Trabecular Ventricular Septal Defects Research Articles

Hybrid perventricular muscular ventricular septal defect closure using the new multi-functional occluder

The hybrid perventricular approach for the closure of trabecular ventricular septal defects is an attractive treatment modality for small children. Worldwide experience has shown that procedure success is influenced by the defect anatomical accessibility, operators' expertise, and device technical features. In May 2018, a new promising device, the KONAR-Multi-functional™ ventricular septal defect occluder (Lifetech, Shenzhen, China), obtained CE-marking for septal defect transcatheter closure after the first-in-man implantation in 2013. Herein, this is the first report of successful perventricular closure of ventricular septal defect using this new device in a child with significant co-morbidities.

Surgical closure of multiple large apical ventricular septal defects: how we do it.

The management of apical ventricular septal defects continues to be challenging because of the difficulty in achieving a complete closure without a left apical ventriculotomy. In this study, we present our innovative technique of closing multiple and/or large muscular apical ventricular septal defects through a right atriotomy. We operated three patients with multiple apical muscular trabecular ventricular septal defects ("Swiss cheese") using a technique that involved exclusion of the right ventricular apex. Their ages ranged between 2 months and 13 years. The VSDs were approached through right atriotomy. The trans right atrial approach using a 5-0 polypropylene purse-string suture or a two-patch procedure is a novel method of closing large apical ventricular septal defects. It was found to be effective with no persistent residual defects and did not have the disadvantages of a ventriculotomy.

Antenatal diagnosis of left atrial isomerism and heterotaxy syndrome in fetus with Meckel-Gruber syndrome

We aimed to present a fetus with Meckel-Gruber syndrome (MKS) who had left atrial isomerism, heterotaxy syndrome and complete heart block. A 26-year-old healthy female was referred to our clinic in the 23rd week of her pregnancy. The fetus had multiple systemic anomalies including fetal heart. Fetal echocardiography revealed a horizontal liver, left-sided stomach and vena cava interruption with azygos continuation. There was also an apical trabecular ventricular septal defect, aorta and pulmonary artery arising from the left ventricle, pulmonary artery hypoplasia, pulmonary valve stenosis and left atrial isomerism. The heart rate was 46/min, consistent with third-degree atrioventricular block. Multiple anomalies including occipital encephalocele, bilateral polycystic kidneys, cleft lip, cleft palate, and polydactyly were also detected in the obstetric ultrasonography. The pregnancy was terminated in the 23rd gestational week based on the consensus of perinatology council. The autopsy examination confirmed the diagnosis of MKS, left atrial isomerism and heterotaxy syndrome. Although some cardiac defects have been reported previously in MKS fetuses, here we expand the cardiac spectrum of anomalies associated with MKS to include left atrial isomerism and heterotaxy syndrome.

Ventricular septal defect originating from an apical left ventricular pseudoaneurysm

We report the case of a 63-year-old man with an apical trabecular ventricular septal defect (VSD) with a restrictive unidirectional left-to-right shunt (peak gradient 80 mmHg), originating from a perfused, dyskinetic apical pseudoaneurysm (PA; 38 × 31 mm) primarily diagnosed by transthoracic echocardiography ( Panels A – C ). The communication between the left ventricular (LV) cavity and the …

Techniques and results in the management of multiple muscular trabecular ventricular septal defects

The management of patients with multiple muscular trabecular ventricular septal defects (VSDs) remains controversial. In the past two decades, innovative techniques including a right ventricular apical infundibulotomy and transcatheter, intraoperative and perventricular device closure have been exploited, and essential right atrial approach and limited apical left ventriculotomy have also been refined. However, specific management guidelines for this difficult disease have not been established. In this article, the benefits and drawbacks of each technique are reviewed and discussed. Primary repair for infants with multiple muscular trabecular VSDs was associated with good late outcomes. The right atrial approach was satisfactory for all muscular VSDs, excluding apical defects that were well seen through a limited apical ventriculotomy. Surgical closure of apical defects could be achieved safely and completely in early infancy through a limited apical left ventriculotomy or a right ventricular apical infundibulotomy. Further follow-up and prudent evaluations of ventriculotomy-associated morbidities are needed. Pulmonary artery banding should be limited to a small infant with complex associated defects. Percutaneous device closure, the most desirable option, is impractical due to limitations between the delivery system and access route. Intraoperative device closure appears less successful than device closure in the catheterization laboratory. Perventricular device closure has a significant advantage of being a non-bypass procedure approach. A less invasive strategy for "true" Swiss cheese septum is needed. All may have an important role, and results obtained by using these techniques are encouraging. These hybrid approaches will promise future success on management guidelines of multiple muscular trabecular VSDs.

Surgical management of muscular trabecular ventricular septal defects

Despite recent advances in diagnosis, surgical techniques, and postoperative care of children with congenital cardiac defects, muscular trabecular ventricular septal defects (VSDs) are still a therapeutic challenge. Among these defects, it is more difficult to achieve secure and complete closure of low trabecular or apical VSDs because of the presence of numerous muscular trabeculations overlying the defect. When they are associated with "Swiss cheese"-type of VSDs, it is almost impossible to visualize the true edges of the defect through the transatrial approach. Consequently, there remains an unacceptable incidence of mortality and morbidity when compared to those that occur with closure of the usual perimembranous VSD. Although various techniques for closure of these difficult trabecular VSDs have been attempted, there is still a significant incidence of complications in the surgical management of trabecular VSDs, mostly significant residual shunts, a need for multiple reoperations, and severe left ventricular dysfunction. This article describes the anatomical details and classification of muscular trabecular VSDs. It also reviews several techniques currently utilized and their outcomes.

Double Aneuploidy: A Case of Trisomy 21 with XYY

Although aneuploidies are common structural chromosomal abnormalities, double aneuploidies involving chromosomes 21 and Y are very rare. Here we report a case of double aneuploidy involving chromosomes 21 and Y in a 5 day old baby with jaundice and polycythemia. The diagnosis was confirmed by karyotype analysis using modified “whole blood” and microtechnique methods followed by Giemsa-Trypsin-Leishman (GTL) banding technique. The patient had typical features of Down syndrome, however, phenotypic features of XYY was not present. In addition, the patient also had atrial septal defect, multiple trabecular small ventricular septal defect, and moderate degree of pulmonary hypertension. Etiological predisposing factor for 48,XYY,+21 is not known. It is difficult to determine the incidence, phenotypic properties, and recurrence risk of 48,XYY,+21.

Spontaneous closure of muscular trabecular ventricular septal defect: comparison of defect positions

To evaluate the timing and frequency of spontaneous closure of the muscular trabecular ventricular septal defect (VSD). We performed a historical cohort study for which 150 patients <3 months of age (median age, 9 days) diagnosed as having a muscular trabecular VSD were selected. Median age at latest follow-up was 2.8 years. Another 32 patients diagnosed after 3 months of age were also reviewed. Using colour Doppler, defects were classified into three groups: anterior, apical and midventricular. Spontaneous closure occurred in 126 patients (84%): anterior, 36 of 47 (83%); apical, 26 of 31 (84%); and midventricular, 64 of 72 (89%). Multivariate analyses showed a lower frequency of spontaneous closure for patients of age of ≥ 20 days at initial echocardiography [hazard ratio 0.60, 95% confidence interval (CI) 0.39-0.89] and for anterior and apical muscular trabecular VSD (hazard ratio 0.66, 95% CI 0.47-0.95). The prevalence of the midventricular muscular trabecular VSD was significantly lower in patients ≥ 3 months of age at initial echocardiography than in those < 3 months (p = 0.010). We infer that midventricular muscular trabecular VSD tends to close spontaneously earlier and more frequently than either anterior or apical muscular trabecular VSD.

Closure of multiple ventricular septal defects by the felt sandwich technique: Further analysis of 36 patients

We previously described a novel technique for closure of trabecular ventricular septal defects by sandwiching the septum with 2 polyester felt patches without requiring left ventriculotomy. We describe the midterm results of this technique and the postoperative cardiac function. This is a retrospective study of 36 consecutive patients. The median age and body weight at the time of repair was 15 months (range: 2-115 months) and 7.8 kg (range: 3.9-51.9 kg), respectively. Sixty-three trabecular ventricular septal defects in 36 patients were closed with the felt sandwich technique. In the early postoperative period, 1 patient died of pulmonary hypertensive crisis. There were 2 late deaths. One patient died of pneumonia 6 months after surgery, and another died suddenly of ventricular arrhythmias 2 years after surgery. Three patients required reoperation (closure of major residual ventricular septal defect, cardiac transplantation for dilated cardiomyopathy, and pacemaker implantation for complete atrioventricular block). Postoperative left ventricular fractional shortening in the group with a body surface area less than 0.4 m2 was significantly lower than that in the group with a body surface area of 0.4 m2 or greater (0.22 +/- 0.09 vs 0.31 +/- 0.06, P = .0027). Moreover, there was a strong correlation between postoperative left ventricular ejection fraction and total patch area/body surface area ratio (R = -0.74, P = .0004). Multiple trabecular ventricular septal defects can be closed with the felt sandwich technique easily and safely. Although this technique can be used in small infants, the use of numerous felt patches disturbs the movement of ventricular septum, which may cause postoperative cardiac dysfunction.

Aneurysmal Formation Induced by Membranous Tissue in Infundibular Ventricular Septal Defect

It is believed that spontaneous closure in infundibular ventricular septal defect (VSD) is induced by the growth and hypertrophy of the muscular portion of the septum around the defect or prolapse of the right aortic cusp.1 On the other hand, autopsy cases with a trabecular VSD have shown fibrous patches, which closed the defect or decreased the defect size.2 We report a patient who had an aneurysm induced by membranous tissue (MT) that formed around an infundibular VSD and underwent sequential echocardiographic examinations.

Surgical management of trabecular ventricular septal defects: The sandwich technique

Background: Surgical closure of trabecular ventricular septal defects is difficult and often unsuccessful. Objective: We performed closure of trabecular ventricular septal defects by sandwiching the septum between 2 polyester felt patches placed in the left ventricle and right ventricle without ventriculotomy. Methods: Eleven patients (7 boys and 4 girls) underwent a sandwiching closure at a mean age of 4.7 years (range, 0.4-9.7 years) and a mean weight of 16.7 kg (range, 4.6-52 kg). Associated cardiac malformations were present in 9 of the 11 patients. Seven patients had undergone previous operations. The trabecular ventricular septal defects are exposed through the tricuspid valve and also from the left ventricular side through a coexisting large perimembranous ventricular septal defect or through the mitral valve through an interatrial septostomy. Two forceps, one each from the right and left ventricular side, lead a 3F Nelaton catheter through the trabecular defect. An oversized circular polyester felt patch mounted on a 3-0 Nespolen suture attached to the Nelaton catheter is then passed into the left ventricle. The suture ends are then passed through a slightly smaller polyester felt patch on the right ventricular side of the septum. The Nespolen suture is then tied, thereby sandwiching the septum between the 2 patches. Results: Time required for the procedure was less than 20 minutes in each case. There were no hospital deaths, and the postoperative course was uneventful in all patients. There was no residual shunt in 3 patients, and a minimal residual shunt was observed in 5 patients. Mild residual shunt was observed in 3 patients. Cardiac catheterization was performed 1 month postoperatively in 8 patients in whom residual shunt was noted on echocardiography. Five of 8 patients had a minimal residual shunt (pulmonary blood flow/systemic blood flow ratio = 1.0). Three patients had a residual shunt (pulmonary blood flow/systemic blood flow ratio = 2.0, 1.6, and 1.2). The patient with a pulmonary blood flow/systemic blood flow ratio of 2.0 had a “Swiss cheese” ventricular septal defect, and a residual shunt remained around the patch. However, the residual shunt decreased to a pulmonary blood flow/systemic blood flow ratio of 1.6 at examination 16 months postoperatively. Echocardiography showed that the residual shunt had also decreased in another 2 patients. Conclusions: We conclude that the sandwich technique is safe and easy. Even in cases with a residual shunt present, the shunt is expected to decrease as time passes. Further experience and longer follow-up of these patients are necessary to conclude whether this technique is applicable to neonates and young infants.J Thorac Cardiovasc Surg 2003;125:508-12

Visceroatrial situs solitus with atrioventricular alignment discordance double outlet right ventricle and superoinferior ventricles: Fetal and neonatal echocardiographic findings

Visceroatrial situs, ventricular morphology, and atrioventricular and ventriculoarterial alignments can reliably be assessed with fetal echocardiography. We herein describe, to our knowledge, the first reported fetal echocardiographic diagnosis of a fetus with visceroatrial situs solitus, atrioventricular alignment discordance, double outlet right ventricle, and superoinferior ventricles of a 22-year-old gravida III para II woman with a previous history of intrauterine fetal death. She had been evaluated at another center and was referred to our unit because of the suspicion about her diagnosis and underwent fetal ultrasonographic evaluation at 39 weeks gestation. Neonatal echocardiographic evaluation and autopsy findings confirmed the diagnosis. Accordingly there was (solitus, L-loop ventricle, and anterior aorta) double outlet right ventricle, superoinferior ventricles with diminutive right ventricle, noncommitted trabecular ventricular septal defect, and pulmonary hypertension. (J Am Soc Echocardiogr 2002;15:749-52.)

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band

OBJECTIVESWe propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations.BACKGROUNDThe alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery.METHODSEleven infants (mean weight 2,560 ± 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 ± 0.7mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure.RESULTSThere were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients.CONCLUSIONSProvided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.

Congenital isolated apical ventricular septal defects.

The diagnosis of apical ventricular septal defects (VSDs) is difficult and the ratio of spontaneous closure is not well known. At the Ankara University Pediatric Cardiology Department, between the years 1992 and 1997, 33 patients were identified by color-flow Doppler (CFD) imaging as having a small defect at the apical portion of the ventricular septum. The age range was 1 day to 13 years. All patients were asymptomatic with normal telecardiograms and electrocardiograms. There were grade 2-3/6, pansystolic murmurs at the apex or closer to the apex in 19 patients. In 30 patients, narrow and short colored jets were seen at the apex of the heart, distal to the moderator band. The jets were wide in three patients. These defects were difficult to image and required specific angulation of the transducer. Of the 23 patients who had follow-up examinations, at 3 months to 3.5 years, there was spontaneous closure in ten (43.5%). To the authors' knowledge, this report is the largest series of isolated apical trabecular ventricular septal defects (t-VSDs). In their experience, color-flow imaging is more sensitive in detecting a small apical VSD. They conclude that although the closure of apical VSD is not the rule, the prognosis is excellent.

Outcomes in patients with interrupted aortic arch and ventricular septal defect

Among 183 neonates with interrupted aortic arch and ventricular septal defect entering a multiinstitutional study between 1987 and 1992, nine died before repair was accomplished. Among the remaining 174, survival at 1 month and 1, 3, and 4 years after repair was 73%, 65%, 63%, and 63%, respectively. The risk factors for death were low birth weight, younger age at repair, interrupted arch type B, outlet and trabecular ventricular septal defects, smaller size of the ventricular septal defect, and subaortic narrowing. Echocardiographically measured dimensions (expressed as Z-values) at all levels of the left heart-aorta complex were small. Two among thirty institutions were risk factors, and two others possibly were. Procedural risk factors for death after repair were (1) repair without concomitant procedures in patients with other important levels of obstruction in the left heart-aorta complex, (2) a Damus-Kaye-Stansel anastomosis, and (3) subaortic myotomy/myectomy in the face of subaortic narrowing. One-stage repair plus ascending aorta/arch augmentation had the highest predicted time-related survival in the 20% of patients with interrupted aortic arch and one or more coexisting levels of obstruction in the left heart-aorta complex, as did initial repair without or with aorta/arch augmentation in the 80% without these.

Surgical management of isolated multiple ventricular septal defects

From January 1980 through September 1990, 130 children underwent surgical closure of isolated multiple ventricular septal defects (mean age 14 +/- 18 months, mean weight 7.0 +/- 4.4 kg). Sixty-one were less than 1 year of age. Sixty-one children had pulmonary protection, 51 had pulmonary artery banding, and 10 had pulmonary valve stenosis. All other patients had severe pulmonary hypertension (mean systolic pressure 75.7 +/- 20.5 mm Hg and already disabling heart failure (New York Heart Association classes III and IV). The surgical management was based on the location of the defects and the ventricular dominance that were assessed preoperatively and intraoperatively. Midtrabecular ventricular septal defects were always centered by the moderator band and were therefore divided into low trabecular, midtrabecular, and high trabecular defects. The perimembranous septum was involved in 102 patients, the trabecular in 121, the inlet septum in 12, and the infundibular septum in 9. Fifty patients had the "Swiss cheese" form of the lesion. Closure of the ventricular septal defects included Dacron patch and mattress sutures. They were always first approached through a right atriotomy, which was sufficient for complete repair in 82 patients. In midtrabecular ventricular septal defects, section of the moderator band (n = 24) allowed closure of all the defects with a single Dacron patch. In 48 patients a right atriotomy and a right (n = 32) or left (n = 14) (particularly for low trabecular ventricular septal defects) or both right and left (n = 2) ventriculotomies were necessary to secure the repair. The hospital mortality rate was 7.7% (10 patients). The causes of deaths were residual ventricular septal defect (n = 5), pulmonary hypertension (n = 2), hypoplastic right ventricle (n = 1) and left ventricle (n = 1), and myocardial infarction (n = 1). Among eighteen survivors with residual ventricular septal defect, six were reoperated on; there were two deaths. A permanent pacemaker was necessary in four patients. Low trabecular ventricular septal defects and left ventriculotomy were significant risk factors for morbidity (death, residual ventricular septal defect), p less than 0.01. At 7 years of follow-up, 90% of survivors were in New York Heart Association class I. Actuarial survival and freedom from reoperation at 7 years were 89.6% and 87.5%, respectively.

Incidence and natural course of trabecular ventricular septal defect: Two-dimensional echocardiography and color Doppler flow imaging study

This study was designed to determine the prevalence of trabecular ventricular septal defect (t-VSD) in neonates and to evaluate the effects of its location, morphologic features, and size on its natural course during infancy. One thousand twenty-eight term newborn infants were examined by color Doppler flow imaging with orthogonal ultrasonographic views. Ten girls and 11 boys (2.0%) were found to have t-VSD. The natural course of the defect was examined in 42 consecutive cases, consisting of this group of 21 neonates and another group of 21 neonates with t-VSD. The morphologic features of the defect within the trabecular septum were classified as one or two defects (36 cases) and as a mesh-like defect (six cases). Reduction in size began from the right ventricular side or from within the trabecular septum. Spontaneous closure occurred most commonly during the first 6 months of life and was observed in 32 cases (76%) by 12 months of age: the frequency of closure was not related to the morphologic features and the initial size of the defect, but apical defects tended to have higher persistent patency than did defects in other locations (p less than 0.05). We conclude that the frequency of t-VSD in neonates and the frequency of spontaneous closure during early infancy are higher than previously believed. This information is important for predicting the natural course of t-VSD and deciding on its proper management.

Morphological characteristics of perimembranous ventricular septal defects and their surgical significance

The recognition of perimembranous ventricular septal defects and their subcategorization into inlet, outlet and trabecular types is important surgically because of the information it gives concerning the relationship with the atrioventricular conduction bundle. Inlet and outlet defects are well defined. The trabecular defect, in contrast, is less well defined. To obtain a better understanding of the perimembranous trabecular defect we studied 30 hearts, each having a ventricular septal defect known to be perimembranous. Of these 30 hearts, 16 were classified as inlet and 10 as outlet types. The “in between” trabecular defect in each of the remaining four hearts was slit-like, extending from the membranous septum into the area of the medial papillary muscle complex. Each was associated with a complex arrangement of the adjacent tricuspid valve leaflet tissues. In three hearts, leaflets and chords were attached to both the upper and lower rim of the defects. In the other specimen a shelf-like anomalous leaflet attachment was present along the lower rim. The atrioventricular conduction tissues were studied in two of the hearts, showing a close relation between the conduction bundle and the rim of the defect and the anomalous tricuspid valve leaflet attachments. In one case a most unusual disposition was encountered. A fan-like right bundle originated from the main axis at the same level as the origin of the left bundle branches. The anomalous fan penetrated into the connective tissue core of the valve leaflet. The bundle was then traced anteriorly to its usual intramyocardial position. The observed features of perimembranous trabecular ventricular septal defects warrant further caution should they require surgical repair.

Morphological characterisation of ventricular septal defects associated with coarctation of aorta by cross-sectional echocardiography.

Cross-sectional echocardiograms of 18 neonates and infants with coarctation of the aorta and ventricular septal defect were retrospectively assessed. With a combination of subcostal and precordial cuts the site and anatomical relations of the defects were determined. In one case there was a muscular trabecular ventricular septal defect. Three had a malalignment defect with associated left ventricular outflow tract obstruction. In two there was a doubly committed subarterial defect with associated malalignment of the point of continuity between the aortic and pulmonary valves and the crest of the trabecular septum. Twelve cases had a perimembranous defect, with varying degrees of extension into the inlet, trabecular, or outlet septum. In this group 10 had associated aortic override, with varying degrees of left ventricular outflow tract narrowing. Abnormal insertion of the tricuspid valve was observed in 10 cases, such that it partly obscured the ventricular septal defect. Thus in most cases of coarctation with ventricular septal defect, the morphology of the ventricular septal defect and ventricular outflow tracts is such that left ventricular ejection is directed towards the pulmonary artery rather than the aorta.