Perimembranous Defect Research Articles

Ventricular septal defects

Ventricular septal defects (VSD's) are the commonest structural congenital heart lesions. Isolated defects make up ∼1/3 of all congenital heart disease with VSD's present in many other more complex lesions. Whilst antenatal diagnosis rate of isolated muscular defects continues to improve, a significant number of perimembranous and inlet defects are still missed. These defects, which are more difficult to identify on basic screening views, are more commonly associated with additional cardiac pathology, extracardiac and chromosomal abnormalities. This session will look at the various types of ventricular septal defects, including atrioventricular septal defects, from both a screening view and advanced fetal echo perspective. It aims to give insight into when and how to go beyond screening views for a more detailed look for VSD's, and what features are important in counselling and ongoing management.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases—Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

The natural history of fetal diagnosed isolated ventricular septal defect.

This study was undertaken to clarify the natural course of ventricular septal defect, and to find an index that would help in prenatal counseling. Between January 2010 and December 2014, 18188 fetuses underwent echocardiographic examinations. Of these, 228 isolated ventricular septal defect cases were retrospectively reviewed. In this retrospective study, the incidence of isolated ventricular septal defect was 1.25% (228/18188). There were 146 patients who underwent echocardiography after delivery in order to confirm the natural course of patients with isolated ventricular septal defect. Of the 146 cases, 64 cases (43.84%) had the ventricular septal defect naturally closed in the fetal period. Of the 82 patients with ventricular septal defect at birth, 25 patients showed natural closure during follow-up. However, four patients (2.74%) required surgical treatment for ventricular septal defect. In case of perimembranous defects, natural closure is more frequent in the fetal period than in the postnatal period. Our results indicate that 60.96% (89/146) of isolated ventricular septal defects diagnosed during the fetal life are closed naturally. Perimembranous type defect, small defect (<2mm) and maternal age less than 35years are the good prognostic factors for the natural closure during fetal life. © 2017 John Wiley & Sons, Ltd.

Evaluation of Ventricular Septal Defect with Special Reference to the Spontaneous Closure Rate, Subaortic Ridge, and Aortic Valve Prolapse II.

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1month to 26years(median 4years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40days to 24.9years (median, 1.8years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p < 0.001). Defect size became small in 306 (16.8%) of patients with VSD at a median of 2.5years. Aneurysmal transformation was detected in 32.9% (600), left ventricular-to-right atrial shunt in 9.7% (176), subaortic ridge in 2.6% (48) of 1823 patients who were followed. In 381 (20.9%) of the 1823 patients, the VSD had been closed by a surgical or transcatheter technique. Surgery is required in one-fifth of patients with subaortic ridge or aortic valve prolapse. In conclusion, isolated VSDs are usually benign abnormalities that tend to shrink and close spontaneously.

Surveillance of ventricular septal defects in Delaware.

The prevalence of ventricular septal defects (VSDs), a birth defect in which there is an opening in the wall that separates the left and right ventricles of the heart, seemed to be substantially higher in Delaware compared with the National Birth Defects Prevention Network (NBDPN). The Delaware Birth Defects Registry (BDR) noted their high prevalence of VSDs in comparison with other states. A subset of children with a VSD born in 2007 through 2010 was identified from the complete reportable statewide defect list that the BDR creates each year. VSDs were categorized by type of VSD (muscular, perimembranous, conotruncal, or atrioventricular septal defect), by either isolated or complex, and then by spontaneously closed, surgically closed, open but clinically insignificant, lost to follow-up, fetal or neonatal death. The BDR team found a prevalence of VSD of 83.4 per 10,000 including fetal/neonatal deaths. Excluding fetal and neonatal deaths the prevalence was 78.7 per 10,000 live births. Excluding small muscular VSDs, the prevalence in Delaware falls to 25.7 per 10,000. The BDR team chose to include all babies with all types of VSDs. Using these criteria Delaware's prevalence of 78.7 was higher than that reported by other states (whose prevalence ranges from 1.6 to 70.0 per 10,000 live births) (National Birth Defects Prevention Network, ). Delaware's prevalence is similar to other states when small muscular VSDs are excluded. Birth Defects Research (Part A) 106:888-893, 2016. © 2016 Wiley Periodicals, Inc.

Cardiac rhythm abnormalities after transcatheter device closure of perimembraneous ventricular septal defects in pediatric patients at intermediate term follow up

Background: Transcatheter closure of perimembranous ventricular septal defects in pediatric patients poses special challenges owing to anatomical relationship between conduction system and perimembraneous ventricular septal defect. Aims: The study aims to assess and evaluate the conduction disturbances after transcatheter device closure of perimembranous ventricular septal defect using different occluders at mid-term follow-up. Methods: We studied 297 patients having PMVSD with clinical and/or echocardiographic evidence of a significant left-to-right shunt. All subjects underwent clinical examination, electrocardiogram (ECG), chest X-rays and transthoracic echocardiography before discharge and at 1, 6 and 12-months after the procedure and yearly thereafter. Platelet anti-aggregation therapy with aspirin 5 mg/kg/day orally and endocarditis prophylaxis was prescribed for six months. Results: The mean age of the patients was 8.64±3.14 years (range 3-17.2 years). Majority (86.2%) had no residual shunt at follow-up. Total rhythm disturbances were seen in about 6% (18/297) of patients with transient complete atrioventricular block (CAVB) occurring in 3 patients. There was no mortality in our study which compares well with the surgical results in which it is between 0-3 percent. Conclusions: This study showed that transcatheter closure of PMVSD using symmetric PMVSD occluders and duct occluders is a safe and effective alternative to surgery. Oversizing of devices should be minimized and low profile devices appropriate to specific morphology of VSD should be used. It has excellent results in experienced hands with minimum morbidity and almost no mortality.

Septal Atrioventricular Junction Region: Comprehensive Imaging in Adults.

The septal atrioventricular junction is a centrally located region of the heart where the septal components of the atria and ventricles meet the aortic, mitral, and tricuspid valves. Important structures in this region include the membranous septum, the central fibrous body, the Koch triangle, the inferior pyramidal space, and the base of the interventricular septum. This small area is the home of the atrioventricular node and the atrioventricular conduction axis and has enormous importance to electrophysiologists owing to its prime role in the conduction system of the heart. The atrioventricular node lies within the triangle of Koch; and the atrioventricular bundle, or bundle of His, exits the atrioventricular node and penetrates the right fibrous trigone and runs underneath the membranous septum. The septal atrioventricular junction is a common location for intracardiac shunts such as membranous and perimembranous septal defects. Imaging classification of these defects can have important implications before surgical closure, because the atrioventricular conduction axis passes along the posteroinferior margin of most perimembranous defects. Extracardiac inflammatory and malignant pathologic conditions can extend from the mediastinum toward the inferior pyramidal space in this region through the epicardial fat planes. Although the anatomic structures are complicated, the components can be shown in exquisite detail with computed tomography (CT). In this review, the anatomic boundaries and important anatomic landmarks are examined with CT and magnetic resonance imaging. Also described are the anatomic variants of the membranous septum pertinent to percutaneous aortic valve implantation, the vascular anatomic variants, and commonly encountered pathologic conditions related to the septal atrioventricular junction. ©RSNA, 2016.

ADO II in Percutaneous VSD Closure in Pediatric Patients.

Main aim of our study to show that ADO II device can be used for the small ventricular septal defects successfully and safely with low complication rates in pediatric population. It is hard to find an ideal device to use for every VSD successfully. If inappropriate device was chosen; complication rate increases, procedure time gets longer that prolongs exposure to ionizing radiation. Therefore interventionalists are in the search for new ideal devices. Between the dates April 2011-October 2014, 21 VSD closures with ADO-II device. were performed. Twenty patients were included, age ranged between 4 months 18 years. Weight of the patients was between 5-76 kg. VSD diameter ranges between 2-6 mm (3.75 ± 1.25). VSD types were muscular in 2 patients, rest of them were perimembranous type. Most of the perimembranous defects (19/21) were aneursymatic and tunnel shaped. All the cases were successfully closed, no major complications were reported. There was no incidence of left bundle branch block, P-R prolongation, or complete heart block. Considering perimembraneous ventricular septal defects as difficult and risky for percutaneous closure because of its proximity to aortic, atrioventricular valves and conduction tissue, we suggest that ADO II device can be safely and effectively used for such defects in particular if an aneurysm formation is present which is also compatible with the literature.

Perimembranöz ve Musküler Ventriküler Septal Defekt: Nadir Bir Birliktelik

Perimembranöz ve Musküler Ventriküler Septal Defekt: Nadir Bir Birliktelik

Perventricular device closure of a doubly committed subarterial ventricular septal defect.

A doubly committed subarterial ventricular septal defect (VSD) used to be a contraindication for device closure. Perventricular device closure has been widely applied in China in the past years, but not in a doubly committed subarterial VSD. Chinese surgeons are now beginning to try this procedure also in a doubly committed subarterial VSD in selected patients. The technique of closure of the perimembranous defect has been described previously in this journal, but we hereby present some additional information on this procedure performed in a doubly committed subarterial VSD.

Insights gained from three-dimensional imaging modalities for closure of ventricular septal defects.

Ventricular septal defects (VSDs) are the commonest group of congenital heart lesions, accounting for ≈30% of congenital heart defects in liveborn infants.1 Defects in the ventricular septum are classified according to their position when the ventricular septum is viewed from the right ventricle and these may vary in their location, size, and number.2,3 VSDs can occur as isolated lesions or as part of more complex disease, for example, transposition of the great arteries or double outlet right ventricle, where the location and size of such defects assume great importance in planning the optimal mode of surgical repair.4 In most cases, surgical closure remains the mainstay treatment for VSDs, but selected lesions may be amenable to catheter closure.5,6 Cross-sectional echocardiography is the most commonly used noninvasive diagnostic method to delineate the position of a VSD before surgery.7 Multiple sonographic cuts and cross-sectional sweeps of the region of interest are used to build a virtual reconstruction of the three-dimensional (3D) anatomy in the mind of the echocardiographer. However, for the surgeon who deals solely with 3D images there can remain an element of uncertainty when called to operate on the patient. The number, size, location of VSDs, and their relationship to adjacent structures are central for selecting whether the defects might be best closed via the tricuspid valve, via the semilunar valves, or via a ventriculotomy or whether these might not be readily approachable and a palliative pulmonary artery banding is desirable.8,9 In this review, we present key 3D echocardiographic projections and examples of different types of VSDs where the images have a direct effect on the approach taken to VSD closure. Comparison of 3D echocardiography (3DE) with other 3D imaging modalities is also described. Images are presented in an …

Follow-up of patients with interventional closure of ventricular septal defects with Amplatzer Duct Occluder II.

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.

Perventricular device closure of a large residual perimembranous interventricular septal defect after previous surgical correction

A 21 years albanian patient was referred with important residual left to right shunt. He was undergone 7 years before conventional surgical correction of a perimembranous ventricular septal defect (VSD). The patient underwent sternotomy and perventricular device closure of the residual employing a 16 mm multifenestrated atrial septal defect occlude, which was positioned through the anterior wall of the right ventricle. across the defect. The previous autologous pericardial patch was compressed into the double umbrella device. We may conclude that perventricular device closure can employed successfully in patients with residual perimembranous VSD after previous surgical repair as an alternative to the conventional surgery with excellent hemodynamic and postoperative outcome. Such a technique should be part of the surgical armamentarum.

SPONTANEOUS CLOSURE OF ISOLATED VENTRICULAR SEPTAL DEFECT IN THE FIRST FIVE YEARS OF LIFE

Background Isolated VSD is the most common congenital heart defect. The defect can be in any portion in the septum and of different size that play an important role in the clinical outcomes of its spontaneous closure. Objective To correlate the size and location (excluding inlet type) of isolated VSD with spontaneous closure rate in the 1 st 5 years of life. Materials and Methods This is a retrospective study for patient’s record with VSD that visited Sulaimani Pediatric Teaching Hospital during 1 st of January 2006 to 30 th of February 2011 in Echocardiography Unit observed and studied by the same operator. The data analysis of 87 children records with isolated VSD had been studied on frequent outpatient clinic visits and followed up for a maximum period of 5 years or until spontaneous closure had occurred. Results from two dimensional, and Doppler (spectral and color) echocardiography examinations were evaluated for each patient. Results The age range of patients at time of initial examination who received follow up was 5 days to 12 months old. Spontaneous closure occurred in 43(49.4%) of the 87 cases that were followed up for 5 years. Thirty six (62.1%) cases of small, 7 (35%) cases of moderate and none of the patients with large VSDs had spontaneous closure. Spontaneous closure was noted in 34 (72.3%) of 47 cases of muscular defects and in 9 (22.5%) of 40 patients with perimembranous VSD. The time of spontaneous closure ranged from 1 to 60 months and it occurred most commonly during the first 24 months of age. By the 1 st , 2 nd , 3 rd and 4 th year cumulative spontaneous closure occurred in 28.7%, 43.6%, 47.1%, and 48.2% respectively and at the 5 th year it was seen in all 43 cases. Conclusion More than half of the small and one third of moderate size VSD had spontaneous closure by 5 year of age. Two thirds of muscular and nearby one fourth of perimembraneous defects have closed spontaneously.The majority of spontaneous closures occurred in the first 2 years of life.

Contribution of Giant Aneurysm of a Membranous Ventricular Septal Defect to Right Ventricular Outflow Tract Obstruction

Perimembranous defects are the most common types of ventricular septal defects (VSDs). In many cases, an aneurysm accompanies this defect. The aneurysm can have advantageous consequences on defect hemodynamics. However, it also has numerous complications that are frequently encountered in clinical practice. In this case, we describe a patient with a membranous VSD with giant aneurysm formation contributing to subpulmonic severe obstruction.

How should I treat a perimembranous interventricular defect following aortic valve replacement surgery?

A 73-year-old man was admitted to our Institute for repeated episodes of congestive heart failure. The patient was affected by arterial hypertension, dyslipidaemia, severe chronic obstructive pulmonary disease, and recurrent atrial fibrillation. Two months earlier he had undergone aortic valve replacement with a biological prosthesis for severe stenosis. Echocardiography showed a left-to-right shunt localised in the left ventricular outflow tract, normal morphology and function of the aortic valve prosthesis, and a hyperkinetic left ventricle. Physical examination, electrocardiography, transthoracic and transoesophageal echocardiography, right heart catheterisation, left ventricular angiography. Post-surgical perimembranous interventricular defect with a clinically significant left-to-right shunt. Percutaneous closure with a ventricular septal defect occluder.

Clarifying the Surgical Morphology of Inlet Ventricular Septal Defects

Different types of ventricular septal defects (VSD) open to the inlet of the right ventricle. The atrioventricular conduction axis is markedly different within these subtypes, a feature of great surgical importance. To clarify these relationships, we have studied hearts with such VSDs from the Idriss archive at Ann and Robert H. Lurie Children's Hospital of Chicago. We selected hearts from the archive showing the different variants of inlet VSD, photographing them to show the presumed disposition of the atrioventricular conduction axis as based on previous histology studies. We differentiated between perimembranous defects, muscular defects, perimembranous defects with straddling of the tricuspid valve, and atrioventricular septal defects with shunting confined at the ventricular level. The atrioventricular conduction axis is different in the four types of inlet VSDs. In perimembranous defects opening to the inlet of the right ventricle, the axis is positioned to the right hand of the surgeon operating through the tricuspid valve, whereas it is to the left hand with the muscular inlet defect. In patients with straddling tricuspid valve, the axis arises from an anomalous posteroinferior atrioventricular node, whereas in patients with atrioventricular septal defect with exclusive ventricular shunting, the axis arises at the crux of the heart from a node located in an inferiorly displaced nodal triangle. An appreciation of these relationships should help surgeons avoid the conduction system when closing inlet VSDs.

Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part I - Acyanotic Heart Defects

The purpose of this review/editorial is to discuss how and when to treat the most common acyanotic congenital heart defects (CHD); the discussion of cyanotic heart defects will be presented in a subsequent editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. Balloon pulmonary valvuloplasty is the treatment of choice for valvar pulmonary stenosis and the indication for intervention is peak-to-peak systolic pressure gradient >50 mmHg across the pulmonary valve. For aortic valve stenosis, balloon aortic valvuloplasty appears to be the first therapeutic procedure of choice; the indications for balloon dilatation of aortic valve are peak-to-peak systolic pressure gradient across the aortic valve in excess of 70 mmHg irrespective of the symptoms or a gradient ≥ 50 mmHg with either symptoms or electrocardiographic ST-T wave changes indicative of myocardial perfusion abnormality. The indications for intervention in coarctation of the aorta are significant hypertension and/or congestive heart failure along with a pressure gradient in excess of 20 mmHg across the coarctation; the type of intervention varies with age at presentation and the anatomy of coarctation: surgical intervention for neonates and young infants, balloon angioplasty for discrete native coarctation in children, and stents in adolescents and adults. Long segment coarctations or those associated with hypoplasia of the isthmus or transverse aortic arch require surgical treatment in younger children and stents in adolescents and adults. For post-surgical aortic recoarctation, balloon angioplasty in young children and stents in adolescents and adults are treatment options. Transcatheter closure methods are currently preferred for ostium secundum atrial septal defects (ASDs); the indications for occlusion are right ventricular volume overload by echocardiogram. Ostium primum, sinus venosus and coronary sinus ASDs require surgical closure. For all ASDs elective closure around age 4 to 5 y is recommended or as and when detected beyond that age. For the more common perimembraneous ventricular septal defects (VSDs) of large size, surgical closure should be performed prior to 6 to 12 mo of age. Muscular VSDs may be closed with devices. Patent ductus arteriosus (PDA) may be closed with Amplatzer Duct Occluder if they are moderate to large and Gianturco coils if they are small. Surgical and video-thoracoscopic closure are the available options at some centers. In the presence of pulmonary hypertension appropriate testing to determine suitability for closure should be undertaken. The treatment of acyanotic CHD with currently available medical, transcatheter and surgical methods is feasible, safe and effective and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

Percutaneous closure of perimembranous and muscular ventricular septal defects in the same session in a pediatric patient

Abstract Ventricular septal defect closure is proposed because of some possible complications such as left heart failure, pulmonary vascular disease, Eisenmenger's syndrome and infective endocarditis. These defects are conventionally closed by surgery in general practice. Percutaneous closure is becoming increasingly common in recent years. While percutaneous closure was first reported in muscular defects, currently it is also used successfully for perimembranous defects. In this paper we presented a pediatric case with a single perimembranous ventricular septal defect detected on transthoracic echocardiography examination. The muscular defect was detected during the intervention and was closed percutaneously in the same session with the perimembranous defect Keywords: Congenital heart disease, ventricular septal defect, percutaneous closure, complication Ozet Ventrikuler septal defektlerin sol kalp yetersizligi, pulmoner vaskuler hastalik, Eisenmenger sendromu ve enfektif endokardit gibi muhtemel komplikasyonlari sebebiyle kapatilmalari onerilmistir. Bu defektler konvansiyonel olarak cerrahi yolla kapatilmaktadir. Son yillarda perkutan yolla kapatma giderek yayginlasmaktadir. Ilk olarak perkutan kapatma muskuler defektler icin bildirilmisken gunumuzde perimembranoz defektlerde basarili bir sekilde kapatilabilmektedir. Bu yazida transtorasik ekokardiyografide tek perimembranoz ventrikuler septal defekt tespit edilen, perkutan kapatma islemi sirasinda muskuler septumda ikinci bir defekt saptanmasi ile ayni seansta perkutan yolla her iki defektin kapatildigi bir cocuk olgu sunulmustur. Anahtar sozcukler: Konjenital kalp hastaligi, ventrikuler septal defekt, perkutan kapatma, komplikasyon

Long term follow-up of 799 children with isolated ventricular septal defects

We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.