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Congenital interventricular septal aneurysms (IVSA) of the muscular septum are rare and can be associated with other familial abnormalities of the ventricular septum, arrhythmias, additional congenital heart disease, and chromosomal abnormalities. IVSA is also linked to ventricular dysfunction and non-compaction, although there are limited reports of this association presenting in utero. We describe a case of fetal ventricular septal aneurysm associated with ventricular dysfunction and pericardial effusion., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: The aim of this study was to examine whether there is a correlation between different types of ventricular septal defects (VSD) and chromosomal abnormalities in the low-risk setting of non-invasive prenatal testing (NIPT) and to evaluate the prognosis of fetuses with varying types of VSD., Methods: Cases of pregnant women who underwent amniocentesis due to fetal VSD were collected by Tianjin Central Hospital of Obstetrics and Gynecology from May 2017 to May 2022. Exclusions were made for those without NIPT, with high-risk NIPT results, genetic disorders, and those lost to follow-up. Data collected included ultrasound classification of VSD, prenatal NIPT results, copy-number variations (CNVs) results, and neonatal outcomes., Results: The prevalence of pathogenic CNVs was investigated in 74 cases of VSDs. Of these cases, 45 were isolated VSDs (9 muscular and 36 non-muscular) and 29 were non-isolated VSDs (10 with intracardiac and 19 with extra-cardiac structural anomalies). The results revealed that the incidence of pathogenic CNVs was lower in isolated VSDs compared to non-isolated VSDs in a low-risk NIPT condition (χ2 = 9.344, P = 0.002). There was no significant difference in the prevalence of pathogenic CNVs between VSDs with intracardiac and extra-cardiac structural anomalies (P = 0.541). Moreover, VSDs associated with intracardiac structural anomalies had the highest rate of surgical intervention., Conclusion: When NIPT is low-risk and VSD is isolated, the likelihood of fetal chromosomal defects is not increased. However, if there are intra- or extra-cardiac structural abnormalities present alongside VSD, the possibility of pathogenic CNV is considerably greater, necessitating invasive prenatal diagnosis. Isolated muscular VSDs usually do not require surgery, which can be used as a basis for prenatal counseling regarding fetal VSD., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm 2 /BSAm 2 ) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Isolated ventricular septal defect (VSD) is often associated with good clinical outcomes. However, infants prenatally diagnosed with VSD are often recommended for delivery at tertiary care centers. The aim of our study was to determine the odds of neonatal intensive care unit (NICU) admission in infants with persistent isolated VSD and complicated VSD, where an infant is affected by VSD and other genetic/structural abnormalities. We performed a retrospective cohort study, with data collected from a single academic institution from June 2018 to March 2023. Individuals with prenatally diagnosed VSD, in the absence of any other heart defects, were included in this study. The primary outcome was admission to the NICU. Multivariable logistic regression was used to assess associations. The association between persistence of VSD and NICU admission was adjusted for maternal age, fetal genetic abnormalities, fetal extracardiac abnormalities, and gestational age at the time of delivery. The association between complicated VSD and NICU admission was adjusted for maternal age and gestational age of the infant at the time of delivery. The odds of NICU admission were similar in infants with persistent isolated VSD and VSD that closed in utero (adjusted OR 1.31, 95% CI 0.30-5.61). However, infants with complicated VSD were at increased risk of NICU admission (adjusted OR 15.52, 95% CI 2.90-82.92). The risk of NICU admission was only increased in infants whose VSD was complicated by another genetic/major structural abnormalities. Therefore, women whose infants are prenatally diagnosed with VSD alone may not require delivery at tertiary care centers., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

To assess the safety and effectiveness of utilizing eccentric occlusion for the treatment of sub-arterial ventricular septal defects, we performed a retrospective study on the classification and analysis of relevant cases. A total of 105 patients with a minimally invasive incision were enrolled in this study, from April 2018 to September 2022. All patients underwent treatment of transthoracic sub-arterial ventricular septal defect occlusion. We analyzed the causes of closure failure, indication, and complication. Briefly, the closure device was successfully implanted in 78 cases (74.2%) with a mean age of 31.4 ± 31.8 months, VSD size of 4.3 ± 0.9 mm, and device size of 6.0 ± 1.1 mm. However, 27 cases (25.8%) required cardiopulmonary bypass due to failure of occlusion. The reasons for failure included 13 cases with worsened aortic regurgitation, two cases with worsened aortic valve prolapse, one case with worsened mitral regurgitation, eight cases with significant residual shunt, and three cases with deviated occlusion morphology. During the 1-36-month follow-up visit, no cases experienced displacement of the eccentric umbrella, shedding, or arrhythmia. All residual shunts resolved during the visit. We concluded that occlusion for sub-arterial VSD has sufficient security and feasibility, under the strict control of surgical indications, appropriate choice of occluder and precise perioperative management., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The experience with percutaneous closure of postoperative residual ventricular septal defects (VSDs) is expanding with improved device technology and techniques. To report our experience with percutaneous closure of residual VSDs after cardiac surgeries. Retrospective clinical data review of patients who had percutaneous closure of postoperative residual VSDs at our institution between 2010 and 2022. Patients' demographics, procedural, and follow-up data were looked at. Twelve patients (50% males) with a median age of 9.2 years (range 0.9-22) were identified. Baseline surgeries were 8 tetralogy of Fallot corrections, 2 pulmonary bandings for large muscular VSD (mVSD) including 1 coarctation repair, 1 atrioventricular septal defect repair, 1 sub-aortic membrane resection-induced iatrogenic VSD, 1 isolated VSD closure, and 1 additional mVSD. Median duration between baseline surgery and percutaneous VSD closure was 2.2 years (range 0.2-8.3). Residual VSD shunting was secondary to surgical patch leakage in 8/12 patients. The median angiographic defect diameter was 6.8 mm (range 4.8-14). The defect was balloon-calibrated in 3/12 patients. Defects were tackled retrogradely in 3/9 patients. Amplatzer Membranous VSD occluder (n = 1), Lifetech Multifunctional (n = 5), Membranous (n = 1) and muscular VSD occluders (n = 2) and Occlutech Membranous (n = 1) and Muscular (n = 2) VSD occluders were used. The procedure was successful in 10/12 patients. Two devices embolized to the pulmonary artery and were snare-retrieved. Both patients were referred for surgery. The median follow-up was 1.3 years (range 0.1-12). Six-month ultrasound showed one trivial residual shunt and one mild right ventricular outflow obstruction. One patient is receiving targeted therapy for pulmonary hypertension at 2 years of follow-up. Transcatheter closure of postoperative residual VSDs is a feasible yet challenging intervention. Procedural complications can be encountered., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Ventricular septal defects (VSD) represent the most common congenital heart defect in newborns. We assessed the electrocardiographic characteristics of newborns with VSDs in a general population sample. The Copenhagen Baby Heart Study is a prospective population-based cohort study offering cardiac evaluation of newborns. Echocardiograms and electrocardiograms were obtained within 30 days after birth and systematically analysed. A VSD was identified in 530 newborns (mean age 11 ± 7 days, 42% boys). Newborns with VSDs had a more left-shifted QRS axis (116 ± 34 vs. 120 ± 3°, p = 0.02), and a higher S-wave amplitude in V1 (721 ± 584 vs. 636 ± 549 µV, p = 0.001) than controls. The largest differences were found in newborns with large or perimembraneous VSDs with a higher frequency of left axis deviation, higher S-wave amplitudes in V1, and higher R- and S-wave amplitudes in V6 compared with controls. R-waves in V1 and V6 were significantly associated to left ventricular mass, whereas S-waves in V1 and V6 were dependent on left ventricular end-diastolic diameter on echocardiography.  Conclusion: Newborns with VSDs showed significant differences in QRS axis, and R- and S-wave precordial amplitudes compared to matched controls. Perimembranous and large VSDs had the greatest effect on the neonatal ECG. What is Known: • Ventricular septal defects in newborns are prevalent and may affect cardiac function and structure. What is New: • The Copenhagen Baby Heart Study is the largest study including a cohort of unselected newborns undergoing postnatal cardiac examination. • We found that newborns with VSD showed significant electrocardiographic differences depending on size and type of VSD compared with healthy newborns., (© 2023. The Author(s).)

Management of pulmonary atresia, ventricular septal defect with major aorto-pulmonary collateral arteries, and hypoplastic native pulmonary arteries focuses on growth of the native pulmonary arteries. One strategy to grow the native pulmonary arteries is through pulmonary valve perforation followed by right ventricular outflow tract stenting, if suitable. We present a unique case of retrograde pulmonary valve perforation and stenting of the right ventricular outflow tract through a major aorto-pulmonary collateral artery., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7 years (interquartile range: 0-17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery were factors associated with post-operative residual aortic regurgitation. Mild pulmonary regurgitation was observed in 12%, 30%, and 40% of patients 5, 10, and 15 years after surgery, respectively. There were no significant differences in age and weight at which surgery was performed between patients with mild pulmonary regurgitation and those with less than mild pulmonary regurgitation. However, the number of sutures across the pulmonary valve was associated with post-operative pulmonary regurgitation (P < 0.01). As some patients with mild pre-operative aortic regurgitation may not improve even after surgery, early surgical intervention is necessary when aortic regurgitation appears. Some patients may develop post-operative pulmonary regurgitation in the long term, suggesting the need for careful follow-up., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Transcatheter closure of Perimembranous VSDs (PMVSD) remains challenging particularly in infants. The aim of this study is to evaluate the efficacy and safety of transfemoral PMVSD device closure in infants weighing ≤ 10 kg in a single centre. Retrospective review of departmental databases and medical charts to define patient cohort and collect demographic, procedural and follow-up data. Between July 2014 and March 2021, 16 patients underwent attempted transfemoral PMVSD device closure (12 retrograde) at a median age of 11 months (interquartile range [IQR] 9-15.5) and a median weight of 8.3 kg (IQR 7.2-9.5). All patients were either symptomatic, had progressive left heart dilation or had VSD associated valve regurgitation. Median defect size on pre-procedural transoesophageal echocardiography was 6.8 mm (IQR 6-8.5). Median device waist size was 6 mm (IQR 4.5-8). Successful device placement was achieved in 14 patients (88%). One patient developed moderate aortic and tricuspid valve regurgitation upon retrograde and antegrade device deployment, respectively, and subsequently underwent surgical closure. The second patient developed progressive aortic regurgitation (AR) 2 days post procedure, and also underwent surgical removal with no residual AR. There was no cases of device embolization and no femoral arterial compromise. On median follow-up of 40.5 months (IQR 25-64), none of the patients developed complete heart block. Three patients (18.75%) had small residual shunts at latest follow-up which have not required any further intervention. Device closure of PMVSD's in children weighing ≤ 10 kg is feasible and safe with good procedural success rates. Use of both the antegrade and retrograde approaches may be necessary depending on anatomical variances., (© 2023. The Author(s).)

Coronary anomalies encompass different conditions in terms of anomalous origin, coursing, and branching, which are occasionally associated with congenital heart disease. This study aimed to explore coronary anomalies associated with ventricular septal defect that was a stereotypical congenital heart disease. We retrospectively reviewed angiographic findings in patients with ventricular septal defect who required corrective surgery, and identified coronary abnormalities, including anomalous origin, coursing, and branching of coronary arteries based on angiography. We studied the prevalence and types of coronary anomalies among them. A total of 998 patients with ventricular septal defect was studied. Age and weight were 2.0 (2.1-15.2) months and 5.7 (4.1-8.7) kg, respectively. There were 115 patients (12%) with syndrome or genetic disorder, and 34 patients (3%) with extracardiac major organ anomalies. The overall prevalence of coronary anomalies was 6.2%. All coronary anomalies were anomalous origin and coursing, among 3 patients accompanied anomalous intrinsic coronary anatomy. Originating within aortic root above the Valsalva sinus was the most common coronary anomaly. Coronary anomalies were independently correlated with bicuspid aortic valve (odds ratio [OR]: 8.02, 95% confidence interval [CI]: 2.34-23.4) and persistent left superior caval vein (OR: 5.02, 95% CI: 1.93-11.7). We showed the possibility that minor cardiac variants, such as bicuspid aortic valve and persistent left superior caval vein, contribute to higher prevalence of coronary anomalies in patients with ventricular septal defect., (© 2022. Springer Japan KK, part of Springer Nature.)

Pulmonary atresia with an intact ventricular septum typically occurs in patients with concordant atrioventricular and ventriculoarterial connections. When it does occur in patients with discordant connections, it is most frequently seen in association with congenitally corrected transposition. We present a rare case of transposition of the great arteries with a ventricular septal defect (VSD) detected in fetal life which evolved throughout pregnancy resulting in the development of pulmonary atresia and severe restriction of the VSD., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Transcatheter ruptured sinus of valsalva aneurysm (RSOVA) closure is an alternative to surgery. When a restrictive ventricular septal defect (rVSD) coexists, there are doubts about support for an occluder and persistent left ventricular (LV) dilatation. We compared the outcomes of patients from a single centre with rVSD and RSOVA after surgery or transcatheter intervention in the past 8 years in this study. Location of rVSD did not alter the approach. Compliant balloon interrogation before transcatheter closure was occasionally used to assess significance of interventricular shunt or aortic regurgitation. Procedural success and LV dimensions before and after treatment were analyzed. Seventeen patients with a mean age of 36.64 ± 12.58 years were analyzed. 12/13 catheter procedures (92%) were successful, of whom eight patients had an outlet VSD. rVSD was closed with device in one patient with perimembranous rVSD, but unaddressed in others. Four patients underwent balloon interrogation. Surgery included the single patient who failed intervention. Transcatheter group had more acute symptoms and advanced functional class than surgical group, though not significant. At a median follow-up of 4.5 (1-7.5) years, the mean LV dimensions (50.3 ± 10.1 mm and 50.1 ± 3.1 mm, p = 0.46) were not different in the transcatheter and surgical groups. Mean end-diastolic volumes (110.5 ± 42. 1 ml and 98.5 ± 37.5 ml, p = 0.91) were not significantly different either. Transcatheter RSOVA closure success was not altered by rVSD despite their location underneath the aneurysms, including outlet rVSD with defect in aortoventricular junction. Persistent LV dilatation was not observed after catheter intervention even when the VSD was not closed., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The aim of this study is to examine the possible high association between multiple ventricular septal defect (mVSDs) and noncompaction cardiomyopathy (NCM) as same embryological origin, and the effect of depressed ventricular function in NCM cases during the follow-up, using echocardiography. A total of 150 patients with mVSDs were diagnosed in a single center in Saudi Arabia; 40 cases with isolated or associated with minor congenital heart disease were recruited. Three specialist echocardiography consultants confirmed the NCM diagnosis separately using Jenni, Chin and Patrick criteria, and myocardial function was estimated by ejection fraction at admission and at follow-up after surgery. Stata-14 to analyze the data was used. In our cohort of 40 cases with mVSD (median age at diagnosis = 0.5 years; mean follow-up = 4.84 years), 13(33%) had criteria of non-compaction confirmed by the three specialist consultants. All were operated by surgery and 11 hybrid approach (interventional & surgery). A significant relationship between abnormal trabeculations and mVSD with or without non-compaction was observed, 34% vs 66% respectively (p < 0.03, Fisher's exact test). A repeated-measures t-test found the difference between follow-up and preoperative ejection-fractions to be statistically significant (t (39) = 2.07, p < 0.04). Further, the myocardial function in the mVSD non-compaction group normalized substantially postoperatively compared with preoperative assessment (mean difference (MD) 11.77, 95% CI: 4.40-19.14), whilst the mVSD group with normal myocardium had no significant change in the myocardium function (MD 0.74, 95% CI: -4.10-5.58). Thus, treatment outcome appears better in the mVSD non-compaction group than their peers with normal myocardium. Acknowledging the lack of genetic data, it is evident the high incidence of non-compaction in this cohort of patients with mVSD and supports our hypothesis of embryonic/genetic link, unlikely to be explained by acquired cardiomyopathy., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Left ventricular outflow tract obstruction (LVOTO) affects survival and reoperation rates after surgical treatment of patients with interruption of the aortic arch (IAA) or coarctation of the aorta (CoA) with ventricular septal defect (VSD). The aim of the study was to determine predictors of LVOTO and to evaluate the relationship between aortic valve (AoV) morphology and the re-intervention rate. Retrospective review of patients, who underwent a conventional repair for IAA/CoA with VSD at a tertiary referral center between 1996 and 2017. The preoperative demographic data as well as pre- and post-operative echocardiographic parameters and re-interventions were reviewed. In the median follow-up of 8.3 years (range of 6.15-10.27) 5 patients (11.9%) from a total of 47 patients included in the study presented with a significant LVOTO. Four of them required reoperation after median period of 2.3 years (range of 0.3-7.9) after the initial surgery. Multivariable logistic regression identified AoV z-score (OR 0.44, p = 0.017) as predictor of LVOTO. The mean AoV z-score before the primary repair was significantly smaller in those with LVOTO as compared to those with unobstructed flow from the LV (- 3.58 ± 1.96 vs. - 1.44 ± 1.55; p = 0.0016). At 1-year follow-up, both groups showed an increase in the AoV z-score (p = 0.98). The re-intervention rate after primary repair (both surgical procedures and percutaneous interventions), either for LVOTO or reCoA, was higher in patients with AoV z-score ≤  - 3 (p = 0.007 vs. p = 0.46) and those, whose aortic annulus was less or equal than patient's weight (kg) + 1.5 mm as compared to those with larger aortic annulus (p = 0.03 vs. p = 0.16). In patients after surgical treatment of IAA/CoA with VSD, the AoV z-score at diagnosis is a significant risk factor for reoperation for LVOTO. With age, AoV growth and z-score improvement is expected. Small AoV at diagnosis is correlated with increased rate of re-intervention for LVOTO and reCoA., (© 2021. The Author(s).)

Residual or iatrogenic ventricular septal defects (VSDs) may result in significant hemodynamic effect. This study aimed to review our center experience in transcatheter closure of post-surgical and post-intervention residual and iatrogenic VSDs and to report on their 12-month long-term outcome. All patients who underwent transcatheter closure of residual/iatrogenic VSDs after surgical or transcatheter CHD interventions between January-2015 and January-2020 were included. Patients' medical records were reviewed and analyzed. Twenty-three patients with a mean age of 14.3 ± 8.8 years were included. The VSD was residual in 18 (78.3%) patients and iatrogenic in 5 (21.7%) patients, post-operative in 19 (82.6%) patients and post-transcatheter in 4 (17.4%) patients. The VSD site was peri-membranous in 9 (39.1%) patients, high-muscular in 6 (26.1%) patients, mid-muscular in 4 (17.4%) patients, and Gerbode shunt in 4 (17.4%) patients. The QP/QS ratio was 2.5 ± 0.7, and the VSD diameter was 6.1 ± 2.1 mm. Most, 16 (71.43%) patients underwent antegrade device deployment, and 7 (28.57%) patients underwent retrograde transaortic device deployment with 3 (13.0%) patients required two devices. Amplatzer™ Muscular VSD devices were used in 16 (69.6%) patients, Amplatzer™ Duct occlude-I devices were used in 4 (17.4%) patients, and Amplatzer™ Duct Occluder-II devices were used in 3 (13.0%) patients with a mean device size of 8.8 ± 2.8 mm. Procedural and fluoroscopy times were 55.1 ± 16.2 and 16.3 ± 4.0 min respectively. During follow-up (23.3 ± 15.9 months), no patient required re-intervention or exhibited mortality. Transcatheter closure of post-operative and post-intervention residual/iatrogenic VSDs represents a safe, feasible, and effective therapeutic approach., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Congenital ventricular outpouchings (CVOs) are rare congenital heart defects with limited data regarding prognosis and outcomes. We aimed to describe the characteristics, outcomes and factors associated with morbidity and mortality of prenatally diagnosed CVOs using our institutional experience and a review of published cases. A total of 86 cases of prenatally diagnosed CVOs were identified, including 3 from our institution and 83 cases identified from a review of the literature. Fetal and postnatal outcomes were analyzed for each case. Pericardial effusions (44%) and ventricular dysfunction (17%) were the most common associated findings. Excluding cases that resulted in pregnancy termination, mortality was 17%, with the majority (11/13) occurring in the prenatal period. Factors associated with mortality included an outpouching located on the left ventricle, a diagnosis of hydrops fetalis, the presence of a pericardial effusion, and an earlier gestational age at diagnosis. Of those that survived to delivery, 57% remained asymptomatic without the need for intervention, and the outpouching regressed or resolved in an additional 15%. Prenatally diagnosed congenital ventricular outpouchings are a dynamic form of congenital heart disease with a high fetal mortality rate. The outcomes associated with the outpouchings appear to be the most variable in the prenatal period and the first year after birth. Serial prenatal and postnatal evaluations should be performed to evaluate for a change in the characteristics of the outpouching.

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication following cardiac surgery, let alone in the pediatric population. Other known causes of LV-PSA are trauma, percutaneous cardiac intervention, and infections. This report describes the development of LV-PSA following surgical repair of ventricular septal defect (VSD) and coarctation of aorta (CoA) in an infant.

Criss-cross heart is a rare congenital cardiac anomaly characterized by the crossing of two ventricular inflow streams. We have demonstrated the utility of 4-dimensional color Doppler rendering in diagnosing the criss-cross heart in a fetus. Four-dimensional color Doppler rendering can demonstrate the relative direction of intracardiac blood flows and facilitate recognition of the crossover of inflow streams in the same plane, confirming the criss-cross heart diagnosis in the fetus.

This retrospective, single-center study evaluated short-term and mid-term results of minimally invasive surgery to occlude ventricular septal defects (VSDs) using a subaxillary approach. The procedure was performed on 429 children (224 boys, 205 girls; age 2.4 ± 2.5 years; mean weight 12.7 ± 10.1 kg) between January 2014 and December 2016 at the Children's Heart Center of Henan Province People's Hospital. An approximately 2-cm subaxillary incision was made between the third and fifth ribs, and the appropriate right atrium or ventricle was punctured under the guidance of transencephalographic echocardiography (TEE). The VSD was then occluded under TEE guidance. The mean size of the VSDs was 4.2 ± 1.0 mm, and the occluder measured 5.3 ± 1.3 mm. Asymmetrical occluders were used in 44 patients and symmetrical occluders in 385 patients. The operative time was 60.7 ± 21.3 min, and time in the intensive care unit was 20.9 ± 6.5 h. Blood loss was 12.4 ± 14.4 ml. There were no deaths among these patients. Occluder displacement occurred in two cases. There were no complications (e.g., third-degree atrioventricular block, new aortic regurgitation, reoperation for massive bleeding, serious infection). All patients were followed for 6-48 months, during which time there were ten cases of a postoperative residual shunt, which self-closed in eight during follow-up. The other two cases are still being followed. No complications occurred during follow-up (e.g., reoperation, aortic regurgitation, atrioventricular block, occluder abscission). Occluding VSDs using the subaxillary approach is safe and effective. Short-term and mid-term results are satisfactory. Further follow-up is required regarding long-term results.

We herein describe, for the first time, the fetal presentation of a case of ventricular inversion, restrictive ventricular septal defect, pulmonary stenosis, hypertensive left ventricle and double outlet right ventricle at 34 weeks of gestational age. Postnatal echocardiography confirmed the prenatal diagnosis. The patient was subsequently successfully palliated with a left ventricle to pulmonary artery conduit. This report illustrates the importance of detailed fetal echocardiography to ensure appropriate delivery and neonatal management, and to optimize outcome.

Decreased coaptation height in adults has been identified as a marker of early valve failure. We evaluated aortic valve coaptation and effective height in healthy children and in children with a ventricular septal defect (VSD) with aortic cusp prolapse (ACP), using echocardiography. We included 45 subjects with VSD with ACP, 27 did not develop aortic regurgitation (AR) by ACP and 18 developed AR by ACP, and 83 healthy children as controls. Aortic root anatomy was estimated using the parasternal long-axis view. We measured the diameter of aortic valve (AV), coaptation height (CH), and effective height (EH) of the aortic valve. We defined the ACH (CH/AV ratio) and AEH (EH/AV ratio) indices as follows: [Formula: see text]. There were significant differences in ACH and AEH between the groups (control vs VSD with ACP vs VSD with ACP and AR, median ACH [%], 35.1 vs 32.0 vs 22.1; median AEH [%], 52.0 vs 48.0 vs 34.4, respectively; P < 0.01]). Intra-cardiac repair (ICR) was performed in 15 cases. Significant increases were observed in ACH and AEH before and after ICR (median ACH [%], before: 27.0, after: 32.7, P < 0.05; median AEH (%), before 38.5, after 45.8, P < 0.05). Measurement of ACH and AEH may allow direct and non-invasive assessment of the severity of VSD with ACP, which could aid clinicians in determining the need and timing for surgical intervention.

Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E'- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.

During the development of so-called aneurysmal transformation of perimembranous ventricular septal defects (pmVSD), tricuspid valve (TV) morphology and function may be altered resulting in left ventricular (LV) to right atrial (RA) shunting. The feasibility and outcome of interventional closure of these pmVSD has not been investigated so far. Interventional closure of pmVSD associated with mild to moderate LV-to-RA shunt was performed in four patients (aged 6.5-12.5 years). pmVSD were closed under fluoroscopic guidance by establishing an arteriovenous wire loop via a femoral artery and advancing the delivery sheath from a femoral vein. Before device release (or withdrawal if necessary), residual shunting across the device and TV valve function was investigated by transthoracic echocardiography and LV angiography. pmVSD sizes of 4, 5.5, 8 and 8.5 mm were closed with a 4/4 and 6/6 Amplatzer duct occluder II and an 8- and 10-mm Amplatzer muscular VSD occluder device, respectively. There were no or only minor residual postinterventional LV-to-RA shunts. No atrioventricular blocks were observed during a mean follow-up of 12.5 months (range 6.5-17 months). Transthoracic echocardiography indicated that the elimination of the VSD jet pushing the antero-superior TV leaflet open is the key mechanism for LV-to-RA shunt reduction after transcatheter pmVSD closure. Interventional closure in pmVSD associated with mild to moderate indirect LV-to-RA shunting is feasible and results in significant reduction in or elimination of LV-to-RA shunting.

The objective of this study was to assess the ability of different parameters to identify fetuses requiring neonatal care for coarctation of the aorta (CoA). Between January 2003 and December 2012, 175 fetuses referred for great vessel disproportion were divided into two groups: group A (n = 51) with high risk of CoA and delivery planned in tertiary care referral center and group B (n = 124) with no increased risk of CoA. In group A, diagnosis of CoA was confirmed in 38/51 (74 %). In group B, 2/124 had CoA. Multiple logistic regression analysis identified the best combination as diffusely hypoplastic and/or angular aortic arches, ventricular septal defect and aortic valve diameter <5 mm at 36-week gestational age (GA). Positive predictive value was 75 % when vessel disproportion was noted before 28-week GA and 73 % in the third trimester. Postnatal diagnosis involved 38 cases of CoA which had not been referred. One case of CoA diagnosed after birth was referred prenatally for difficulty of screening without any defect. The results of our prospective study are in agreement with those of previous series, but our false positive rate was lower especially when the diagnosis of vascular disproportion was made at third trimester. The performance of fetal cardiac screening does not seem to be very good, but prenatal diagnosis is probably not always possible: Among our three false negative cases, two had isolated vascular disproportion and the third no risk factors.

The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p < 0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p < 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values <0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

Nonsurgical closure of congenital ventricular septal defects (VSD) has become increasingly acceptable with the availability of different occlusion systems. Transcatheter device treatment is used for perimembranous and muscular defects. Atrio-ventricular block remains the most troublesome complication of device closure. The aim of this study was to describe our experience with closure of VSD using the Amplatzer Duct Occluder II (ADO II) as an "off-label" approach in children and adults. Between 2004 and 2012 transcatheter closure of 31 VSD (20 perimembranous, 10 muscular VSD and 1 ruptured sinus valsalva) with ADO II was undertaken in patients between 3 months and 55 years of age and with a body weight ranging from 4 to 105 kg in our institution. In 29 of 31 procedures, the defect was successfully closed (93.5%) without any significant complications. No increase of aortic or tricuspid valve regurgitation was found in any after procedure. Small residual shunts were observed immediately after the device implantation, but disappeared during a median follow-up period of 38 months (0.4-63) in 27 of 31 patients. There was no incidence of AV block or other conductance abnormalities during implantation or follow-up. The ADO II device is safe and effective for transcatheter VSD closure, but this is still an "off-label" use. After long-term follow-up in a large number of patients this device may be approved for VSD closure in the future.

We herein report the advantages of retrograde ventricular septal defect (VSD) closure using a muscular VSD device for perimembranous VSDs. Perimembranous VSDs are conventionally closed by an antegrade technique (arteriovenous looping technique) using a patent ductus arteriosus or asymmetric perimembranous VSD device. However, we used a symmetrical muscular VSD device in all cases described in this report. Use of the antegrade technique for the first few patients with VSD resulted in prolonged procedural and fluoroscopic times and frequent slippage of the device into the right ventricle. Subsequent use of the retrograde technique shortened the procedural time and allowed for easier closure of the perimembranous VSD. We performed retrograde closure of perimembranous VSDs using a symmetrical muscular VSD device in 130 patients. We obtained a high rate of successful deployment (88.5%) and a low rate of complications (6.0%). We also achieved shorter procedural and fluoroscopic times than those associated with the antegrade technique.

Combination of right and left ventricular outflow tracts obstruction is extremely rare. Neonates with combined aortic stenosis (AS) and pulmonary stenosis (PS) present in critical condition and required urgent treatment. The management approach is not well defined. We report five female neonates with combined AS and PS presented to our institute in the last 5 years, age (1-18 days), weight (2.2-3.4 kg). Two had associated muscular ventricular septal defects. The mean Doppler gradient across the aortic valve (AV) was 73 mmHg (53-105 mmHg) and across the pulmonary valve was 62 mmHg (44-76 mmHg). Three had balloon dilatation and one surgical repair. The fifth patient was managed conservatively, but had sudden cardiac death at age of 3 months. One patient arrived in shock and sepsis, underwent emergency balloon dilation of the AV in the ICU. Despite decreasing the gradient, she died next day after the procedure. The surviving children were well at median follow-up age of 3.4 years. This is an extremely rare combination which needs early intervention. The management approach is not well defined. Interventional catheterization is possibly the better option.

This report summarizes the authors' clinical experience with perventricular closure of the perimembranous ventricular septal defect (PmVSD) using the concentric occluder as a minimally invasive technique without cardiopulmonary bypass and following transesophageal echocardiography (TEE) guidance. Between July 2011 and March 2013, 61 patients with PmVSD underwent perventricular concentric device closure using a minimally invasive inferior sternotomy approach. The basal diameter of the PmVSD ranged from 2.5 to 7 mm. The diameter of the occlusion device waist ranged from 4 to 8 mm. A ventricular septal aneurysm or an adhesive tricuspid valve was present in 49 patients. Multiple orifices in the aneurysm were treated in ten patients, including dispersed orifices in four patients and comparatively concentrated orifices in the remaining six patients. The occlusion devices were deployed via the right ventricle with TEE guidance and no cardiopulmonary bypass. Complete shunt occlusion was achieved for all the patients in the operating room. The orifices of the ventricular septal aneurysm were closed in 46 patients, with the left disc of the concentric devices placed in the aneurysms, whereas the PmVSDs in the remaining 15 patients were closed from the ventricular septal defect (VSD) basal part following the rule that the upper rim of the PmVSD be more than 2 mm from the aortic valve. Ventricular premature beats occurred in one patient. The follow-up period ranged from 1 to 21 months (median 13.5 ± 8.2 months), and the patients had stable heart function postoperatively. None of the patients had more than mild valvular regurgitation, and no worsening regurgitation was observed in those who had tricuspid or aortic regurgitation before surgery. No complete atrioventricular block, position shift of the occlusion devices, thrombosis, or residual VSD occurred during the follow-up period. The perventricular concentric device closure of PmVSD with an inferior sternotomy in selected patients is a safe, feasible, and simple treatment. The concentric device is easier to anchor than the eccentric device and has proved to be a reasonable choice for PmVSD occlusion, especially in patients with a perimembranous aneurysm formation or PmVSD extending to the inflow tract.

Gerbode defect, a left ventricle to right atrium (LV-RA) communication, is usually congenital. Acquired LV-RA communications are rare and only few case reports of successful trans-catheter closure have been published though none of them were on infants. We hereby report a rare case of LV to RA shunt acquired following surgical repair of Tetralogy of Fallot (TOF). The defect was successfully closed percutaneously with an Amplatzer duct occluder. This is the first reported case of device closure of an acquired Gerbode defect in an infant.

The arterial switch operation (ASO) remains a challenging procedure, especially in cases with a complicated coronary anatomy. In recent years, the autologous flap extension technique has been used for coronary implantation in complicated ASOs. Operative techniques and indications are discussed in this report. From January 2006 to June 2011, ASO with the autologous flap extension technique for coronary implantation was used for 21 patients, including five cases involving transposition of the great arteries (TGA) with an intact ventricular septum, eight cases involving TGA with a ventriculoseptal defect, five cases of Taussig-Bing anomaly, and three cases involving corrected TGA. Age at operation varied between 3 and 314 days (median, 110 days). Body weight varied between 3.1 and 14 kg (median, 5.4 kg). Three patients underwent a two-stage operation. In all the patients, the main trunk of the right coronary artery or the dilated right ventricular conus branch originated from the left- or right-facing sinus and followed an abnormal course of anterior looping to the aorta. The operative techniques included a long coronary button excised from the aorta and a pedicle flap on the pulmonary artery (neoaorta) cut as a cuff extended to the button of the coronary artery with equal distance. The side edges of the flap and the button were sutured to each other, thus forming an extension tube that lengthened the coronary artery. No in-hospital operative mortality occurred. Delayed sternum closure occurred in five cases. The average mechanical ventilator time was 101.6 h. The average intensive care unit stay was 9.5 days. Follow-up evaluation after discharge was complete in 17 cases. Growth and development were improved in all patients. No ischemic electrocardiographic changes occurred. One patient underwent reoperation for supravalvular pulmonary stenosis 2 years later. The autologous flap extension technique for coronary implantation in complicated ASOs can decrease hospital mortality due to abnormal coronary arteries, especially for patients undergoing two-stage ASOs or patients whose main trunk of the right coronary artery or dilated right ventricular conus branch originates from the left- or right-facing sinus and follows an abnormal course of anterior looping to the aorta.

A pacemaker insertion through the azygos vein in a child is presented. When both the low weight of the child and mediastinitis precluded standard approaches, the azygos approach proved to be an alternative and a straightforward bailout solution.

Paroxysmal non-reentrant supraventricular tachycardia due to double ventricular response through antegrade dual atrioventricular nodal pathways by a single atrial excitation has been reported in limited adult cases but not in pediatric patients with structurally normal hearts or with congenital heart defects. We report the case of a 5-year-old boy with non-reentrant double-ventricular response (DVR) supraventricular tachycardia (SVT) after repair of ventricular septal defect. To the best of our knowledge, this is the first pediatric report about an electrophysiologic study and successful selective radiofrequency (RF) catheter ablation for the slow pathway leading to tachyarrhythmia that is difficult to manage medically. In conclusion, non-reentrant DVR SVT is a rare form of tachycardia that should be considered in the differential diagnosis of SVT in children after repair of congenital heart diseases. It is amenable to mapping and RF catheter ablation.

Ventricular tachycardia (VT) arising from the coronary sinus cusps and mimicking right-ventricular morphology is a rare entity. In this report, we report the successful cryoablation of left-coronary sinus cusp VT.

This report describes the case of an 18-year-old post-Senning patient noted to have a residual ventricular septal defect and severe tricuspid regurgitation together with a shunt from the right atrium to the left ventricle detected several years after surgery.

This report describes a 3-year-old boy who presented with hemoptysis and a history of congenital heart disease detected at the age of 3 months. Clinical and laboratory evaluation showed a large true congenital pulmonary artery aneurysm of the left main pulmonary artery occupying the entire left hemithorax and two small aneurysms in the right lung. In addition, a restricted ventricular septal defect was detected. It was evident on clinical and histologic grounds that the multiple aneurysms seen in this child stemmed from congenital weakness of the arterial wall in conjunction with increased pulmonary blood flow and elevated pulmonary artery pressure.

Background: Double-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum., Methods/results: A full-term infant girl (2.9 kg) referred for hypoxia (80% with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation., Conclusions: This report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.

Closure of perimembranous ventricular septal defects (PmVSDs) [4] with the original Amplatzer membranous VSD occluder (mVSD1) has been associated with an increased risk of complications, the most notable of which has been complete heart block. This has led to the introduction of a new device to close PmVSDs. The authors describe their experience with the new Amplatzer membranous VSD occluder (mVSD2) in two patients and critique the new device design and delivery system, comparing it with the mVSD1 occluder. Two patients underwent attempted closure of PmVSDs with the new mVSD2 device. One patient had successful closure of the defect with no residual shunt and no evidence of heart block at follow-up evaluation. Trace to mild aortic insufficiency was observed at the time of closure and at the last follow-up visit. The rhythm remained sinus. In the second patient, the device was placed but removed before release because of inability to orient the device in optimal position. Percutaneous closure of PmVSD is feasible with the new mVSD2 occluder. The change in the design may reduce the incidence of complete heart block. It remains to be seen whether the device will be suitable for smaller patients.

Objective: To investigate the value of spatiotemporal image correlation (STIC) technique in the ventricular septal defect diagnosis., Methods: A total of 1,163 fetuses were enrolled in this study, diagnosed by fetal echocardiography and analyzed by STIC technique. We obtained effective STIC volumes from the fetus according to the Goncalves' report, and judged fetal cardiac structure from STIC volume information again with a double-blind method. Another echocardiography was obtained during neonatal or infant period as follow-up., Results: (1) As much as 1,062 cases were diagnosed to have normal fetal heart by fetal echocardiography, 43 cases had congenital heart disease without VSD, and 58 cases had VSD [21 cases (36.2%) were simple VSD and 37 cases (63.8%)were VSD with other heart malformation]. (2) Three fetal VSD cases (0.26%) were missed and one normal case was diagnosed as VSD after two-dimensional (2D) echocardiography. STIC technique corrected the diagnosis for two cases: one case of VSD after fetal 2D echocardiography was confirmed to be normal with STIC and neonatal heart examination; one case that was diagnosed as normal by fetal 2D echocardiography was confirmed to have VSD with STIC and neonatal heart examination., Conclusions: The special value of STIC technique in the diagnosis of VSD had been confirmed by this study, and it had been proved as the best additional method to fetal echocardiography.

Aortic regurgitation is a common complication of ventricular septal defects. The most common mechanism is right or noncoronary cusp prolapse. Other mechanisms are right or noncoronary cusp fibrosis resulting in thickening and restricted motion of the leaflets or infective endocarditis leading to cusp perforation. We describe a case of subacute and severe aortic regurgitation due to noncoronary cusp prolapse resulting in the development of a large aneurysm and rupture of the noncoronary sinus of Valsalva into the right atrium.

This report describes a premature newborn with pulmonary atresia and an intact ventricular septum who presented with a significant fistula between the right ventricle and the left anterior descending coronary artery (LAD). The right ventricle was not decompressed to prevent myocardial damage. After 2 months, the fistula had closed spontaneously. The pulmonary valve was opened, and biventricular circulation was achieved.