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Background: Tetralogy of Fallot is a congenital heart disease mostly diagnosed and treated in early childhood. However, there are some adult cases receiving treatment. Case presentation: We describe a 78-year-old Japanese woman who presented with severely hypertrophic right ventricle, ventricular septum defect, overriding aorta, and severe infundibular stenosis in the right ventricular outflow tract. As hypoxemia was mild and daily exertion was sufficiently possible, home oxygen therapy was introduced. After 1 month, she was referred because of a positive blood culture. The blood culture test was positive four times, therefore, the antibacterial drug was administered according to active infective endocarditis. SpO2 repeatedly decreased during hospitalization, thus oxygen was needed. As there were infective endocarditis onset and progressive hypoxemia, we planned a surgical correction. Conclusion: Tetralogy of Fallot was diagnosed and successfully treated with complete surgical correction, and the development of infective endocarditis was the definitive indication for surgery at this late age. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67–1.06) than in the univentricular-repair group (median 1.79, IQR 1.53–2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction. [ABSTRACT FROM AUTHOR]

Background: As double-outlet right ventricle has a wide pathophysiology spectrum, its comprehensive treatment strategy is determined based on relevant factors, such as the location and size of the ventricular septal defect, ventricular volume, and relationship of the great arteries. However, for borderline double-outlet right ventricle cases, it is occasionally difficult to decide the treatment strategy preoperatively. Recently, advances in 3D printing technology based on computed tomography have enabled the creation of 3D heart models of congenital heart disease that can precisely reproduce the anatomical structure of each patient even for complex anomalies. Herein, we describe a young patient in whom univentricular repair could be decided after confirming the 3D heart model and intracardiac structure under direct vision. Case presentation: We describe a 3-year-old girl who was diagnosed with double-outlet right ventricle and severe pulmonary valve stenosis at birth and who underwent a left modified Blalock–Taussig shunt at 2 years of age. Preoperative examination revealed a borderline condition for biventricular repair characterized by a small left ventricle volume and side-by-side relationship of the great artery. After a preoperative discussion using a 3D heart model, we concurred that an intraoperative assessment would be made as to whether biventricular repair was possible or not. After confirming the intracardiac structure under direct vision, we assessed that intraventricular rerouting was not possible owing to the high risk of subvalvular aortic stenosis as there was no tissue that could be incised between the right ventricular free wall and the primary interventricular foramen, as indicated in the 3D heart model. Thus, atrial septostomy and Glenn anastomosis were performed. Conclusions: We report a 3-year-old girl with a borderline double-outlet right ventricle in whom a univentricular repair strategy could be decided after confirming the 3D heart model and intracardiac structure under direct vision. A 3D-printed heart model can be useful in patients whose repair strategy is difficult to judge for the borderline double-outlet right ventricle. [ABSTRACT FROM AUTHOR]

Background: Postoperative fluid management is important after open heart surgery, because cardiopulmonary bypass evokes an abnormal inflammatory response and increases vascular permeability, especially in pediatric patients. We assessed the safety and effectiveness of tolvaptan for management of postoperative fluid retention after congenital heart surgery. Methods and results: This retrospective study analyzed data from 43 children with uncomplicated congenital heart disease who underwent open heart surgery between September 2013 and August 2016. The patients were divided into two groups. Group N ( n = 18; September 2013 through May 2014) received the conventional oral diuretics alone, and Group T ( n = 25; June 2014 through August 2016) received a single dose of tolvaptan (0.45 mg/kg) in addition to the conventional oral diuretic therapy. Data were collected, while patients who received intensive care were assessed and compared between groups. Add-on tolvaptan use was associated with increased urinary output standardized by body weight (54.3 ± 4.5 vs 47.3 ± 19.1 mL/kg; p = 0.043), decreased additional intravenous diuretic dose standardized by body weight (0.26 ± 0.23 vs 0.62 ± 0.48 mg/kg; p = 0.001), and a smaller decrease in central venous pressure (1.3 ± 2.7 vs 1.9 ± 3.8 mmHg; p = 0.019). Laboratory analysis showed that electrolyte concentrations in blood and urine did not significantly differ between groups. Conclusions: Tolvaptan appears to be effective and safe for management of postoperative fluid retention after congenital heart surgery. [ABSTRACT FROM AUTHOR]

A 38-year-old woman underwent atriopulmonary Fontan surgery at age 18 years and subsequently successfully delivered a girl by cesarean section at age 34. Her condition later deteriorated due to atrial tachyarrhythmia and progressed to New York Heart Association (NYHA) class IV heart failure. Her treatment, at age 36, comprised total cavopulmonary connection conversion, direct right atrial ablation with bipolar radiofrequency devices, the creation of an atrial septal defect, and placement of a dual-chamber permanent pacemaker. Three years after the conversion, her condition has improved to NYHA class I. [ABSTRACT FROM AUTHOR]