Single Ventricular Septal Defect Research Articles

Transesophageal echocardiography-assisted transcatheter device closure of apical muscular ventricular septal defect.

Transesophageal echocardiography-assisted transcatheter device closure of apical muscular ventricular septal defect.

Association of maternal diseases during pregnancy with the risk of single ventricular septal defects in the offspring – a population-based case-control study

Objective: In general, the analytical epidemiological studies evaluated cases with congenital heart defects (CHDs) together. However, different CHD entities have different etiology, and in the vast majority of patients the underlying causes are unclear. Thus the objective of the study was to evaluate the possible etiological factors in the origin of single ventricular septal defect (VSD) after surgical intervention or lethal outcome, i.e. as homogeneous as possible.Method: In the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities acute and chronic maternal diseases with related drug treatments and pregnancy supplements in early pregnancy were evaluated in the mothers of 1661 cases with isolated/single VSD and their 2534 matched and 38 151 all controls without defect, and 19 833 malformed controls with other isolated non-cardiac defect.Results: There was a higher risk of VSD in the children of mothers with high fever related influenza during the critical period of VSD and this risk was limited by antifever therapy. In addition paroxysmal supraventricular tachycardia and epilepsy treated with anticonvulsant drugs associated with higher risk of VSD. Finally, the high doses of folic acid alone in early pregnancy.Conclusions: High-fever-related maternal diseases may have a role in the origin of VSD which is preventable with antifever drug therapy, and the high doses of folic acid in early pregnancy reduced the risk of VSD.

Ventricular septal defects in function of maternal sociodemographic aspects

Abstract The objective of our project is to reveal the possible etiological factors of different congenital cardiovascular abnormalities. In this study, we evaluated single ventricular septal defect (VSD) after surgical correction or with lethal outcome. The birth outcomes of these cases in the function of maternal socio-demographic features were evaluated. Data are based on 1,659 VSD cases, 2,534 matched controls and 38,151 all controls without any defects, in addition in the mothers of 19,393 malformed controls with other isolated defects in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities. VSD had mild female excess with a higher rate of preterm birth and mainly low birth weight indicating intrauterine growth restriction of affected fetuses, particularly in males and full-term or average weighted cases. The mothers of cases with VSD had lower socioeconomic status and higher rate of smoking and particularly drinking habit. The evaluation of medically recorded pregnancy complications showed an association of gestational diabetes with a higher risk of VSD. In conclusion, the association of small localized size of VDS and obvious fetal growth restriction needs further explanation in these cases, while gestational diabetes, lower socioeconomic status and adverse lifestyle of pregnant women may have a role in the origin of VSD.

Percutaneous device closure of congenital and iatrogenic ventricular septal defects in adult patients

We report our 10-year experience with percutaneous closure of adult congenital and acquired (non-post-infarct) ventricular septal defects (VSDs) using different types of Amplatzer occluder devices. Adult congenital and acquired VSDs may produce significant morbidity and mortality. Furthermore, such VSDs pose a significant surgical challenge. Between February 2000 and August 2009, data were retrospectively reviewed from 28 patients who underwent 29 procedures for percutaneous device closure of hemodynamically significant VSDs. Seventeen had unrepaired congenital VSDs, 10 had post-operative VSDs (5 with residual patch-margin defects, 4 post-aortic valve replacement, 1 post-myomectomy), and one had an acquired traumatic VSD. INDICATIONS FOR CLOSURE INCLUDED: symptoms related to significant shunt (dyspnea on exertion); unexplained deterioration of LV function, and/or LV dilation; recurrent endocarditis, and pulmonary hypertension. Outcome parameters were procedural success, procedure-related complications, evidence of residual shunt by echocardiography, and improvement in the signs/symptoms for which the procedure was performed. The mean follow-up interval was 68 months. Of the 28 patients studied, a single VSD was present in 26 patients, while one patient had two defects, and one patient had one defect on the LV side with three openings at the RV side. The median size of the defects by echocardiography was 6 mm. A device was successfully implanted in 28 of 29 (97%) procedures and 28 of 28 (100%) patients. PROCEDURE-RELATED COMPLICATIONS OCCURRED IN TWO CASES: one involving an access site hematoma not requiring transfusion as well as nonsustained ventricular tachycardia that resolved spontaneously and the other involving acute mitral regurgitation due to inadvertent trapping of the anterior mitral valve leaflet between the left ventricular disk and the septum that was resolved by recapturing of the disk. There was immediate complete closure in 20 patients (71%). In six cases there was trivial residual shunt and in two patients the residual shunt was mild. At the latest follow-up, four of the eight with a residual shunt had no shunt and in the remaining four the residual shunt was trivial. Among symptomatic patients 18 (64%), there was marked improvement in symptoms and for those patients 17 (61%) for whom the procedure was performed to address left ventricular enlargement, there was reduction or stabilization in LV size on serial echoes. Percutaneous closure of VSDs in the adult patient appears to be safe and effective.

Transcatheter closure of high‐risk muscular ventricular septal defects with the CardioSEAL occluder: Initial report from the CardioSEAL VSD Registry

The CardioSEAL VSD registry was created to track safety of the device to close high-risk Ventricular Septal Defect (VSD). This is the first report from the multi-centered CardioSEAL VSD registry reviewing demographics and initial results. Centers recruited patients with VSD who were high-risk for surgery due to medical condition or anatomic features. 18 centers contributed data on 55 high-risk patients who had 61 VSD-occlusion procedures, with age of range of 5 days to 65 years and using one to six devices. Implantation approach was transvenous in 48, perventricular in five, and by combined approach in two patients. Ninety-two percent of intended VSD device implants were judged successful. Twenty-two patients had single VSD closed by single device in 18 and by two devices in four patients. All patients <8 kg underwent perventricular device implantation. Thirty-three patients had multiple VSDs which were closed by a single device in 23, and multiple devices in 10. At discharge echocardiography showed total residual flow through all VSDs in which devices were used was classified as "Small" or less in 74%, "More than small" in 11%, and "Uncertain" in 15%. Eight major adverse events occurred in 5/61 cases (8% event rate), with 3/81 devices embolized (4% embolization rate), 5/81 devices surgically explanted (6% explant rate), and no deaths judged to be procedure-related. This initial report from the multi-centered CardioSEAL VSD registry demonstrates the safety of the device to close high-risk VSDs.

Surgical results in patients with double outlet right ventricle: a 20-year experience

Background. The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. Methods. Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle. Results. Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively ( p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 ± 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively ( p = 0.11). Conclusions. Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy.

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpulmonary stenosis. A single patch technique, with closure of the zone of apposition ('cleft') in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosuperior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

Anatomic features and surgical strategies in double-outlet right ventricle.

The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.

Repair of interrupted aortic arch: A ten-year experience

Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any attempt at operation and 79 underwent surgical repair. Median age at operation was 9 days (range 1 day to 6 years) and median weight was 3.0 kg (range 1.8 to 20 kg). All but one were in severe congestive heart failure and 31.5% had oliguria or anuria. Preoperative pH varied between 6.8 and 7.4 (median 7.3). Sixty-nine received prostaglandin E 1 infusion and 54 received mechanical ventilation. Aggressive preoperative ressucitation was necessary in 43 cases. Preoperative transfontanellar echography (performed routinely) since 1987 revealed intracerebral bleeding in six patients. Type A interrupted aortic arch was present in 37 cases, 41 patients had type B, and one had type C. Interrupted aortic arch was associated with single ventricular septal defect in 35 cases, 24 patients had associated complex heart defects, and 30 had significant subaortic stenosis (six had both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricles were found in one. Sixty-four patients underwent single-stage repair and 15 underwent multistage repair. Aortic arch repair consisted of direct anastomosis in 59 cases, patch augmentation in eight, and conduit interposition in 12. Ten patients underwent associated pulmonary artery banding and 19 underwent concomitant repair of complex associated lesions. The subaortic stenosis was addressed by four surgical techniques: myotomy or myectomy in five patients; creation of a double-outlet left ventricle, aortopulmonary anastomosis, and conduit insertion between the right ventricle and pulmonary artery bifurcation in four; no direct attempt to relieve the subaortic stenosis in six; and left-sided ventricular septal defect patch in 15. Mean duration of deep hypothermic circulatory arrest, crossclamp time, and cardiopulmonary bypass time were 38.8 ± 15.6 min, 60.5 ± 24.7 min, and 143 ± 40.1 min, respectively. Postoperative mortality rate was 18.9% (70% confidence limits 14% to 24.6%), and overal mortality rate was 31% (70% confidence limits 20.9% to 42.2%). The results have improved with time, with an overall operative mortality rate of 12% since 1990. Univariate statistical analysis revealed that early survival was influenced by preoperative renal function, detection of cerebral bleeding by transfontanellar echography, the number of cardioplegic injections, and the date of operation. Multivariate analysis revealed that preoperative renal function and the number of cardioplegic injections were independent risk factors for early mortality. Echocardiographic measurements of the left heart–aorta complex with preoperative Z values as low as -4 demonstrated rapid growth after repair. In the presence of subaortic stenosis, better survival was obtained with a left-sided patch for ventricular septal defect closure ( p < 0.05). Twenty-three patients underwent 26 reoperations for recoarctations (seven), left bronchial compression (two), second-stage repair (eight), right ventricle–pulmonary artery conduit replacement (three), and miscellaneous (four). One of the survivors was reoperated on for subaortic membrane. Survival at 5 years for the entire series was 70%. For isolated forms, it was 73.5% (90% for 1990 to 1995), for complex forms it was 70%, and in the presence of subaortic stenosis it was 60%. In conclusion, interrupted aortic arch remains a surgical challenge with continually improving results. Early diagnosis with preoperative resuscitation and adequate myocardial protection seem extremely important for further improvements. Associated subaortic stenosis or complex lesions can be treated in a single-stage repair with satisfactory medium-term results. (J T HORAC C ARDIOVASC S URG 1996;112:1150-60)

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.

Outcomes in seriously ill neonates with coarctation of the aorta

Among 326 severely symptomatic neonates with coarctation with or without ventricular septal defect, four died before an initial procedure was performed. Among the 322 undergoing an initial procedure, survival for at least 24 months was 84%; the hazard function for death was lower initially but more prolonged in patients without than in those with ventricular septal defect. Important mitral valve anomalies coexisted in 5% of patients, left ventricular hypoplasia in 5% (more commonly in patients without ventricular septal defect), narrowing of the left ventricular outflow tract in 9% (more common in patients without ventricular septal defect), and narrowing of the proximal arch in 1%; one or more of these anomalies was present in most patients without ventricular septal defect who died. Five percent of the 322 patients had more than one of these coexisting anomalies, and 8% had just one. The most commonly used technique of repair of the coarctation was resection and end-to-end anastomosis, but no technique was a risk factor for death by multivariable analysis. Extension of the area of resection so that the end-to-end anastomosis was proximal to the left subclavian artery but distal to the left common carotid artery did not increase risk; extensions beyond this, and in the case of patch graft repair, extensions proximal to the left subclavian artery, did increase risk. Patch graft repair was associated with the highest prevalence (21%) of reintervention to the coarctation repair. Among patients with coexisting moderate-sized or large ventricular septal defects, repair of the coarctation, pulmonary trunk banding, and subsequent repair of the defect were associated with the highest 2-year survival, 97% in those with single ventricular septal defect. The risk-adjusted outcomes in two institutions were less good than in all others.

Clinical outcomes after the arterial switch operation for transposition. Patient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society.

As the probability increases that the arterial switch operation is optimal treatment for transposition, detailed information about outcomes and the circumstances in which they are suboptimal becomes important. A multi-institutional prospective study with annual detailed follow-up included 513 neonates with simple transposition or transposition and ventricular septal defect entering for diagnosis and treatment at < 15 days of age and undergoing an arterial switch repair. The 1-month and 1- and 5-year survivals were 84%, 82%, and 82%, respectively. The hazard function for death had a rapidly declining single phase that approached zero by 12 months after surgery. Among the eight patients who died > or = 3 months after the operation, four had severe ventricular dysfunction, probably related to imperfect coronary arterial transfer. Coexisting single ventricular septal defect was not a risk factor for death. Origin of the left main coronary artery or only the left anterior descending or the circumflex artery from the right posterior sinus (sinus 2) was a risk factor that was even stronger when an intramural course was present; multiplicity of ventricular septal defects was a risk factor. Longer global myocardial ischemic time and total circulatory arrest time were risk factors. Certain institutions were shown to be risk factors for death; the results in some improved with increasing experience, in some they did not, and in some they worsened. Good early and intermediate-term clinical outcomes can be obtained in neonates with simple transposition and transposition and ventricular septal defect by use of the arterial switch operation. Certain coronary artery patterns and certain institutions lessen the goodness of outcome.

Atypischer Ventrikelseptumdefekt

A case of a congenital cardiac malformation in form of a single ventricular septal defect is reported and histopathologic findings are presented. The possible origin with regard to its untypical localisation is discussed.

Incremental risk factors in hospital mortality rate after repair of ventricular septal defect

A total of 312 patients with single or multiple ventricular septal defects (VSD) with or without major associated lesions underwent surgical repair between 1967 and 1979. In the overall experience in patients without major associated defects, six hospital deaths (3.6%) occurred among 166 patients undergoing primary repair of single large VSDs, but in the last 5 years, one death (1.1%) occurred among 94 patients. Among the 29 patients with multiple VSDs, nine deaths (31%) occurred, but only one (7%) among the 14 operated upon in the last 5 years. In both groups, young age was an incremental risk factor for hospital death overall, but not in the last 5 years. Neither the location of the VSD nor the surgical approach were risk factors. Among 58 patients with major associated lesions and either single or multiple VSDs, 14 (24%) died after operation. In this subset, risk of hospital death did not decline during the 12 year period, and young age remained a significant incremental risk factor throughout. Of 261 patients with a single VSD and without a straddling tricuspid valve, one (0.4%) developed complete heart block, as did two (4%) of 47 patients with multiple VSDs. The decrease in hospital mortality rate with time and the neutralization of young age and multiplicity of VSDs as important incremental risk factors resulted from improvements such as more precise preoperative diagnosis of location and number of VSDs and improved intraoperative and postoperative care. The incremental risk of major associated lesions, especially in the very young and in those with multiple VSDs, should be neutralized in the future by-further research and development. However, in the meantime, management in most subsets should be individualized.

Multiple Ventricular Septal Defects

Multiple ventricular septal defects (VSD) were closed in 24 children aged 3 to 13 years (mean 6.7). The number of defects ranged from 2 to 7 (mean 4). There were 3 hospital deaths and 1 late death. Three patients required reoperation to close defects which had reopened or had been overlooked previously, and 1 of these died (the late death in the series). High defects in addition to low muscular ones were present in 20 of the patients. Cardiac failure due to large shunts had necessitated previous pulmonary artery banding in 10 patients (42%), compared to 13% of patients with single VSDs. Preoperative angiocardiography correctly predicted multiple defects in 13 patients. Closure of multiple low muscular defects was found to be facilitated by an external buttress technique.