Residual Ventricular Septal Defect Research Articles

Our Experience in 33 Patients of Multiple Ventricular Septal Defect Closure

Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.

Fluorescein dye-guided intraoperative identification and closure of muscular ventricular septal defect.

Various techniques for intraoperative identification of muscular ventricular septal defects (VSDs) are cumbersome, difficult to use in infants, and inefficient in confirming the completeness of closure. We used simple technique of intraoperative fluorescein saline injection into the left ventricle to locate the muscular VSDs and to confirm their closure. This is a prospective observational study conducted between April 2013 to December 2013, involving 22 patients with multiple VSDs either in isolated form or in association with other congenital cardiac anomalies. Following the closure of all visible VSDs, an 8F infant feeding tube was introduced into the left ventricle through the fossa ovalis and fluorescein saline was injected. Right ventricle was observed for saline leakage and VSD site tracking. Saline injection was repeated till appearance of no significant leak. A total of 63 defects were closed in 22 patients. Only two patients required re-endocardialization or septal exclusion technique for Swiss cheese septum. None of them required division of moderator band or major trabeculae. Eleven patients had insignificant residual shunting found on postoperative echocardiography. There were two deaths. In the remaining 20 patients, the mean of intensive care unit (ICU) and hospital stay was 5.4 ± 3.72 and 10.2 ± 4.64 days, respectively. Comparative study between patients with and without residual VSD showed no significant difference in mean inotropic score, ICU, and hospital stay, confirming the absence of significant residual shunting. Intraoperative fluorescein saline injection into the left ventricle is safe, effective in precisely localizing muscular VSDs and confirming the completeness of their closure.

Abstract 14058: Intramural Ventricular Septal Defects After Repair of Conotruncal Anomalies Are Associated With Postoperative Morbidity

Introduction: Intramural ventricular septal defects (VSDs) are tunnel-like communications between a great artery and right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We sought to assess the prevalence of residual intramural VSDs and their impact on postoperative course. Methods: We reviewed all patients at our institution who underwent biventricular repair of a conotruncal anomaly involving VSD baffle to a great artery from the left ventricle from 1/1/11 to 6/30/13. Perioperative echocardiographic images were reviewed for residual intramural or non-intramural VSDs. The primary outcome was a composite of ECMO use, cardiac transplantation, or mortality. Secondary outcomes included hospital length of stay (LOS) and need for repeat catheter-based or surgical VSD closure. Results: Of 158 total subjects, 92 (58%) were male and diagnoses were tetralogy of Fallot (n=111), d-transposition of the great arteries (14), truncus arteriosus (13) and other (20). Median age at surgery was 51 days (IQR 6,107). Residual VSD was present in 111 subjects; 29 (26%) had an intramural defect, and 82 (74%) had non-intramural defects (71 peripatch defects, 6 small muscular defects, and 5 with both). Of the non-intramural VSDs, 79 (96%) were hemodynamically insignificant lesions (<2mm). Residual intramural VSDs were more likely than non-intramural VSDs to be greater than 2mm (48% vs 4%, p<0.001). There was no difference in weight, age at operation, or type of conotruncal anomaly based on type of residual VSD. The primary composite outcome was more prevalent in subjects with residual intramural defects compared to non-intramural defects (5/29 [17%] vs 4/82 [5%], p = 0.05). In addition, subjects with residual intramural VSDs had longer LOS (24 days [IQR 13,67] vs 11 days [IQR 5,23], p=0.002). The rates of repeat catheter-based or surgical closure were low overall but higher in subjects with intramural VSDs (8/29 [28%] vs 5/82 [6%], p=0.005). Conclusions: Intramural VSDs are common after repair of conotruncal anomalies and tend to be larger than non-intramural VSDs. Although most subjects still have favorable outcome, intramural VSDs are associated with greater postoperative morbidity, longer LOS, and need for reintervention.

A Novel Technique for Repair of Complete Atrioventricular Canal Defect: The Central Patch Technique.

Two-patch, single-patch, and modified single-patch repairs are accepted techniques for repair of complete atrioventricular (AV) canal defects. We propose a novel, alternative technique: the central patch technique. For the central patch technique, the superior and inferior bridging leaflets are attached with simple sutures to the right and left of their coaptation point. Both bridging leaflets are incised along a line above the ventricular crest, similar to a traditional single-patch technique. An oval pericardial patch is sewn to the central defect created in the AV valve tissue. Interrupted, horizontal mattress sutures are placed along the ventricular crest, through the midline of the central patch and through the edge of the atrial septal defect (ASD) patch. Tying the sutures simultaneously closes the ventricular septal defect (VSD) and secures the ASD patch to the ventricular crest. Repair of the left AV valve and ASD closure are performed in the routine fashion. Five patients underwent the central patch technique repair of complete AV canal defect. Weight was 4.8 to 6.3 kg; age was four to eight months. Cardiopulmonary bypass and myocardial times averaged 137 minutes and 109 minutes, respectively. No patient had more than mild left AV valve regurgitation or trivial residual VSD at completion of repair. No patient developed left ventricular outflow tract obstruction. The central patch technique is applicable to all forms of complete AV canal defect. Subjectively, it offers technical advantages compared to standard techniques and may result in a shorter learning curve for junior congenital heart surgeons. Results are preliminary but are consistent with standard techniques.

Complete Atrioventricular Septal Defect: Comparison of Modified Single-Patch Technique with Two-Patch Technique in Infants

The purpose of this study was to compare the modified single-patch technique with the two-patch technique for infants with complete atrioventricular septal defects (AVSDs). Between December 2001 and October 2011, 98 infants underwent complete AVSD primary repair. Forty-six patients had a modified single-patch technique; 59 patients had a two-patch technique. Eighty-seven patients had follow-up by echocardiography to measure the degree of valve regurgitation. There were two deaths (one in modified single-patch group and one in two-patch group). Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (70.56 ± 21.05 vs. 83.76 ± 22.74 minutes, p=0.004; 95.02 ± 19.73 vs. 109.9 ± 34.07, p=0.011). There was no patient with third-degree atrioventricular (AV) block in the modified single-patch group, while two patients in the two-patch group required a pacemaker (3.85%, p=NS). During follow-up, one death occurred in the single-patch group and three deaths in the two-patch group. At last follow-up, ten patients had more than moderate left AV valve regurgitation (four in single-patch group vs. six in two-patch group, p=0.886) and eight patients required reoperation (three in single-patch group vs. five in two-patch group, p=0.841). One patient in the single-patch group required reoperation for a residual ventricular septal defect and none in the two-patch group. Modified single-patch repair in infants with complete AVSD is a safe and reproducible technique. The results are as good as the two-patch technique. Among long-term survivors, most have very good clinical and functional results and minimal or no regurgitation of either AV valve.

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.

Technical Performance Score as Predictor for Post-discharge Reintervention in Valve-Sparing Tetralogy of Fallot Repair

Recognition of late problems following repair of tetralogy of Fallot (TOF) with a transannular patch has stimulated modifications to preserve pulmonary valve (PV) function. This study assesses the ability of technical performance score (TPS) to determine the need for post-discharge reinterventions (RIs) in valve-sparing TOF repair. We retrospectively reviewed 157 patients following valve-sparing repair of TOF from 2007-2012. We assigned TPS as Class 1 (optimal), Class 2 (adequate), or Class 3 (inadequate) based on discharge echo and clinical criteria. Preoperative, discharge, and follow-up PV Z scores and post-discharge RIs were documented. Reasons for Class 2 or 3 designation were right ventricular outflow tract (RVOT) gradient in 52, pulmonary regurgitation in 13, residual ventricular septal defects in 7, both RVOT gradient and ventricular septal defects in 13, and both RVOT gradient and pulmonary regurgitation in 37 patients. Median follow-up was 19.6 (range: 0.1-86.1) months. Class 3 patients had a significantly longer median intensive care unit and hospital stay compared with Class 1 (3 vs 2 days [P = 0.015] and 7 vs 5 days [P < 0.001], respectively). Post-discharge RIs were significantly lower in Class 1 vs Class 2 and Class 3 (P = 0.003). Class 1 patients had significantly larger PV Z scores compared with Class 2 or Class 3 patients (P < 0.001). TPS is associated with post-discharge RI rate after valve-sparing TOF repair. Preoperative PV Z score is highly correlated with Class I TPS. Patient selection based on preoperative PV Z scores may help determine if valve-sparing approach is appropriate, thus minimizing the need for RIs.

Female patient after correction of tetralogy of Fallot with severe pulmonary regurgitation and significant left-to-right shunt at the ventricular septal level (RCD code: IV-5A2)

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease after 1 year of age. Less than 5% of all patients with uncorrected ToF live beyond the age of 40 years. Prognosis after surgical correction is good with a 32-year survival of 86% of the cases. Predictors of long-term mortality include older age at operation and postoperative right-to-left ventricular peak systolic pressure ratio of 0.5 or higher [1]. Surgery is performed to close ventricular septal defect (VSD) and relieve right ventricular outflow tract obstruction(RVOTO). Adult patients with surgical repair of tetralogy of Fallot in history often develop complications, which include severe pulmonary regurgitation, RVOTO, right ventricular dilation and dysfunction, residual VSD, aortic root dilation with aortic regurgitation, left ventricular dysfunction, endocarditis, atrial and ventricular tachycardia, and sudden cardiac death. We present a case of a female patient with several long-term complications after ToF repair. JRCD 2014; 1 (7): 24–28

Modified Single-Patch Compared With Two-Patch Repair of Complete Atrioventricular Septal Defect

We compared the outcomes of modified single-patch and two-patch surgical repair of complete atrioventricular septal defect (CAVSD) on left ventricular outflow tract (LVOT) diameter and on left atrioventricular valve (LAVV) coaptation. We reviewed retrospectively postoperative 2-dimensional echocardiograms of all CAVSD patients who underwent modified single-patch or two-patch repair between 2005 and 2011. We measured the leaflet coaptation length of the LAVV in the apical four-chamber view. The LVOT was measured in the long axis view. Fifty-one patients underwent CAVSD repair at a median age of 4 months (range, 1 to 9 months) (single-patch, n=29; two-patch, n=22). The images from 46 echocardiograms were adequate for analysis. Modified single-patch repair required significantly shorter bypass time (102.0±33.6 vs 152.9±39.5 minutes, p<0.001) and ischemic time (69.0±21.7 vs 106.9±29.7 minutes, p<0.001) than did two-patch repair. The indexed coaptation length of the septal and lateral leaflets was not different between single-patch and two-patch (3.1±2.3 vs 4.1±3.1 mm/m2, p=0.25; 2.3±2.3 vs 3.3±3.0 mm/m2, p=0.21). Indexed LVOT diameter was not different in the two groups (26.1±5.2 vs 28.5±7.1 mm/m2, p=0.22). There was no hospital or late death during the median follow-up time of 35 months (range, 1 to 69 months). Five patients underwent reoperation after single-patch repair (3 with residual ventricular septal defect [VSD] and LAVV regurgitation, 1 with residual VSD, 1 with pacemaker implantation). After the two-patch repair, 1 patient required reoperation for a residual VSD and right atrioventricular valve regurgitation (p=0.22). The modified single-patch repair was performed with significantly shorter bypass time and myocardial ischemic time. The postoperative LVOT diameter and LAVV leaflet coaptation length were not significantly different between techniques.

Perventricular Closure of Perimembranous Ventricular Septal Defects Using the Concentric Occluder Device

This report summarizes the authors' clinical experience with perventricular closure of the perimembranous ventricular septal defect (PmVSD) using the concentric occluder as a minimally invasive technique without cardiopulmonary bypass and following transesophageal echocardiography (TEE) guidance. Between July 2011 and March 2013, 61 patients with PmVSD underwent perventricular concentric device closure using a minimally invasive inferior sternotomy approach. The basal diameter of the PmVSD ranged from 2.5 to 7 mm. The diameter of the occlusion device waist ranged from 4 to 8 mm. A ventricular septal aneurysm or an adhesive tricuspid valve was present in 49 patients. Multiple orifices in the aneurysm were treated in ten patients, including dispersed orifices in four patients and comparatively concentrated orifices in the remaining six patients. The occlusion devices were deployed via the right ventricle with TEE guidance and no cardiopulmonary bypass. Complete shunt occlusion was achieved for all the patients in the operating room. The orifices of the ventricular septal aneurysm were closed in 46 patients, with the left disc of the concentric devices placed in the aneurysms, whereas the PmVSDs in the remaining 15 patients were closed from the ventricular septal defect (VSD) basal part following the rule that the upper rim of the PmVSD be more than 2 mm from the aortic valve. Ventricular premature beats occurred in one patient. The follow-up period ranged from 1 to 21 months (median 13.5 ± 8.2 months), and the patients had stable heart function postoperatively. None of the patients had more than mild valvular regurgitation, and no worsening regurgitation was observed in those who had tricuspid or aortic regurgitation before surgery. No complete atrioventricular block, position shift of the occlusion devices, thrombosis, or residual VSD occurred during the follow-up period. The perventricular concentric device closure of PmVSD with an inferior sternotomy in selected patients is a safe, feasible, and simple treatment. The concentric device is easier to anchor than the eccentric device and has proved to be a reasonable choice for PmVSD occlusion, especially in patients with a perimembranous aneurysm formation or PmVSD extending to the inflow tract.

Long-term outcome of correction of tetralogy of Fallot in 56 adult patients

Background Although most patients with tetralogy of Fallot undergo radical repair during infancy and childhood, patients that remain undiagnosed and untreated until adulthood can still be treated. This study aimed to evaluate long-term outcomes of adult patients with tetralogy of Fallot who were treated surgically, and to determine the predictors of postoperative pulmonary regurgitation. Methods Fifty-six adult patients underwent complete surgical repair. Forty-three patients (76.8%) required a transannular patch. Systolic, diastolic, and mean pressure in the main pulmonary artery were measured after repair. Results The early mortality rate was 3.6%. The 16-year survival rate was (84.4±11.5)%. Late echocardiography revealed 41 patients with transannular patch who had pulmonary regurgitation, consisting of mild pulmonary regurgitation in 28 patients, moderate in eight, and severe regurgitation in five patients. In addition, there was right ventricular outflow tract stenosis in nine patients, moderate/severe tricuspid valve regurgitation in seven, and residual ventricular septal defect in five. Logistic regression analysis demonstrated that the mean pulmonary pressure measured just after repair predicted late pulmonary regurgitation. Conclusions The long-term survival of surgically treated adult patients with tetralogy of Fallot is acceptable. The mean pressure &gt;20 mmHg in the main pulmonary artery measured right after surgical repair may be a feasible reference to time the reconstruction of the pulmonary valve.

Desinserción parcial de válvula tricúspide para cierre de comunicación interventricular

IntroductionVentricular septal defect closure through the right atrium might be cumbersome in the presence of redundant subvalvar apparatus. Partial tricuspid valve detachment to ease the defect closure is presented. MethodsRetrospective review of 156 patients with ventricular septal defect (82 isolated, 74 Fallot). 20 cases (9 isolated, 11 Fallot) had the tricuspid valve detached (12,8). Mean age and weight was 10,6 months and 7,13kg, respectively. The anterior tricuspid leaflet was electively, partially detached, thus providing a straightforward closure of the defect with Dacron patch (19 patients) or directly (1). Associated procedures: mid-ventricle muscle bundle resection (2), aortic valve re-attachment (2), Alfieri stitch (1) and mitral commisurotomy (1). ResultsMean extracorporeal time was 107minutes (range 61-153) and mean ischemic time was 58minutes (range 31-99). No residual ventricular septal defect, tricuspid regurgitation or aortic regurgitation was detected in intra-operative echographic assessment. One patient died due to sepsis. Another patient had a definite pacemaker implanted for complete heart block. Mean follow-up was 25 months, with no residual defects. ConclusionsPartial detachment of the anterior tricuspid leaflet is useful for right atrium closure of ventricular septal defect in difficult cases. This approach is optimal, rendering good results and little morbidity.

Early- and mid-term results of off-pump pulmonary valve implantation: a single-center experience

Background: This study aims to assess the early- and midterm results of off-pump pulmonary valve implantation (PVI). Methods: Between October 2006 and February 2012, nine patients (5 males, 4 females; mean age 18.4±6.3 years; range 8 to 27 years) underwent off-pump PVI with an injectable stent. Echocardiography revealed severe pulmonary valve regurgitation, progressive right ventricular dilatation and dysfunction in all patients. No residual ventricular septal defect (VSD) was seen. Results: We implanted a 23 mm valve in one patient, a 25 mm valve in two patients, a 27 mm valve in two patients, a 29 mm valve in three patients and a 31 mm valve in one patient. Postoperative echocardiographic examination revealed no regurgitation in seven patients with mild valve regurgitation in two patients. The mean peak systolic gradient was 12.6±3.7 mmHg, while the mean systolic gradient was 8.3±2.4 mmHg. The mean follow-up was 34±17 months (range, 1.5 to 58 months). None of the patients needed reoperation. Conclusion: The advantages of off-pump PVI include the applicability without cardiopulmonary bypass, availability of large-sized valves, the absence of any interventional limitation and the opportunity for fixation into the pulmonary artery to avoid valve migration. No-react nature of the valve also minimizes the degeneration risk of the valve.

Stable right ventricular size and function during short-term follow-up in patients with pulmonary regurgitation after tetralogy of Fallot repair

To assess changes in ventricular size and function over time in conservatively treated adult patients with repaired tetralogy of Fallot (TOF) and significant pulmonary regurgitation (PR). Patients with repaired TOF who had undergone more than one cardiac magnetic resonance study were identified. To confine the cause of ventricular size and function deviation to PR, patients with residual ventricular septal defect, more than mild regurgitation at a valve other than the pulmonary valve, and known coronary artery disease were excluded. The final analysis included 27 adults with PR fraction >20%. During a follow-up of mean 2.1 ± 0.8 years, there was no change in right ventricular (RV) end-diastolic volume (EDV; 162.1 ± 27.6 versus 164 ± 29.6 ml/m(2), p = 0.5). Left ventricular (LV) EDV showed a small decrease (85.1 ± 16.2 versus 81.5 ± 14.1 ml/m(2), p = 0.02). The mean PR fraction, PR volume, and peak RV outflow tract gradient did not change. Additionally, both RV ejection fraction (EF) and LVEF remained stable over the follow-up period (48.1 ± 6.5 versus 48.4 ± 6.7%, p = 0.83, and 57.3 ± 5.4 versus 57.2 ± 5.1 %, p = 0.91, respectively). Only two asymptomatic patients (7.4% of the study group) developed symptoms and the remaining did not deteriorate. The RVEDV, RVEF, and LVEF remained stable over a mean follow-up of approximately 2 years in the majority of adult patients after TOF repair with significant PR and a wide range of RVEDV.

Cardiovascular MR Imaging after Surgical Correction of Tetralogy of Fallot: Approach Based on Understanding of Surgical Procedures

Tetralogy of Fallot (TOF) is one of the most common congenital heart diseases for which patients are referred for postoperative magnetic resonance (MR) imaging evaluation. The most common surgical procedures for TOF repair include infundibulectomy, transannular pulmonary artery patch repair, and right ventricle-pulmonary artery conduit placement. In the past few decades, surgery has proved successful, but most patients require repeat imaging throughout their lives. MR imaging is now frequently used for morphologic and functional evaluation after TOF repair. The most common late postoperative sequelae and residual lesions include right ventricular outflow tract aneurysm and dyskinesis, conduit failure, pulmonary regurgitation, tricuspid regurgitation, right ventricular failure, residual main and branch pulmonary artery stenosis, branch pulmonary artery aneurysm, left pulmonary artery kinking, and residual or recurrent ventricular septal defect. The imaging approach for the evaluation of patients with repaired TOF should be guided by the surgical procedure used and the complications that are expected. Knowledge of the most common postoperative problems and their cardiovascular MR imaging appearances is essential for good radiology practice in this clinical setting.

Reoperation After Arterial Switch: A 27-Year Experience

The long-term outcome and spectrum of reoperation after the arterial switch operation (ASO) has not been fully defined, and there are limited data in the literature. We reviewed our institutional experience with reoperation(s) after ASO. Between January 1984 and January 2012, 32 patients (23 male) underwent reoperation(s) after ASO. Anatomy included simple transposition of the great arteries in 14, complex transposition of the great arteries in 14, and Taussig-Bing in 4. Mean age was 6.7 ± 1.4 years at first operation and 10.8 ± 13.4 years at the second operation. Isolated pathology was present in 11 (34.3%) and multiple pathologies in 21 (65.6%). Abnormalities at first reoperation were right-sided pathology in 18 (56.3%), left-sided pathology in 10 (31%), coronary artery in 3 (9%), mitral valve in 3 (9%), residual ventricular septal defect in 4 (12.5%), and recoarctation in 2 (6.3%). It was the second reoperation in 12 and the third reoperation in 3 patients. The first reoperation included pulmonary artery patch plasty in 18, aortic valve operation in 8 (4 valve replacement, 3 root replacement, and 1 repair), pulmonary valve replacement in 4, coronary artery bypass grafting in 3, and mitral valve repair in 3. Multiple reoperations occurred in 15 patients, comprising right-sided procedures (11), left-sided (2), and other (2). Pulmonary artery reconstruction occurred earlier than neoaortic intervention (5.4 ± 6.8 vs 13.8 ± 7.7 years, p < 0.001). There were 2 early deaths (6.2%); both patients had complex transposition of the great arteries and both were at early reoperation after ASO. Median follow-up was 14.5 years (maximum, 27 years). There were no late deaths. Freedom from reoperation at 1, 5, and 15 years was 88%, 78%, and 41%, respectively. The most common indication for reoperation after ASO is right-sided pathology, followed by neoaortic root pathology. Late survival after ASO is excellent and risk of late reoperation is low. Life-long medical surveillance is required.

Management of multiple ventricular septal defects: Evolution of surgical technique

Background Residual ventricular septal defects (VSDs), ventricular and septal dysfunction and heart block are frequent complications in surgical management of multiple VSDs (Swiss cheese heart). The technique with the lowest risk of morbidity and mortality has still to be identified. Methods Between January 200 and September 2012, 45 patients with a median age of 4.1 years (2 months to 15 years) underwent surgical closure of multiple VSDs. In 22 cases there were associated lesions. Two-stage repair, i.e. pulmonary artery banding (PAB) followed by VSDs closure using different techniques, was adopted in 29 cases (group A). Primary repair was performed in 15 cases (group B). Six out of these 15 cases were managed by the so-called sandwich technique (group B1), whereas in the other nine other techniques of closure were used (group B2). Results The overall mortality was 8.9% (4/45). Three of the four deaths occurred in group A and one in group B2. Follow-up of group A showed that 26 patients had residual VSDs (2 of whom needing early redo surgery), 2 patients had left ventricular dysfunction and one patient developed heart block. In group B1, there were five cases with residual VSDs. In group B2, there were five cases with residual VSDs (only one needing redo surgery), one case with left ventricular dysfunction and one case with heart block. Conclusion Primary repair of multiple VSDs is associated with less morbidity and mortality than staged repair. The sandwich technique has the lowest risk of residual VSDs, septal dysfunction and heart block compared to the other techniques. However, particular attention is needed in positioning the left-sided patch to avoid septal bulging with consequent left ventricular outflow tract obstruction and interference with mitral valve chordae with secondary mitral regurgitation.

Combined transcatheter device closure of ruptured sinus of valsalva and a post‐surgical residual ventricular septal defect

Sinus of Valsalva aneurysms are commonly associated with ventricular septal defects (VSDs). We describe a 14-year-old boy who presented with rupture of right sinus of Valsalva (RSOV) into the right ventricular outflow tract (through two openings) along with a residual VSD, four years after surgical closure of the VSD. Both antegrade and retrograde approaches were used to close the RSOV defects. The larger defect was closed from the venous side using an Amplatzer Duct Occluder (ADO-I) device while the smaller defect was closed from the arterial end using and ADO-II device. The residual VSD was also closed percutaneously using a ADO-I device. Successful transcatheter closure of all defects was possible thereby preventing the morbidity and mortality associated with a repeat surgery.

Hybrid melody pulmonary valve replacement in an adult with severe pulmonary hypertension and pulmonary artery aneurysm

A 48-year-old female with D-TGA, ventricular septal defect (VSD), pulmonary stenosis, pulmonary hypertension (PAH), and total anomalous pulmonary venous connection underwent hybrid intervention for a pulmonary artery (PA) aneurysm and replacement of a dysfunctional pulmonary valve (PV). She underwent a hemi-Mustard procedure at 9 years of age but remained cyanotic. She developed atrial fibrillation, heart failure, and functional decline at 43 years of age. A chest CT demonstrated a 6 cm PA aneurysm that upon re-imaging at 48 years had increased to 11 cm. A catheterization procedure revealed severe PS, PR, residual VSD, severe PAH with a pulmonary vascular resistance of 30 Wood units. She was evaluated and turned down for heart-lung transplantation at another institution. She was subsequently referred to our institution for heart-lung transplantation but was felt to be at unacceptably high risk given the complexity of her anatomy, imaging suggesting liver cirrhosis and liver biopsy with extensive fibrosis. After extensive discussion of risk and benefits, the patient agreed to proceed with a hybrid intervention, consisting of surgical aneurysm resection/PA repair, tricuspid valve repair; PV replacement with a Melody valve, and VSD closure. There were no complications and she was discharged home within 2 weeks. Six months post procedure, she is not on oxygen, her resting room air saturation is 94%, and echocardiography shows stable Melody valve function. This case highlights the utility of a hybrid approach in the treatment of an adult with complex congenital heart disease, heart failure and severe PAH, considered at the highest risk for adverse surgical outcomes. The short-term efficacy of the Melody valve in severe PAH is reassuring.

Pitfalls in Repair of Conotruncal Anomalies

Despite a wide anatomic diversity, the complete repair of all conotruncal anomalies includes two surgical steps. 1) An intracardiac tunnel is created to connect the left ventricle to one of the arterial orifices (usually the aortic, sometimes the pulmonary), through the conoventricular ventricular septal defect. Any conal septum should be resected to create a short, large, and straight tunnel. Abnormal insertions of the atrioventricular valves (tricuspid and mitral) on the conal septum should be preserved. "Intramural" residual ventricular septal defects must be avoided by anchoring the intracardiac patch directly to the arterial annulus. 2) To connect the right ventricle to the pulmonary artery, either an intracardiac or an extracardiac reconstruction is carried out, according to the distance between the tricuspid valve and the pulmonary orifice. When extracardiac reconstruction is indicated, it is usually performed without prosthetic conduit (with or without French maneuver, eventually using the left atrial appendage). In most patients, complete repair can be performed as a primary operation during infancy.