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Background: Both percutaneous and perventricular device closures of perimembranous ventricular septal defects (Pm-VSDs) are alternatives to surgical procedures，but they all present certain drawbacks., Objective: To report our clinical experiences and midterm follow-up results of minimally invasive peratrial device closure of Pm-VSDs under the guidance of transesophageal echocardiography(TEE) in patients <12 months of age., Methods: Between January 2015 and December 2020，268 patients <12 months of age with Pm-VSDs underwent peratrial device closure in our institute. The procedure was performed under TEE guidance via a small right subaxillary incision. The delivery pathways is established by manipulating the hollow probe, and then the device is installed., Results: A total of 263 cases (98.1%) underwent successful closure, whereas five cases failed and were converted to cardiopulmonary bypass operation via the original incision during the procedure. The mean age was 9.5 ± 2.0 months and the mean body weight was 8.8 ± 1.4 kg. The mean diameter of the VSD was 4.4 ± 0.5 mm. One patient (0.4%) underwent a second thoracotomy for postoperative intercostal hemorrhage on the second day after surgery. The mean diameter of the occluder size was 5.5 ± 0.6 mm. During the follow-up (4.3 ± 1.4 y), there was no mortality, no new aortic valve regurgitation and atrioventricular block., Conclusion: Peratrial device closure of Pm-VSDs via the right subaxillary route under TEE guidance is safe and effective at midterm follow-up, confirming this is an valuable alternative method for patients <12 months of age., Competing Interests: Declaration of Competing Interest The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: The objective of this study was to evaluate the safety and efficacy of transcatheter closure of ventricular septal defects (VSD) using the LifeTech™ multifunctional occluder device (MF-Konar)., Methods: Clinical features and demographic characteristics and follow-up findings were evaluated retrospectively from three centers., Results: MF-Konar was used in 98 patients. The median age and weight of the patients were 3.8 years (range 5.4 months-50 years) and 15.3 kg (range 5.5-80 kg), respectively. The mean fluoroscopy time was 13.7 ± 8.2 min (range 3.4-42.6 min). Procedural success was obtained for 96 out of 98 patients (98%). In 54 out of 98 patients, closure was performed via the antegrade route. Major complications occurred in four patients (embolization in two, complete heart block in one, and device dislocation needing surgical treatment in one). All of the complications were treated successfully, and there was no mortality. Mild residual flow in eight patients (8%), new onset tricuspid valve insufficiency in one (moderate), and new onset aortic valve insufficiency in one (mild) were observed during a mean follow-up duration of 224 ± 149 (10-515) days. Minor rhythm disturbances were observed in eight patients., Conclusions: Transcatheter closure of VSDs in selected patients using the LifeTech MF-Konar device seems effective. Its advantages are softer design, use of both an antegrade and retrograde approach, and an advanced smaller delivery system. Increasing the number of usage and the experience will provide more accurate data and low complication rates., Competing Interests: Declaration of competing interest Three of the authors are (OB, AG and EO) are proctors of the Lifetech company., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Patients with simple shunt lesions, such as atrial septal defect (ASD), ventricular septal defect (VSD) and persistent arterial duct (PDA) remain at risk of developing pulmonary hypertension (PH) even after correction of their cardiac defect. We aimed to assess the contemporary prevalence of PH in a well characterized nationwide group of patients based on the German National Register for Congenital Heart Defects., Methods and Results: We included all patients >16 years of age with an isolated diagnosis of ASD, VSD or PDA. Only patients with previous surgical or interventional closure of the defect were included. Patients with genetic syndromes were excluded. Out of 49,597 CHD patients in the register we identified 825 patients with closed, isolated simple defects (52% ASD, 41% VSD, 7% PDA). Of these, 25 (3%) developed PH after a median follow-up of 16 years from defect closure. The risk of PH increased significantly with age at follow-up (p < 0.0001) and age at repair (p < 0.0001) on logistic regression analysis Patients with PH were significantly more likely to be symptomatic (59% vs. 9% in NYHA class ≥2, p < 0.0001) and had significantly higher mortality (hazard ratio 13.4, p < 0.0001) compared to the remaining patients., Conclusions: Based on data from the German National Register CHD Register we report a PH prevalence of 3.0% in patients with corrected, simple lesions. Patients with PH were more symptomatic and had significantly increased mortality risk. Life-long surveillance and low threshold for workup is recommended to ascertain diagnosis of PH, which has important prognostic and clinical implications., Competing Interests: Declaration of competing interest The authors and the institutions involved have received support by Actelion, Pfizer and GlaxoSmithKline Group of Companies (GSK)., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: To compare transthoracic device closure via a minimal lower-sternal incision or minimal right thoracic incision and surgical repair via midsternal incision for perimembranous ventricular septal defect (VSD)., Methods: We retrospectively analyzed the clinical data of 147 patients with isolated perimembranous VSD who were treated in our hospital from June 2017 to December 2017. According to the therapeutic approaches, the patients were divided into group A(transthoracic device closure via a minimal lower-sternal incision), group B((transthoracic device closure via a minimal right thoracic incision) and group C(surgical repair via midsternal incision). The clinical data of the three groups were statistically analyzed., Results: The three groups of patients had satisfactory outcomes for VSD closure. No complications, including third-degree atrioventricular block, large residual shunt requiring re-operation, newly moderate-severe aortic or tricuspid regurgitation, occluder detachment were reported. Compared with group C, the operative time, duration of mechanical ventilation, length of ICU stay, drainage volume, blood transfusion volume, length of the incision, and length of postoperative hospital stay were significantly lower in the device groups (A and B)., Conclusion: Transthoracic device closure via a minimal lower-sternal incision or minimal right thoracic incision and surgical repair via midsternal incision are sufficiently safe procedures for the treatment of isolated perimembranous VSD and can achieve satisfactory early clinical efficacy. Both device approaches have the advantages of a quick recovery and good cosmetic appearance of the incision., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: There are increasing reports of cardiac and exercise dysfunction in adults with small, unrepaired ventricular septal defects (VSDs). The primary aim of this study was to evaluate pulmonary function in adults with unrepaired VSDs, and secondly to assess the effects of 900 μg salbutamol on lung function and exercise capacity., Methods: Young adult patients with small, unrepaired VSDs and healthy age- and gender-matched controls were included in a double-blinded, randomised, cross-over study. Participants underwent static and dynamic spirometry, impulse oscillometry, multiple breath washout, diffusion capacity for carbon monoxide, and ergometer bicycle cardiopulmonary exercise test., Results: We included 30 patients with VSD (age 27 ± 6 years) and 30 controls (age 27 ± 6 years). Patients tended to have lower FEV 1 , 104 ± 11% of predicted, compared with healthy controls, 110 ± 14% (p = 0.069). Furthermore, the patient group had lower peak expiratory flow (PEF), 108 ± 20% predicted, compared with the control group, 118 ± 17% (p = 0.039), and showed tendencies towards lower forced vital capacity and increased airway resistance compared with controls. During exercise, the patients had lower oxygen uptake, 35 ± 8 ml/min/kg (vs 47 ± 7 ml/min/kg, p < 0.001), minute ventilation, 1.5 ± 0.5 l/min/kg (vs 2.1 ± 0.3 l/min/kg, p < 0.001) and breath rate, 48 ± 11 breaths/min (vs 55 ± 8 breaths/min, p = 0.008), than controls., Conclusion: At rest, young adults with unrepaired VSDs are no different in pulmonary function from controls. However, when the cardiorespiratory system is stressed, VSD patients demonstrate significantly impaired minute ventilation and peak oxygen uptake, which may be early signs of parenchymal dysfunction and restrictive airway disease. These abnormalities were unaffected by the inhalation of salbutamol., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: The long-term evolution of pulmonary vascular resistance (PVR) after ventricular septal defect (VSD) repair is unknown. This study serially evaluated resting and exercise PVR after VSD repair in childhood., Methods: Patients were enrolled from the outpatient Adult Congenital Heart Disease clinic of the University Hospitals Leuven and compared to age- and gender-matched controls. Participants underwent resting and exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. Total PVR was calculated as the ratio of mean pulmonary artery pressure (mPAP) to cardiac output (CO). The slope of the mPAP-CO curve (exercise PVR) was obtained using linear regression analysis., Results: Twenty-seven patients (mean age 31 ± 7 years, 70% male) and 18 controls were included. At baseline, patients had larger right ventricular (RV) end-diastolic areas (10 ± 2 vs 9 ± 1 cm 2 /m 2 , p = 0.001) and lower tricuspid annular plane systolic excursion (TAPSE) (17 (17-19) vs 26 (22-28) mm, p < 0.001). After 1.1 (1.0-1.5) years follow-up, similar differences in RV areas and TAPSE were found. Patients reached lower peak workload and cardiac index compared to controls at each time point. Peak total PVR was higher (Baseline: 2.7 ± 0.8 vs 2.2 ± 0.3 mm Hg/L/min, p = 0.005; Follow-up: 2.9 ± 0.9 vs 2.1 ± 0.3 mm Hg/L/min, p < 0.001) and the mPAP-CO slope was steeper (Baseline: 2.2 ± 0.8 vs 1.7 ± 0.3 mm Hg/L/min, p = 0.008; Follow-up: 2.5 ± 0.9 vs 1.6 ± 0.3 mm Hg/L/min, p < 0.001) in patients. The mPAP-CO slope in patients correlated inversely with peak oxygen uptake (R = -0.41 and - 0.45, p = 0.036 and 0.022, baseline and follow-up, respectively)., Conclusion: Despite repair, VSD patients seem to show altered pulmonary hemodynamics and RV impairment at rest and exercise, supporting life-long follow-up., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The prevalence of congenital heart disease (CHD) in the adult population is steadily increasing. A substrate of prosthetic material and residual lesions, constantly evolving as surgical techniques change over time, predispose these patients to the potentially devastating complication of infective endocarditis (IE)., Methods: We retrospectively reviewed 2935 patients in our adult CHD database for all cases of endocarditis between 1991 and 2016. Incidence, clinical course and predictors of outcomes were analysed., Results: We document 74 episodes in 62 patients, with an incidence of 0.9 cases/1000 patient years (py). IE was more common in complex CHD (1.4 cases/1000py) and ventricular septal defects (VSDs) (1.9 cases/1000py). Prosthetic material was involved in 47% and left-sided infection predominated (66%). The incidence in bicuspid aortic valves post aortic valve replacement (AVR) was significantly higher than in unoperated valves, being 1.8 and 1.1 cases/1000 patient years respectively. Streptococcus was the most frequently implicated causative organism (37%). Emboli occurred in 34% of cases with a cerebral predilection. 46% of patients required surgery during the admission for IE, most frequently to replace a severely regurgitant bicuspid aortic valve. Early endocarditis-related mortality was 15%, associated with cerebral emboli and acute renal failure., Conclusions: In a contemporary adult CHD cohort, those with complex underlying lesions, VSDs or an AVR were at higher risk for IE. Mortality remains substantial and is more likely in patients with cerebral emboli and/or acute renal failure., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Vector flow mapping is a novel echocardiographic flow visualization method, and it has enabled us to quantitatively evaluate the energy loss in the left ventricle (intraventricular energy loss). Although intraventricular energy loss is assumed to be a part of left ventricular workload itself, it is unclear what this parameter actually represents. The aim of the present study was to elucidate the characteristics of intraventricular energy loss., Methods: We enrolled 26 consecutive children with ventricular septal defect (VSD). On echocardiography vector flow mapping, intraventricular energy loss was measured in the apical 3-chamber view. We measured peak energy loss and averaged energy loss in the diastolic and systolic phases, and subsequently compared these parameters with catheterization parameters and serum brain natrium peptide (BNP) level., Results: Diastolic, peak, and systolic energy loss were strongly and positively correlated with right ventricular systolic pressure (r=0.76, 0.68, and 0.56, p<0.0001, = 0.0001, and 0.0029, respectively) and right ventricular end diastolic pressure (r=0.55, 0.49, and 0.49, p=0.0038, 0.0120, and 0.0111, respectively). In addition, diastolic, peak, and systolic energy loss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, p<0.0001, < 0.0001, and=0.0116, respectively)., Conclusions: In children with VSD, elevated right ventricular pressure is one of the factors that increase energy loss in the left ventricle. The results of the present study encourage further studies in other study populations to elucidate the characteristics of intraventricular energy loss for its possible clinical application., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Pulmonary arterial hypertension (PAH) is virtually absent after closure of ventricular septal defect (VSD) in the first six months of life. However the prevalence of PAH in patients, who underwent VSD closure later, is not clear. The aim of this study was to analyse the prevalence of PAH after a successful VSD closure after the age of 6months and whether there are risk factors for developing PAH., Methods: Echocardiographic and right heart catheter data of patients with VSD or complete atrioventricular septal defect, who underwent VSD closure after the age of 6months in our institution between 01/2005 and 06/2014, were retrospectively analyzed. PAH was defined as mean pulmonary arterial pressure (mPAP) of ≥25mmHg or tricuspid regurgitation jet velocity of ≥3.5m/s., Results: In 228 patients (median age at shunt closure 4.0years, range 0.5-69) and 174 complete follow-up data (median follow-up 3.7years, range 0.5-39.4), 9 patients needed pulmonary vasodilator therapy after shunt closure, 4 of them temporarily for up to 79months. Three patients are still on vasodilator treatment 1, 2.6 and 6years after surgery, other two were lost to follow-up. Another 6 patients with preoperatively borderline hemodynamics due to elevated mPAP and pulmonary vascular resistance, recovered well without signs of postoperative PAH., Conclusion: With the current practice for safe late VSD closure, PAH is very rare at least in the first years of follow-up. In most patients with perioperative PAH, this condition appears to be transient and shows good response on pulmonary vasodilator treatment., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Small ventricular septal defects (VSDs) are considered to be without hemodynamic influence and most remain unrepaired. However, studies recently described late cardiac adverse consequences that could potentially affect functional capacity. Yet, this has never been assessed in adulthood. Therefore, the aim was to determine peak exercise capacity in adults with small VSDs compared with healthy, matched controls., Methods: In a prospective, cohort study we included patients with unrepaired VSDs and healthy controls, (age 18 to 40years). Functional capacity was determined through incremental bicycle tests and gas exchange was measured breath-by-breath with Jaeger MasterScreen CPX®. Primary endpoint was peak oxygen uptake, while secondary endpoints were anaerobic threshold and health-related quality-of-life., Results: In total, 34 VSD patients (age 26.5±6years) and 28 controls (age 26.9±5years) were included. There were no differences between groups in demographic characteristics or habitual exercise levels. At peak exercise, patients reached lower peak oxygen uptake, 36.2±9ml/kg/min, compared with controls, 43.8±6ml/kg/min (p=0.002) along with lower maximal workload; patients 3.2±1 watt/kg and controls 3.8±1 watt/kg (p=0.001). Aerobic capacity was also poorer in patients, 24.5±8ml/kg/min compared with controls, 31.2±7ml/kg/min (p=0.005). Lastly, patients had lower health-related quality-of-life in terms of physical (p=0.017) and social functioning (p=0.003) compared with controls. In the patient group physical functioning was directly correlated to the impaired peak oxygen uptake (r=0.473, p=0.005)., Conclusion: We demonstrated reduced subjective and objective functional capacity in small, unrepaired VSDs compared with controls. Furthermore, a correlation was seen between the impaired peak exercise capacity and lower self-estimated physical health., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: To determine the risk and predictors of progression of aortic regurgitation (AR) and other adverse events (AE) in adults with supracristal ventricular septal defect (VSD)., Methods: Retrospective observational study of 62 adults with supracristal VSD followed at the Mayo Clinic from 1994 to 2013. Freedom from AR progression was compared by age and racial groups (Asian vs non-Asian). Predictors of AR progression were determined using Cox proportional hazard model. Composite AE endpoint was defined as AR progression, endocarditis, aneurysm of sinus of Valsalva (aSOV), and rupture of aSOV. Risk of AE was compared between group 1 (no surgical intervention) and group 2 (surgical intervention)., Results: Sixty-two patients aged 47(SD 12) years were followed for 13 (SD 5) years. Group 1=42/62 and group=20/62. Endpoint of AR progression and aSOV occurred in 13% and 20% respectively. Freedom from AR progression for the entire cohort was 88%, 86% and 86% at 5, 10 and 15 years respectively. Risk of AR progression was significantly lower in patients (aged ≤40 years, p=0.008) but similar between Asians and non-Asians (p=0.57). Age ≤40 years was an independent predictor of AR progression (hazard ratio [HR] 3.5, 95% CI 2.3-5.4, p=0.001). Composite AE endpoint occurred in 33% and 40% of group1 and 2 cohorts respectively, p=0.69., Conclusion: Adults with supracristal VSD are at lower risk for AR progression but higher risk for aSOV formation compared to historical pediatric cohorts. Younger age was a predictor of AR progression. Surgical intervention had no protective effect on complication rate., (Copyright © 2015. Published by Elsevier Ireland Ltd.)

Recent studies have shown markedly reduced functional capacity in long-term survivors of ventricular septal defect (VSD), and in order to explore possible mechanisms, we performed non-invasive assessment of ventricular force-frequency relationships during exercise in adults operated for VSD in early childhood. We conducted a prospective study at a tertiary referral center. Patients (n=23) and healthy controls (n=20) underwent continuous Doppler-echocardiographic imaging during supine bicycle ergometry. The cycling workload was individually and manually incremented during the test session in response to heart rate. The heart was imaged in an apical 4-chamber view, and color-coded myocardial velocities were recorded. Post hoc, peak systolic velocity and isovolumetric acceleration (IVA) were blindly determined in the basal ventricular segments. VSD-operated patients differed markedly in all right ventricular endpoints compared with controls. IVA was lower prior to the test, 70 ± 30 cm/s(2) vs. 150 ± 60 cm/s(2) among controls, and during the entire test session ending at a heart rate of 160 beats/min; 140 ± 50 cm/s(2) vs. 300 ± 30 cm/s(2), p < 0.01 at both points. A similar pattern was revealed in terms of peak right ventricular systolic velocity. Left ventricular and septal measurements showed a similar, although less significant, tendency with a clearly lower left ventricular optimum heart rate among patients: 140 beats/min vs. 154 beats/min among controls. In the diseased cohort biventricular force-frequency relationships were directly correlated to peak oxygen uptake. VSD repair in early childhood is associated with disruption of the right ventricular force-frequency relationship, which may contribute to the previously observed reduction in functional capacity., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: Real-time three-dimensional echocardiography (RT3DE) is a new image modality, and it can display a unique image reconstruction in a variety of heart diseases. However, clinical assessment of ventricular septal aneurysm (VSA) by RT3DE has not been reported. This pilot prospective study is to survey what kinds of new insights of VSA can be provided by RT3DE as compared with conventional 2-dimensional echocardiography (2DE)., Methods: We investigated the diagnostic value of RT3DE and 2DE in 60 consecutive patients with VSA. From different transthoracic windows, structures of interest can be displayed from any orientation through adjusting cropping and slicing the RT3DE datasets. The results were compared with those in 2DE., Results: RT3DE reconstruction of VSA was feasible in 56 of 60 patients (93%). When compared with 2DE, additional information provided by RT3DE included blood flow through left ventricle to right ventricle, visualization of VSD enface border in 56 patients (93%), morphology of the VSA from apical short axis view in 48 patients (86%), hypertrophied margin of the interventricular septum in 26 patients (43%), dynamic changes of VSA and tricuspid valve in 18 patients (30%), adhesion of chordae tendineae in VSA in 16 patients (26%)., Conclusions: Structures of interest can be evaluated from unique RT3DE in any orientation during scanning. RT3DE offers additional novel views and has the advantages of not only displaying better visualization of VSA, but also adequately showing the spatial relationship with its adjacent structures. It can provide novel and useful anatomic insights than 2DE while assessing patients with VSA.

Background: The purpose of this study was to evaluate the accuracy of AV valve morphology assessed by real-time transthoracic 3D echocardiography (RT-3DE) compared to surgical findings and to assess whether RT-3DE is applicable in clinical practice., Methods: Between June 2004 to May 2005, 19 patients with an atrioventricular septal defect (AVSD) undergoing surgical treatment at our institution were enrolled in the study. RT-3DE was performed with Philips Sonos 7500 echo-system and off-line analysis with TomTec Echoview software. The AVSD was assessed for the morphology of AV valve, with particular interest to the superior and inferior bridging leaflets. 3D data were compared with measurements and descriptions acquired during the surgical procedure., Results: Acquisition of RT-3DE datasets was feasible in all patients. Of the 19 patients, there were 11 infants (age <1 year). The duration of 3D data acquisition was 12+/-3 min for patients above 1 year and 4+/-2 for infants. Reconstruction time was 22+/-8 min. In all patients the AV valve orifice and RT-3DE observations of the superior and inferior bridging leaflets were all correctly identified by RT-3DE compared with the surgical findings., Conclusion: Real-time transthoracic 3D echocardiography provides accurate assessment of AVSDs and correctly depicts the AV valve morphology. After a short learning curve, RT-3DE is easily applicable during daily clinical practice.

Background: Few data are available with regard to the impact of aortic cusp herniation on the evolution of aortic regurgitation (AR) in patients with a perimembranous ventricular septal defect (VSD)., Methods: One hundred and two patients with a perimembranous ventricular septal defect with right coronary cusp prolapse were divided to two groups depending on the development of aortic regurgitation. The original defect diameter, the right coronary cusp deformity index (RCCD), and the right coronary cusp imbalance index were obtained as we reported previously., Results: Mild aortic regurgitation was detected in 35 patients, and moderate in three. No aortic regurgitation was observed in 64 patients. A significantly larger number of patients had noncoronary cusp prolapse and the right coronary cusp imbalance index >/=1.30 in the aortic regurgitation group than in the no regurgitation group. Relative risk and odds ratio of noncoronary cusp prolapse and the right coronary cusp imbalance index >/=1.30 for development of aortic regurgitation were 3.69 (95% CI, 0.91-15.03) and 27.90 (95.94-130.85), and 2.23 (0.83-5.98) and 4.70 (1.44-15.27), respectively. Surgical closure was performed in 29 patients. All patients with no noncoronary cusp prolapse underwent simple patch closure of the ventricular septal defect, while five patients with noncoronary cusp prolapse and aortic regurgitation underwent aortic valvuloplasty. Among these, one patient needed aortic valve replacement., Conclusions: The additional complication of noncoronary cusp prolapse is a strong risk factor for the development of aortic regurgitation in patients with a perimembranous ventricular septal defect with right coronary cusp prolapse.

The authors describe the cross-sectional echocardiographic and angiographic features besides the postmortem findings of a newborn with aortic atresia associated with the aortopulmonary window and an interrupted aortic arch, as the third known similar case reported in the literature. Anatomical and clinical similarities with common arterial trunk are emphasized and a more correct management is established to avoid risks, in which Norwood-type techniques would be preferable.

Right ventricular infarction is frequently seen with inferior myocardial infarction, but it rarely presents in isolation. We describe a case of isolated right ventricular infarction complicated with ventricular septal defect. Clinical, hemodynamic, echocardiographic, and surgical features are discussed. Twenty months following surgical repair of the ventricular septal defect and two vessel coronary artery bypass surgery, the patient continues to do well and is free from angina and exertional dyspnea.