Single-stage arterial switch operation conversion with dextro-transposition of the great arteries and pulmonary arterial hypertension due to baffle leak after a Senning procedure.

Patients who have undergone an atrial switch operation for dextro-transposition of the great arteries (dTGA) sometimes suffer from right ventricular dysfunction, tricuspid regurgitation, arrhythmias, or baffle leaks. We report the first case of single-stage arterial switch operation conversion in an adult patient with dTGA and pulmonary arterial hypertension (PAH) due to a baffle leak after a Senning procedure. Perioperative mechanical circulatory support was required for 3 weeks. Although chronic kidney disease persisted, her left ventricular function and pulmonary hypertension improved over the first postoperative year. This is a rare case, and we believe that the patient's PAH might have helped the left ventricle withstand systemic pumping for over two decades. Learning objective: Patients with dextro-transposition of the great arteries may face problems after an atrial switch operation because their right ventricle is responsible for systemic pumping. Staged arterial switch operation conversion is a strategy that can combat these problems but increases the risk of perioperative mortality. Pulmonary arterial hypertension allows for single-stage arterial switch operation, but the operative risk may be higher.

Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study.

BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ±â€¯7 years old, follow-up: 18 ±â€¯6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ±â€¯1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

Mechanisms of Left Ventricular Dysfunction Assessed by Layer-Specific Strain Analysis in Patients With Repaired Tetralogy of Fallot.

BACKGROUND: Left ventricular (LV) dysfunction in patients with repaired tetralogy of Fallot (rTOF) is an important risk factor for adverse outcomes. The aim of this study was to assess the details and time course of such LV dysfunction using layer-specific strain analysis by echocardiography.Methods and Results:The 66 patients with rTOF (mean age, 16.3±9.3 years) were divided into 3 groups (T1: 4-10 years, T2: 11-20 years, T3: 21-43 years), and 113 controls of similar age (mean age, 17.2±9.3 years) were divided into 3 corresponding groups (C1, C2, and C3). Layer-specific longitudinal strain (LS) and circumferential strain (CS) of 3 myocardial layers (endocardial, midmyocardial, and epicardial) were determined by echocardiography. Basal and papillary endocardial CS values were decreased in T1 compared with C1. With the exception of papillary epicardial CS, basal/papillary CS and LS of all 3 layers decreased in T2 compared with C2. Excepting papillary epicardial CS, all other values were decreased in T3 compared with C3. CONCLUSIONS: Potential myocardial damage was found in the endocardium at the basal and papillary levels of the LV in young patients with rTOF, extending from the endocardium to the epicardium and from the base to the apex. This is the possible time course of LV dysfunction in patients with rTOF.

Dual origin multiple plexus-like coronary to pulmonary artery fistulas - Consideration of their etiology and therapeutic strategy.

BACKGROUND: Multiple plexus-like coronary to pulmonary fistulas (CAPFs) originating from bilateral coronary arteries are a rare type of coronary artery fistula (CAF). Their etiology and therapeutic strategy are discussed. METHODS AND RESULTS: Three patients were diagnosed with dual origin plexus-like CAFs drained to the pulmonary artery trunk. Their ages ranged from 40 to 78 years. Enhanced computed tomography could clearly demonstrate three-dimensional anatomy of CAPFs. Four catheter interventions were performed in all 3 patients. Antegrade approach was applied in 2 procedures and retrograde approach in 2 procedures. A detachable coil was used in 3 procedures and a combination of a vascular plug and detachable coils for 1 procedure. The effective occlusion was achieved in all patients without complications. Follow-up myocardial scintigraphy showed no perfusion defect in all patients. CONCLUSIONS: CAPFs in our cases, which developed in their adulthood, anatomically correspond with the vasa vasorum of the proximal of the great arteries. The dual origin multiple plexus-like CAPFs might develop from the vasa vasorum of the proximal portion of the great arteries with age. The antegrade approach would be effective because even dual origin multiple plexus-like CAFs converged and drained to a single major exit of the pulmonary trunk. Catheter occlusion could be feasible and safe for dual origin multiple plexus-like CAPFs.

Blood coagulation abnormalities and the usefulness of D-dimer level for detecting intracardiac thrombosis in adult Fontan patients.

BACKGROUND: Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown. METHODS: We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients. We measured various biomarkers of blood coagulation, fibrinolysis, and platelet activity in 122 Fontan patients (Fontan group: median age [P25-P75]: 27 [20-34] years) and compared them to those in 50 patients with atrial septal defect (ASD group: 31 [24-40] years). RESULTS: Regardless of whether the patient had ICT, the Fontan group showed significantly lower levels of antithrombin III, thrombomodulin, and α2-antiplasmin; lower protein C and protein S activities; and significantly higher levels of thrombin-antithrombin complex and α2-plasmin inhibitor complex than the ASD group. Among various biomarkers, D-dimer level measured by using latex immunoassay was significantly higher in the patients with ICT (thrombus group: n=21) than in the patients without ICT (non-thrombus group: n=101). Fifteen (26%) of 57 patients on warfarin achieved prothrombin time international normalized ratios (PT-INRs) of >2. The proportion of patients with PT-INRs of >2 tended to be lower in the thrombus group than in the non-thrombus group (13% vs 31%). Persistent atrial arrhythmia and D-dimer level were significant risk factors associated with ICT formation in the multivariate analysis (persistent atrial arrhythmia: hazard ratio [HR], 6.89; 95% confidence interval [CI], 1.44-34.5; D-dimer: HR, 0.29; 95% CI, 0.13-0.50). Receiver-operating characteristic curve analysis revealed that the appropriate cutoff D-dimer level for screening for ICT was 1.8µg/mL (area under the curve, 0.94), with a negative predictive value of 95%. CONCLUSIONS: In the adult Fontan patients, blood coagulation abnormalities existed regardless of the absence of ICT. D-dimer level may be a useful biomarker for screening for ICT in adult Fontan patients.

Fontan circulation over 30 years. What should we learn from those patients?

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 c 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.

Sildenafil reduces pulmonary vascular resistance in single ventricular physiology.

BACKGROUND: High pulmonary vascular resistance (PVR) may be a risk factor for early and late mortality in both Glen shunt and Fontan operation patients. Furthermore, PVR may increase long after the Fontan operation. Whether pulmonary vasodilators such as phosphodiesterase 5 inhibitors can decrease PVR in patients with single ventricular physiology remains undetermined. METHODS AND RESULTS: This was a prospective, multicenter study. Patients with single ventricular physiology who have a PVR index higher than 2.5 Wood units·ãŽ¡ (WU) were enrolled. Cardiac catheterization was performed before and after administration of sildenafil in all patients. After the Fontan operation, a six minute walk test (6MWT) was also performed. A total of 42 patients were enrolled. PVR was significantly decreased in each stage of single ventricular physiology after sildenafil administration: from 4.3±1.5WU to 2.1±0.6WU (p<0.01) in patients before a Glenn shunt, from 3.2±0.5WU to 1.6±0.6WU (p<0.001) in patients after a Glenn shunt, and from 3.9±1.7WU to 2.3±0.8WU (p<0.001) in patients after Fontan. In patients after Fontan, the 6MWT increased from 416±74m to 485±72m (p<0.01), and NYHA functional class improved significantly (p<0.05) after sildenafil administration. No major side effects were observed in any patients. CONCLUSIONS: Sildenafil reduced PVR in patients with single ventricle physiology. Sildenafil increased exercise capacity and improved NYHA functional class in patients after a Fontan operation. This implies that pulmonary vasodilation is a potential therapeutic target in selected patients with elevated PVR with single ventricle physiology. Long-term clinical significance warrants further study.

Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.

OBJECTIVES: This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. METHODS: Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). RESULTS: The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. CONCLUSIONS: In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

Efficacy of pulmonary vasodilator therapy in patients with functionally single ventricle.

This study retrospectively evaluated the effectiveness of pulmonary vasodilator therapy with bosentan (n = 14) and/ or sildenafil (n = 23) in 34 patients with a functionally single ventricle. Vasodilator therapy was initiated before the Fontan procedure in 18 patients and after the procedure in 16 patients. The reasons for vasodilator treatment included high pulmonary artery pressure or pulmonary vascular resistance (n = 8), high central venous pressure after the Fontan or bidirectional Glenn procedure (n = 7), and ventilatory impairment (n = 8). In the 11 patients who underwent right heart catheterization before and after the initiation of therapy, the mean pulmonary artery pressure decreased significantly from 19.5 ± 5.5 mmHg to 14.3 ± 3.0 mmHg (P = 0.023) and the transpulmonary pressure gradient decreased significantly from 10.9 ± 4.6 mmHg to 7.2 ± 3.3 mmHg (P = 0.046). Of the 18 patients who started vasodilator therapy before the Fontan procedure, 10 survived surgery, 4 are awaiting surgery, 3 had not been evaluated for the Fontan procedure at the end of the study period, and 1 died of heart failure after discontinuing bosentan therapy. There were no deaths among the patients who started therapy after the Fontan procedure. Two of the 14 patients receiving bosentan discontinued treatment because of adverse effects (hepatic dysfunction and increased serum brain natriuretic peptide level). Bosentan or sildenafil therapy is usually safe and may contribute to reducing pulmonary vascular resistance in patients with a functionally single ventricle before and after a Fontan type operation.

Aircraft transfer of pediatric patients with intractable cardiac or airway problems ­ single-institutional experience of a specialty hospital.

BACKGROUND: There is currently a well-established network for the allocation of donor organs for transplantation in Japan, and emergency patients are often transported by the "Doctor Helicopter". However, interhospital transfer of patients, which can require aircraft with specialized equipment, depends on arrangement by each responsible hospital. METHODS AND RESULTS: Since 2009 there were 41 interhospital aviation transfers of pediatric patients with intractable cardiac or airway diseases seeking surgical treatment at Sakakibara Heart Institute. Of these, 22 were newborns, 21 were on continuous drip infusion and 14 on mechanical ventilator support. In 15 cases (36.6%), a commercial airliner was used, with the remaining using chartered emergency aircraft (eg, local fire department helicopter, Self-Defense-Forces of Japan and the Doctor Helicopter). The median transfer time was 239 min for commercial airliners, 51 min for chartered aircraft departing directly from the referring hospital and 120.5 min for chartered aircraft departing from a nearby location. The efficiency of the transfer exemplified by the percentage of the time on board the aircraft was significantly lower for commercial airliners compared with chartered emergency aircraft. CONCLUSIONS: Further efforts and cooperation with government are required to obtain geographically uniform availability of carriers with optimal medical equipment to improve pediatric patient outcomes.

Outcome of univentricular repair in patients with Down syndrome.

OBJECTIVE: Total cavopulmonary connection (TCPC) is rarely performed for a functional single ventricle in children with Down syndrome; therefore, the postsurgical outcomes are not well known. We evaluated mortality and related factors after TCPC in children with Down syndrome. METHODS: Between January 2004 and March 2010 we identified 8 patients with Down syndrome among 235 patients who had undergone TCPC. The preoperative clinical course, preoperative data, and postoperative clinical course were evaluated. In addition, clinical parameters and postoperative clinical course were compared between children with Down syndrome (n = 8) and a non-Down syndrome group (n = 227). RESULTS: The median age at the time of TCPC was 4.1 years (range, 3.4-5.5 years), and the preoperative mean pulmonary artery pressure was 13.9 ± 1.81 mm Hg. We observed respiratory complications in 2 patients, surgical site infection in 3 patients, and chylothorax in 2 patients. No significant difference was observed in preoperative data and mortality rate (1 of 8 patients [12.5%] in the Down syndrome group; 5 of 227 patients [2.2%] in the non-Down syndrome group) between the groups of children with and without Down syndrome. However, when the postoperative clinical course was examined, the durations of intensive care unit stay (P = .009) and hospital stay (P = .007) were found to be significantly prolonged in the Down syndrome group. CONCLUSIONS: Patients with Down syndrome tend to show prolonged recovery after TCPC. However, as opposed to previous reports, the mortality rate of patients undergoing TCPC is lower with no significant difference from that of children without Down syndrome.

Outcome of implantable cardioverter defibrillator therapy for congenital heart disease.

BACKGROUND: The use of implantable cardioverter defibrillator (ICD) therapy for congenital heart disease (CHD) has been increasing, but few studies have reported on the efficacy of ICD therapy in Japanese CHD patients. METHOD: Twelve CHD patients (median age, 35 years) with first ICD implantation were examined. Median follow-up duration was 2.9 years. Demographic information, implant electrical parameters, appropriate and inappropriate discharge data and complications were recorded for all implants from 2003 to 2010. RESULTS: Implant indication was primary prevention in two patients and secondary prevention in 10. Overall four patients received one or more discharges; three patients (25%) with secondary prevention received nine appropriate discharges. Inappropriate discharge attributed to sinus tachycardia occurred in two patients (16.7%). Only one patient experienced the late complication of skin erosion at the generator implantation site. CONCLUSIONS: Patients with CHD experienced significant rates of appropriate discharges and lower complications. But given that the indications of ICD implantation were mostly for secondary prevention, the ratio of appropriate shocks might be lower than in previous studies. In the primary prevention patients, the benefit of ICD was not clear because no appropriate discharges were seen during follow up. Although ICD implantation for CHD is beneficial for preventing sudden cardiac death, careful decision making and a large, long-term prospective study is required for the determination of the efficacy of ICD therapy in Japanese patients with CHD.

Effect of carvedilol on heart failure in patients with a functionally univentricular heart.

BACKGROUND: The effect of carvedilol on heart failure (HF) in patients with a functionally univentricular heart (UVH) remains unclear. METHODS AND RESULTS: Carvedilol was used to treat HF in 51 patients with a UVH, classified into 3 groups: after the Fontan operation (F), after the bidirectional Glenn operation (G), and patients who had not undergone Fontan or Glenn operation (NF). Carvedilol therapy was started at a mean age of 10 ± 12 years (range: 1 month to 34 years). The initial and maximum doses of carvedilol were 0.04 ± 0.03 and 0.42 ± 0.29 mg · kg(-1) · day(-1), respectively. After a mean follow-up of 11 months, the cardiothoracic ratio improved from 60 ± 8 to 58 ± 8% (P<0.01), and the dosage of furosemide was reduced from 1.4 ± 0.9 to 0.7 ± 0.7 mg · kg(-1) · day(-1) (P < 0.01). The ejection fraction also improved from 35 ± 12 to 40 ± 11% (P < 0.05), and this improvement was prominent in the F group (from 35 ± 15 to 45 ± 9%; P < 0.05). Clinical signs, symptoms, and New York Heart Association functional class also improved. CONCLUSIONS: Carvedilol may play an important role in treating HF associated with a UVH.

Efficacy and safety of carvedilol for heart failure in children and patients with congenital heart disease.

There have been few reports describing the use of carvedilol in children or patients with congenital heart disease. Therefore, its optimal regimen, efficacy, and safety in these patients have not been adequately investigated. Subjects were 27 patients with two functioning ventricles, for whom carvedilol was initiated (from December 2001 to December 2005) to treat heart failure. All patients had failed to respond to conventional cardiac medication. They consisted of 12 males and 15 females, aged 23 days to 47 years (median age: 2 years). Heart failure due to ischemia (myocardial infarction, intraoperative ischemic event) or due to myocardial disease (cardiomyopathy, myocarditis), and heart failure with atrial or ventricular tachyarrhythmia represented 70% of all cases. Carvedilol was initiated at a dose of 0.02-0.05 mg/kg/day, which was increased by 0.05-0.1 mg/kg/day after 2 days, 0.1 mg/kg/day after 5 days, and 0.05-0.1 mg/kg/day every month thereafter with a target dose of 0.8 mg/kg/day. This study retrospectively assessed the efficacy and adverse reactions based on changes of symptoms, cardiothoracic ratio (CTR), left ventricular ejection fraction (LVEF), and human atrial natriuretic peptide (hANP)/b-type natriuretic peptide (BNP) blood levels. The mean follow-up period was 10.2 months (range: 1-46 months). Twenty-six (96.3%) patients showed improvement in symptoms and were discharged from the hospital. However, the remaining one patient failed to respond and died. Significant cardiovascular adverse reaction was seen in none of the patients. The mean CTR decreased from 61.8% +/- 5.3% before treatment to 57.6% +/- 7.4% after treatment (P < 0.05, n = 25), and the mean LVEF improved from 41.4% +/- 23.1% to 61.1% +/- 10.1% (P < 0.05, n = 10), respectively. Mean hANP and BNP levels showed a decrease from 239.1 pg/ml to 118.3 pg/ml and a significant decrease from 437.9 pg/ml to 120.5 pg/ml, respectively (P < 0.05, n = 10). Improvements in these data were also demonstrated when analyzed individually among the pediatric group (aged younger than 18) and the congenital heart disease group. Initiation of carvedilol at a lower dose with more gradual dose escalation, compared with previously reported regimens, might have efficacy with low incidence of adverse effects in pediatric patients and patients with congenital heart disease. Carvedilol may be effective in treating heart failure in children due to ischemia, myocardial disease, and complicated by tachyarrhythmia.

Single ventricle repair in children with Down's syndrome.

OBJECTIVE: There is a paucity of information regarding appropriate management of children with Down's syndrome and a functional single ventricle. We report the results of bidirectional superior cavopulmonary shunts in six patients with Down's syndrome with a functional single ventricle. METHODS: Between January 1991 and December 2004, we identified six patients with Down's syndrome among 263 who had undergone bidirectional superior cavopulmonary shunts (BCPSs). There were four males and two females. The age at BCPS ranged from 1 to 12 years (mean 4.3 +/- 3.9 years), and body weight varied between 8.2 and 29.4 kg (mean 13.8 +/- 7.8 kg). All six patients had an unbalanced complete atrioventricular septal defect, with right ventricular hypoplasia present in five and left ventricular hypoplasia in one. RESULTS: There were no operative deaths, but one case required takedown of the BCPS. Except for this case, postoperative courses were generally uneventful. The median duration of follow-up was 46 months (range 12-80 months). Only two of five survivors after BCPS underwent a subsequent Fontan procedure, and one of these patients died of pulmonary hypertension post-operatively. The remaining three patients appeared to have significant risk factors for the Fontan procedure due to severe common atrioventricular valve regurgitation or persistent pulmonary vascular obstructive disease, including one who has completely dropped out from the Fontan track. CONCLUSION: Down's syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down's syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully.

Effectiveness of carvedilol for congestive heart failure that developed long after modified Fontan operation.

We report a case of a patient with severe heart failure after Fontan procedure in whom carvedilol was very effective. A 27-year-old man had intractable congestive heart failure due to severe ventricular dysfunction after Fontan operation. Central venous pressure was elevated to 29 mmHg. A right-to-left shunt was noted across a large collateral vessel between the innominate vein and the pulmonary vein. He was administered carvedilol (initial dose, 2 mg/day; maximum dose, 30 mg/day). Cardiac catheterization performed 1 year after carvedilol administration revealed a decrease in atrial pressure and improvement of ventricular function. He underwent a conversion operation to total cavopulmonary connection (TCPC) and ligation of a collateral vein communicating with the innominate and pulmonary veins. Carvedilol may be a legitimate treatment before TCPC conversion or heart transplantation for the high-risk group of patients with a failed Fontan circulation.

Arterial switch operation using aortic homograft for transposition of the great arteries with pulmonary regurgitation.

We report a case of a 15 month-old boy who underwent the arterial switch operation using cryopreserved aortic homograft for transposition of the great arteries with pulmonary regurgitation, with coexisting right ventricular outflow tract obstruction precluding atrial switch operation. Follow-up echocardiography at 6 months showed trivial neoaortic valve regurgitation, no significant systemic outflow obstruction, with good cardiac function. In small children, the choice of material for left ventricular outflow tract reconstruction is one of the most crucial issues. Cryopreserved homograft has been one of the primary options for the aortic valve replacement in small children because of the ease of suturing and excellent hemostasis.

Mitral valve replacement in children under 3 years of age.

OBJECTIVE: This study was undertaken to review our experience of mitral valve replacement in children under 3 years of age. METHODS: Between January 1990 and May 2004, 18 patients under 3 years of age underwent a total of 20 mitral valve replacements using a bileaflet mechanical prosthetic valve. There were 9 males and 11 females. The age at surgery ranged from 3 months to 3 (mean=1.02 +/- 0.72) years and body weight varied between 3.4 and 13.2 (mean=7.08 +/- 2.74) kg. RESULTS: There were 4 early and 2 late deaths, and these occurred in severe cases aged less than 1 year of age. Re-replacement of mitral valve was required in 3 patients (valve thrombosis in 2 and pannus formation in 1). Orifice size of the implanted prosthesis (OS) as compared with the predicted normal size of the mitral valve (NS) was well correlated with maximum transprosthetic flow velocity estimated by Doppler echocardiography. In this study, the OS/NS>0.65 was maintained in all patients, and none required re-replacement because of prosthesis-patient mismatch. CONCLUSION: Patients less than 1 year of age had significant mortality and morbidity. The results were satisfactory in the remainder (1-3 years). During this follow-up period, none required re-replacement due to somatic growth, but it will be an unavoidable problem in the future. The OS/NS, which can be checked with a regular physical examination, may serve as a guide to determine the most appropriate timing for the second surgery.

Steroid supplementation: a legitimate pharmacotherapy after neonatal open heart surgery.

BACKGROUND: An inflammatory response together with multiple organ failure subsequent to cardiopulmonary bypass is especially prominent in neonates. The behavior of glucocorticoids during this period in these patients is not known. If adrenal insufficiency should exist, it could considerably compromise postoperative recovery. METHODS: Twenty neonates undergoing biventricular repair were enrolled. Ten patients were assigned to receive hydrocortisone treatment and the other 10 to receive placebo. The treatment group received stress-dose hydrocortisone sodium succinate after discontinuation of cardiopulmonary bypass: 0.18 mg.kg(-1).hr(-1) for 3 days, 0.09 mg.kg(-1).hr(-1) for 2 days, and 0.045 mg.kg(-1).hr(-1) for 2 days. The placebo was 5% glucose solution. RESULTS: Patients had adrenal insufficiency (cortisol < 5 microg/dL) from 24 to 72 hours in the placebo group. This was associated with a simultaneous reduction of left ventricular shortening fraction (p < 0.0001, analysis of variance; p = 0.0203, Student's t test), the necessity to increase inotropic agents (p = 0.043, analysis of variance), and an increase in serum lactate level (p = 0.049, Student's t test). During this period, serum cortisol level was maintained above the normal level (> 23 microg/dL) in the hydrocortisone group. The placebo group had a greater positive fluid balance (p = 0.027, Student's t test) and greater total body edema in the immediate postoperative period (p = 0.065, Student's t test). Blood oxygenation constantly improved, and the duration on mechanical ventilation was shorter (83.5 +/- 42.1 versus 138.2 +/- 89.7 hours; p = 0.098) in the hydrocortisone group. CONCLUSIONS: Adrenal insufficiency may occur after neonatal open heart surgery. Stress-dose hydrocortisone supplementation blunts other organ dysfunction and can be considered a legitimate pharmacotherapy in this cohort.