Aortic root replacement in a patient with unoperated tricuspid atresia.

A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Aortic root replacement was performed and then his heart failure was controlled.

A Case of Thoracic Endovascular Aortic Repair Using Carotid Access with Axillary-Carotid Bypass for Descending Aortic Aneurysm in a Patient with Aortoiliac Occlusive Disease.

The authors report a 71-year-old male with descending thoracic aortic aneurysm and multiple risk factors (aortoiliac occlusive disease, obesity, ascending aorta dilatation, and history of left ventriculoperitoneal shunt for hydrocephalus) who was treated with thoracic endovascular aortic repair (TEVAR) via left common carotid artery (LCCA) access and left axillary-carotid artery (Ax-CA) bypass; this approach shortened the LCCA clamp time during the procedure. The patient was discharged without any complications. TEVAR via LCCA access with left Ax-CA bypass is a useful and safe procedure for patients in whom conventional femoral artery access is not feasible.

Surgical treatment for adult congenital heart disease: consideration for indications and procedures.

The number of the adult patients with congenital heart diseases (ACHD) continues to grow owing to improvement of surgical results and medical management. Corrective surgery for complex CHD does not always mean complete cure. It is not rare that the patients will visit the cardiology institutes because of secondary lesions due to residua or sequela in adults. Some patients with CHD remain unrepairable with different degree of heart failure and pulmonary arterial hypertension. Association of arrhythmias is common in ACHD patients and sometimes critical. We experienced 265 surgical procedures for ACHD patients at our center between 1999 and 2015. Of these procedures, palliative surgery was performed in 3%, palliation to corrective surgery in 6%, primary repair in 57%, and redo surgery in 34%. Hospital mortality within 30 days in this period was 1.1%. Surgery for ACHD patients is safe, beneficial and low-risk treatment; however, tailored procedures for the individual patient are essential to obtain the optimal quality. A comprehensive multidisciplinary approach is required to fulfill this goal.

Surgical angioplasty for ostial atresia of left main coronary artery in child.

Ostial atresia of the left main coronary artery (LMCA) in children without any primary disease is extremely rare. We present here a case of occlusion of the LMCA in a 9-year-old girl. Myocardial scintigraphy showed poor perfusion in both domains of the left anterior descending artery (LAD) and left circumflex artery (LCx). Coronary artery graphy (CAG) showed complete ostial atresia of the LMCA and retrograde perfusion from the thin collateral arteries into the LAD. We performed angioplasty using an autologous pericardium onlay patch. Her postoperative course was unremarkable. Postoperative CAG showed vanishing collateral arteries, confirming anterograde flow through the LAD and LCx, and myocardial scintigraphy showed improvement in perfusion.

Patient-specific assessment of cardiovascular function by combination of clinical data and computational model with applications to patients undergoing Fontan operation.

The assessment of cardiovascular function is becoming increasingly important for the care of patients with single-ventricle defects. However, most measurement methods available in the clinical setting cannot provide a separate measure of cardiac function and loading conditions. In the present study, a numerical method has been proposed to compensate for the limitations of clinical measurements. The main idea was to estimate the parameters of a cardiovascular model by fitting model simulations to patient-specific clinical data via parameter optimization. Several strategies have been taken to establish a well-posed parameter optimization problem, including clinical data-matched model development, parameter selection based on an extensive sensitivity analysis, and proper choice of parameter optimization algorithm. The numerical experiments confirmed the ability of the proposed parameter optimization method to uniquely determine the model parameters given an arbitrary set of clinical data. The method was further tested in four patients undergoing the Fontan operation. Obtained results revealed a prevalence of ventricular abnormalities in the patient cohort and at the same time demonstrated the presence of marked inter-patient differences and preoperative to postoperative changes in cardiovascular function. Because the method allows a quick assessment and makes use of clinical data available in clinical practice, its clinical application is promising.

Valve-sparing root replacement surgery for the truncal valve in an adult: report of the initial successful case.

We describe here an initial successful case of valve-sparing surgery using reimplantation technique in a 24-year-old male with aortic root dilatation with truncal valve insufficiency after common arterial trunk repair. Concomitant right ventricular outflow tract reconstruction with expanded polytetrafluoroethylene was also successfully performed. He was discharged home on postoperative day 10 without stenosis or regurgitation of repaired valves. He is in New York Heart Association class I condition without any anticoagulant agents 6 months after operation. Of course, careful follow-up will be needed though our early result is acceptable.

Fontan completions over 10 years after Glenn procedures.

OBJECTIVE: Despite the broadened indications for Fontan procedure, there are patients who could not proceed to Fontan procedure because of the strict Fontan criteria during the early period. Some patients suffer from post-Glenn complications such as hypoxia, arrhythmia, or fatigue with exertion long after the Glenn procedure. We explored the possibility of Fontan completion for those patients. METHODS: Between 2004 and 2010, five consecutive patients aged between 13 and 31 years (median 21) underwent Fontan completion. These patients had been followed up for more than 10 years (10 to 13, median 11) after Glenn procedure as non-Fontan candidates. We summarise these patients retrospectively in terms of their pre-operative physiological condition, surgical strategy, and problems that these patients hold. RESULTS: Pre-operative catheterisation showed pulmonary vascular resistance ranging from 0.9 to 3.7 (median 2.2), pulmonary to systemic flow ratio of 0.3 to 1.6 (median 0.9), and two patients had significant aortopulmonary collaterals. Extracardiac total cavopulmonary connections were performed in three patients, lateral tunnel total cavopulmonary connection in one patient, and intracardiac total cavopulmonary connection in one patient, without a surgical fenestration. Concomitant surgeries were required including valve surgeries--atrioventricular valve plasty in three patients and tricuspid valve replacement in one patient; systemic outflow tract obstruction release--Damus-Kaye-Stansel procedure in two patients and subaortic stenosis resection in one patient; and anti-arrhythmic therapies--maze procedure in two patients, cryoablation in two patients, and pacemaker implantation in two patients. All patients are now in New York Heart Association category I. CONCLUSION: Patients often suffer from post-Glenn complications. Of those, if they are re-examined carefully, some may have a chance to undergo Fontan completion and benefit from it. Multiple lesions such as atrioventricular valve regurgitation, systemic outflow obstruction, or arrhythmia should be surgically repaired concomitantly.

Survey of reoperation indications in tetralogy of fallot in Japan.

BACKGROUND: Although the survival rate for repaired Tetralogy of Fallot (TOF) is dramatically improving, anatomical and functional residua and sequelae followed by arrhythmias and sudden death are still challenging issues to be resolved. Reoperation can reduce the incidence of arrhythmias and sudden death, but there is no consensus on the indications of reoperation for patients with TOF, especially in Japan. METHODS AND RESULTS: A cross-sectional questionnaire survey of reoperation indications in patients with TOF was performed through a Japanese multicenter study. The questionnaire, which focused on the number of repaired TOF patients aged >15 years old, reoperation indications and management, was sent to the institutions belonging to Japanese Society for Adult Congenital Heart Disease. In total, 41.5% (78/188) of the institutions replied. The total number of repaired TOF patients was 4,010, and sudden cardiac death was observed in 45.236/4,010 (5.9%) experienced reoperation. Pulmonary stenosis (32%) and pulmonary regurgitation (29%) were the most common reasons for reoperation. There were only 2 implantable cardioverter defibrillator or resynchronization therapy defibrillator implantations. The physiological/anatomical indications of reoperation differed among the hospitals. CONCLUSIONS: Approximately 1.1% of patients suffered sudden death and 6% of repaired TOF patients had reoperation. The indications of reoperation, however, varied among the institutions. Therefore, detailed information for each case of sudden death or reoperation should be collected and analyzed in order to establish guidelines for reoperation.

Outcomes of one-lung Fontan operation: a retrospective multicenter study in Japan.

BACKGROUND: The Fontan operation for patients with one available lung is an extremely challenging situation. However, few reports are available on this procedure. The purpose of this study was to describe outcomes of one-lung Fontan operation. METHODS: A retrospective multicenter study was performed. Twelve of 1,142 patients whose data were recorded here underwent one-lung Fontan operation between September 1989 and October 2009. Preoperative, operative, and postoperative data were reviewed. RESULTS: Median age at operation was 3.5 years (range, 1.0 to 22.8), the preoperative mean pulmonary pressure was 11.5±3.3 mm Hg (range, 7.0 to 18.0), the ventricular ejection fraction was 58%±13% (range, 39 to 76), and end-diastolic ventricular pressure was 7.5±3.5 mm Hg (range, 1.0 to 12.0). The available lung was right in 9 patients and left in 3 patients. Eleven patients underwent a two-staged Fontan completion. Extracardiac conduit total cavopulmonary connection, intraatrial extracardiac conduit total cavopulmonary connection, and atriopulmonary connection were performed in 10 patients, 1 patient, and 1 patient, respectively. The estimated actuarial survival was 83% at 1 year, 73% at 5 years, and 73% at 10 years. Impaired ventricular function was found to be a significant risk factor for mortality by univariate analysis (43.0%±9.5% versus 64.0%±9.5%, p<0.01), but not by multivariate analysis. CONCLUSIONS: One-lung Fontan operation can be performed with an acceptable midterm to long-term mortality rate in patients without impaired ventricular function. Thus, absence of one lung itself is not a contraindication to the Fontan operation.

Intra-atrial rerouting and maze procedure for an adult patient in cor triatriatum, persistent left superior vena cava, and atrial fibrillation.

A combination of cor triatriatum and persistent left superior vena cava without communication to the coronary sinus is uncommon. A 62-year-old male with this diagnosis in conjunction with atrial fibrillation underwent successful intracardiac repair done with a unique method. After a maze procedure and enlargement of the route from the pulmonary veins to the mitral valve, a GoreTex graft was used to reroute the left superior vena cava into the right atrium and to close two thirds of the circumference of the patient's atrial septal defect; the rest of the defect was closed with another GoreTex patch.

Intracardiac total cavopulmonary connection in an asplenic adult male 12 years after Glenn procedure for total anomalous pulmonary venous return (1b).

Although indications for a Fontan procedure have broadened, some patients, in the past, were ineligible for the Fontan completion after a Glenn procedure and thus suffered the limitations of the Glenn procedure-namely desaturation, arrhythmia and reduced quality of life. If examined more closely, however, completion may yet be feasible for such patients. We present here a complex case of asplenia, dextrocardia and total anomalous pulmonary venous return (1b) where the Fontan procedure was successfully completed 12 years after the Glenn procedure. A unique surgical strategy incorporating intra-atrial total cavopulmonary connection and atrioventricular valve repair contributed to our success.

Prevalence of adult patients with congenital heart disease in Japan.

BACKGROUND: Today most patients with congenital heart disease (CHD) can be expected to survive into adulthood. Reports regarding the number of adults with CHD in Japan are scarce. Our study aims to define the number of these adults. MATERIAL AND METHODS: The estimated number of infants born in Japan with major CHDs since 1947 was calculated together with mortality rates. We estimated the number of CHD survivors from data on survival rates of unoperated and postoperative patients. The number of deaths from 1968 to 1997 was analyzed using individual death certificates held by the Japanese Government. RESULTS: In 1967, 163,058 patients with CHD including 53,846 adults were assumed to be alive. From 1968 to 1997, 548,360 patients with CHD were born and 82,919 died. A total of 622,800 patients, including 304,474 children (49%) and 318,326 adults (51%) were estimated to be alive in 1997. From 1997 to 2007, there has been an estimated increase of 9000 adults every year, and in 2007, 409,101 adults are estimated to be alive. CONCLUSIONS: The prevalence in adults with CHD in Japan has explosively increased from 1967 to 2007. There were 409,101 adults with CHD in 2007 with an annual increase of 9000. These data are crucial for planning the establishment in Japan of special facilities and resources necessary for the care of these patients.

The prevalence and risk factors for cholelithiasis and asymptomatic gallstones in adults with congenital heart disease.

PURPOSE: Cyanosis is considered to be a risk factor for cholelithiasis which is an important complication of cyanotic congenital heart disease (CCHD) in adults. In this study, the prevalence of cholelithiasis and asymptomatic calcium bilirubinate gallstones was evaluated in adults with congenital heart disease (CHD). Furthermore, risk factors for this potentially high risk complication were assessed. MATERIALS AND METHODS: Subjects were derived from 114 consecutive congenital patients who visited our center from May 2008 to January 2009. For analyses of risk factors, we divided them into 4 groups: group A, 15 CCHD patients without reparative surgery (7 men, 31.8 ± 7.0 years old); group B, 41 CCHD patients rendered acyanotic by reparative surgery (21 men, 32.5 ± 11.8 years old); group C, 23 unoperated acyanotic CHD patients (11 men, 42.4 ± 16.4 years old); and group D, 35 patients who were acyanotic before and after operation (18 men, 36.3 ± 14.8 years old). Gallstones were identified by abdominal ultrasound and risk factors were analyzed by a multivariate logistic regression model. RESULTS: Cholecystectomy was performed in 5/114 (4.3%), asymptomatic gallstones were seen in 16/114 (14%), and symptomatic gallstones except for patients after cholecystectomy were seen in 7/114 (6.1%). In group A, 4 (27%) with gallstones underwent cholecystectomy (p<0.01). Non-cholesterol gallstones were observed in 5 patients (33%) in group A, 12 patients (29%) in group B, nobody in group C, and 3 patients (8.6%) in group D. By a multivariate logistic regression model, CCHD by nature regardless of repair, prolonged cyanosis periods, higher frequency of cardiopulmonary bypass (CPB), and lower platelet counts were significant factors predicting gallstones (odds ratio 4.48, 1.08, 3.96, and 0.87, 95% CI, 1.14-17.5, 1.00-1.18, 1.65-9.54, and 0.75-0.99, respectively). CONCLUSIONS: The prevalence of cholelithiasis and asymptomatic gallstones is significantly high in CCHD patients regardless of cardiac repairs. CCHD by nature, prolonged cyanosis durations, high frequency of CPB and low platelet counts have influences on gallstone formation in adults with CHD.

Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies.

OBJECTIVE: The extracardiac conduit procedure is widely used for patients with heterotaxy syndrome with complex systemic and pulmonary venous anomalies; however, it lacks conduit-growth potential and requires long-term anticoagulation. We present the intracardiac Fontan procedure, which eliminates the above-mentioned disadvantages. PATIENTS AND METHODS: Twenty-four patients (mean age, 4.1 years; weight, 13.0 kg) with heterotaxy syndrome underwent intracardiac Fontan operations between March 1995 and March 2008. In each patient, the anomalous systemic venous return with the isolated hepatic vein was redirected to the pulmonary artery using an intra-atrial baffle without obstructing the pulmonary venous pathway; this was accomplished by anterior (n=5), lateral (n=15) or posterior tunnel methods (n=4), depending on the anatomical relationship of the systemic and pulmonary venous pathways. RESULTS: There was one (4%) in-hospital and two (8%) late deaths in total. Five (21%) patients underwent re-operation for either pulmonary venous obstruction or supraventricular tachycardia. The actuarial 12-year survival was 86% (the Kaplan-Meier survival plot). The freedom from re-operation at 10 years was 77%. Anticoagulation was not required except for two patients (8%) who had prosthetic valves and coagulation disorder. Obstruction of the systemic venous pathway was not observed in any patient; however, five (20%) patients had clinically significant postoperative arrhythmias. At the final follow-up, all survivors were categorised as the New York Heart Association class I. CONCLUSIONS: Aided by detailed preoperative anatomical and physiological diagnoses, intracardiac Fontan procedures were technically feasible in patients with complex systemic and pulmonary venous anomalies. The specific cardiac anatomy in these patients warranted this procedure; however, taking into consideration the improved outcomes of the modified Fontan procedure, this method should be performed with deliberation.

Nationwide survey of care facilities for adults with congenital heart disease in Japan.

BACKGROUND: The number of adults with congenital heart disease (CHD) is increasing rapidly, but care programs have not been fully established in Japan. METHODS AND RESULTS: Questionnaires regarding current status and resources of outpatient and in-hospital services, and management of pregnancy in patients with adult CHD (ACHD) were sent to 1,033 training hospitals for board-certified cardiologists. Useful replies were obtained from 458 hospitals (44%). In 417 hospitals (91%), at least 1 patient was followed in the outpatient clinic; however, only 14 hospitals (3%) had specialized outpatient clinics; 354 hospitals (77%) had in-hospital patients, but only 6 hospitals (2%) admitted >50 patients per year. Surgery for ACHD was performed in 232 hospitals (51%), but in 135 of these (58%), the number of operations was <5 per year. Pregnant women with CHD were managed in 157 hospitals (34%), although only 3 hospitals (2%) managed >10 cases per year. CONCLUSIONS: In most hospitals in Japan, a limited number of ACHD patients have been followed up and specialized multi-disciplinary facilities for ACHD need to be established.

Arrhythmia and reduced heart rate variability during pregnancy in women with congenital heart disease and previous reparative surgery.

OBJECTIVES: Tachyarrhythmia during pregnancy may have an adverse effect on the mother and the fetus. Heart rate variability (HRV) is a significant marker of autonomic nervous function and may predict tachyarrhythmia. HRV and incidence of arrhythmia in women with repaired congenital heart disease (CHD) during pregnancy were examined. METHODS: Twenty-eight patients with repaired CHD (35 pregnancies, age: 26+/-3.5 years at their first pregnancy, 22+/-6.2 years post-repair) and 19 healthy pregnant volunteers (controls, 19 pregnancies, age: 28+/-5.5 years) were enrolled. Holter monitoring and laboratory tests were examined at 28+/-4 weeks of gestation and 22+/-13 weeks postpartum in patients and 28+/-3 weeks of gestation in controls. Time and frequency variables of HRV were analyzed. RESULTS: Compared with controls and postpartum, there was a significantly higher incidence of tachyarrhythmia during pregnancy in women with CHD (4 episodes of supraventricular tachyarrhythmia (SVT) and 5 of non-sustained ventricular tachycardia, P=0.02). HR response in tetralogy of Fallot was impaired (781+/-50 ms, P=0.02). Most of HRV variables were significantly suppressed (average RR 740+/-64 ms, SDRR 99+/-22 ms, low and high frequency domains (341+/-165 ms2 and 256+/-181 ms2) (P<0.05). Reduced SDRR and high frequency domains were observed in patients with tachyarrhythmia. Furthermore, there were increased left ventricular end-diastolic dimensions 48+/-5 mm (P=0.001) and atrial natriuretic peptide levels 33+/-13 pg/ml (P=0.01) in the CHD patients compared with postpartum levels. CONCLUSIONS: Impaired autonomic nervous activity, volume overload of the heart and operative scar all play a contributory role in higher incidence of tachyarrhythmia during pregnancy in patients with repaired CHD compared to healthy pregnant controls. Close monitoring of patients with CHD for tachyarrhythmia during pregnancy is, thus, warranted.

Hemiazygos venous additional pulmonary flow for successful total cavo-pulmonary connection.

Patients who underwent only Glenn procedure after being deemed unsuitable candidates for Fontan completion are not small in number, and may develop arterio-venous (AV) pulmonary malformations during the follow-up period. We present the case of a 17-year-old woman with severe systemic desaturation 13 years after Glenn procedure and repair of total anomalous pulmonary venous return. Among other anomalies, the patient disclosed hemiazygos continuation to a persistent left superior vena cava. This case supports the concept that returning flow from the abdominal vein plays an important role in the well-balanced growth of the pulmonary artery and in the inhibition of the pulmonary AV malformation.

Shaggy aorta syndrome after acute arterial macroembolism: report of a case.

We report the case of a patient who underwent treatment for a macroembolism in the right lower leg, which led to shaggy aorta syndrome. Anticoagulant therapy for the macroembolism and intra-aortic catheterization exacerbated the patient's renal function and triggered another massive microembolization of the visceral arteries, with a fatal outcome. To minimize the incremental complications inherent to this syndrome, awareness and prompt diagnosis with enhanced computed tomography or intravenous digital subtraction aortography are essential. Axillo-bifemoral bypass with bilateral external iliac artery ligations, performed with optimal timing, could save patients with shaggy aorta syndrome.