Restrictive Ventricular Septal Defect Research Articles

Experience of Morphologic Left Ventricle Training in Congenitally Corrected Transposition of the Great Arteries in Children: Evaluation of the Operation Results

The aim. To evaluate the efficacy of morphologic left ventricle (mLV) training through pulmonary artery banding (PAB) in pediatric patients with congenitally corrected transposition of the great arteries.
 Materials and methods. From 2003 to 2023, 12 patients underwent PAB for mLV training at the Ukrainian Children`s Cardiac Center. The mean age of the patients was 30.3 ± 25.1 months, the mean weight was 11.6 ± 5.2 kg. Hemodynamic parameters were assessed to determine the size of the band applied to the pulmonary artery (PA) trunk during the operation. Intraoperative transesophageal echocardiography was performed in 6 patients. Transthoracic echocardiography (TTE) was performed in the early postoperative period. In the remote period, all the patients underwent TTE and X-ray angiography.
 Results. During PAB, the mLV pressure averaged 58 ± 12.6% (from 40% to 80%) of the systemic arterial pressure. PAB dilatation was required for one patient in the early postoperative period (postoperative day 2) because of significant hemodynamic changes. In the early postoperative period, the maximum gradient across PA measured by TTE averaged 39.3 ± 10.0 mm Hg (from 25 to 60 mm Hg). There was no hospital mortality.
 The follow-up period averaged 47.2 ± 26.0 months (8 to 113 months). In the long-term observation period, 2 patients (16.6%) died, one 8 months and the other 5 years after the operation. The double switch operation was performed in 6 patients, on average, 48.2 ± 6.9 months after PAB. According to angiography, the mLV pressure before the double switch operation was 90.2 ± 11.6% of that in the systemic right ventricle. The end-diastolic pressure in the mLV averaged 14 ± 4.1 mm Hg. The peak pressure gradient across the PA measured by echo was 81.3 ± 28.8 mm Hg. The mLV mass index averaged 62.9 ± 8.8 g/m2, the mLV ejection fraction averaged 61.2 ± 2.9%. Overall radiographic and echocardiographic data in these patients indicated that mLV is trained and capable of performing the role of a systemic ventricle after anatomical correction.
 Conclusions. PA banding as a method of mLV training can be used in patients with congenitally corrected transposition of the great arteries with intact ventricular septum, restrictive ventricular septal defects and severe tricuspid regurgitation.
 To evaluate the effectiveness of PAB for mLV training, a multimodal diagnostic approach is used, including non-invasive and invasive methods such as TTE, transesophageal echocardiography, and cardiac catheterization. The optimal age for PAB in patients of this group for the purpose of training the LV was on average 2-2.5 years.

An alternative surgical approach for isolated pulmonary valve infective endocarditis secondary to restrictive ventricular septal defect: a case report

BackgroundNative pulmonary valve (PV) infective endocarditis (IE) is a rare condition with an incidence of 1.5–2%. Although medical therapy is the mainstay of treatment, surgical intervention is still indicated in cases that fail to respond to antibiotics. To date, there is lack of consensus about the best surgical approach for isolated native PV IE. While valve repair is sometimes feasible, most of the cases require valve replacement depending on the extent of tissue damage. A stented bioprosthesis can be used when infection is confined to the valve leaflets. However, extension of damage to the pulmonary root or right ventricular outflow tract usually requires debridement and root replacement.Case presentationA 30-year-old lady with a long history of restrictive ventricular septal defect (VSD) presented with fever and shortness of breath shortly after vaginal delivery that was diagnosed as isolated PV IE with pulmonary septic emboli. After 1 week of antibiotic therapy, there was no response with persistent infection and complete valve destruction. Heart team discussion recommended PV replacement using a Freestyle valve along with VSD repair. On follow-up after 1 year, she was doing fine with well-functioning Freestyle valve.ConclusionUnrepaired restrictive VSD can be a predisposing factor for native PV IE. A Freestyle valve in the pulmonary position is a valid option for such cases, with good early outcomes and reasonable durability, particularly when there is extensive tissue infection and homograft is not available. More evidence is still required to evaluate the long-term outcomes of PV Freestyle in cases of IE.

Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining. This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present). Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation. The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

Laubry-PEZZI Syndrome Surgical Repair in Adult: A Case Report

The presence of aortic regurgitation (AR) in the setting of the ventricular septal defect (VSD) has always been a management challenge. A 21-year-old male patient presented to the Cardiovascular surgery department with a restrictive perimembranous VSD with severe aortic regurgitation (AR). VSD was first diagnosed at 3 years of age. The surgical correction was decided when he was symptomatic of severe aortic regurgitation. Trans-aortic approach was performed to close the ventricular septal defect and correct the aortic regurgitation simultaneously. Surgical management was followed by a complete heart block.

Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid valve: repair with accessory valve preservation

BackgroundRestrictive ventricular septal defect resulting in suprasystemic right ventricular pressure is a rare entity in tetralogy of Fallot patients. It is often caused by accessory tricuspid valve tissue protruding through the defect. Surgical resection of the accessory tricuspid valve has been recommended, but the risk of tricuspid regurgitation caused by the resection has not been widely discussed.Case presentationA 5-month-old male who had been diagnosed with tetralogy of Fallot was referred for progressive decline in oxygen saturation. Preoperative echocardiogram revealed restrictive ventricular septal defect and severe pulmonary stenosis, suggesting suprasystemic right ventricular pressure (> 160 mmHg). Intraoperatively, accessory tricuspid valve tissue was found to be attached to the superior edge of the ventricular septal defect and partially occlude the aortic valve. The risk of tricuspid regurgitation caused by accessory tricuspid valve resection was high due to the complicated relationship between the true tricuspid valve and the accessory tricuspid valve. Therefore, the accessory tricuspid valve was preserved on the right ventricular side of the ventricular septum to avoid the risk of tricuspid regurgitation. Postoperative echocardiogram did not show tricuspid regurgitation or left ventricular outflow tract obstruction, and the patient was discharged to home on postoperative day 35 without major complications.ConclusionsWe herein reported a high-risk patient of tetralogy of Fallot with restrictive ventricular septal defect caused by accessory tricuspid valve who successfully underwent definitive repair with accessory tricuspid valve preservation considering the risk of tricuspid regurgitation.

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction

BackgroundStudies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era. MethodsThis is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA–LVOTO, or double-outlet right ventricle TGA type–LVOTO from June 2004 to June 2021. ResultsThere were 34 patients stratified by Nikaidoh (n = 16) or Rastelli (n = 18) operation. The incidence of all postoperative complications and mortality was low, and the incidence of complications between the groups was similar. Patients were more likely to have undergone a Nikaidoh than a Rastelli if they had a pulmonary annulus >5 mm (87.5% vs 11.1%), anteriorly/posteriorly oriented great vessels (88% vs 8%), remote (80% vs 11%) or restrictive (75% vs 6%) ventricular septal defect, and right ventricular hypoplasia (50% vs 0%; all, P < .05). The resulting rates of reoperation were similar between the groups (44.0% vs 37.5%; P = .24) and largely composed of conduit replacements in the Rastelli patients and valvular repairs or replacements in the Nikaidoh group. Rates of catheter-based interventions were also similar. ConclusionsThese findings suggest that for the optimal treatment of conotruncal anomalies with discordant ventriculoarterial connections, procedural selection should be based on pathoanatomic criteria that can ensure patients undergo the operation most suited to their anatomy.

P406 LAUBRY–PEZZI SYNDROME: EVERYTHING IS NOT AS IT SEEMS

Abstract Introduction Laubry–Pezzi syndrome is a congenital heart disease characterized by the prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD), mostly supracristal, leading to severe aortic regurgitation (AR). It has been reported nearly always in childhood, the Venturi effect is the predominant factor and onset symptoms may be variable. Case Report A 61–years–old male was admitted to our intensive case unit for chest pain radiated to interscapular region and exertional dyspnea. Hemodynamic parameters were normal. At ECG sinus rytm with heart rate 92 bpm and non–specific repolarization abnomalities were reported. Hs troponin I was 276 ng/L, myoglobin 30.2 ng/mL and BNP 325 pg/mL. Transthoracic echocardiography showed a dilated left ventricle (LV EDV 107 mL/m2) with normal ejection fraction (55%), severe AR without aortic root dilation and a suspicious shunt left to right at level of interventricular septum. Therefore, transesophageal echocardiography was performed and confirmed severe AR by probable bicuspid valve and right cusp prolapse, in absence of calcifications and/or vegetations by endocarditis, restrictive perimembranous VSD (maximum diameter 0.6 cm) and ostium secundum ASD with shunt L–R. Right chambers were not dilated and non invasive systolic pulmonary artery pressure was 43 mmHg. Coronary angiography was performed and showed a significant stenosis of distal left anterior descending (LAD) artery and of proximal circumflex artery. Right heart catetherization excluded significant high values of pulmonary pressures (mPAP=18 mmHg), of pulmonary capillary wedge pressure (9 mmHg), with a mild increase of pulmonary vascular resistances (1.16 HRU; nv &amp;lt; 96). Coronary artery bypass grafting (left internal mammary artery on LAD and Y–graft radial artery on obtuse marginal artery), aortic valve replacement with bioprothesis (Inspiris Resilia 23 mm), ASD closure by direct suture and VSD closure with pericardial patch were performed. No post–surgical complications were reported. Interestingly, during the replacement of aortic valve, it resulted tricuspid, confirming the suspicion of Laubry Pezzi syndrome. Conclusions our case was object of great debate because restrictive VSD and severe AR were not previously known, were diagnosed in middle age and until then paucisymptomatic. Laubry–Pezzi syndrome is a very rare disease that may explain controversial cases of severe AR.

Practice Patterns in the Management of Pressure Restrictive Perimembranous Ventricular Septal Defects: A Multinational Survey.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z-score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z-score and QP:QS ratio cut-offs (p-value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.

Stenting of Ductus Arteriosus for Duct-Dependent Pulmonary Circulation: An Emergency and Life Saving Procedure

BACKGROUND: Critical congenital heart defect with ductal-dependent pulmonary flow presents as a life-threatening condition. The patency of ductus arteriosus is required to supply blood flow into the lung. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. CASE DESCRIPTION: We present full-term newborns who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male newborn had Tetralogy of Fallot-Pulmonary Atresia, restrictive Ventricular Septal Defect (VSD) and patent ductus arteriosus (PDA). Patient B, a 5-days-old female newborn had Tricuspid Atresia, restrictive VSD, PDA, and multiple congenital anomaly. Patient C, a 2-days-old female newborn had PA-IVS, PDA with stretched Patent Foramen Ovale (PFO). Prostaglandin-E1 infusion was started at first and stopped 6 hours prior to the procedure. All patients underwent ductus arteriosus stenting via femoral artery approach with drug-eluting stent. Pulmonary vascular markings were increased and oxygen saturations were improved in all of the patient. 5-month follow up, patients were in stable condition and prepared for surgical correction. DISCUSSION: Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides bridging palliation until the time of definitive surgery. The effectiveness of the procedure was highlighted by the fact that all the patients showed a significant improvement in arterial saturation and pulmonary vascular marking. CONCLUSION: Stenting of ductus arteriosus is feasible and safe with great result. Early detection and timely management are imperative to save the life. KEYWORDS: critical congenital heart defect, ductus arteriosus, ductal stenting, ductal-dependent pulmonary blood flow

Abstract 15341: Persistent Fifth Arch as an Aortopulmonary Connection in a Case of Tetralogy of Fallot

Persistent fifth aortic arch is a heterogeneous vascular anomaly with variable anatomical presentations and physiological consequences. This unusual embryological remnant represents a management challenge, particularly in critical right sided obstructive heart lesions where accurate delineation of sources of pulmonary blood flow is essential. This is a newborn 2 kg premature male born at 34 weeks of gestation with prenatally diagnosed tetralogy of Fallot. Post-natal echocardiogram confirmed diagnosis with evidence of moderate-to-severe pulmonary stenosis, restrictive ventricular septal defect, and a right aortic arch. CT angiogram ordered to delineate the head and neck vessel anatomy showed an exceedingly rare finding of an isolated connection of the left subclavian artery (LSCA) to the main pulmonary artery (MPA) via a left sided ductus arteriosus. Blood flowed from the common carotid arteries, through the circle of Willis, and, in a retrograde direction, into the LSCA. Prostaglandin E was initiated due to concern for ductal dependent pulmonary blood flow, but after clinical observation it was discontinued without subsequent onset of cyanosis. Repeat echocardiograms were reassuring in showing persistence of the left ductus arteriosus. The patient was discharged home at 5 weeks-old and at 4 months-old he underwent complete repair after developing progressive desaturations and worsening sub-valvar and valvar pulmonary stenosis. The surgical repair included ventricular septal defect closure, transannular patch, extensive right ventricular myomectomy and reimplantation of LSCA onto the left common carotid artery. The post-operative course was uncomplicated. This rare case of persistent fifth aortic arch with retrograde flow to the LSCA demonstrates the complementary roles of noninvasive imaging modalities in guiding medical therapy and surgical management. This unusual blood flow pattern and initial concern for duct dependent pulmonary blood flow complicated early management, but a conservative approach with repeat imaging allowed for eventual complete repair.

Abstract 11386: Double Whammy Complicating Management of Pulmonary Hypertension

Introduction: When presented with challenging cardiac cases, in addition to obvious diagnoses, it is critical to maintain high level of suspicion about other subtle conditions. We describe one such case of pulmonary arterial hypertension (PAH) from severe mitral stenosis (MS) and another less obvious congenital cardiac condition that significantly impacted the clinical course. Case: 32-year-old immigrant male with known heart murmur presented with palpitations due to atrial fibrillation. His resting oxygen saturation was 95% in all extremities and he had no clubbing. He had pectus carinatum and scoliosis, prominent right ventricular lift, normal S1, loud S2, opening snap, rumbling diastolic murmur at the apex, and an ejection systolic murmur at the left upper sternal border. Echocardiogram revealed normal biventricular size and function, severely enlarged left atrium, rheumatic severe MS, moderate mitral regurgitation, a restrictive ventricular septal defect (VSD), and elevated right ventricular systolic pressure. Cardiac catheterization demonstrated mixed pre- and post-capillary PAH with mean pulmonary artery pressure of 80 mmHg, wedge pressure 30 mm Hg and pulmonary vascular resistance of 3.0 woods unit which was underestimated. Since pre-capillary component of the PAH could not be entirely explained by the small VSD, a congenital cardiac CT was performed revealing moderate sized patent ductus arteriosus (PDA). Further management decisions were complicated since MS was not amenable to percutaneous intervention and surgical risks were high given severe pre-capillary PAH- management of which with pulmonary vasodilators was itself high risk in the setting of severe MS and high wedge pressures. After optimal medical management, a multidisciplinary team carefully considered the risks and benefits of multiple interventions. Ultimately, the patient underwent surgical mitral valve replacement, fenestrated patch closure of the PDA, and then initiation of pulmonary vasodilators. The patient continues to do well on oral pulmonary vasodilator therapy 6 months after surgery. Conclusion: We describe an astute critical thought process of a multidisciplinary team utilized to manage a challenging case of mixed PAH from severe rheumatic MS and PDA.

P245 A RARE CAUSE OF OVERESTIMATION OF PULMONARY PRESSURE BY ECHOCARDIOGRAPHY: WHEN HEMODYNAMICS RELIEVES ECHOCARDIOGRAPHY AND ECHOCARDIOGRAPHY EXPLAINS HEMODYNAMICS

Abstract Echocardiography is the gatekeeper of the diagnostic workflow of pulmonary hypertension (PH). However, it lacks precision, and right heart catheterization (RHC) may be needed in selected cases. A morbidly obese 47–year–old woman (BMI= 58 Kg/m2) with dyspnea NYHA II was referred to our Center after a high probability of PH was found at echocardiography, this latter performed as a routine assessment before bariatric surgery. Despite a suboptimal acoustic window, the tricuspid regurgitant jet profile was well–defined, with an estimated systolic pulmonary artery pressure (PAP) of 100 mmHg. There were no overt echocardiographic signs of left heart disease. During RHC, mean PAP was just above normal values, with filling pressures at the upper limit of normal (Figure 1), high cardiac index (3.6 L/min/m2) and normal pulmonary vascular resistance (1.4 WU). The echocardiographic estimate of high systolic PAP was explained by an intraventricular RV pressure gradient of about 100 mmHg (Figure 2). Oximetry ruled out a significant intracardiac shunt. Since resting hemodynamics did not explain the patient’s symptoms, the patient pre–test probability of having heart failure with preserved ejection fraction (HFpEF) was intermediate–high, and PAWP was in a grey–zone. Accordingly, we performed an exercise RHC. The test eventually unmasked HFpEF, as witnessed by an abnormal increase of PAWP and LV end–diastolic pressure (Figure 1 panel C and D). Exercise capacity was mildly reduced (71% of predicted) due to peripheral limitation with normal cardiac and respiratory reserves. Transesophageal echocardiography was then performed. It showed a muscular obstruction below the infundibulum dividing the RV into a high–pressure apical portion and a low–pressure infundibulum (Figure 3). This rare congenital anomaly was also associated with a restrictive perimembranous ventricular septal defect. Double–chambered RV has been reported to present rarely in adults, but it might be a cause of false Doppler echocardiographic estimate of PAP. Based on a meticulous invasive hemodynamic characterization at rest and during exercise, integrated with cardiovascular imaging, we could perform such a diagnosis. This congenital anomaly did not fully explain the patient’s symptoms, which may better be attributed to cardiovascular complication of obesity (HFpEF). Accordingly, we referred the patient for bariatric surgery first, postponing the indication to cardiac surgery during the follow–up.

Spontaneous closure of an isolated congenital perimembranous ventricular septal defect in two dogs

BackgroundThough spontaneous closure of isolated congenital ventricular septal defects in humans is very common, it has been rarely reported in dogs.Case presentationA 4 month old Havanese dog and a 4.5 month old Chihuahua x Jack Russell terrier cross were presented for murmur evaluation to the authors’ institution. Both puppies were clinically healthy and had a loud systolic murmur on the right hemithorax. Echocardiography in both dogs revealed a small, isolated, restrictive perimembranous congenital ventricular septal defect. No echocardiographic signs of left ventricular volume overload or pulmonary hypertension were present. Re-check auscultation in both dogs revealed the absence of a murmur, and echocardiography showed no flow through the interventricular septum. In the 9 kg Havanese dog and the 4 kg mixed breed dog, spontaneous closure occurred at 13–17 months and 12–30 months, respectively.ConclusionsIn both dogs the spontaneous closure of a congenital perimembranous ventricular septal defect took place in a young adult age. The mechanism of closure remains unclear.

Transcatheter Versus Surgical Closure of Acute Ruptured Sinus of Valsalva Aneurysms with Associated Ventricular Septal Defects.

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 8years 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 ± 12.58years 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 ± 10.1mm and 50.1 ± 3.1mm, p = 0.46) were not different in the transcatheter and surgical groups. Mean end-diastolic volumes (110.5 ± 42. 1ml and 98.5 ± 37.5ml, 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.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

WeChat-assisted health education and preoperative care improve the mental state of parents of children with ventricular septal defect

ABSTRACT Many studies have shown that parents of children with congenital heart disease have more stress, anxiety and depression. This study was aimed to explore the effect of implementing WeChat-assisted health education and preoperative care on parents of children with the restrictive ventricular septal defect to improve the psychological state. A prospective randomized controlled study was conducted in a provincial hospital in China. Participants were randomly divided into an intervention group and a control group to explore the psychological state of parents of children with the restricted ventricular septal defect. Before surgery, the state-trait anxiety inventory scale score (STAI) of the WeChat group were 26.8 ± 8.2 and 27.3 ± 7.0, which were significantly higher than those of the leaflet group (37.6 ± 12.9 and 39.3 ± 11.7). Compared with the STAI score at the first visit, the WeChat group preoperative score was significantly lower (P < 0.05). The rate of loss to follow-up in the WeChat group (0%) was significantly lower than that of the leaflet group (14.3%). The complication of the leaflet group was significantly higher than that of the WeChat group. Health education and preoperative care for parents of children with restrictive ventricular septal defect through WeChat can effectively improve the parents’ mental state and reduce the incidence of complications and the rate of loss to follow-up.

Percutaneous closure of ventricular septal defect using LifeTechTM Konar-MF VSD Occluder: initial and short-term multi-institutional results.

Transcatheter ventricular septal defect closure remains a complex procedure with potential complications like complete heart block and aortic regurgitation. The ideal device design for such intervention is still evolving. To assess the safety, efficacy, and short-term outcome of ventricular septal defect closure using LifeTechTM multifunctional (KONAR-MFTM) VSD Occluder. In a multicenre study, 44 patients with haemodynamically significant, restrictive ventricular septal defects underwent closure with the KONAR-MFTM device from April, 2019 to March, 2020. Clinical, echocardiographic, and angiographic data were collected and reviewed. Patients were followed up at 1, 3, 6, and 12 months. The median age and weight were 8 (1.7-36) years and 20 (11-79) kg. Of 44 patients, 8 (18%) had a high muscular and 36 (82%) had a perimembranous defect, of which 6 had mild prolapse of the right coronary cusp. The median ventricular septal defect size was 8.8 (3.9-13.4) mm. A retrograde approach was adopted in 39 (88.6%) patients. Nine patients (20.5%) had a small residual leak and there was a slight increase in aortic regurgitation in one patient. One device, which embolised to pulmonary artery was retrieved, and the defect was closed with a larger device. At a median follow-up of 13 (5-18) months, the residual leak persisted in 1 (2.3%) patient. Mild aortic regurgitation in one patient remained unchanged. There were no major complications. Percutaneous closure of ventricular septal defect using KONAR-MFTM device is safe and effective in short and midterm follow-up including selected patients with perimembranous defect and mild prolapse of the right coronary cusp.

WeChat-assisted pre-operative health education improves the quality of life of parents of children with ventricular septal defects: A prospective randomised controlled study.

This study aimed to explore the effect of implementing pre-operative WeChat-assisted health education for parents of children with restrictive ventricular septal defects to improve their quality of life. A prospective randomised controlled study was conducted in a provincial hospital in China. Participants were randomly divided into the intervention group (WeChat group, n= 35) and the control group (leaflet group, n= 35). The quality of life of parents of children with restricted ventricular septal defects was studied. Compared with the leaflet group, the WeChat group had a significantly lower score for the pre-operative Self-Rating Anxiety Scale (SAS) (P< 0.05). The pre-operative SAS score in the WeChat group was significantly lower than that at the first visit (P< 0.05). However, the SAS score in the leaflet group was similar at the time of preoperation and the first visit (P> 0.05). The pre-operative scores of the World Health Organization Quality of Life-BREF (WHOQOL-BREF) scale in the physiological, psychological, social, and environmental fields and the total life quality score in the WeChat group were significantly higher than those of the leaflet group (P< 0.05). Implementing pre-operative WeChat-assisted health education for parents of children with restrictive ventricular septal defects can effectively relieve their anxiety and improve their quality of life.

Prenatal diagnosis of tetralogy of Fallot with a restrictive ventricular septal defect

Objective Tetralogy of Fallot (TOF) usually presents with a large non-restrictive ventricular septal defect (VSD). Case(s) We hereby report a rare case of prenatal diagnosis of TOF with a restrictive VSD secondary to redundant tricuspid valve tissue. Partial occlusion of the VSD resulted in prenatal progression to myocardial hypertrophy and postnatally to right heart failure with difficult management. Conclusion Prenatal signs of cardiac dysfunction in TOF should prompt a detailed exam of the VSD. Parents and neonatologists must be advised and delivery must be guaranteed in a tertiary center.