough preoperative examinations sometimes causes a poor prognosis.

We report a successful arterial switch operation for complete transposition of great arteries with atrial and visceral situs inversus totalis and mirror image dextrocardia in a 12-day-old infant girl. The aorta was located left side-by-side to the pulmonary trunk with a single coronary artery (mirror image of 1RLCx). After French maneuver, the posterior circumference of the neo-aorta was reconstructed. Then the coronary button was transplanted into the neo-aorta with a trap door technique carefully avoiding any twist and over-stretch. The neo-pulmonary trunk was reconstructed with an autologous pericardial patch and sutured to the longitudinal incision made into the left central pulmonary artery. The baby was discharged from hospital and has been doing well without any morbidity relating myocardial ischemia.

We report a pediatric case of successful cardiac resynchronization therapy (CRT) for acute relapsed deterioration of dilated cardiomyopathy with cardiac dyssynchrony due to a noncompacted left ventricle (LV). A 20-month-old female developed severe congestive heart failure, which was classified as New York Heart Association class IV. She failed to thrive despite intravenous inotropic support. Her LV function and dimension improved markedly after CRT. The beneficial effect of CRT in this patient has remained for more than 3 years.

Objective: Establishing a new continuity between the right ventricle and the pulmonary artery is the mainstay of repair for persistent truncus arteriosus. We used the Tran Viet—Neveux technique without a Lecomte maneuver to construct the connection without a conduit. Here, we retrospectively review the mid-term surgical results to examinethe effectiveness of this approach. Methods:A cylindrical segment incorporating both pulmonary artery branches was sleeve-resected from the truncal artery. The cylindrical segment was cut in the middle and two truncal arterial flaps were combined to form the posterior floor of the new pulmonary arterial trunk. The edge of the floor was attached directly to the superior margin of an oblique incision made in the left-anterior wall of the right ventricle. A polytetrafluoroethylene monocusp was attached to the lower half margin of the right ventricular incision. A large glutaraldehyde-treated pericardial patch was used to form the anterior hood of the new right ventricular outflow tract. Both great arteries were located in a normal spiral configuration. Results: Ten babies (range: 3 days to 9 months of age) underwent this procedure.The Collett—Edwards classification of persistent truncus arteriosuswas type I in five cases and type II in five others. There was one hospital death due to severe respiratory distress. During follow-up (36—60 months, median 54 months), only one re- operation was required to enlarge a left branch pulmonary artery stenosis. Follow-up echocardiography showed pulmonary regurgitation (mild two, moderate seven, and severe one) and mild flow acceleration in the left pulmonary artery branch and right ventricle—pulmonary artery connection in one case. Conclusion: This simple modification for surgical correction of persistent truncus arteriosus may be an effective alternative that overcomes conduit-related problems. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Murine studies have been a mainstay of analyzing the effects of genetic manipulation on cardiac phenotype. Reliable and efficient methods to evaluate cardiac phenotype are necessary and need to be established. However, cardiac catheterization techniques for obtaining such data have not been standardized. The goal of this study was to establish a simple and less invasive technique for cardiac catheterization in a closed-chest mice model. Mice were anesthetized with a combination of ketamine and xylazine. The right common, internal, and external carotid arteries were isolated microscopically, and a 1.4 Fr. conductance catheter was inserted and advanced retrograde into the left ventricle (LV). LV hemodynamic parameters were measured. This simple technique enabled us to obtain more physiologic LV hemodynamics compared with those from open-chest models reported in literature. When combined with other techniques, such as echocardiography, more precise assessment of cardiac function can be achieved. Although potentially associated with a modest learning curve, this technique likely will be useful as a standard procedure for cardiac catheterization in mice.

Cardioplegic arrest has been the main mechanism of myocardial protection during open-heart surgery; however, it causes myocardial injury during ischemia-reperfusion. Free radical scavengers are widely known to attenuate ischemia-reperfusion injury in various settings. We investigated the effects of edaravone, a novel free radical scavenger that was originally used for cerebral protection, on myocardial function during ischemia-reperfusion after cardioplegic arrest. Rat hearts were excised and perfused using Langendorff apparatus. The hearts were cardioplegically arrested for 90 min using St. Thomas' Hospital cardioplegic solution (ST solution) at 4 degrees C every 45 min and then reperfused for 20 min. The hearts were divided into 4 groups (n = 13 in each group). In Group ST, the hearts were arrested using the ST solution alone. In Groups L, M, and H, the hearts were arrested using the ST solution supplemented with a low-dose (1 microM), moderate dose (10 microM), and high dose (100 microM) of edaravone, respectively. Left ventricular function (+dp/dt (max)) and the levels of the cardiac enzymes released were measured before and after cardioplegic arrest. At the end of the study, the water content and the tissue oxidative stress (8-hydroxy-2'-deoxyguanosine) of the heart were measured. During reperfusion, the edaravone-treated groups showed a greater functional recovery with regard to the +dp/dt (max) (P0.05). The lactate level was the lowest (P0.01) in Group M. The water content of the hearts in the edaravone-treated groups was significantly lower (P0.05) than that in Group ST. Oxidative stress was significantly lower (P0.01) in the edaravone-treated hearts than in Group ST, and it was the lowest in Group M. The addition of edaravone to the cardioplegic solution ameliorates the impairment in myocardial function by reducing the oxidative stress after cardioplegic arrest. In this study, the maximum improvement in the myocardial function was achieved by addition of a moderate dose (10 microM) of edaravone.

We report an initial experience of successful application of bosentan (BOS), a endotheline-1 dual-receptor blocker, in the treatment of sustained pulmonary hypertension (PH) early after surgical correction of congenital heart disease. Two infants (5 and 9-months-old girls undergone repair of complete atrioventricular septal defect and closure of ventricular septal defect, respectively) suffered from PH-relating symptoms after extubation despite oral sildenafil (SIL) treatment. After administration of BOS (1.5 mg/kg/day), the symptoms were dramatically relieved and SIL was uneventfully discontinued. Although BOS caused a transient increment of liver transaminase in the 5-month-old girl, BOS is a possible alternate in the treatment of the postoperative sustained PH as reported in a various type of chronic PH.

We report a case of successful administration of oral sildenafil (ie, a phosphodiesterase-5 inhibitor) in an infant for impaired pulmonary circulation that caused early clinical deterioration after a bicavopulmonary shunt. The transpulmonary pressure gradient (ie, a clinical indicator of pulmonary circulation) was initially normalized by inhaled nitric oxide; however, an increase in transpulmonary pressure gradient and oxygen desaturation occurred after extubation and discontinuation of inhaled nitric oxide on postoperative day 1. Subsequent administration of oral sildenafil in stepwise doses resulted in normalization of transpulmonary pressure gradient and improved oxygen saturation with successful discontinuation of intravenous vasodilators. Our results suggest that oral sildenafil may be a potent adjunctive therapy for impaired postoperative pulmonary circulation after right heart bypass surgery.

ObjectiveThe purpose of this study was to evaluate the therapeutic efficacy of the combined growth factor therapy with an omental flap in a rabbit model of chronic myocardial ischemia.MethodsChronic ischemia was created in rabbits by placing a constrictor on the left circumflex artery. Four weeks later the animals were divided into 3 groups: group FG, in which a gelatin hydrogel sheet incorporating 100 μg of basic fibroblast growth factor was placed over the left circumflex region followed by covering with the omental flap including the intact gastroepiploic artery; group F, in which only the basic fibroblast growth factor sheet was placed; and group N, in which no treatment was done.ResultsCine magnetic resonance imaging analysis showed a greater percentage wall thickening in the left circumflex region in group FG than in other groups (group FG, 49.2% ± 4.5%; group F, 41.2% ± 3.8%; group N, 32.1% ± 2.5%, P =.035, group FG vs group F). A colored microsphere assay showed higher perfusion in the left circumflex region in group FG than in group F. Perfusion in the left circumflex region was decreased after clamping the gastroepiploic artery pedicle in group FG (before clamping, 2.83 ± 0.72 mL · min−1 · g−1; after clamping, 1.93 ± 0.59 mL · min−1 · g−1; P < .01). In vivo angiography via gastroepiploic artery showed direct “to-and-fro” visible collaterals between the gastroepiploic and occluded left circumflex coronary arteries in group FG.ConclusionThe combined growth factor therapy with an omental flap induced arteriogenesis and provided additional perfusion via the gastroepiploic artery to ameliorate regional dysfunction in the chronically ischemic myocardium.

Acute or subacute occlusion of a fenestration between the Fontan chamber and the atrium causes low cardiac output status, sustained pleural effusion, and protein losing enteropathy in critical cases. To achieve reliable and long patency of the fenestration in extracardiac Fontan operations, we modified our technique for creating a fenestration, namely "kissing" anastomosis, in which the atrial incision is directly sutured to the surface of the extracardiac conduit leaving a few millimeters outside from the edge of the punched-out hole (fenestration). We applied this modification in 2 cases. The diameter of the hole is 4 mm (case 1) and 3 mm (case 2). The patients were anticoagulated by warfarin potassium after the surgery. The fenestrations were patent at 3- (case 1) and 1-month (case 2) after the surgery. Peripheral oxygen saturations at discharge were 86 (case 1) and 88% (case 2). There was no death or major complication. The described procedure is an effective alternative to maintain patency and function of the fenestration in extracardiac Fontan operations.

The mechanisms that underlie the development of myocardial dysfunction after resuscitated hemorrhagic shock (HS) are not known. Recent studies suggest that systemic activation of inflammatory mediators may contribute to cellular dysfunction and/or cell death in various organs, including the heart. However, the precise role that inflammatory mediators play in the heart in the setting of resuscitated HS is not known. Accordingly, the purpose of the present study was to use a well-defined murine model of resuscitated HS to characterize the functional significance of inflammatory mediators in the heart in vivo. Mice were subjected to sham operation or resuscitated HS. Left ventricular (LV) function was assessed by two-dimensional echocardiography 6 h after resuscitation. Myocardial TNF, IL-1β, and IL-6 proteins were measured 1 and 6 h after resuscitation. To determine the role of TNF in HS-induced LV dysfunction, mice were treated with a soluble TNF receptor antagonist (etanercept) before HS or at the time of resuscitation. LV fractional shortening was significantly depressed ( P < 0.05) in resuscitated HS mice (28 ± 1.5%) compared with sham controls (35.8 ± 1.0%). TNF and IL-1β levels were significantly increased ( P < 0.05) in resuscitated HS mice. Pretreatment with etanercept abrogated resuscitated HS-induced LV dysfunction, whereas treatment at the time of resuscitation significantly attenuated, but did not abrogate, LV dysfunction. Together, these data suggest that TNF plays a critical upstream role in resuscitated HS-induced LV dysfunction; however, once the deleterious consequences of reperfusion injury are initiated, TNF contributes to, but is not necessary for, the development of LV dysfunction.

A five-month-old girl of Down syndrome underwent a corrective surgery for complete atrioventricular septal defect. Her postoperative course was complicated with pulmonary hypertensive (PH) crises despite nitroglycerin (NTG) infusion and inhaled nitric oxide (NO). Sildenafil citrate, a phosphodiesterase 5 inhibitor, was administered through a nasogastric tube at a starting dose of 0.3 mg/kg by stepwise increment to the maximum dose of 2 mg/kg 4 hourly. Sildenafil citrate dramatically lowered pulmonary arterial pressure and the patient was weaned from NTG and NO without PH crisis. There was no side effect after sildenafil citrate administration. Oral sildenafil citrate is a safe and potent adjunct to the existing therapies for postoperative PH in infants after open heart surgery.