Association between pregnancy and long-term cardiac outcomes in individuals with congenital heart disease.

BACKGROUND: As early life interventions for congenital heart disease improve, more patients are living to adulthood and are considering pregnancy. Scoring and classification systems predict the maternal cardiovascular risk of pregnancy in the context of congenital heart disease, but these scoring systems do not assess the potential subsequent risks following pregnancy. Data on the long-term cardiac outcomes after pregnancy are unknown for most lesion types. This limits the ability of healthcare practitioners to thoroughly counsel patients who are considering pregnancy in the setting of congenital heart disease. OBJECTIVE: We aimed to evaluate the association between pregnancy and the subsequent long-term cardiovascular health of individuals with congenital heart disease. STUDY DESIGN: This was a retrospective longitudinal cohort study of individuals identifying as female who were receiving care in two adult congenital heart disease centers from 2014 to 2019. Patient data were abstracted longitudinally from a patient age of 15 years (or from the time of entry into the healthcare system) to the conclusion of the study, death, or exit from the healthcare system. The primary endpoint, a composite adverse cardiac outcome (death, stroke, heart failure, unanticipated cardiac surgery, or a requirement for a catheterized procedure), was compared between parous (at least one pregnancy >20 weeks' gestation) and nulliparous individuals. By accounting for differences in the follow-up, the effect of pregnancy was estimated based on the time to the composite adverse outcome in a proportional hazards regression model adjusted for the World Health Organization class, baseline cardiac medications, and number of previous sternotomies. Participants were also categorized according to their lesion type, including septal defects (ventricular septal defects, atrial septal defects, atrioventricular septal defects, or atrioventricular canal defects), right-sided valvular lesions, left-sided valvular lesions, complex cardiac anomalies, and aortopathies, to evaluate if there is a differential effect of pregnancy on the primary outcome when adjusting for lesion type in a sensitivity analysis. RESULTS: Overall, 711 individuals were eligible for inclusion; 209 were parous and 502 nulliparous. People were classified according to the World Health Organization classification system with 86 (12.3%) being classified as class I, 76 (10.9%) being classified as class II, 272 (38.9%) being classified as class II to III, 155 (22.1%) being classified as class III, and 26 (3.7%) being classified as class IV. Aortic stenosis, bicuspid aortic valve, dilated ascending aorta or aortic root, aortic regurgitation, and pulmonary insufficiency were more common in parous individuals, whereas dextro-transposition of the great arteries, Turner syndrome, hypoplastic right heart, left superior vena cava, and other cardiac diagnoses were more common in nulliparous individuals. In multivariable modeling, pregnancy was associated with the composite adverse cardiac outcome (36.4%% vs 26.1%%; hazard ratio, 1.83; 95% confidence interval, 1.25-2.66). Parous individuals were more likely to have unanticipated cardiac surgery (28.2% vs 18.1%; P=.003). No other individual components of the primary outcome were statistically different between parous and nulliparous individuals in cross-sectional comparisons. The association between pregnancy and the primary outcome was similar in a sensitivity analysis that adjusted for cardiac lesion type (hazard ratio, 1.61; 95% confidence interval, 1.10-2.36). CONCLUSION: Among individuals with congenital heart disease, pregnancy was associated with an increase in subsequent long-term adverse cardiac outcomes. These data may inform counseling of individuals with congenital heart disease who are considering pregnancy.

Mechanical circulatory support for early surgical repair of postinfarction ventricular septal defect with cardiogenic shock.

Postinfarction ventricular septal defect (pVSD) due to acute myocardial infarction complicated by cardiogenic shock (CS) is associated with high mortality. The aim of this study was to determine the outcome of primary surgical repair of pVSD in patients with CS and examine whether it is influenced by the use of mechanical circulatory support (MCS) devices. Between October 1994 and April 2016, primary surgical repair of pVSD complicated by CS was performed in 53 patients. Thirty-six (68%) were implanted pre-operatively with an intra-aortic balloon pump (IABP), 4 (8%) with extracorporeal life support (ECLS), and 13 (24%) received no MCS device. Prospectively collected demographic and perioperative data were analyzed retrospectively. All-cause, 30-day mortality rates were analyzed and multivariate analysis was performed to differentiate independent risk factors. No pre-operatively implanted MCS device was able to improve 30-day survival, whereas pre-operatively implanted ECLS tended to have a positive effect (P = .106). The post-operative need for a MCS device or escalation of MCS invasiveness (IABP upgrade to ECLS) was associated with a higher 30-day mortality (P = .001) compared with patients without any MCS device or those with pre-operatively implanted MCS devices. An independent risk factor for 30-day mortality was the interval between acute myocardial infarction and surgery <7 days (OR 5.895, CI 1.615-21.515; P = .007). Pre-operative implantation of ECLS for CS tends to improve the outcome of early primary surgical pVSD repair. The need for a post-operative MCS device is associated with a worse 30-day survival after early primary surgical pVSD repair.

Short- and Long-term Outcome After Interventional VSD Closure: A Single-Center Experience in Pediatric and Adult Patients.

Interventional closure of congenital ventricular septal defects (VSD) is recording a continuous rise in acceptance. Complete atrioventricular block (cAVB) and residual shunting are major concerns during follow-up, but long-term data for both are still limited. We retrospectively evaluated the outcome of patients with interventional VSD closure and focused on long-term results (> 1 year follow-up). Transcatheter VSD closures were performed between 1993 and 2015, in 149 patients requiring 155 procedures (104 perimembranous, 29 muscular, 19 residual post-surgical VSDs, and 3 with multiple defects). The following devices were used: 65 × Amplatzer™ Membranous VSD Occluder, 33 × Duct Occluder II, 27 × Muscular VSD Occluder, 3 × Duct Occluder I, 24 × PFM-Nit-Occlud®, and 3 × Rashkind-Occluder. The median age at time of implantation was 6.2 (0.01-66.1) years, median height 117 (49-188) cm, and median weight 20.9 (3.2-117) kg. Median follow-up time was 6.2 (1.1-21.3) years and closure rate was 86.2% at last follow-up. Complications resulting in device explantation include one case of cAVB with a Membranous VSD occluder 7 days after implantation and four cases due to residual shunt/malposition. Six (4%) deaths occurred during follow-up with only one procedural related death from a hybrid VSD closure. Overall, our reported results of interventional VSD closure show favorable outcomes with only one (0.7%) episode of cAVB. Interventional closure offers a good alternative to surgical closure and shows improved performance by using softer devices. However, prospective long-term data in the current era with different devices are still mandatory to assess the effectiveness and safety of this procedure.

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18.

BACKGROUND: Surgical management has not been encouraged in patients with trisomy 18 (T18) and congenital heart diseases due to poor survival. This study aimed to investigate (1) the appropriateness of palliative surgeries followed by intracardiac repair (ICR) (i.e., two-stage ICR) for patients with a large ventricular septal defect (VSD) and T18, and (2) its impact on their long-term outcomes. METHODS: Medical charts of patients with VSD and T18 who underwent two-stage ICR at the Japanese Red Cross Medical Center between January 2005 and December 2019 were retrospectively reviewed. Demographic data, timing, and types of palliative surgeries, information related to ICR, peri- and postoperative clinical information, postoperative survival, and cause of death were collected. The long-term prognosis of patients treated with two-stage ICR was compared with that of patients treated with primary ICR and palliative surgery without ICR. RESULTS: Overall, 18 (2 male, 16 female) patients underwent two-stage ICR. Pulmonary artery banding was the initial palliative surgery in all patients after a median duration of 19.5 (range 6-194) days of life. The median age and the mean body weight at the time of ICR were 18.2 (7.6-50.7) months and 6.0 ± 1.0 kg, respectively. The mean pulmonary artery pressure and pulmonary vascular resistance index before ICR were 19.1 ± 7.3 mmHg and 3.4 ± 2.0 U m2, respectively. Overall, 17/18 (94%) patients were discharged after ICR. Fourteen (78%) patients were alive during data collection. None of the patients died of cardiac insufficiency, and the median duration of survival was 46.3 (14.3-186.4) months since birth. Most patients required cardiac medications rather than pulmonary vasodilators at the last follow-up. During the study period, three patients underwent primary ICR, and 46 underwent palliative surgery without ICR. Of those who underwent primary ICR, two died in the hospital on the first and 48th day following ICR, and the third died 179 days after the ICR. The Log-rank test revealed a significantly longer survival for the patients treated with two-stage ICR compared with those treated with palliative surgery without ICR (P = 0.003). CONCLUSION: Two-stage ICR improves the long-term survival of patients with VSDs and T18. This safe surgical strategy can also prevent pulmonary hypertension in such patients.

Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis: Modified REV versus Rastelli.

The objective of this study was to evaluate and compare the results of the modified réparation à l'ètage ventriculaire (REV) and the Rastelli operation for the treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS). Records of 38 patients who underwent the modified REV (n = 16) or the Rastelli operation (n = 22) for the treatment of TGA, VSD, and PS between 2010 and 2019 were reviewed. The median age was 2.2 years (range 0.6-8.0 years) and the median weight was 11.3 kg (range 6.4-22.0 kg). No in-hospital death occurred and there were 4 early reoperations (two in each group). Overall survival at 10 years was 97.4% (100% in Modified REV group and 95.5% in Rastelli group, P = 0.39). Freedom from left ventricular outflow tract (LVOT) reoperation was 100% in both groups. Freedom from right ventricular outflow tract (RVOT) reoperation was 100% in Modified REV group and 75.4% in Rastelli group (P = 0.073). Event-free survival was 100% in Modified REV group and 72.0% in Rastelli group (P = 0.048). The most recent echocardiography showed that LVOT peak gradient was less than 10 mmHg in all patients. In Modified REV group, 30.8% of patients (4/13) had either RVOT obstruction (RVOT peak gradient more than 40 mmHg) or moderate or severe pulmonary insufficiency, while conduit stenosis (peak gradient more than 40 mmHg) was found in 25.0% of patients (3/12) in Rastelli group. The modified REV and the Rastelli operation provide satisfactory early results, as well as long-term survival and LVOT performance. However, the modified REV has better RVOT performance.

Predictors of Mortality in Primary Surgical Repair of Postinfarction Ventricular Septal Defects: The Leipzig Experience.

BACKGROUND: A postinfarction ventricular septal defect (pVSD) as a complication of acute myocardial infarction (AMI) is associated with high mortality. This retrospective, single-center study aimed to identify predictors of early and long-term outcomes in patients undergoing primary surgical repair of pVSD managed by various surgical techniques. METHODS: We reviewed data from 77 consecutive patients who underwent primary surgical repair after pVSD in our institution. Prospectively collected demographic and perioperative data were analyzed retrospectively. Predictors of 30-day mortality and long-term outcome were assessed. RESULTS: pVSD was anterior in 45 patients (58.4%) and posterior in 32 (41.6%). Buttressed mattress suture (n = 9, 11.7%), simple single septal patch (n = 34, 44.2%), simple double septal patch (n = 2, 2.6%), sandwich double patch (n = 1, 1.3%), and the infarct exclusion technique (n = 31, 40.3%) were performed for surgical closure. Fifty-three patients (68.8%) had preoperative cardiogenic shock. The 30-day mortality was 42.8% (33 patients). Independent risk factors of 30-day mortality were duration between AMI and surgery <7 days (odds ratio [OR] 5.229, P = .011), preoperative absence of diuretics (OR 6.913, P = .005), and preoperative cardiogenic shock (OR 3.558, P = .011). Cumulative survival rates at 1, 5, and 10 years were 57.1%, 57.1%, and 31.2%, respectively. CONCLUSION: In pVSD, the 30-day mortality remains high, and preoperative cardiogenic shock significantly influenced mortality in our study. None of the surgical techniques or materials used in our investigation influenced the outcome.

Repair of ischemic ventricular septal defect with and without coronary artery bypass grafting.

BACKGROUND AND AIM OF THE STUDY: Ventricular septal defect (VSD) following myocardial infarction (MI) is a relatively infrequent complication with high mortality. We sought to investigate the effect of concomitant coronary artery bypass graft (CABG) on outcomes following post-MI VSD repair. METHODS: Electronic search was performed to identify all relevant studies published from 2000 to 2018. Sixty-seven studies were selected for the analysis comprising 2174 patients with post-MI VSD. Demographic information, perioperative variables, and outcomes including survival data were extracted and pooled for systematic review and meta-analysis. RESULTS: Single-vessel disease was most common (47%, 95% confidence interval [CI], 42-52), left anterior descending coronary artery was the most commonly involved vessel (55%, 95% CI, 46-63), and anterior wall was the most commonly affected territory (57%, 95% CI, 51-63). Concomitant CABG was performed in 52% (95% CI, 46-57) of patients. Of these, infarcted territory was re-vascularized in 54% (95% CI, 23-82). A residual/recurrent shunt was present in 29% (95% CI, 24-34) of patients. Of these, surgical repair was performed in 35% (95% CI, 28-41) and transcatheter repair in 11% (95% CI, 6-21). Thirty-day mortality was 30% (95% CI, 26-35) in patients who had preoperative coronary angiogram, and 58% (95% CI, 43-71) in those who did not (P < .01). No significant survival difference observed between those who had concomitant CABG vs those without CABG. CONCLUSIONS: Concomitant CABG did not have a significant effect on survival following VSD repair. Revascularization should be weighed against the risks associated with prolonged cardiopulmonary bypass.

Prenatally Diagnosed Congenital Ventricular Outpouchings: An Institutional Experience and Review of the Literature.

Congenital ventricular outpouchings (CVOs) are rare congenital heart defects with limited data regarding prognosis and outcomes. We aimed to describe the characteristics, outcomes and factors associated with morbidity and mortality of prenatally diagnosed CVOs using our institutional experience and a review of published cases. A total of 86 cases of prenatally diagnosed CVOs were identified, including 3 from our institution and 83 cases identified from a review of the literature. Fetal and postnatal outcomes were analyzed for each case. Pericardial effusions (44%) and ventricular dysfunction (17%) were the most common associated findings. Excluding cases that resulted in pregnancy termination, mortality was 17%, with the majority (11/13) occurring in the prenatal period. Factors associated with mortality included an outpouching located on the left ventricle, a diagnosis of hydrops fetalis, the presence of a pericardial effusion, and an earlier gestational age at diagnosis. Of those that survived to delivery, 57% remained asymptomatic without the need for intervention, and the outpouching regressed or resolved in an additional 15%. Prenatally diagnosed congenital ventricular outpouchings are a dynamic form of congenital heart disease with a high fetal mortality rate. The outcomes associated with the outpouchings appear to be the most variable in the prenatal period and the first year after birth. Serial prenatal and postnatal evaluations should be performed to evaluate for a change in the characteristics of the outpouching.

Outcomes of infants and children undergoing surgical repair of ventricular septal defect: a review of the literature and implications for research with an emphasis on pulmonary artery hypertension.

BACKGROUND: Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect. METHODS: We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes. RESULTS: Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair. CONCLUSION: Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Pulmonary artery banding and venous bidirectional cava-pulmonary shunt for two-stage arterial switch procedure in late referral of patients with transposition of the great arteries and intact ventricular septum: midterm results.

OBJECTIVE: Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late. MATERIAL AND METHODS: Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic "banana-shaped" left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28). RESULTS: The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (p<0.001, p < 0.001, and p < 0.00, respectively). CONCLUSION: Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.

Management of post-myocardial infarction ventricular septal defects: A critical assessment.

BACKGROUND: Post-myocardial infarction (MI) ventricular septal defects (PIVSD) are an uncommon but life-threatening complication of acute MI. Although surgical closure has been the standard of care, mortality, and recurrence of VSD remain high even after emergent surgery. Transcatheter VSD closure (TCC) devices have become an alternative or adjunct to surgical closure. METHODS: Online database search was performed for studies that included adults with PIVSD who underwent medical treatment (MT) alone, surgical closure (SC) (early or late), and TCC (early, late, or for post-surgical residual VSD). RESULTS: Twenty-six studies were included with a total of 737 patients who underwent either MT (N = 100), SC (early (n = 167), late (n = 100)), and TCC (early (n = 176), late (n = 115), or post-surgical residual VSD (n = 79)). The 30-day mortality among MT group was 92 ± 6.3%, among SC was 61 ± 22.5% (early 56 ± 23%, late 41 ± 30%), and for all TCC patients was 33 ± 24% (early 54 ± 32.7%, late 16 ± 26%), and TCC for post-surgical residual VSD 11 ± 34.9%. The mortality among overall SC, overall TCC and early TCC groups was significantly lower as compared with the MT (P < 0.001 for all comparisons). The overall mortality among all TCC, and late TCC groups was significantly lower when compared with the late SC (P < 0.0001, P < 0.0001, respectively). CONCLUSION: Closure of PIVSD decreases mortality as compared with MT alone and should be attempted as early as possible after diagnosis. Selection of TCC versus SC should be based on factors including complexity of the defect, availability of closure devices, expertise of the operator, and clinical condition of patient.

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis.

OBJECTIVES: The main aim of this systematic review was to evaluate the prevalence and type of associated anomalies in fetuses with heterotaxy diagnosed prenatally on ultrasound; the perinatal outcome of these fetuses was also studied. METHODS: An electronic search of MEDLINE, EMBASE and CINAHL databases was performed. Only studies reporting the prenatal diagnosis of isomerism were included. Outcomes observed included associated cardiac and extracardiac anomalies, fetal arrhythmia, abnormal karyotype, type of surgical repair and perinatal mortality. The analysis was stratified according to the type of heterotaxy syndrome (left (LAI) or right (RAI) atrial isomerism). Meta-analyses of proportions were used to combine data. Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale for cohort studies. RESULTS: Sixteen studies (647 fetuses) were included in the analysis. Atrioventricular septal defect was the most common associated major cardiac anomaly found both in fetuses with LAI (pooled proportion (PP), 59.3% (95% CI, 44.0-73.7%)), with obstructive lesions of the right outflow tract occurring in 35.5% of these cases, and in fetuses with RAI (PP, 72.9% (95% CI, 60.4-83.7%)). Fetal arrhythmias occurred in 36.7% (95% CI, 26.9-47.2%) of cases with LAI and were mainly represented by complete atrioventricular block, while this finding was uncommon in cases with RAI (PP, 1.3% (95% CI, 0.2-3.2%)). Abnormal stomach and liver position were found, respectively, in 59.4% (95% CI, 38.1-79.0%) and 32.5% (95% CI, 11.9-57.6%) of cases with LAI, and in 54.5% (95% CI, 38.5-70.1%) and 45.9% (95% CI, 11.3-83.0%) of cases with RAI, while intestinal malrotation was detected in 14.2% (95% CI, 2.5-33.1%) of LAI and 27.1% (95% CI, 7.9-52.0%) of RAI cases. Hydrops developed in 11.8% (95% CI, 2.9-25.6%) of fetuses diagnosed prenatally with LAI. Biventricular repair was accomplished in 78.2% (95% CI, 64.3-89.4%) of cases with LAI, while univentricular repair or palliation was needed in 17.0% (95% CI, 9.7-25.9%); death during or after surgery occurred in 26.8% (95% CI, 4.6-58.7%) of LAI cases. Most children with RAI had univentricular repair and 27.8% (95% CI, 15.5-42.1%) died during or after surgery. CONCLUSIONS: Fetal heterotaxy is associated with a high prevalence of cardiac and extracardiac anomalies. Approximately one quarter of fetuses with heterotaxy died during or after surgery. Abnormal heart rhythm, especially heart block, is common in fetuses with LAI, while this finding is uncommon in RAI. Biventricular repair was common in LAI while univentricular repair was required in the majority of children affected by RAI. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Evaluation of Different Minimally Invasive Techniques in Surgical Treatment for Ventricular Septal Defect.

BACKGROUND: Minimally invasive cardiac surgery is becoming a safe and cosmetic alternative to standard median sternotomy (SMS). This retrospective study reviews our results and experience with the lower mini-sternotomy (LMS) technique and the right lateral thoracotomy (RLT) technique for ventricular septal defect (VSD) closure compared with SMS. METHODS: Between January 2013 and Dec 2015, 198 patients underwent repair VSD through lower mini-sternotomy (LMS Group, n=66), right lateral thoracotomy (RLT Group, n=59), standard median sternotomy (SMS Group, n=73). Cardiopulmonary bypass was achieved directly in the three different approaches. RESULTS: Procedures were performed successfully in all patients among the three groups and no in-hospital mortality occurred. No patient was reverted to standard median sternotomy in the LMS Group and RLT Group. The CPB time was 37.73±11.46 mins in the LMS Group, 41.3±13.97 mins in the RLT Group and 36.99±10.84 mins in the SMS Group (p=0.078); the cross-clamp times were 23.85±9.78 mins in the LMS Group, 22.54±9.08 mins in the RLT Group and 19.23±6.92 mins in the SMS Group (p=0.009). The total incision length of the procedure in the SMS Group (7.45±1.54cm) was longer than the other groups (LMS Group, 5.58±0.8cm and RLT Group, 5.96±1.48cm) and the difference was significant (p<0.001). CONCLUSIONS: Both the LMS and RLT approach can be performed with favourable cosmetic and acceptable clinical results for closing VSD. They are the promising alternatives to standard median sternotomy and merit further study.

Percutaneous Closure of Post-Infarction Ventricular Septal Defects-An Over Decade-long Experience.

OBJECTIVES: To report an over decade-long experience with percutaneous post-infarction ventricular septal defect (PIVSD) closure. BACKGROUND: PIVSDs remains a major clinical challenge with extremely high mortality. Data concerning interventional closure of PIVSD is scarce. METHODS: All percutaneous PIVSD closures performed between 2003 and 2016 in 8 participating centres were identified. Data concerning patients and procedures was acquired. Patients were divided into two groups, based on the time interval between VSD diagnosis and closure (≤14 days-acute phase, >14 days-non-acute phase). RESULTS: Twenty-one percutaneous PIVSD closures were performed on 20 patients (9 females, mean age: 70 years). Mean interval between the diagnosis and the procedure was 182.6 ± 500 days (range: 7-2228). Defects were mostly located in apical (55%) segments of the septum. In 7 cases (33%) the procedure was performed in the acute phase. The closure was technically successful in 17 cases (81%). Four patients died within 48 hours after the procedure. 30-days survival rate of the entire cohort was 70%. Univariate analysis revealed impact of technical success of the procedure (HR 0.13, CI 0.03-0.68 P = 0.016) and white blood cell count (HR 1.36 per unit increase, CI 1.1-1.69, P = 0.005) on 30-day mortality. CONCLUSIONS: In a selected population of patients percutaneous PIVSD closure is feasible and provides satisfactory survival rate. Procedural success has a protective impact on survival. Timing of the closure remains controversial. Procedure in the non-acute phase carries lower mortality, but at the same time introduces a selection bias. Larger registry-based studies are required.

Summary of Clinical Experience of Modified Double Root Translocation in the Management of Complete Transposition of Great Arteries With Left Ventricular Outflow Tract Obstruction.

To summarize the therapeutic effects of modified double root translocation (MDRT) in the management of congenital heart disease-transposition of great arteries (TGA) with ventricular septum defect (VSD) and left ventricular outflow tract obstruction (LVOTO). From May 2013 to March 2015, we treated 6 patients (4 males, 2 females, aged from 1 year and 8 months old to 5 years old) with complete transposition of great arteries with left ventricular outflow tract obstruction, SaO2 54 ± 7.3%; the outflow velocity of the left ventricular or pulmonary valve measured by Doppler was 4.46 ± 0.15 m/s, and the Nakata index was 217 ± 32 cm(2)/m(2). We carried out a double root translocation operation on these 6 patients.One patient developed low cardiac output syndrome 4 hours after the operation. Extracorporeal membrane oxygenation (ECMO) was performed, but the patient died of multiple organ failure. The other 5 patients all recovered and were discharged from the hospital. During the 3-month to 2-year follow-up period, these 5 patients all demonstrated NYHA Class I or NYHA Class II LVEF (65 ± 2.7) %; 4 had mild pulmonary regurgitation, 1 moderate pulmonary regurgitation; 3 no aortic regurgitation, and 2 micro aortic regurgitation, SaO2 99 ± 0.4%.Modified double root translocation is an effective treatment method in the management of complete transposition of great arteries with left ventricular outflow tract obstruction.

Percutaneous closure of postinfarction ventricular septal defect: in-hospital outcomes and long-term follow-up of UK experience.

BACKGROUND: Postinfarction ventricular septal defect carries a grim prognosis. Surgical repair offers reasonable outcomes in patients who survive a healing phase. Percutaneous device implantation represents a potentially attractive early alternative. METHODS AND RESULTS: Postinfarction ventricular septal defect closure was attempted in 53 patients from 11 centers (1997-2012; aged 72±11 years; 42% female). Nineteen percent had previous surgical closure. Myocardial infarction was anterior (66%) or inferior (34%). Time from myocardial infarction to closure procedure was 13 (first and third quartiles, 5-54) days. Devices were successfully implanted in 89% of patients. Major immediate complications included procedural death (3.8%) and emergency cardiac surgery (7.5%). Immediate shunt reduction was graded as complete (23%), partial (62%), or none (15%). Median length of stay after the procedure was 5.0 (2.0-9.0) days. Fifty-eight percent survived to discharge and were followed up for 395 (63-1522) days, during which time 4 additional patients died (7.5%). Factors associated with death after postinfarction ventricular septal defect closure included the following: age (hazard ratio [HR]=1.04; P=0.039), female sex (HR=2.33; P=0.043), New York Heart Association class IV (HR=4.42; P=0.002), cardiogenic shock (HR=3.75; P=0.003), creatinine (HR=1.007; P=0.003), defect size (HR=1.09; P=0.026), inotropes (HR=4.18; P=0.005), and absence of revascularization therapy for presenting myocardial infarction (HR=3.28; P=0.009). Prior surgical closure (HR=0.12; P=0.040) and immediate shunt reduction (HR=0.49; P=0.037) were associated with survival. CONCLUSIONS: Percutaneous closure of postinfarction ventricular septal defect is a reasonably effective treatment for these extremely high-risk patients. Mortality remains high, but patients who survive to discharge do well in the longer term.

Clinical Implications of a Multivariate Stratification Model for the Estimation of Prognosis in Ventricular Septal Defect.

OBJECTIVES: To derive and validate a multivariate stratification model for prediction of survival free from intervention (SFFI) in ventricular septal defect (VSD). A secondary aim is for this model to serve as proof of concept for derivation of a more general congenital heart disease prognostic model, of which the VSD model will be the first component. STUDY DESIGN: For 12 years, 2334 subjects with congenital heart disease were prospectively and consecutively enrolled. Of these, 675 had VSD and form the derivation cohort. One hundred seven other subjects with VSD followed in another practice formed the validation cohort. The derivation cohort was serially stratified based on clinical and demographic features correlating with SFFI. RESULTS: Six strata were defined, the most favorable predicting nearly 100% SFFI at 10 years, and the least favorable, a high likelihood of event within weeks. Strata with best SFFI had many subjects with nearly normal physiology, muscular VSD location, or prior intervention. In the validation cohort, the relation between predicted and actual SFFI at 6 months, 1 year, 2 years, and 5 years follow-up had areas under the receiver operating characteristic curves 0.800 or greater. CONCLUSIONS: A prediction model for SFFI in VSD has been derived and validated. It has potential for clinical application to the benefit of patients and families, medical trainees, and practicing physicians.

Treatment of Perimembranous Ventricular Septal Defect in Children Weighing Less than 15 kg: Minimally Invasive Periventricular Device Occlusion versus Right Subaxillary Small Incision Surgical Repair.

BACKGROUND: To compare the treatment outcomes between minimally invasive periventricular device occlusion (MIPDO) and right subaxillary incision surgical repair (RSISR) on perimembranous ventricular septal defect (PmVSD) in children weighing less than 15 kg. METHODS: From January 2010 to January 2013, 538 infants (age < 3 years, weight < 15 kg) with PmVSD were randomly divided into two groups according to different treatment methods. Group 1 (265 cases) had periventricular device occlusion through a lower partial median sternotomy under transesophageal echocardiography (TEE); group 2 (265 cases) underwent surgical repair on cardiopulmonary bypass (CPB) through a right subaxillary incision. A prospective randomized controlled study was performed regarding success rate, operation time, volume of blood loss and transfusion, length of intubation and intensive care unit (ICU) stay, complications, expenses, and follow-up results. RESULTS: All patients had effective treatment with no death or serious life-threatening complications. In group 1, 255 cases (96.23%) underwent successful periventricular device occlusion. The remaining 10 cases (3.77%) were successfully converted to conventional operation. Different arrhythmias arose in 30 cases (11.76%), trivial residual shunt (RS) in 18 cases (7.06%), and new trivial tricuspid regurgitation (TR) in 29 cases (11.37%). In group 2, all patients (100%) underwent successful surgical repair. Different arrhythmias occurred in 116 cases (43.77%), trivial RS in 16 cases (6.04%), new trivial TR in 11 cases (4.15%), and heart dysfunction in 17 patients (6.42%). All patients were followed for more than 12 months. The final treatment effects were similar in both groups, but group 1 was significantly superior to group 2 regarding operation time, volume of blood loss, length of intubation and ICU stay, hospitalizations, and costs (all p < 0.05). TR incidence was higher in group 1 (p < 0.05), and that of right bundle branch block was higher in group 2 (p < 0.05). The incision was longer in group 2, but in a less exposed location. CPB was not needed in group 1, but anticoagulants were required for 3 to 6 months. CONCLUSION: Both RSISR and MIPDO are effective treatment methods for PmVSD. Though having some limitations, MIPDO not only minimized the surgical trauma to patients but also ensured safety to the maximum extent. However, patient selection is vital. For selected patients, especially those with moderate PmVSDs with obvious clinical symptoms and no valve regurgitation, it seems an ideal approach.

Staged Biventricular Repair-Oriented Strategy in Borderline Biventricular Repair Candidates with Ventricular Septal Defect.

Although borderline biventricular repair (BVR) candidates unsuitable for primary BVR are often subjected to single-ventricle repair (SVR), some of them reach BVR by staged strategy. We evaluated our staged BVR-oriented strategy in borderline BVR candidates with ventricular septal defect (VSD) in whom a BVR/SVR decision was deferred beyond the neonatal period. Forty-two patients were treated with the approach between 1991 and 2012. They had been followed toward BVR until it was judged impossible. Outcomes, time course toward definitive repair (DR: BVR, SVR, or 1 + 1/2 ventricle repair), and hemodynamics were reviewed. A total of 57 palliative surgeries were performed before BVR or bidirectional Glenn (BDG), namely procedures to control pulmonary blood flow in 40, to improve mixing in 5, and to promote left ventricle (LV) growth in 5. LV growth was achieved in four patients. There were three cardiac deaths. Except for four awaiting patients, 19 reached BVR (50 %), 11 patients were converted to other than BVR, and 28 patients achieved DR (74 %) at the median age of 30.9 months. Cardiac cath before BVR or BDG performed at the median age of 22.5 months revealed well-preserved pulmonary vasculature with the median pulmonary artery pressure of 14 mmHg, except three patients unsuitable for SVR. In conclusion, our staged BVR-oriented strategy required longer time course and more complex palliative surgeries compared with a simple SVR strategy. Leaving open the possibility of a late crossover to an SVR pathway is mandatory when adopting staged BVR-oriented strategy in these complex patients.