Concomitant Ventricular Septal Defect Research Articles

The results of single–stage repair for coarctation with ventricular septal defect in neonates

Background: The association of coarctation of the aorta (CoA) with a ventricular septal defect (VSD) in neonates is seriously who needs to surgery if not control heart failure by medicine in neonates. There are 2 procedures for repair includes: Two-stage repair (the repair coarctation and closure of the concomitant VSD as a secondary procedure), One-stage repair (the repair coarctation and closure of the concomitant VSD). Methods: We retrospectively reviewed patients (in neonate) undergoing repair of CoA with closure of VSD from 1/2015 to 12/2023. There were sixteens patients who underwent one-stage repair. The mean of age and body’s weight were 21 days (range from 14 days to 29 days) and 3,3 kilograms (range from 2,9 to 3,6). All patients had severe heart failure, lager VSD and gradient through aortic isthmus that was 54 mmHg. Results: The time of cardiopulmonary bypass, aortic clamping and selective cerebral perfusion time were: 126 mins; 87 mins and 18 mins. There were 5 patients with open chest. One patient died at ICU by multiple organ failure and one patient with cerebral haemorrhage. The gradient postopration of aortic arch was 7 mmHg with no residual VSD. During follow-up time 57 months after surgery, there was no late death. The mean gradient of aortic arch was 12 mmHg and one patient with 25 mmHg Conclusion: The results of single–stage repair for coarctation with ventricular septal defect in neonates was good with low re-coarctation.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The studyis aretrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1years) than those with d-TGA (37.5 ± 8.3years) or complex ccTGA (40.8 ± 13.4years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Being Overweight Is Related to Neoaortic Sinus Dilatation After Arterial Switch Operation.

Neoaortic sinus dilatation is a late comorbidity after an arterial switch operation in patients with dextro-transposition of the great arteries. We aim to explore whether neoaortic sinus dilatation is related to overweight or obesity, as these affect aortic remodeling in such patients. We measured neoaortic diameters including those for the annulus, sinus, sinotubular junction, and ascending aorta by echocardiography and studied the relationship between these diameters and body mass index in patients aged 15 years and older after an arterial switch operation. A total of 45 patients were studied. Median (interquartile range) age, body mass index, and systolic blood pressure at echocardiography were 20 (17-28) years, 21.2 (18.4-24.2) kg/m2, and 120 (112-127) mm Hg, respectively. Echocardiogram revealed the following median values (interquartile ranges): neoaortic annulus diameter, 22.2 (19.8-23.8) mm; sinus diameter, 34.6 (31.8-39.8) mm; sinotubular junction diameter, 27.5 (22.4-30.0) mm; and ascending aortic diameter, 20.4 (19.4-22.7) mm. The neoaortic sinus diameter was significantly correlated with body mass index (r = 0.41, P = .004) and was significantly larger in patients with concomitant ventricular septal defect (P < .001) and those who were over-weight (body mass index ≥25 kg/m2; P = .006). Neoaortic sinus dilatation after an arterial switch operation is associated with being overweight during adolescence and young adulthood. It is important to prevent obesity in patients after an arterial switch operation and educate them on a healthy lifestyle.

Long-term results of biventricular correction for patients with double outlet right ventricle.

The purpose of this study was to report outcomes of double outlet right ventricle biventricular repair at a single institution in developing countries and to investigate post-operatively determined risk factors for mortality and late intervention. Patients with double outlet right ventricle who underwent biventricular repair in our centre from January 2009 to December 2021 were included. A total of 96 patients (male/female = 61/35) with biventricular repair were included. According to its specific anatomical type, the appropriate individual surgical plan was selected. Multivariate analysis indicated that prolonged cardiopulmonary bypass time(OR = 1.044; p = 0.012) and pre-operative moderate or above pulmonary hypertension(OR = 24.558; p = 0.023) were independent risk factors for early mortality. Univariate analysis showed that different anatomical types and different surgical methods had similar late intervention and late mortality. Concomitant coarctation of the aorta(OR = 40; p = 0.020) and concomitant ventricular septal defect enlargement(OR = 26.667; p = 0.005) were independent risk factors for late intervention by multivariate analysis. Selection of appropriate surgical techniques based on different anatomical types often results in similar late outcomes. For patients with concomitant ventricular septal defect enlargement during the operation, it is necessary to fully expand and avoid damage to the conduction bundle. We should timely intervention in patients with coarctation of the aorta and pay attention to the occurrence of left ventricular outflow tract obstruction during follow-up.

Aortic valve repair in patients with ventricular septal defect.

The aim of the study is to evaluate early and late clinical outcomes of patients undergoing the aortic valve repair in patients associated with ventricular septal defect and to analyze whether age is a risk factor predicting repair failure. All patients who were underwent aortic valve repair with concomitant ventricular septal defect closure from 2006 to 2018 were included and divided into two groups: less than 10 years (Group 1) and more than or equal to 10 years (Group 2). About 159 patients were included (Group 1 = 92; Group 2 = 67). Overall median age was 8 years, 70% were males. The most common ventricular septal defect was perimembranous in both groups. Two patients in Group 2 had to undergo aortic valve replacement subsequently which resulted in early mortality. Univariable analysis showed prolonged ventilation (p < .001), postoperative intensive care unit stay (p = .019) as significant factors for early mortality. Estimated survival was 97.6% ± 2.4 at the end of 5 years and 10 years in Group 1 and 93.6% ± 3.6 in Group 2. Freedom from residual aortic regurgitation more than or equal to moderate at 5 years and 10 years was 85.6% ± 4.9 and 55% ± 13.8 in Group 1 and 76.9% ± 6.7 and 47.4% ± 12.2 in Group 2, however, it was not significant (p = . .084). The aortic valve repair in patients with ventricular septal defect provides satisfactory early and late results in terms of mortality and need for reintervention in all age groups.

Prognostic Model to Predict Postoperative Adverse Events in Pediatric Patients With Aortic Coarctation.

Background: Postoperative adverse events remain excessively high in surgical patients with coarctation of aorta (CoA). Currently, there is no generally accepted strategy to predict these patients' individual outcomes.Objective: This study aimed to develop a risk model for the prediction of postoperative risk in pediatric patients with CoA.Methods: In total, 514 patients with CoA at two centers were enrolled. Using daily clinical practice data, we developed a model to predict 30-day or in-hospital adverse events after the operation. The least absolute shrinkage and selection operator approach was applied to select predictor variables and logistic regression was used to develop the model. Model performance was estimated using the receiver-operating characteristic curve, the Hosmer–Lemeshow test and the calibration plot. Net reclassification improvement (NRI) and integrated discrimination improvement (IDI) compared with existing risk strategies were assessed.Results: Postoperative adverse events occurred in 195 (37.9%) patients in the overall population. Nine predictive variables were identified, including incision of left thoracotomy, preoperative ventilation, concomitant ventricular septal defect, preoperative cardiac dysfunction, severe pulmonary hypertension, height, weight-for-age z-score, left ventricular ejection fraction and left ventricular posterior wall thickness. A multivariable logistic model [area under the curve = 0.8195 (95% CI: 0.7514–0.8876)] with adequate calibration was developed. Model performance was significantly improved compared with the existing Aristotle Basic Complexity (ABC) score (NRI = 47.3%, IDI = 11.5%) and the Risk Adjustment for Congenital Heart Surgery (RACHS-1) (NRI = 75.0%, IDI = 14.9%) in the validation set.Conclusion: Using daily clinical variables, we generated and validated an easy-to-apply postoperative risk model for patients with CoA. This model exhibited a remarkable improvement over the ABC score and the RACHS-1 method.

Surgical repair of concomitant ventricular septal defect and aortic cusp prolapse or aortic regurgitation, also known as the Laubry-Pezzi syndrome

Surgical repair of concomitant ventricular septal defect and aortic cusp prolapse or aortic regurgitation, also known as the Laubry-Pezzi syndrome

Midterm Echocardiographic Assessment of Right Ventricular Function After Midline Unifocalization

Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function. Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study-aided repair of PA/VSD/MAPCAs. For patients who underwent either concomitant or delayed intracardiac repair, echocardiographic RV systolic function was retrospectively calculated to assess (1) RV fractional area change (RVFAC) and (2) two-dimensional RV longitudinal strain (RVLS) of the free wall of the right ventricle. QLAB cardiac analysis software version 10.3 (Philips Medical Systems, Andover, MA) was used for analysis. A total of 64 patients underwent concomitant VSD closure at the time of unifocalization, and 16 patients underwent delayed VSD closure at a median of 2.3 years (range: 3 days to 7.4 years). At a median follow-up of 8.1 years (range: 0.1 to 19.5 years) for the concomitant repair group versus 7.4 years (range: 0.01 to 15.3 years) for the delayed repair group, no differences in RVFAC and RVLS were observed (RVFAC: 41.0% ± 6.2% versus 41.2% ± 7.6%, p= 0.91; RVLS:-18.7 ± 4.3 versus-18.9 ± 4.0, p= 0.87). Patients (83%) with PA/VSD/MAPCAs underwent complete repair at intermediate-term follow-up with preserved RV function. Delayed VSD closure was accomplished in 50% of the patients initially deemed unsuitable for repair. Delayed VSD closure did not affect survival and did not portend impaired RV systolic function.

D-TGA Combined With Left Arch Atresia of a Double Aortic Arch

A three-month-old female underwent an arterial switch operation for transposition of the great arteries (TGA) with concomitant ventricular septal defect (VSD). After the operation, the patient suffered from stubborn pulmonary infection with increased airway resistance, and could not be weaned from a ventilator. Multispiral computed tomography (CT) scanning suggested a double aortic arch with left arch atresia. The patient underwent a second procedure to resection the left aortic arch. The patient gradually recovered and was successfully weaned from the ventilator.

Right ventricular outflow tract stenting in double outlet right ventricle with critical pulmonary stenosis and hypoplastic pulmonary arteries.

Double outlet right ventricle (DORV) is a congenital heart disease in which the great arteries (aorta and main pulmonary artery) arise from the right ventricle (RV), with concomitant ventricular septal defect (VSD), usually non-restrictive. Double outlet right ventricle can be accompanied by other cardiovascular anomalies, such as ventricular hypoplasia, restrictive septal defects and various pulmonary vascular bed anomalies, including hypoplastic pulmonary arteries (PA). Critical right ventricle outflow tract (RVOT) stenosis and hypoplasia of pulmonary arteries in newborns with DORV necessitates the need to maintain pulmonary blood flow to provide optimal systemic oxygenation in severely cyanotic newborns. Routinely recommended treatment for cyanotic newborns is a surgical procedure with implantation of a Blalock-Taussig systemic-to-pulmonary artery shunt (BT shunt), or interventional patent arterial duct (PDA) stenting. In complex RVOT stenosis with hypoplastic pulmonary arteries alternative percutaneous multilevel RVOT and pulmonary trunk stenting could serve as an alternative treatment [1, 2]. We report multistage treatment of a hypotrophic infant suffering from DORV with RVOT stenosis and a hypoplastic pulmonary vascular bed. Initial diagnostics with angiography revealed multilevel obstruction of the RVOT with severe hypoplasia of pulmonary arteries. The baby, who presented severe cyanosis, did not meet anatomic criteria for surgical palliation while the ductal flow remained insufficient. Her initial morphology determined our alternative strategy with initial RVOT stenting, despite the hindrance caused by extremely low body weight.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: primary repair

Primary repair of pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collaterals based on single-stage unifocalization was first reported in 1995. From a midline approach, all collaterals are extensively dissected, translocated in front of the oesophagus and/or the trachea, when required, and directly anastomosed to each other or to the native pulmonary arteries, whenever present, without interposition of prosthetic material. The need for concomitant VSD closure is assessed intraoperatively with a pulmonary flow study according to a standardized protocol. Pulmonary blood supply is established by valved conduit interposition in all patients, regardless of the suitability for VSD closure. Palliation with systemic-pulmonary shunt is reserved for selected cases. Between 1994 and 2015, 94 patients with a median age of 1.09 years (range 0.03-19) underwent single-stage unifocalization at our institution. In 78 (82.1%) of them, an intraoperative pulmonary flow study was utilized to assess acceptability for concomitant VSD closure, which was accomplished in 69 cases (73%). Intraoperatively, following VSD closure, the mean right ventricle-to-aortic pressure ratio was 0.49 ± 0.14. The overall mortality rate was 11.2% (n = 10), with an 82% survival at 12.5 years. At a median follow-up interval of 5.8 years, the right ventricle-to-aortic pressure ratio did not differ significantly from the early postoperative phase. The surgical results of primary repair of PA with VSD and major aortopulmonary collaterals based on single-stage unifocalization and an intraoperative pulmonary flow study are satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions.

Pentalogy of Cantrell accompanied by scoliosis and pes equinovarus deformity at 12 weeks gestation

Cantrell's pentalogy (CP) is a rare syndrome characterized by defects in the lower sternum with ectopia cordis, anterior diaphragm defects, midline supraumbilical abdominal wall defects, defects in the diaphragmatic pericardium, and congenital heart disease. The authors report a 12-weeks gestation with multiple fetal anomalies suggesting the diagnosis of CP (a large thoraco-abdominal defect with herniating liver and bowel, heart deviated anteriorly with concomitant ventricular septal defect), and the 'S' shaped fetal spine due to increased lumbar lordosis and scoliosis with accompanying pes equinovarus deformity. Chorionic villus sampling was performed due to increased nuchal translucency (3.7 mm). The fetal karotype was found to be 47, XX,+21 (trisomy 21). In the literature, three scoliosis cases have been reported accompanying the CP along with multiple anomalies and one concomitant pes equinovarus deformity has been reported previously.

Management of an Associated Ventricular Septal Defect at the Time of Coarctation Repair

Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD. We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality. There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p < 0.001). Mean age at repair for group 1 (n= 286) and group 2 (n= 472) was 87.4days and 21.6 days, respectively (p= 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p= 0.130). Discharge mortality for group 1 and group 2 was similar, at8.7% and 9.1%, respectively (p= 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%. The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach.

Anatomic Repair for Corrected Transposition With Left Ventricular Outflow Tract Obstruction

We investigated the long-term outcomes of anatomic repair for congenitally corrected transposition of great arteries (ccTGA) and its variant associated with left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD). From 1987 to 2011, 47 patients (27 with pulmonary atresia and 20 with pulmonary stenosis) with ccTGA and its variant associated with LVOTO and VSD underwent anatomic repair. The mean operative age was 5.5 ± 3.7 years old (range, 1.6 to 21.3). The preoperative right ventricular end-diastolic volume was 133% ± 31% (81 to 222) of their normal size. The atrial switch procedure was Mustard in 31 patients and Senning in 16, with the latter used in all from 2002. The mean follow-up period was 11.6 ± 7.3 years (maximum, 22.7). The VSD was concomitantly enlarged in 4 patients and Damus-Kaye-Stansel (DKS) anastomosis was added in 9 patients with pulmonary stenosis and restrictive VSD. The overall survival rate at 20 years was 70.2% and no mortality has been observed in 21 consecutive patients since 1997. No patient required reoperation for the postoperative systemic ventricular outflow tract obstruction. A surgical heart block developed in 1 patient (2.1%) who underwent concomitant VSD enlargement. None of the patients developed a moderate or greater aortic or neo-aortic regurgitation. Recent outcomes after anatomic repair for congenitally corrected transposition of great arteries associated with LVOTO and VSD were excellent. For patients with pulmonary stenosis and restrictive VSD, additional DKS anastomosis seems to be an effective approach to avoid postoperative systemic ventricular outflow tract obstruction and surgical heart block.

Monochorionic triplet with concordant congenital cardiac defects

The chances of congenital cardiac defects are often increased in multiple birth orders. However, there is a paucity of data on its incidence amongst triplets in Nigeria. Here is a report of a set of triplet with concordant atrial septal defects and the third triplet found to have a concomitant ventricular septal defect.

Heart failure in a patient with corrected transposition of the great arteries. When is biventricular pacing indicated?

This report presents a patient with complex congenitally corrected transposition of the great arteries (ccTGA) and concomitant ventricular septal defect (VSD), who developed symptomatic heart failure further aggravated after dual-chamber pacemaker implantation.The initial disqualification from surgery based on high vascular pulmonary resistance was verified in repeated catheterization. The oxygen test was positive, therefore the patient underwent surgical correction. Despite successful repair with the use of two prosthetic valves (mechanical and biological) and an intraventricular patch, the symptoms of heart failure persisted. In view of the prolonged QRS complex on the ECG and the signs of significant intraventricular mechanical dyssynchrony measured by Tissue Doppler Imaging (TDI), the patient was referred for cardiac resynchronization therapy (CRT). A biventricular pacemaker allowed a larger synchronicity of systemic RV systolic function and the improvement of the patient’s general condition.The case described indicates that optimal care in patients with ccTGA can require implementation of both surgical and electrophysiological methods of treatment.

Swinging door flap technique for endoscopic transeptal repair of bilateral choanal atresia

We describe and evaluate the outcome of an endoscopic transeptal approach for the repair of bilateral choanal atresia in neonates. The study was a prospective case series of neonates with a confirmed diagnosis of bilateral choanal atresia who were treated by the endoscopic transeptal approach between July 2003 and December 2005. The endoscopic approach was performed using 4 mm 0 degrees telescope. A laterally based trapezoid shaped septal mucosal flap was created on each side using a radiofrequency needle. The flaps were elevated in a swinging door fashion to expose the atretic plate and the vomer. After removal of the posterior bony septum and widening of the choana, the flaps were trimmed and applied to the exposed lateral bony rim of the choana. All patients were stented bilaterally for 3-4 weeks postoperatively. Fourteen neonates (10 females and 4 males) were recruited. Their age at the time of surgery ranged from 2 to 25 days. Recovery was uneventful in all cases except for one case that developed bleeding in the immediate postoperative period and was controlled under endoscopic guidance. One case died 3 months following stent removal from uncompensated heart failure due to concomitant ventricular septal defect. Follow-up ranged from 4 to 36 months with a mean of 17.3 +/- 9.3 months. An adequate functional nasal breathing and appropriate feeding with sufficient weight gain were maintained during the entire follow-up period. The described endoscopic technique offered excellent visualization of the choana and allowed maximal widening of the choana while preserving the mucosa along the entire circumference of the newly created choana and hence less risk of postoperative stenosis.

Repair of ruptured sinus of valsalva aneurysm: Determinants of long-term survival

Background. Ruptured sinus of Valsalva aneurysm is a rare cardiac anomaly and long-term survival after surgical treatment is not well established. This study was designed to investigate the determinants of long-term survival after repair of ruptured sinus of Valsalva aneurysm. Methods. From April 1978 to April 1996, 53 patients underwent operation for ruptured sinus of Valsalva aneurysm. The incidence among our cardiac surgical population was 0.56%. Long-term survival was investigated in 46 patients (13 to 65 years) who survived the operation, with 96.2% follow-up completeness (mean ± standard deviation, 6.5 ± 4.9 years; maximum, 17.2 years), by univariate and multivariate analyses. Results. There was no early operative death and no recurrence after the initial repair. Actuarial survival was 83.8% ± 8.4% at 15 years. Reoperation, aneurysm draining into the left ventricle, aortic prosthetic dehiscence, bacterial endocarditis, and aortic cross-clamp time (<70 minutes) were significant factors in long-term survival ( p < 0.05). Multivariate analysis revealed that only aortic prosthesis dehiscence was the significant factor influencing late survival ( p = 0.0001). Conclusions. Surgical treatment for ruptured sinus of Valsalva aneurysm is safe and has satisfactory results. Aortic prosthesis dehiscence is the independent determinant for long-term survival. Other factors including bacterial endocarditis, concomitant ventricular septal defect repair, and aortic valve replacement did not independently influence long-term survival.

Morbidity and Mortality of Intra-aortic Balloon Pumps Placed Through the Aortic Arch

Twenty-four patients required an intra-aortic balloon pump placed through the aortic arch during cardiac operations from 1985 to 1993. The operative procedures of the 24 patients requiring arch balloon pumps included aortocoronary bypass (14), redo aortocoronary bypass (3), valve replacement (3), aortocoronary bypass with concomitant ventricular septal defect repair (1), heart transplantation (2), and aortic to right ventricle fistula repair (1). Mortality was 54%. Morbidity included cerebral vascular accident (17%), acute renal failure (29%), left ventricular thrombus (4%), sternal wound infection (4%), and mediastinal exploration secondary to bleeding from the balloon pump site (4%). This review suggests that (1) mortality for patients requiring arch balloon is significant, (2) complications of cerebral vascular accident and renal failure may be increased, and (3) severe peripheral vascular disease is associated with arch balloon placement and subsequent increased morbidity and mortality.