Simultaneous Transcatheter Closure of a Ventricular Septal Defect and Pulmonary Valvuloplasty: A Case Report.

BACKGROUND Congenital heart diseases (CHDs) are the most common form of birth defects, affecting the structure and function of neonatal hearts. Pulmonary valve stenosis (PVS) and ventricular septal defects (VSD) are 2 of the more prevalent forms, both of which can lead to significant morbidity if left untreated. The emergence of transcatheter techniques has revolutionized the therapeutic landscape, presenting minimally invasive yet effective alternatives to open-heart surgery and significantly reducing associated patient morbidity and recovery time. CASE REPORT The presented case details the management of a 19-year-old man with complex CHDs, highlighting the nuanced decision-making process that led to a transcatheter approach. The patient's clinical presentation, marked by symptoms reflective of significant cardiac compromise, demanded a tailored approach that utilized the latest advancements in non-surgical intervention. The successful closure of the VSD with an Amplatzer device and the resolution of PVS via balloon valvuloplasty were achieved without complications, showcasing the potential of these techniques in managing similar cases. The post-intervention period was marked by a noteworthy recovery, confirming the procedural efficacy and enhancing the patient's quality of life. CONCLUSIONS The favorable outcome of this case highlights the pivotal role of transcatheter interventions in treating complex CHDs and suggests a shift towards less invasive approaches in cardiac care. This case contributes valuable insights to the existing body of evidence, reinforcing the potential of transcatheter techniques to become the preferred treatment modality. With promising immediate and short-term results, these techniques highlight the need for continued research into their long-term efficacy and application across diverse patient demographics.

Repair of a fistula between the aorta and right ventricular outflow tract secondary to infective endocarditis of a unicuspid aortic valve and previously repaired ventricular septal defect.

BACKGROUND: Infective endocarditis of the aortic valve can result in a wide range of destructive pathology beyond the valve leaflets and annulus which require careful surgical planning to provide appropriate debridement and reconstruction. Failure to do so can result in a failure of surgical treatment, recurrent infection and cardiac failure with concomitant high morbidity and mortality. CASE REPORT: We describe the case of a 45-year-old male with previous patch repair of a ventricular septal defect, who was diagnosed with sub-acute bacterial endocarditis of the native aortic valve and developed a new fistula from the aorta to the right ventricular outflow tract which. This was managed surgically. CONCLUSION: This unique case highlights another spectrum of infective endocarditis with a unique approach to repair and management.

En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.

Clinical Outcomes and Echocardiographic Predictors of Reintervention After Interrupted Aortic Arch Repair.

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 (CSAcm2/BSAm2) ≤ 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.

Simultaneous surgical repair of a cardiac myxoma causing left ventricular outflow tract obstruction and a ventricular septal defect in a small dog.

Background: Cardiac myxomas are benign tumours that can occur in any heart chamber or valve. They are extremely rare in dogs. We present a novel case involving a cardiac myxoma in the left ventricular outflow tract (LVOT) and a ventricular septal defect (VSD) in a small dog. Case Description: A female miniature dachshund (age, 7 months; weight, 2.88 kg) presented with growth insufficiency, lethargy, and a cardiac murmur. Echocardiography revealed a small polypoid mass in the LVOT and a membranous VSD. Simultaneous surgeries were performed to resect the mass (aortotomy) and close the VSD (right atriotomy) using low-flow cardiopulmonary bypass with surface-cooling hypothermia and retrograde cardioplegia. The tumour was histopathologically identified as a myxoma. The dog survived with no cardiac complications for 11 years after surgery. Conclusion: To our knowledge, this is the first report of ante-mortem diagnosis and simultaneous surgical repair of a cardiac myxoma obstructing the LVOT and a VSD in a small-breed dog. In addition to describing this complicated case, this report presents what we believe is the first reported use of retrograde cardioplegia during open-heart surgery in a small-breed dog.

Complete Percutaneous Retrieval of Migrated VSD Occluder Device from the Pulmonary Artery: Management of a Catastrophic Complication.

Transcatheter closure of muscular ventricular septal defects (VSD) remains a safe and effective method with low complication rates. However, device migration can pose a significant challenge to interventional cardiologists due to potential mortal consequences. A 21-year-old female presented to our clinic with exertional dyspnea and was diagnosed with a muscular VSD. The defect was percutaneously closed using an Amplatzer occluder device. On the first post-procedural day, the patient experienced repeated episodes of coughing and mild hemoptysis. Imaging revealed migration of the VSD occluder device to the right pulmonary artery (PA). Percutaneous retrieval of the device was then decided upon. The right PA was accessed using a hydrophilic guidewire and a pigtail catheter. This catheter was exchanged for an 8-Fr sheathless guide catheter, and a 6-Fr Judkins right catheter was advanced into the right PA through the sheathless guide catheter using the mother-and-child technique. Multiple attempts using a snare were made to retrieve the migrated device. Eventually, the proximal marker point, the hub of the device, was grasped and pulled back from the PA, then externalized through the sheath without the need for surgical cutdown. Our report represents a case of complete percutaneous retrieval of an embolized VSD occluder device from the PA.

Successful interventional occlusion of muscular ventricular septal defect in a dog.

Ventricular septal defect (VSD) is a rare congenital heart disease in dogs. Hemodynamically important interventricular defects must be closed to improve the prognosis. This case report describes successful interventional transcatheter closure of a muscular VSD in a young Maltese and poodle mixed-breed dog with a large muscular interventricular defect (~5 mm in diameter) with a high rate of left-to-right shunt flow. The VSD was closed with a customized Amplatzer-type VSD occluder via a percutaneous transvenous (jugular) approach. We concluded that interventional occlusion of a muscular VSD with an Amplatzer-type occluder is a viable treatment option for dogs. A regular follow-up study for this dog is ongoing and has not detected complications. Key clinical message: Interventional occlusion of a muscular VSD with an Amplatzer-type occluder is a viable treatment option for dogs.

Occlusion interventionnelle réussie d'une communication interventriculaire musculaire chez un chien. La communication interventriculaire (VSD) est une maladie cardiaque congénitale rare chez le chien. Les anomalies interventriculaires hémodynamiquement importantes doivent être fermées pour améliorer le pronostic. Ce rapport de cas décrit la fermeture interventionnelle réussie par cathéter d'un VSD musculaire chez un jeune chien de race mixte (maltais et caniche) présentant un défaut interventriculaire musculaire important (~5 mm de diamètre) avec un débit de shunt élevé de gauche à droite. Le VSD a été fermé avec un obturateur VSD personnalisé de type Amplatzer via une approche trans-veineuse percutanée (jugulaire). Nous avons conclu que l'occlusion interventionnelle d'un VSD musculaire avec un obturateur de type Amplatzer est une option de traitement viable pour les chiens. Une étude de suivi régulière de ce chien est en cours et aucune complication n'a été détectée.Message clinique clé :L'occlusion interventionnelle d'un VSD musculaire avec un obturateur de type Amplatzer est une option de traitement viable pour les chiens.(Traduit par Dr Serge Messier).

Risk Factors of Long-Term Sequelae After Transcatheter Closure of Perimembranous Ventricular Septal Defect in Young Children.

BACKGROUND: Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years.Methods and Results: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure. CONCLUSIONS: Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.

Differential Clubbing and a Triad of Patent Ductus Arteriosus, Ventricular Septal Defect and Supravalvular Ring Mitral Stenosis: A Case Report.

It is not uncommon for congenital heart defects to occur in clusters. Those involving a right to left heart shunt commonly cause cyanosis and finger clubbing. Differential clubbing involving only the lower limb digits is a strong pointer to the presence of patent ductus arteriosus with reversal of shunt. We report a case of 25-year-old man with effort intolerance and differential clubbing. He was found to have the uncommon triad of patent ductus arteriosus, ventricular septal defect and supravalvular ring mitral stenosis. The presence of differential clubbing on a background of patent ductus arteriosus usually indicates a reversal of shunt and negates surgical intervention. This general rule may however not apply with co-existing mitral stenosis as the elevated pulmonary pressure may be predominantly post-capillary. The finding of mitral stenosis in a patient with patent ductus arteriosus and differential limb clubbing may signify a good prognostic surgical outcome.

Il n'est pas rare que des malformations cardiaques congénitales surviennent en clusters. Celles impliquant un shunt cardiaque droitegauche provoquent souvent une cyanose et un hippocratisme digital. L'hippocratisme digital différentiel touchant uniquement les orteils des membres inférieurs est un indicateur fort de la présence d'un canal artériel persistant avec inversion du shunt. Nous rapportons le cas d'un homme de 25 ans présentant une intolérance à l'effort et un hippocratisme digital différentiel. Il a été diagnostiqué avec la triade peu commune de canal artériel persistant, de communication interventriculaire et de sténose mitrale à anneau supravalvulaire. La présence d'un hippocratisme digital différentiel sur un fond de canal artériel persistant indique généralement une inversion du shunt et exclut une intervention chirurgicale. Cependant, cette règle générale peut ne pas s'appliquer en présence d'une sténose mitrale concomitante, car la pression pulmonaire élevée peut être principalement post-capillaire. La découverte d'une sténose mitrale chez un patient atteint de canal artériel persistant et d'un hippocratisme digital différentiel peut indiquer un bon pronostic pour l'intervention chirurgicale.

[Communication interventriculaire compliquant un infarctus de myocarde antérieur : un cas de fermeture percutanée]. / Ventricular septal defect complicating anterior acute myocardial infarction : A Case of transcatheter closure.

INTRODUCTION: Post-infarction ventricular septal defect (PIVSD) is one of the most serious mechanical complications of acute myocardial infarction (AMI). Over the last decade, percutaneous closure is increasingly undertaken, with results similar to cardiac surgery. We present a case of ST-elevated anterior AMI, complicated by apical PIVSD successfully treated with transcatheter closure. CASE REPORT: An 83-year-old man was hospitalized for chest pain occurred 18 hours before, during the night time. He was an active smoker. Clinical examination revealed normal heart sounds and pulmonary bibasilar crackles. ST-segment elevation with deep T waves inversion in anterior leads were detected on the electrocardiogram. A mildly-reduced ejection fraction (40%) was found by transthoracic echocardiogram. The patient underwent emergency coronary angiography, which revealed a subocclusive stenosis of the mid left anterior descending artery with a TIMI 2 flow, treated by balloon angioplasty and drug-eluting stent. Four days after revascularization, the patient developed an acute deterioration with signs of decompensated heart failure and a new holosystolic murmur with large irradiation. Inotropic agents' administration was required to maintain a precarious hemodynamic condition. A bedside Echo revealed an apical VSD, measuring 15 × 10 mm, with left-to-right shunting, and pulmonary hypertension. The patient was scheduled for transcatheter PIVSD closure. The procedure was performed under fluoroscopic guide. Two vascular access sites were placed, femoral arterial and right internal jugular vein. Through the right internal jugular vein, a 24-mm Amplatzer atrial septal occluder on a 9 French Amplatzer TREVISIO™ intravascular delivery system was advanced via right ventricle into the PIVSD. Contrast fluoroscopy was used to assess apposition and the degree of shunt reduction before release. Echocardiographic evaluation performed 48 hours later confirmed a correct apposition of the device with insignificant residual shunt. At 6 months follow-up, he was asymptomatic, with unchanged prosthetic findings. CONCLUSION: Percutaneous closure has been emerged as a valid cost-effective alternative to surgery and should be advised. However, debate remains on the optimal preprocedural optimization, timing of repair and modality of treatment.

Percutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.

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.

Ventricular septal defect following mitral valve replacement.

Mitral valve surgery (MVS), with repair preferred to replacement, is a common procedure for the treatment of severe primary mitral regurgitation related to leaflet prolapse. Structural complications after MVS include left ventricular outflow obstruction, paravalvular leak and atrial septal defect. Intraoperative transoesophageal echocardiography and predischarge transthoracic echocardiography (TTE) specifically screen for these complications. Ventricular septal defect (VSD), a known complication after aortic valve surgery, is rarely reported after MVS. Recently, unsuccessful valvuloplasty prior to replacement was suggested as a risk factor. We present such a case and explore mechanisms with advanced cardiac imaging. In this case, the patient was found to have an elongated membranous septum that likely predisposed her to septal injury. Finally, we provide guidance on specific transoesophageal/transthoracic echocardiography views to avoid a missed diagnosis.

How best can we name the channels seen in the setting of deficient ventricular septation?

Surgical repair of channels between the ventricles is enhanced when the surgeon knows precisely where to place a patch, or baffle, so as to restore septal integrity. The paediatric cardiologist should provide the necessary information. Communication will be enhanced if the same words are used to account for the structures in question. Currently, however, the same term, namely "ventricular septal defect," is used to account for markedly different areas within the heart. Closure of perimembranous defects found in hearts with concordant or discordant ventriculo-arterial connections restores the integrity of the ventricular septum, at the same time separating the systemic and pulmonary blood streams. When both arterial trunks arise from the right ventricle, in contrast, the surgeon when placing a baffle so as to separate the blood streams, does not close the channel most frequently described as the "ventricular septal defect." In this review, we show that the perimembranous lesions as found in hearts with concordant or discordant ventriculo-arterial connections are the right ventricular entrances to the areas subtended beneath the hinges of the leaflets of the aortic or pulmonary valves. When both arterial trunks arise from the right ventricle, and the channel between the ventricles is directly subaortic, then the channel termed the "ventricular septal defect" is the left ventricular entrance to the comparable space subtended beneath the aortic root. We argue that recognition of these fundamental anatomical differences enhances the appreciation of the underlying morphology of the various lesions that reflect transfer, during cardiac development, of the aortic root from the morphologically right to the morphologically left ventricle.

Preliminary experience with transcatheter closure of wind-sock-like perimembranous ventricular septal defects using Amplatzer Vascular Plug IV devices.

Amplatzer Vascular Plug IV (Abbott, USA) is usually used for the occlusion of abnormal tortuous vessels and has not been tried for the transcatheter closure of perimembranous ventricular septal defects with wind-sock morphology. Here, we report on three successful cases of perimembranous ventricular septal defect transcatheter closure using Amplatzer Vascular Plug IV. We did not observe residual shunting or new onset of complications during follow up. These preliminary positive results advocate the application and suitability of Amplatzer Vascular Plug IV for closing wind-sock-like perimembranous ventricular septal defects.

Bex-Nikaidoh operation and the impact of double root translocation on outcomes.

OBJECTIVES: The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes. METHODS: We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019. RESULTS: Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9-2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270-259) vs 209 min (IQR 179-281), P = 0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3-10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P = 0.03). CONCLUSIONS: The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients.