Ventricular Septal Defect Patch Research Articles

Ventricular septation for double inlet ventricle: Avoiding conduction injury

ObjectivesAlthough conduction location can be reliably predicted in double inlet ventricle, ventricular septation continues to carry a significant risk of complete heart block. This study describes our experience using intraoperative conduction mapping during ventricular septation. MethodsPatients undergoing ventricular septation from 2017 to 2023 were identified. Conduction mapping was performed on the open, decompressed, beating heart to identify the His bundle. Mapped His bundle locations were compared with those predicted from magnetic resonance imaging diagnoses by a senior pediatric cardiac electrophysiologist blinded to mapping results. ResultsVentricular septation was performed in 31 patients, 25 with hypoplastic right ventricles and 6 with hypoplastic left ventricles. Two-stage septation was performed in 25 patients, and single-stage septation was performed in 6 patients. Mapped conduction location was performed in the last 25 consecutive patients. Mapped conduction location was concordant with expected location in 21 of 22 patients with predictions. Complete heart block requiring a permanent pacemaker occurred in 4 patients despite successful mapping, whereas 10 patients required other reoperations, most commonly to address residual atrioventricular valve regurgitation or subpulmonary obstruction from the ventricular septal defect patch. There has been no perioperative mortality, need for single-ventricle palliation, or heart transplantation. All 13 patients who have progressed to complete septation and 18 patients with interstage circulation have acceptable hemodynamics, preserved ventricular function, and no heart failure symptoms at latest follow-up. ConclusionsVentricular septation represents an alternative to the Fontan that can be performed safely in a subset of patients with acceptable early outcomes. Conduction mapping is an adjunct strategy that may add precision to well-established rules for reliably predicting conduction location.

Clinical Course of Residual Ventricular Septal Defects After Congenital Heart Disease Repair.

The clinical course of residual ventricular septal defects after congenital heart disease repair is not completely elucidated in the medical literature. This study assessed the incidence, size, and clinical course of residual defects.This single-center retrospective study included 132 patients who survived after ventricular septal defect patch closure (n = 107) and intracardiac repair of double-outlet right ventricle (n = 16) and tetralogy of Fallot (n = 9). Residual defect was evaluated on transthoracic echocardiogram upon hospital discharge and at outpatient clinic visits.The median age at surgery was 1.2 (0.3-13.9) years. In total, 45 (34.1%) patients presented with residual defects upon hospital discharge. The residual defects were within 2mm (n = 27), 2-3mm (n = 15), and > 3mm (n = 3), and the median size was 1.5 (0.5-3.8) mm. There was no late mortality during a median follow-up of 5.4years. Among 42 residual defects measuring < 3mm upon hospital discharge, 37 (82.2%) spontaneously closed. Further, five defects decreased in size (1.8 ± 0.6mm upon hospital discharge vs1.2 ± 0.8mm at the latest visits, p = 0.15). However, the size of three residual defects measuring > 3mm upon hospital discharge increased, and two patients required re-surgery for residual defect.Significant residual defect requiring reoperation was rare. In most cases, residual defects measuring < 3mm upon hospital discharge spontaneously closed within 5years, and the size of the other defects decreased.

Pre-surgical three-dimensional transesophageal echocardiography commissural view of the right and non-coronary commissure of the patient described in Figure 6. The retracted and thickened right coronary l aflet is visualized, which is restricted in its motion both related to fusion with the left coronary leaflet, but also extension of fibrotic tissue from the ventricular septal defect patch over the proximal portion

Pre-surgical three-dimensional transesophageal echocardiography commissural view of the right and non-coronary commissure of the patient described in Figure 6. The retracted and thickened right coronary l aflet is visualized, which is restricted in its motion both related to fusion with the left coronary leaflet, but also extension of fibrotic tissue from the ventricular septal defect patch over the proximal portion

Ventricular tachycardia substrates in young patients with repaired tetralogy of fallot

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): We acknowledge the support from the Netherlands Cardiovascular Research lnitiative: An initiative with support of the Dutch Heart Foundation and Hartekind. Background Patients with repaired tetralogy of Fallot (rTOF) have a time-dependent increase in risk of ventricular tachycardia (VT) after the age of 30 years (Y), with slow conducting anatomical isthmus (SCAI) 3, located between the pulmonary annulus and the ventricular septal defect (VSD) patch, as dominant VT substrate (1-4). However, SCAI may be already present before 30Y. Purpose To assess (1) the prevalence of SCAI, (2) the associated factors in SCAI development, and (3) the occurrence of clinical VT, in rTOF patients &amp;lt;30Y. Methods From a database of 146 patients with rTOF or related lesions who underwent electroanatomical mapping (EAM) and programmed electrical stimulation (PES) between 2007 and 2022 for treatment of ventricular arrhythmias (VA), risk stratification or before pulmonary valve replacement (PVR), those aged &amp;lt;30Y at time of procedure were selected and analysed. Results Of 146 rTOF patients, 55 (38%) were &amp;lt;30Y (median 16Y [IQR 14-22]); 33/55 (60%) had TOF, 9/55 (16%) TOF with double outlet right ventricle (DORV), and 13/55 (24%) complex TOF variants, including 8 patients with pulmonary atresia with VSD, 2 patients with absent pulmonary valve, and 3 with other variations. Initial intracardiac surgical repair (median age 0.8Y [IQR 0.4-1.4]) was performed via a ventriculotomy in 10/55 (18%) after palliative shunts in 16/55 (29%). An RV to pulmonary artery (RV-PA) conduit was inserted during initial or early after (&amp;lt;1Y) initial repair in 12/55 (22%). In 14/55 (25%) pulmonary valve replacement (PVR) was performed later in life (median age 10.1Y [IQR 2.7-14.3]). Five of 55 (9%) underwent EAM/PES after a spontaneous VA, 40/55 (73%) before PVR, and 10/55 (18%) for risk stratification. Monomorphic VT was inducible in 8/55 (15%), including 4/5 patients with spontaneous VT, and was proven related to a SCAI 3 in 7/8 (88%). In 16/55 (29%) SCAI 3 could be identified; no other SCAI (1,2,4) was present. Of note, 9/16 (56%) of patients with SCAI 3 had initial RV-PA conduit repair/early PVR, in comparison with only 3/39 (8%) of patients without SCAI. In univariable analysis complex TOF (OR 6.8 [95% CI 1.8-26.4]), ventriculotomy (OR 5.3 [95% CI 1.2-22.3]), any prior PVR (OR 4.8 [95% CI 1.4-16.7]), and initial RV-PA conduit/early PVR (OR 15.4 [95% CI 3.3-71.8]) were significantly associated with SCAI 3. In multivariable analysis initial RV-PA conduit/early PVR remained the only independent predictor of SCAI 3 (OR 13.0 [95% CI 1.2-142.4]). Conclusions A SCAI 3 is present in 29% of rTOF patients &amp;lt;30Y referred for EAM/PES and the dominant VT substrate for spontaneous and inducible VTs. RV-PA conduit placement during initial repair or early PVR (&amp;lt;1Y after repair) is associated with SCAI 3 in young rTOF. Whether the early conduit/valve placement causes scarring at the infundibulum with consecutive slow conduction and/or whether the complex variants contribute to SCAI requires additional studies.

Systematic Electrophysiological Study Prior to Pulmonary Valve Replacement in Tetralogy of Fallot: A Prospective Multicenter Study.

Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes. A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021. A standardized stimulation protocol was used across all centers. A total of 120 patients were enrolled, mean age 39.2±14.5 years, 53.3% males. Sustained ventricular tachycardia was induced in 27 (22.5%) patients. When identifiable, the critical isthmus most commonly implicated (ie, in 90.0%) was between the ventricular septal defect patch and pulmonary annulus. Factors independently associated with inducible ventricular tachycardia were history of atrial arrhythmia (OR, 8.56 [95% CI, 2.43-34.73]) and pulmonary annulus diameter >26 mm (OR, 5.05 [95% CI, 1.47-21.69]). The EPS led to a substantial change in management in 23 (19.2%) cases: 18 (15.0%) had catheter ablation, 3 (2.5%) surgical cryoablation during PVR, and 9 (7.5%) defibrillator implantation. Repeat EPS 5.1 (4.8-6.2) months after PVR was negative in 8 of 9 (88.9%) patients. No patient experienced a sustained ventricular arrhythmia during 13 (6.1-20.1) months of follow-up. Systematically performing programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR yields a high rate of inducible ventricular tachycardia and carries the potential to alter management. It remains to be determined whether a standardized treatment approach based on the results of EPS will translate into improved outcomes. URL: https://www. gov; Unique identifier: NCT04205461.

Severe form of Ebstein’s anomaly associated with ventricular septal defect

Although the ventricular septal defect (VSD) that coexists with the severe form of Ebstein’s anomaly was thought to be beneficial for hemodynamics, it is unclear whether biventricular repair is always possible. Ebstein’s anomaly with VSD was diagnosed at 4 days of age based on a heart murmur and mild cyanosis. The cardiothoracic ratio was 78% and the Celermajer index was 1.6 (grade 4), but the hemodynamics were stable and tricuspid valve regurgitation was less than mild. Pulmonary overcirculation developed, and therefore, biventricular repair was attempted at 4 months of age, consisting of VSD patch closure, atrial septal defect partial closure, and the cone reconstruction of the tricuspid valve. Due to persistent cyanosis, however, nitric oxide inhalation and high flow nasal oxygen inhalation could not be discontinued. Catheter examination showed a cardiac index of 2.1 L/min/m2, moderate tricuspid regurgitation, and a pulmonary-to-systemic blood flow ratio of 0.64, and thus, takedown to one and one-half ventricular circulation using bidirectional superior cavopulmonary anastomosis, atrial communication closure, and right pulmonary artery banding was performed. The patient was discharged on postoperative day 25. Four months after discharge, the patient is doing well with home oxygen therapy.

Realignment of the ventricular septum in tetralogy of Fallot using (partial) direct closure of the ventricular septal defect: Long-term follow-up and comparison to conventional patch repair.

Aortic dilatation and regurgitation after surgical repair of tetralogy of Fallot (TOF) is known, and beside other factors, mainly addressed to an intrinsic aortopathy. In 2011, we reported the influence of realingement of the left ventricular outflow tract (LVOT) by (partial) direct closure of the ventricular septal defect (VSD) in TOF on aortic structures and function. We now evaluated the further follow-up of this cohort and compared the results to a matched group of TOF patients with classical VSD patch closure. Forty patients with TOF treated between 2003 and 2008 are included in the study, with 20 patients each in the VSD (a) (partial) direct closure and (b) patch closure group. Follow-up time after surgery was 12.3 years (11.3-13.0). Patient characteristics, echocardiographic measurements, and surgical and intensive care unit parameters were not significantly different between both groups. After surgery and during long-term follow-up, realignement of the LVOT, shown by the angle between the interventricular septum and the anterior aortic annulus in long axis view in echocardiography, was lower in Group A (34 vs. 45°, P < 0.0001). No differences in LVOT or aortic annulus size, aortic regurgitation, or dilation of the ascending aorta and right ventricular outflow tract gradients were found. Transient rhythm disturbances were found in 3 patients in each group, with only one persistent complete atrioventricular block in Group B. (Partial) direct closure of the VSD in TOF leads to a better realignement of the LVOT and showed comparable short- and long-term results without higher risk for rhythm disturbances during follow-up.

Feasibility analyses of virtual models and 3D printing for surgical simulation of the double-outlet right ventricle.

Accurate diagnosis and surgical selection of the double-outlet right ventricle (DORV) is both critical and difficult. Virtual models and three-dimensional (3D) printing have been used to provide morphological copies to doctors as reference. However, the existing methods have shortcomings in visualization of the surgical results, optimal surgical design, and accurate surgical scheme measurements. To overcome this problem, we performed surgical predictions by designing the intraventricular baffle and ventricular septal defect patch to evaluate surgical options and using 3D printing to guide the trimming of the baffle or patch. A complete set of processes including scanning, modeling, designing, 3D printing, and guiding the trimming of the baffle for the diagnosis and surgical planning of DORV was established. Six cases were used to evaluate the feasibility of this method. The average rate of misdiagnosis of the six cases by computed tomography and echocardiography was 42.5%, which was reduced to 4.6% when the diagnosis was established using the virtual models and 3D printing as auxiliary tools. The approach effectively improved diagnostic accuracy, guided the operation, and simplified the process of patch trimming. The proposed method can thus be used for improving the surgical simulation and guiding of the DORV surgery.

Triple-valve replacement for Rhizobium radiobacter endocarditis with septic shock in an adult with ventricular septal defect. A case report.

Introduction and importanceTriple-valve replacement in active infective endocarditis has rarely been reported. This paper is the first report of a triple-valve replacement performed in endocarditis with septic shock and the first presentation of multivalvular endocarditis due to Rhizobium radiobacter.Case presentationA 26-year-old patient with a neglected ventricular septal defect referred to us in septic shock, with multiple organ failure, severe biventricular dysfunction, and pulmonary hypertension, due to Rhizobium radiobacter infective endocarditis affecting the aortic, tricuspid and pulmonary valves. Initially, he was deemed unfit for surgery. However, after clinical stabilization, triple-valve replacement, aortic annular abscess repair, membranous septum aneurysm resection, and ventricular septal defect patch closure were performed. The postoperative evolution was good; both ventricles showed functional recovery after six months.Clinical discussionAlthough surgery provides the best chances of survival in endocarditis with septic shock, reportedly, most cases are considered inoperable. Clinical stabilization under intensive care using specific therapies to manage septic shock, myocardial dysfunction, and pulmonary hypertension was crucial for surgery success. Custodiol® cardioplegia, and replacement of the right-sided valves using a beating-heart technique were used to reduce the myocardial ischemic time.ConclusionRhizobium radiobacter, an opportunistic gram-negative bacterium, potentially may cause multiple valve endocarditis. Patients with endocarditis and septic shock initially considered inoperable can still benefit from surgery after tenacious intensive care (cytokine hemoadsorption and levosimendan are helpful in this process). In complex multivalvular procedures, a beating heart technique to replace the right-sided valves should be considered to minimize the duration of myocardial ischemia.

Non-invasive identification of slow conducting anatomical isthmuses in patients with tetralogy of Fallot by 3D late gadolinium enhancement cardiovascular magnetic resonance

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with repaired tetralogy of Fallot (rTOF) remain at risk of sudden cardiac death due to reentrant sustained monomorphic ventricular tachycardia (SMVT). Slow conducting anatomical isthmuses (SCAI), in particular SCAI3 at the outlet septum, bordered by the pulmonary annulus and the ventricular septal defect patch, are the dominant substrate for SMVT. Electroanatomical mapping (EAM) is the invasive gold standard to identify SCAIs, and transection of SCAI by catheter ablation has been correlated with favorable long-term outcome. Non-invasive identification of SCAI for risk stratification and treatment planning is needed but has not been established yet. Three-dimensional (3D) late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) facilitates accurate visualization of morphologically complex hearts with high-spatial resolution. Objective The study thought to determine whether 3D LGE-CMR can identify SCAIs. Methods Consecutive patients with rTOF who underwent right ventricular (RV) EAM and 3D LGE-CMR were included. LGE-CMR-derived 3D RV reconstructions were created (ADAS-3D) and merged with 3D RV EAM data. Mapping points were superimposed on the CMR-derived 3D reconstruction allowing for direct comparison of EAM data and local signal intensity (SI). The optimal SI cut-off to identify low bipolar voltage (LBV, BV&amp;lt;1.76mV) was determined by receiver operating characteristic carve. An abnormal AI on LGE-CMR was defined as AI with continuous SI above the obtained cut-off connecting AI borders. Results Forty-eight rTOF patients (34±16 years, 58% male) were included. At EAM, 21 patients had normal AI, and 20 and 7 had a SCAI (&amp;lt;0.5m/s) or blocked AI, which was AI3 in all. Patients with SCAI showed low BV of AI3 (median 0.7 [range 0.25-2.59] mV). In 11 patients, 14 SMVTs could be induced, all related to SCAI3. A total of 9240 points were analyzed, showing a significant correlation between BV and SI (R=0.4, P&amp;lt;0.001). The optimal SI cut-off to identify LBV was 42% of the maximal SI (MSI) (AUC 0.80; sensitivity, 74%; specificity, 78%). Using this cut-off of MSI, a SCAI or blocked AI3 could be correctly identified by LGE-CMR in all 27 patients, and a normal AI3 could be correctly confirmed by LGE-CMR in 14/21 patients with normal EAM findings (Figure). The sensitivity and specificity of 3D LGE-CMR for identifying SCAI or blocked AI3 were 100% and 67%, respectively. Of note, among patients with normal EAM findings, those with abnormal AI3 on LGE-CMR had significantly lower BV of AI3 than those with normal AI3 on LGE-CMR (2.06 [Range, 1.62-2.60] vs. 3.53 [2.22-5.67] mV, P&amp;lt;0.01). Conclusion 3D LGE-CMR can identify SCAI with 100% sensitivity and may identify diseased AI3 even before critical conduction delay occurs. This technique may allow for non-invasive risk stratification of VT and can refine patient selection for invasive EAM.

PE-565-03 NON-INVASIVE IDENTIFICATION OF SLOW CONDUCTING ANATOMICAL ISTHMUSES IN PATIENTS WITH TETRALOGY OF FALLOT BY 3-DIMENSIONAL LATE GADOLINIUM ENHANCEMENT CARDIOVASCULAR MAGNETIC RESONANCE IMAGING

Patients with repaired tetralogy of Fallot (rTOF) remain at risk of sudden cardiac death due to sustained monomorphic ventricular tachycardia (SMVT). The majority of SMVTs are related to slow conducting anatomical isthmuses (SCAI) in particular to SCAI3 at the outlet septum, bordered by the pulmonary annulus and the ventricular septal defect patch. Electroanatomical mapping (EAM) is the invasive gold standard to identify SCAIs; non-invasive characterization of AIs has not been established.

Iatrogenic ventricular septal defect during right ventricular assist device insertion in congenitally corrected transposition of the great arteries

This case report describes the management of a large iatrogenic ventricular septal defect (VSD) created by the coring device during systemic ventricular assist device (right ventricular assist device [RVAD]) insertion in a 16-year-old patient with congenitally corrected transposition of the great arteries. The VSD was closed by a bovine pericardial patch and the ventriculotomy was extended laterally to relocate the VAD sewing ring. After RVAD implantation, the patient initially remained cyanotic, potentially due to a tiny VSD patch leak with right-to-left shunting. Hypoxia was successfully corrected by rescue nitric oxide infusion and the patient was bridged to transplant after 91 days.

Pseudoaneurysm formation between ascending aorta and main pulmonary artery following ventricular septal defect patch dehiscence

Complications of ventricular septal defect (VSD) repair include arrhythmias, tricuspid valve insufficiency, and residual shunt. We are presenting a unique and extremely rare complication of rupture of VSD patch, leading to the formation of pseudoaneurysm between the ascending aorta and main pulmonary artery following VSD patch closure.

Sildenafil’s Early, Late Impact on Ventricular Septal Repair: Older Children Using the Double Patch

BackgroundDelayed diagnosis in children with a ventricular septal defect (VSD) is common in low- and middle-income countries. Consequently, these children present with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). The study investigators introduced the double-flap valve VSD patch closure technique (DFV) in 1996 to reduce early postoperative risk. Long-term results are presented in this report. MethodsThis was a retrospective single-institution study of patients who underwent DFV between May 1996 and July 2015. Beginning in 2005, all candidates for DFV received sildenafil preoperatively and postoperatively. Preoperative catheterization data and operative, postoperative, hospital, and follow-up data were analyzed. ResultsA total of 40 patients underwent the DFV procedure. Patients’ demographics were comparable between the sildenafil and nonsildenafil groups. One of 39 patients (2.6%) was lost to follow-up. Early mortality was 2.5% (1 of 40), and late mortality was 2.6% (1 of 38). Sildenafil improved preoperative oxygen saturation, improved preoperative hemodynamics, and shortened postoperative ventilation time. In both groups, abnormal hemodynamic values improved with a 100% oxygen challenge. The median age at late follow-up was 26.3 years (interquartile range [25%, 75%], 20.9, 29.9 years), and the median time since operation was 19.2 years (interquartile range, 11.4, 22.7 years). Current discharge survival was 97.3%. A total of 18% of patients had severe PAH in late follow-up. Multivariate analysis revealed only a baseline PVR-to–systemic vascular resistance ratio of 0.8 or greater as a significant predictor of late severe PAH. ConclusionsLong-term follow-up demonstrated that 60% of the patients will achieve normal or nearly normal pulmonary artery pressures. Furthermore, the study demonstrated that sildenafil improves preoperative hemodynamics and postoperative management. Children with VSD, elevated PVR, and PAH should not be denied operation.

Hybrid Transthoracic Periventricular Device Closure of Ventricular Septal Defects: Single- Center Experience.

ObjectiveTo evaluate the efficacy of hybrid transthoracic periventricular device closure of ventricular septal defects (VSDs) in a single center.MethodsAll patients who underwent hybrid transthoracic periventricular device closure of VSDs between January 2018 and December 2019 were retrospectively analyzed. The preoperative, operative and postoperative findings and clinical follow-ups were reviewed.ResultsA total of 59 patients underwent the procedure. Transesophageal echocardiographic guidance was used in all procedures. The procedure was successful in 57 procedures (97%). The procedures of two patients were changed to open-heart surgery during the same intervention due to severe aortic insufficiency (the device was not deployed) and significant residual shunt after device deployment. One major complication (1.7%) was observed after the procedure. The patient’s device was dislodged within 12 hours after the procedure, and this patient underwent device extraction and VSD patch closure due to significant residual shunt. Eight (14%) minor complications were observed after the procedure, and three of them persisted during follow-up. Three of these eight complications were incomplete right bundle branch block, one of which resolved during follow-up; two were mild residual shunts, one of which resolved during follow-up; two were mild new-onset tricuspid valve insufficiencies; and one was mild new-onset mitral valve insufficiency; all valvular insufficiencies were resolved during follow-up.ConclusionsHybrid transthoracic periventricular device closure of VSD seems to be a good alternative approach due to its procedural success and low risk rates. The best advantage of the procedure is the possibility of switching to open-heart surgery, if necessary.

Long-Term Follow-Up of Pericardium for the Ventricular Component in Atrioventricular Septal Defect Repair.

Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material. Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019. A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death <30 days occurred in two (3%) patients. Reinterventions occurred in eight patients (early [within 30 days] in two, early and late in one, and late in five), all in the autologous pericardium group. Log-rank tests showed no significant difference in mortality (P = .892), LAVV reinterventions (P = .228), or LAVV regurgitation (P = .770). In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.

P263 Paradoxical embolus causing myocardial infarction in a young adult with congenital heart disease

Abstract Case Report A 17 year old girl presented to her local hospital 24 hours after the onset of severe central chest pain that lasted for 30 minutes and spontaneously resolved. Her congenital heart disease background was of Ebstein’s anomaly of the tricuspid valve, coarctation of the aorta and ventricular septal defect (VSD). She underwent surgical repair of the coarctation and PA band at two months of age, followed by VSD closure when she was a year old.. An atrial communication was deliberately left open at the time of this operation and she had a known residual VSD and mild tricuspid regurgitation. Her resting saturations were 97% in air . On admission, she had a significantly raised Troponin T of 24,000ng/L with no significant ST segment elevation. An echocardiogram demonstrated new left ventricular (LV) systolic impairment with an ejection fraction of 33%. She underwent a ‘triple rule out’ cardiac CT scan and subsequently a cardiac MRI. On retrospective review, the CT demonstrated a sizeable atrial communication with left to right shunt, focal tight mid left anterior descending (LAD) artery stenosis aftersecond diagonal branch with mid to apical anterior and anteroseptal hypoperfusion. The MRI demonstrated extensive full thickness late gadolinium enhancement in mid to apical septum extending to the apical cap and apical inferior segment with microvascular obstruction (MVO) in the mid septum with possible VSD patch breakdown. No thrombi was notes on the gadolinium enhancement images. She was transferred to our hospital and underwent an invasive coronary angiogram that demonstrated recanalization of her coronary artery. She remained haemodynamically well throughout the admission. Anticoagulation with a direct acting oral anticoagulant (DOAC) was started alongside ACE-I and Beta blocker therapy. Discharge transthoracic echocardiogram showed flow across the atrial septum, a small residual VSD and mild LV dysfunction with akinesia of mid-to-distal LAD territory and very good function of remaining LV segments. She was discharged home with a plan for elective outpatient admission for a closure of her interatrial communication in a few months once she has recovered. Figure 1 summarises the key aspects of the multimodality imaging in this patient. Discussion Coronary embolus is the underlying cause of 3% of acute coronary syndromes with a small proportion being from a paradoxical embolus from the venous circulation through a right to left shunt across an atrial communication. Ebstein’s anomaly is a rare disorder, accounting for &amp;lt;1% of all congenital heart defects. Right-to-left shunt across the atrial communication is common in this condition due to RA dilatation and increased RA pressures secondary to tricuspid regurgitation. Our case illustrates the importance of having a high index of suspicion in patients with congenital heart disease for this rare complication of paradoxical coronary embolus, especially given the significant morbidity. Abstract P263 Figure 1

Conduction system disorders after surgical patch closure of ventricular septal defects; a comparison between two suturing techniques

Background: Ventricular septal defect (VSD) is the most common congenital heart disease, and conduction disorder is one of the frequent complications after VSD closure. Suturing technique used for VSD closure may affect the occurrence of this complication. The aim of this study was to compare the outcome of VSD surgical patch closure using continuous versus interrupted suture techniques.&#x0D; Methods: The study included 150 VSD patients who had surgical patch closure between December 2014 and March 2017. They were subclassified into two groups according to the suture technique; continuous suture technique (n= 75) and interrupted suture technique (n= 75). Preoperative, operative, and postoperative variables were reviewed and analyzed. The postoperative rhythm was recorded using contіnuous electrocardiogram during intensive care unit stay. 12-leads electrocardiogram and echocardiography were performed immediately after surgery and repeated before discharge and after 3, 6, 12 months then yearly. The follow-up period ranged from 1 to 3.25 years (2.04 ± 0.84 years).&#x0D; Results: Aortіc cross-clamp tіme was longer in the interrupted technique group (51.40±15.21 vs. 42.32±13.86 minutes; p &lt;0.01). 7 (9.3%) patients in the continuous technique group had an atrioventricular block during ICU stay, and 2 (2.7 %) had complete heart block. However, one patient (1.3%) in the interrupted technique group had an atrioventricular block, and no patient had complete heart block (p=0.006). Incidence of conduction defects during follow-up was insignificantly different between the groups. There was no significant difference in the postoperative complications between the groups.&#x0D; Conclusions: Complete heart block is an infrequent complication after VSD patch closure. The interrupted suture technique was associated with a lower incidence of conduction defects during the early postoperative period

Successful Ablation of Ventricular Tachycardia in Repaired Tetralogy of Fallot via Transjugular and Subclavian Approach.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Surgical repair with ventricular septal defect (VSD) patch and right ventricular outflow tract (RVOT) reconstruction is the only curative therapy. The population of adults with repaired Tetralogy of Fallot (rTOF) is growing due to the improvement of surgical technique. As a consequence, the increase burden of age-dependent ventricular arrhythmias (VAs) in patients with rTOF becomes an important reason for late morbidity and mortality. Notably, the vast majority of VAs in patients with rTOF are monomorphic ventricular tachycardias (VTs).1 Given the better understanding of the potential 4 anatomic isthmuses for VT circuits,2 catheter ablation has been implemented to be an effective and curative strategy to manage VTs in patients with rTOF, while clinical approaches are frequently achieved using femoral vein. However, clinical hurdle persisted once if patients with rTOF had limited vascular access. Here, we reported a challenging case with difficult vascular access owing to multiple occluded thrombosis and successful ablation of multiple monomorphic VT was performed using superior approach guided by merged intracardiac echocardiography (ICE) and reconstructed cardiac computed tomography (CT) image reconstruction in a case of rTOF.

Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease.

Background:Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.Methods:The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis.Results:Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m2 or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0–100%), 98.4% (95% CI: 96.0–100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively.Conclusions:Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m2 and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.