Yasutaka Kawasoe

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.

Aortic pressure wave reflection in patients after successful aortic arch repair in early infancy

Despite the apparently successful surgical repair of aortic coarctation, subsequent cardiovascular complications have sometimes been encountered. Aortic pressure wave reflection is one of the risk factors for developing cardiovascular diseases, and an enhancement of the pressure wave reflection has been reported in patients after aortic arch repair. To clarify this issue, the increase in pressure wave reflection was evaluated in patients <15 years old who underwent aortic arch repair. This study enrolled 35 patients after aortic arch repair in early infancy. All patients underwent cardiac catheterization, and in 20 patients, there was no pressure difference within the repaired aortic arch. The aortic pressure waveforms in patients after successful aortic arch repair were recorded using a pressure sensor-mounted catheter, and the augmentation index in the ascending aorta was calculated. The augmentation index in patients after an aortic arch repair was increased compared with control subjects, although there was no pressure difference between the ascending and descending aorta (P<0.0001). The increase in the augmentation index was correlated with the patient’s age (r=0.8932, P<0.0001) and with the left ventricular posterior wall thickness (r=0.4075, P=0.0373). In patients who undergo aortic arch repair, the pressure wave reflection is accelerated, even when the aortic arch repair is ‘successful’. This increase is one of the possible causes of left ventricular hypertrophy.

Magnetic resonance imaging of myocardial infarction in kawasaki disease

We performed magnetic resonance imaging in patients with Kawasaki disease following myocardial infarction to assess the usefulness of the technique in detecting myocardial infarction and coronary artery lesions. In six patients (group A), the interval after myocardial infarction was from 7 days to 7 months, and in five patients (group B), it was from 1 to 4 years. Imaging was performed with a superconducting magnet operating at 0.5 T with spin-echo sequence and ECG-gated multiple slices of 5 mm thickness. Myocardial signals were increased in group A, and the region of high signal intensity corresponded to the site of myocardial infarction. The signal intensity within the myocardium was homogeneous in five patients in group B. Coronary arteries were visualized in 20 of 22 instances. Signals within the coronary artery were observed in all 14 instances with poor contrast runoff from the coronary aneurysm, and 11 of these vessels showed high signal intensity. In all six instances in which large aneurysms with severe stenosis were present, signals in the coronary artery were increased. In contrast, high signal intensity in the coronary artery was not observed in five of six instances with good contrast runoff. Signals in the coronary arterial cavity and high signal intensity in the coronary artery persisted in five of six instances with turbulent coronary flow. The findings of increased coronary arterial signals suggested stagnant blood flow in the coronary aneurysm. In conclusion, magnetic resonance imaging was a useful modality for assessment of myocardial infarction and coronary artery lesions in Kawasaki disease.

Aortic surgery is one of the risk factors for enhancement of pressure wave reflection in adult patients with congenital heart disease

BACKGROUND Excessive aortic pressure wave reflection is one of the risk factors of cardiovascular diseases. In some clinical course of congenital heart diseases, the elevated pressure wave reflection has been reported. The purpose of this study is to elucidate the risk factors of the enhanced pressure wave reflection in adult patients with congenital heart disease. METHODS We enrolled 99 adult (≥20 years) patients with congenital heart disease. We measured their radial pressure augmentation index and examined the relationship between it and various clinical variables. RESULTS The radial pressure augmentation index was 77.1 ± 19.1% and it had a significant correlation with the history of aorto-pulmonary shunt (t=4.194; p<0.0001), age (t=4.091; p<0.0001), height (t=-3.580; p=0.001) and the history of direct aortic surgery (t=2.253; p=0.027). Forty-four patients (44.4%) demonstrated high radial augmentation index (>1SD of age- and gender matched control) and the determinants of the elevated radial augmentation index were the history of aorto-pulmonary shunt (odds ratio, 21.32; 95% confidence interval, 5.47-83.14; p<0.0001) and the direct aortic surgery (4.18; 1.38-12.72; p=0.012). CONCLUSIONS The history of aortic surgeries enhances aortic pressure wave reflection in adult patients with congenital heart disease. The enhanced aortic pressure wave reflection is one of the risk factors for cardiovascular diseases. Therefore, the adult patients with congenital heart disease after aortic surgeries should be carefully observed and tightly controlled concerning the risk factors for cardiovascular diseases.

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.

Syndrome of Inappropriate Secretion of Antidiuretic Hormone Associated With Angiotensin-Converting Enzyme Inhibitor Administration

Angiotensin-converting enzyme inhibitors (ACEI’s) are an important medication in the treatment of congestive heart failure. However, ACEIs may cause harmful side effects, such as the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), which is a rare but important side effect. We describe here a case of SIADH associated with ACEI administration in a 6-year-old boy with restrictive cardiomyopathy. After recovery from acute exacerbation of congestive heart failure by tolvaptan administration, an ACEI (cilazapril) was started to decrease the production of angiotensin II, which upregulates serum antidiuretic hormone secretion. The patient’s heart failure symptoms worsened, including accumulation of right pleural effusion and ascites, after the initiation of ACEI administration. Cessation of ACEI administration dramatically improved his symptoms. Because it is difficult to distinguish SIADH associated with ACEI from worsening congestive heart failure, the possibility of fluid retention due to ACEI administration should always be considered when this agent is administered to patients with heart failure.

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.

Successful Selective Intra‐arterial Thrombolytic Therapy for Embolic Stroke in a Patient with Asplenia Syndrome and Unrepaired Cyanotic Congenital Heart Disease

We report successful selective local intra-arterial thrombolytic therapy for thromboembolic occlusion of right middle cerebral artery in a patient with asplenia syndrome and unrepaired cyanotic congenital heart disease.

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.