Pregnancy and Delivery in Patients With Repaired Congenital Heart Disease　- A Retrospective Japanese Multicenter Study.

BACKGROUND: Although advances in cardiac surgery have led to an increased number of survivors with congenital heart disease (CHD), epidemiological data regarding the pregnancies and deliveries of patients with repaired CHD are scarce.Methods and Results:In this study, we retrospectively reviewed the clinical outcomes of pregnancies and deliveries of women with repaired CHD. Overall, 131 women with repaired CHD were enrolled and there were 269 gestations. All patients were classified as New York Heart Association (NYHA) Class I or II. The prevalence of cesarean sections was higher in patients with (CyCHD) than without (AcyCHD) a past history of cyanosis (51% vs. 19%, respectively; P<0.01). There were 228 offspring from 269 gestations and the most prevalent neonatal complication was premature birth (10%), which was more frequent in the CyCHD than AcyCHD group (15.7% vs. 5.6%, respectively; P<0.01). Five maternal cardiac complications during delivery were observed only in the CyCHD group (8%); these were classified as NYHA Class II and none was fatal. CONCLUSIONS: Delivery was successful in most women with repaired CHD who were classified as NYHA Class I or II, although some with CyCHD and NYHA Class II required more attention. Cesarean sections were more common in the CyCHD than AcyCHD group, and CyCHD may be a potential risk for preterm deliveries.

Preferences Regarding Transfer of Patients With Congenital Heart Disease Who Attend Children's Hospital.

BACKGROUND: The aim of this study was to determine preferences regarding transfer of patients with congenital heart disease (CHD) attending a children's hospital in Japan and related factors. Methods and Results: We conducted a self-administered questionnaire survey with CHD patients >15 years of age treated at the pediatric cardiology outpatient clinic of a children's hospital. Logistic regression analysis was used to identify factors related to patient preferences regarding the transfer. One hundred and eleven of the 122 patients given a questionnaire provided valid responses (valid response rate, 91.0%). Sixty-six subjects (64.9%) reported "not being told anything specific" by their physicians about the transfer from the children's hospital, and 72 (59.5%) stated that they "wished to continue attending the children's hospital". Visiting outpatient clinic with parents (OR, 11.00; 95% CI: 2.01-60.97), having low uncertainty about continuing to attend the children's hospital (OR, 0.95; 95% CI: 0.92-0.98), and having high uncertainty about leaving the current physician (OR, 1.04; 95% CI: 1.01-1.07) were significantly related to the patient's wish to continue to attend the children's hospital. CONCLUSIONS: There is a need to improve patient education regarding the opportunities for transfer, and to develop a systematic transition program for children's hospitals and aligned specialized adult CHD centers.

[Spontaneous Closure of Atrial Septal Defect Caused Severe Heart Failure in a Child with Pulmonary Atresia and Intact Ventricular Septum Having an Extracardiac Total Cavopulmonary Connection;Report of a Case].

Unobstructed atrial communication is a prerequisite for favorable pulmonary circulation in patients with single-ventricle physiology and atrial-communication-dependent pulmonary venous drainage. It might also be a prerequisite for favorable coronary circulation in patients with single-ventricle physiology and atrial-communication-dependent coronary venous drainage. Obstruction of atrial communication, on which coronary venous drainage depends, has not yet been documented. We report a patient with pulmonary atresia and an intact ventricular septum having an extracardiac total cavopulmonary connection. Spontaneous closure of the atrial septal defect( ASD), measuring 10 mm before the total cavopulmonary connection, led to a leftward ventricular septal shift and left ventricular wall thickening, causing severe left ventricular diastolic dysfunction which resolved after surgical ASD creation. Detection of spontaneous ASD closure by ultrasound was challenging because the to-and-fro blood flow in the dilated coronary sinus mimicked a patent ASD. Prophylactic ASD enlargement accompanying the cavopulmonary connection procedure is advised in such patients.

Acute progressive mitral regurgitation resulting from chordal rupture in infants.

This report describes six previously healthy infants with acute mitral valve chordal rupture. Although their initial symptoms were mild respiratory distress and fever, all the infants presented with acute progressive left heart failure and required urgent surgical repair. Mitral valve annuloplasty and mitral valve replacement were performed (for three patients each). Two patients died, both of whom had cardiac arrest and required resuscitation before surgery. Histologic examination of excised mitral valve leaflets showed infiltration of gram-positive bacteria in these two patients. All the patients were 4-6 months old, which may suggest underlying congenital factors. The authors believe early diagnosis and surgery are crucial as a life-saving measure for infants with acute mitral valve chordal rupture.

Paclitaxel-coated drug-eluting balloon for pulmonary vein stenosis after repair of total anomalous pulmonary venous return with asplenia.

We used a paclitaxel-coated drug-eluting balloon (DEB) for pulmonary vein stenosis (PVS) after repair of total anomalous pulmonary venous return with asplenia. No adverse effect of paclitaxel was revealed and plasma concentration of paclitaxel was not elevated after intervention. Although progression of PVS slowed after dilatation with the DEB, stenosis recurred relentlessly, resulting in patient demise. This strategy may be safe for infantile cases, but requires further effective strategy, such as usage of larger sizes of DEBs. .

Ascending aortic approach for balloon aortic valvuloplasty with concomitant bilateral pulmonary artery banding in a very low-birth-weight neonate with critical aortic stenosis and poor left ventricular function.

An 1186-g infant was born at 27 weeks' gestation. Echocardiography showed critical aortic stenosis, a dilated left ventricle (left ventricular end-diastolic diameter, 16.9 mm), and poor left ventricular function (left ventricular ejection fraction, 8.5%). Due to duct-dependent systemic circulation, the patient underwent a hybrid intervention consisting of bilateral pulmonary artery banding (PAB) and balloon aortic valvuloplasty (BAV) via the ascending aorta on day 2. A stainless steel tip and a 6-French introducer were combined to create an access device. The access device was inserted into the ascending aorta, through which balloon catheters were manipulated. On day 4, echocardiography showed a left ventricular ejection fraction of 48% and dominant antegrade blood flow in the aortic arch. The pulmonary artery bands were removed and the ductus was clipped on day 8 to establish in-line circulation. After re-balloon aortic valvuloplasty for restenosis, the patient was discharged from the hospital at 7 months of age. The clinical implications of this case are: the ascending aortic approach is feasible for BAV in low-birth-weight neonates; and bilateral PAB performed concomitantly with BAV may be efficient for neonates with critical aortic stenosis and poor left ventricular function.

Complete atrioventricular block as a complication of varicella in children.

Although varicella is a benign and self-limited disease in children, serious complications can occur. We herein report a case of a 15-month-old boy who required a permanent pacemaker because of complete atrioventricular block as a complication of varicella. Universal vaccination is warranted to prevent such a potentially fatal complication in Japan where varicella is still endemic.