Early vascular aging in adult patients with congenital heart disease.

Many studies have reported that adults with congenital heart disease suffer from hypertension and cardiovascular disease even at younger ages. Therefore, we analyzed early vascular aging, which is defined as pulse wave velocity values higher than the 95th percentile for age and sex, and clarified the relationship between early vascular aging and many parameters related to cardiovascular disorders in adults with congenital heart disease. We enrolled 72 adult patients with congenital heart disease and measured their brachial-ankle pulse wave velocity.

Employment status and contributing factors among adults with congenital heart disease in Japan.

Background: Working is an important concern in transitional care for adults with congenital heart disease (ACHD) because work connects people with society. Employment status is correlated with gender, but studies on patient employment by gender have rarely been conducted. This study therefore aimed to examine the employment status of ACHD by gender and to explore the factors influencing this status.

Relationship between non-osmotic arginine vasopressin secretion and hemoglobin A1c levels in adult patients with congenital heart disease.

Arginine vasopressin (AVP), which induces vasoconstriction and conserves solute-free water when released during high plasma osmolality, is secreted through 2 mechanisms: osmoregulation and baroregulation. This study aims to clarify the mechanisms and influencing factors for non-osmotic AVP secretion in adult patients with congenital heart disease (CHD). AVP levels were measured in 74 adults with CHD.

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.

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.

The cardiac blood supply-workload balance in children.

It is well known that the reflected pressure wave in small children returns earlier than that in adolescent. The reason of early return of the reflected pressure wave in infancy is their height. The short distance between heart and reflection point makes the reflected pressure wave returning to the heart earlier.

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.

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.

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.

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.

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.

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%).

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.

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.

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.