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.

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 1 month to 26 years (median 4 years) 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 40 days to 24.9 years (median, 1.8 years). 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 2.5 years. 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.

Interventional VSD-Closure with the Nit-Occlud® Lê VSD-Coil in 110 Patients: Early and Midterm Results of the EUREVECO-Registry.

In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Anatomy of the ventricular septal defect in outflow tract defects: similarities and differences.

OBJECTIVE: The study objective was to analyze the anatomy of the ventricular septal defect found in various phenotypes of outflow tract defects. METHODS: We reviewed 277 heart specimens with isolated outlet ventricular septal defect without subpulmonary stenosis (isolated outlet ventricular septal defect, 19); tetralogy of Fallot (71); tetralogy of Fallot with pulmonary atresia (51); common arterial trunk (54); double outlet right ventricle (65) with subaortic, doubly committed, or subpulmonary ventricular septal defect; and interrupted aortic arch type B (17). Special attention was paid to the rims of the ventricular septal defect viewed from the right ventricular side and the relationships between the tricuspid and aortic valves. RESULTS: The ventricular septal defect was always located in the outlet of the right ventricle, between the 2 limbs of the septal band. There was a fibrous continuity between the tricuspid and aortic valves in 74% of specimens with isolated outlet ventricular septal defect, 66% of specimens with tetralogy of Fallot, 39% of specimens with tetralogy of Fallot with pulmonary atresia, 4.6% of specimens with double outlet right ventricle, 1.8% of specimens with common arterial trunk, and zero of specimens with interrupted aortic arch type B (P < .005). When present, this continuity always involved the anterior tricuspid leaflet. CONCLUSIONS: The ventricular septal defect in outflow tract defects is always an outlet ventricular septal defect, cradled between the 2 limbs of the septal band. However, there are some differences regarding the posteroinferior and superior rims of the ventricular septal defect. These differences suggest an anatomic continuum from the isolated outlet ventricular septal defect to the interrupted aortic arch type B rather than distinct physiologic phenotypes, related to various degrees of abnormal rotation of the outflow tract during heart development: minimal in isolated outlet ventricular septal defect; incomplete in tetralogy of Fallot, tetralogy of Fallot with pulmonary atresia, and double outlet right ventricle; absent in common arterial trunk; and excessive in interrupted aortic arch type B.

Use of diagnostic information submitted to the United Kingdom Central Cardiac Audit Database: development of categorisation and allocation algorithms.

OBJECTIVE: To categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery. DESIGN: Codes from the International Paediatric Congenital Cardiac Code were mapped to recognisable primary cardiac diagnosis groupings, allocated using a hierarchy and less refined diagnosis groups, based on the number of functional ventricles and presence of aortic obstruction. SETTING: A National Clinical Audit Database. Patients Children undergoing cardiac interventions: the proportions for each diagnosis scheme are presented for 13,551 first patient surgical episodes since 2004. RESULTS: In Scheme 1, the most prevalent diagnoses nationally were ventricular septal defect (13%), patent ductus arteriosus (10.4%), and tetralogy of Fallot (9.5%). In Scheme 2, the prevalence of a biventricular heart without aortic obstruction was 64.2% and with aortic obstruction was 14.1%; the prevalence of a functionally univentricular heart without aortic obstruction was 4.3% and with aortic obstruction was 4.7%; the prevalence of unknown (ambiguous) number of ventricles was 8.4%; and the prevalence of acquired heart disease only was 2.2%. Diagnostic groups added to procedural information: of the 17% of all operations classed as "not a specific procedure", 97.1% had a diagnosis identified in Scheme 1 and 97.2% in Scheme 2. CONCLUSIONS: Diagnostic information adds to surgical procedural data when the complexity of case mix is analysed in a national database. These diagnostic categorisation schemes may be used for future investigation of the frequency of conditions and evaluation of long-term outcome over a series of procedures.

Clarifying the surgical morphology of inlet ventricular septal defects.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: An appreciation of these relationships should help surgeons avoid the conduction system when closing inlet VSDs.

Device closure of congenital ventricular septal defects.

Ventricular septal defect is the most common congenital heart malformation. Surgical closure, when indicated, has been practiced for over 50 years with good results; however, surgical closure is still associated with significant morbidity and mortality. Over the past decade, several occluding devices have been developed that made catheter device closure an attractive alternative to surgery with widely satisfactory results. In this article, a comprehensive review of percutaneous and perventricular (hybrid) device closure of each type of ventricular septal defect is presented.

Aortic valve prolapse associated with outlet-type ventricular septal defect.

BACKGROUND: Aortic valve prolapse is frequently associated with juxta-arterial ventricular septal defect. The significance of its association with other outlet types of ventricular septal defect, however, remains unclear. METHODS: From 1987 to 2002, 677 patients (male:female ratio, 424:253) who received surgical repair for ventricular septal defect extending to the outlet septum were reviewed. Based on surgical findings, ventricular septal defects were classified as juxta-arterial, perimembranous outlet, or muscular outlet type. RESULTS: Aortic valve prolapse occurred in 373 of 677 patients (57.2%) with 209 juxta-arterial, 103 perimembranous outlet, and 61 muscular outlet type. Significant aortic regurgitation developed in 51 of 373 (14%). Among 252 patients with regular follow-up, the mean onset ages of aortic valve prolapse in juxta-arterial, perimembranous outlet, and muscular outlet type were 4.9, 5.0, and 5.1 years, respectively (no statistical difference). The presence of larger shunt and probably anterior malalignment predicted an earlier onset of aortic valve prolapse. Perimembranous outlet and muscular outlet type ventricular septal defect were frequently associated with infundibular hypertrophy and subaortic ridge, and perimembranous outlet type was associated with anterior septal malalignment. In juxta-arterial ventricular septal defect and ventricular septal defect with anterior malalignment, prolapsed cusp was always the right coronary cusp, but noncoronary cusp involvement was also common in perimembranous outlet type (17 of 103, 16.5%). CONCLUSIONS: The association with anterior septal malalignment, infundibular stenosis and subaortic ridge is related to the location of the outlet ventricular septal defect. The age of onset of aortic valve prolapse in each type was quite similar, and a larger shunt may predict an earlier onset.

Will a handheld ultrasound scanner be applicable for screening for heart abnormalities in newborns and children?

BACKGROUND: There is significant interest in opportunities to provide echocardiography services for detection of congenital heart disease with portable, or even handheld, devices in remote areas or third world countries where conventional ultrasound systems may not be available. We tested a handheld system (HHS) (SonoHeart, SonoSite Inc, Bothell, Wash) equipped with a broadband, 7- to 4-MHz, miniaturized, curved, linear-array transducer and implemented with an improved directional Doppler flow map. METHODS: All echocardiography scanning was performed in the neonatal nursery, pediatric intensive care department, or pediatric echocardiography laboratory of our institution. We reviewed limited echocardiography view sequences sequentially obtained by the same expert examiner (D.J.S.) in 50 infants and children (age: 1 day to 6 years), with preoperative or postoperative forms of congenital heart disease. Each patient was studied twice, once with a conventional full-feature system (FFS) and then a limited scan with the HHS using similar frequency transducers. The cardiologist (D.J.S.) and blinded research laboratory reviewers (X.L., G.K.M., R.A.R.) read the FFS and HHS image sequences for diagnosis and for grading the quality of the anatomic and flow feature images. The studies were performed and reviewed with the examiner and reviewers blinded to patient diagnosis. RESULTS: The major diagnoses (eg, patent ductus arteriosus, atrio-ventricular (AV) canal, peripheral pulmonary valve stenosis, aortic coarctation, atrial septal defect, ventricular septal defect, preoperative or postoperative tetralogy of Fallot, and mitral regurgitation) were made by both readers, who were unaware of each other's diagnosis results. Furthermore, the average composite HHS cardiac anatomic feature score on a scale of 0 (not visualized) to 3 (visualized precisely) from the parasternal long-axis and 4- or 5-chamber view for cardiac anatomy were 2.67 +/- 0.49 (SD) and 2.50 +/- 0.55, respectively, versus 2.73 +/- 0.45 and 2.55 +/- 0.54 for the FFS. The mean flow feature score, comprising all views, was 2.67 +/- 0.45 (HHS) versus 2.72 +/- 0.48 (FFS). The P values for all above comparisons were >.05. Image quality of the FFS anatomic structures were, thus, not statistically different from the HHS. Although the color cosmetic was different for the HHS directional (nonvelocity) map, only 9% of 150 total findings (including structural abnormalities and flow features, none of which were critical) were missed, whereas the other 91% regurgitant, shunt, stenosis flow features or heart structure were imaged adequately by the HHS in this population. CONCLUSIONS: Implementing high-frequency transducers and programs optimized for tissue and flow imaging on the HHS should provide images of sufficient quality for targeted echocardiography examinations to determine the presence, absence, or status of congenital heart disease in newborns and young children.

The surgical anatomy of the left ventricular outflow tract in hearts with ventricular septal defect and aortic arch obstruction.

BACKGROUND: Profound understanding of the left ventricular outflow tract (LVOT) anatomy is crucial to improve surgical results in patients with aortic arch obstruction, ventricular septal defect, and subaortic stenosis. METHODS: We studied the morphology of the LVOT in 32 postmortem hearts with aortic arch obstruction and a ventricular septal defect. In case of subaortic obstruction, the length of the subaortic muscular component was measured anteriorly and posteriorly within the left ventricle. RESULTS: Seven of the 32 hearts had no subaortic stenosis. Nine had aortic override, which caused LVOT narrowing. Sixteen hearts contained a subaortic shelf, downstream to the ventricular septal defect, which deviated into the left ventricle in 15. In 10 of these the shelf was muscular; in 6 it was a fibrous ridge. In cases with a muscular shelf, the posterior part was significantly shorter than the anterior part (p < 0.004). In 9 hearts the LVOT was further narrowed because of the abnormal relationship between the mitral valve and the subaortic shelf. CONCLUSIONS: The present study confirms the complexity of LVOT stenosis in aortic arch obstruction and ventricular septal defect and provides a better understanding of the options to achieve surgical relief.

Prevalence, relation to spontaneous closure, and association of muscular ventricular septal defects with other cardiac defects.

Previous studies on muscular ventricular septal defect (VSD) have not taken into account the specific defect location in the septum. We retrospectively reviewed all patients with a muscular VSD, with and without associated malformations, diagnosed over 32 months to determine the prevalence and rate of spontaneous closure of single defects in relation to location in the muscular septum. Defects were classified into 4 groups: midmuscular, apical, anterior, and posterior. Two hundred seven patients were identified, of whom 125 had a single defect. The relative prevalence of single muscular VSD was: midmuscular 55 (44%), apical 31 (25%), anterior 33 (26%), and posterior 6 (5%). Thirty patients had signs of spontaneous closure and only 1 underwent surgery. There was no difference in rate of closure with respect to anatomic locations. Patients with multiple muscular VSD were either referred for surgery in the first year of life or had a course similar to patients with a single VSD. Muscular VSD associated with other cardiac malformations was more often encountered in patients with conoventricular VSD and coarctation of the aorta. The distribution of anatomic groups of muscular VSD in association with malformations was similar to the single VSD.

The surgical anatomy of ventricular septal defects with univentricular atrioventricular connection.

Hearts that do not possess one-to-one connections at the segmental junctions almost always produce a univentricular atrioventricular connection. One ventricle is usually large and dominant and the other small, lacking one or two of its components. The ventricular septal deficiency forms part of the circulatory pathway. We take the stance that only hearts that possess a truly solitary ventricular chamber are univentricular. They cannot have a ventricular septal defect, and so are excluded from this study. This review, therefore, is concerned with the morphology of septal defects in hearts in which both atrioventricular junctions are connected exclusively to a dominant left or a dominant right ventricle, and those lacking one atrioventricular connection, where the remaining valve is connected to a dominant ventricle. This morphology in the absence of one atrioventricular connection can be modified when there is overriding of the solitary atrioventricular valve. The ventricular septal defects are analyzed and categorized for the various groups, and the position of the conduction axis is described for the well-recognized entities.

[Surgical treatment of left ventricular right atrial fistula (II-type): report of 12 cases].

12 cases of left ventricular right atrial fistula (II-type) were identified and repaired by open heart surgery. Our date showed that defects of the septal leaflet of tricuspital valve can be divided into three groups: multiple-opening, single-opening and split defects. When there were VSD and fistula at the same position, the VSD was not repaired completely if the opening of tricuspital septal leaflet was mistaken as the VSD itself. It is important to expose the edge of the VSD completely by cutting tricuspital septal leaflet when repairing the VSD. The cutting septal leaflet can be sutured after closure of the VSD.

Risk factors for ventricular septal defect in Finland.

The possible effect of genetic and environmental factors during pregnancy on the occurrence of ventricular septal defect (VSD) in the offspring was studied in 150 cases and 756 controls. The cases represented all verified VSDs in Finland during 1982-1983. The controls were randomly selected from all babies born during the same period. Case and control mothers were interviewed by midwives approximately three months after delivery using a structured questionnaire. Congenital heart disease was more prevalent among parents of cases than those of controls. Maternal alcohol consumption during the first trimester of pregnancy was more common among the mothers of VSD infants (47.0%) than among those of controls (38.0%, P less than 0.05). Exposure to organic solvents at work showed in logistic regression analysis an adjusted relative odds ratio of 1.8 (95% confidence interval 1.0-3.4). The risk of VSD was not associated with any of the maternal habits monitored, e.g. smoking, or coffee, tea, cola, acetosalicylic acid or diazepam consumption. Whether the mother was employed during the first trimester of pregnancy, and her exposure to anesthetic gases, disinfectants, pecticides, wood preservatives or video display terminals were not factors associated with the risk of ventricular septal defect.

Ruptura del septum interventricular postinfarto miocardico / Rupture of interventricular septum post-myocardial infarction

Se informan los resultados de la intervencion quirurgica tardia en doce casos de ruptura postinfarto del septum interventricular. En promedio los pacientes fueron intervenidos despues de doce dias de producida la ruptura. Se presentaron dos muertes: una debida a sindrome de bajo gasto cardiaco y otra secundaria un accidente cerebrovascular ppostoperatorio. El manejo inicial se practico con inotropicos, vasodilatadores, diureticos y balon de contrapulsacion intraaortico. E lprocedimiento quirurgico incluyo parche con suturas reforzadas de teflon,aneurismectomia e injertos aortocoronarios cuando fueron necesarios. Se discute la fisiopatologia de la entidad y los resultados obtenidos con intervencion temprana y tardia.

Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. I. Criteria of surgical decision.

The feasibility of anatomic repair (defined as the reconstruction of normal ventriculoarterial connection) was investigated in 104 patients who underwent an operation for anomalies of ventriculoarterial connection associated with ventricular septal defect. Three types of anatomic repair were used: intraventricular rerouting, REV (association of intraventricular rerouting with translocation of the pulmonary arterial trunk on the right ventricle), and arterial switch associated with closure of the ventricular septal defect. Intraventricular repair was considered to be the best and simplest method when possible. In the other cases, REV was indicated if pulmonary outflow tract obstruction was present, and arterial switch was performed when the tract was patent. The feasibility of intraventricular repair was related to the distances between the tricuspid valve and the semilunar valves. Preoperative measurement of these distances is an essential criterion to choose the appropriate repair of anomalies of ventriculoarterial connection associated with ventricular septal defect.

Of clefts, commissures, and things.

A full understanding of the morphology of atrioventricular septal defects ("endocardial cushion defects," "atrioventricular canal malformations") demands knowledge of the exact nature of three specific breaches in the skirt of valve leaflet tissue guarding the atrioventricular junction. One of these is the space between the left ventricular components of the two leaflets that are enclosed in both the right and left ventricles (the bridging leaflets). Traditionally described as a cleft, it has been suggested more recently that this gap functions as a commissure. The second space is that which is found anteriorly and superiorly in the so-called Rastelli type A malformation. This is also called a cleft in a common anterior leaflet, but it has been suggested that it too is a commissure. The final breach is that produced by the surgeon when he divides the free-floating superior bridging leaflet if repairing the so-called Rastelli type C malformation with a one-patch technique. It is generally agreed that this breach be considered a division. To adjudicate the nature of the other breaches, it is necessary to compare them with commissures in atrioventricular and arterial valves and with the isolated cleft that exists in the aortic leaflet of a normal mitral valve. These considerations show that the gap between the left ventricular components of the bridging leaflets functions as a commissure even though it is not supported by a papillary muscle. This would not be expected, since the commissural attachments of the leaflets are in the left and right ventricle, respectively. The gap seen anteriorly in the Rastelli type A malformation is also a commissure, being supported in typical fashion by the medial papillary muscle of the right ventricle.