Surgical repair of tetralogy of Fallot in a 78-year-old woman: a case report.

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.

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

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.

Untreated atrial septal defect after intracardiac repair for tetralogy of Fallot.

Tetralogy of Fallot was repaired previously in a preschooler through right ventriculotomy, providing excellent exposure for ventricular septal defect closure and right ventricular outflow tract obstruction relief. Herein, we describe the preschooler as a 50-year-old man with untreated atrial septal defect found at pulmonary valve replacement in the remote period after tetralogy of Fallot repair. It was inferred intraoperatively that the previous tetralogy of Fallot was repaired only through right ventriculotomy, and atrial septal defect closure was performed together with pulmonary valve replacement.

[Cardiac Strangulation Induced by Epicardial Pacemaker Leads Causing Heart Failure;Report of a Case].

A 5-year-old girl has a history of epicardial VVI-pacemaker implantation due to congenital heart block at the age of 2 months. Five years later, she developed heart failure at the same time of battery depletion. The chest X-ray indicated the loop formation of the epicardial leads and the echocardiogram demonstrated paradoxical movement of ventricles. The 3-dimensional computed tomography finally revealed strangulation of biventricular apex caused by loop of the leads. She underwent reoperation. Cardiac strangulation was relieved by total removal of the loop and repositioning of right atrial and ventricular electrodes in a gentle curve of the leads. She was discharged and doing well. Cardiac strangulation is a rare, but it can be lethal. Therefore epicardial pacemaker leads should not be positioned around the ventricle with excessive redundancy.

Safety and effectiveness of tolvaptan for fluid management after pediatric cardiovascular surgery.

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.

Open-square technique using a novel pre-cuffed, spiral-ringed conduit for the Norwood procedure.

To avoid stenotic conduit events, 2 modifications were added to Norwood reconstruction with a right ventricle-to-pulmonary artery conduit: open-square insertion of a proximal conduit end and use of a pre-cuffed, spiral-ringed conduit. Three consecutive patients treated with this technique successfully underwent bidirectional Glenn with no stenotic events. These modifications are beneficial and safe.

Inhibition of EP4 signaling attenuates aortic aneurysm formation.

BACKGROUND: Aortic aneurysm is a common but life-threatening disease among the elderly, for which no effective medical therapy is currently available. Activation of prostaglandin E(2) (PGE(2)) is known to increase the expression of matrix metalloproteinase (MMP) and the release of inflammatory cytokines, and may thus exacerbate abdominal aortic aneurysm (AAA) formation. We hypothesized that selective blocking of PGE(2), in particular, EP4 prostanoid receptor signaling, would attenuate the development of AAA. METHODS AND FINDINGS: Immunohistochemical analysis of human AAA tissues demonstrated that EP4 expression was greater in AAA areas than that in non-diseased areas. Interestingly, EP4 expression was proportional to the degree of elastic fiber degradation. In cultured human aortic smooth muscle cells (ASMCs), PGE(2) stimulation increased EP4 protein expression (1.4 ± 0.08-fold), and EP4 stimulation with ONO-AE1-329 increased MMP-2 activity and interleukin-6 (IL-6) production (1.4 ± 0.03- and 1.7 ± 0.14-fold, respectively, P<0.05). Accordingly, we examined the effect of EP4 inhibition in an ApoE(-/-) mouse model of AAA infused with angiotensin II. Oral administration of ONO-AE3-208 (0.01-0.5 mg/kg/day), an EP4 antagonist, for 4 weeks significantly decreased the formation of AAA (45-87% reduction, P<0.05). Similarly, EP4(+/-)/ApoE(-/-) mice exhibited significantly less AAA formation than EP4(+/+)/ApoE(-/-) mice (76% reduction, P<0.01). AAA formation induced by periaortic CaCl(2) application was also reduced in EP4(+/-) mice compared with wild-type mice (73% reduction, P<0.001). Furthermore, in human AAA tissue organ cultures containing SMCs and macrophages, doses of the EP4 antagonist at 10-100 nM decreased MMP-2 activation and IL-6 production (0.6 ± 0.06- and 0.7 ± 0.06-fold, respectively, P<0.05) without increasing MMP-9 activity or MCP-1 secretion. Thus, either pharmacological or genetic EP4 inhibition attenuated AAA formation in multiple mouse and human models by lowering MMP activity and cytokine release. CONCLUSION: An EP4 antagonist that prevents the activation of MMP and thereby inhibits the degradation of aortic elastic fiber may serve as a new strategy for medical treatment of AAA.

Mechanisms of recurrent regurgitation after valve repair for prolapsed mitral valve disease.

BACKGROUND: We assessed mitral valve (MV) function using serial echocardiography as an indicator of the durability of MV repair. The aim of this study was to analyze the mechanisms of recurrent regurgitation after MV repair for degenerative disease. METHODS: From 1991 to 2007, 736 patients had valve repair for mitral regurgitation caused by leaflet prolapse: 346 patients had posterior and 390 had anterior leaflet prolapse. The mean age was 54.6±14.6 years, with 495 males. The durability and mechanisms of recurrent regurgitation were evaluated by the findings of echocardiography and reoperation. Follow-up and late echocardiography averaged 5.7±3.9 and 5.1±3.6 years, respectively. RESULTS: Survival was 91.9%±1.5% at 10 years. Freedom from reoperation and moderate or severe regurgitation at 10 years were 91.2%±1.7% and 84.5%±2.1%, respectively. Reoperations were performed for recurrent regurgitation in 29 patients, hemolysis in 5, and endocarditis in 1. Based on the findings of reoperation, the mechanisms of repair failure were procedure related in 9 (25.7%), valve related in 25 (71.4%), and unknown in 1. Late echocardiography revealed none to trivial regurgitation in 511 patients, mild in 153, moderate in 26, and severe in 40. Anterior leaflet prolapse, preoperative atrial fibrillation, and no use of annuloplasty ring were independent predictors of recurrent regurgitation. The main causes of moderate or severe regurgitation were leaflet thickening in 34 patients, leaflet prolapse in 20, dehiscence in 10, and unknown in 2. CONCLUSIONS: The main mechanism of recurrent regurgitation after MV repair is progressive degeneration that is characterized by leaflet thickening and prolapse, especially in patients with anterior leaflet prolapse.

Increased graft occlusion or string sign in composite arterial grafting for mildly stenosed target vessels.

BACKGROUND: Composite grafting is a useful technique that avoids the need for aortic manipulation and enables a wide range of target vessels to be revascularized, effectively using the limited arterial grafts available. However, it has not been clarified whether composite grafting can achieve angiographic outcomes equivalent to those obtained with individual grafting for specific target vessels. METHODS: We retrospectively reviewed 830 distal arterial graft anastomoses in 256 patients who underwent off-pump coronary artery bypass surgery and also underwent 1-year follow-up coronary angiograms. Four hundred and ten anastomoses using a composite grafting technique were compared with 420 anastomoses using individual grafting. RESULTS: In target vessels with mild stenosis, the incidence of graft occlusion or string sign was significantly higher in composite internal thoracic arteries (ITA) than in individual ITA grafts (composite 20.3% versus individual 7.3%; p = 0.018) and showed a higher tendency in composite radial arteries (RA) than in individual RA grafts (59.3% versus 36.4%, p = 0.09). In contrast, the incidence was similar between composite and individual ITA grafts (5.7% versus 3.3%, p = 0.278) and composite and individual RA grafts (11.5% versus 29.6%, p = 0.297) in target vessels with severe stenosis. CONCLUSIONS: The angiographic outcomes of composite grafts were closely related to the severity of stenosis of the target coronary artery. In target vessels with mild stenosis, composite grafting resulted in a higher incidence of graft occlusion or string sign than individual grafting did.