Early and mid-term outcomes of double-chambered right ventricle repair: An 8-year experience.

BACKGROUND/AIM: Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle. METHODS: Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively. RESULTS: An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths. CONCLUSIONS: The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Implications of left atrial enlargement and patent foramen ovale creation on early surgical outcomes in patients with total anomalous pulmonary venous connection.

INTRODUCTION: In patients with total anomalous pulmonary venous connection (TAPVC), left atrium (LA) is small and suprasystemic pulmonary artery (PA) pressures may be present in some patients. In our study, we studied the relationship between surgical LA enlargement and patent foramen ovale (PFO) creation separately on the outcomes of patients with TAPVC. MATERIALS AND METHODS: Out of the 130 patients operated in our institute between January 2014 and December 2020, LA was enlarged in 60 patients. LA enlargement was done using a larger patch for atrial septal defect (ASD) closure. Thus, the LA volume was increased by shifting the patch towards the right atrium (RA). Suprasystemic or high PA pressures were present in 60 patients. In 33 patients, PFO was created. Early surgical outcomes were determined on the basis of vasoactive inotropic score (VIS), hours of ventilation, hours of inotropic support, intensive care unit (ICU) stay, and hospital stay. RESULT: Between the LA enlarged and nonenlarged group there was statistically significant less VIS score (18 [13-27.5] vs. 24 [18-30], p value .019), hours of ventilation (23 [16-46.5] vs. 26 [18-60], p value .039), hours of inotropic support (45.5 [30-72] vs. 55 [38-84], p value .038), and ICU stay (7 [5-9] vs. 8 [7-10] p value .0352) and statistically nonsignificant less hospital stay (11.5 [9-13] vs. 12 [9-14], p value .424). In patients with preoperative suprasystemic or high PA pressures, there was a statistically significant less VIS score (16 [11-23.5] vs. 18 [13-25], p value .044), hours of ventilation (20 [14-37] vs. 22 [18-39], p value .038), hours of inotropic support (34 [29.5-71] vs. 38 [30-78], p value .042), and hospital stay (9 [5-12] vs. 11 [9-14], p value .038) and statistically nonsignificant less ICU stay (7 [5.5-9] vs. 7 [6-9], p value .886) in the group with a PFO with respect to the other group in which no PFO was created. CONCLUSION: In patients with TAPVC, LA can be enlarged by using a large ASD patch and thus shifting the septum towards RA. Early surgical outcomes were improved with LA enlargement. In patients with suprasystemic or high PA pressures, leaving a PFO improved the postoperative outcomes.

Sub-pulmonic stenosis caused by a right ventricular outflow tract vegetation in a children with restricted ventricular septal defect.

Infective endocarditis involving the right heart is rarely observed in the pediatric population. Echocardiography plays an important role in its diagnosis, and surgery is indicated in patients with heart failure and persistent sepsis not responding to medical treatment. Here, we report a rare case of restricted ventricular septal defect complicated by a vegetation developed in the right ventricular outflow tract and causing sub-pulmonic stenosis in a 3-year-old male child.

Giant Eustachian valve and Thebesian valve-A highly deceptive structures as atrial septal defect rims.

The Eustachian valve (EV) of the inferior vena cava and the Thebesian valve (TV) of the coronary sinus are incompletely regressed structures of embryonic sinus venosus. In the majority of cases, the EV and TV disappear completely after birth or are represented only by a thin crescentic fold. On echocardiography, these vestiges may mimic abnormal structures. We report a case with giant EV and TV which were initially misinterpreted as rims of an atrial septal defect (ASD) leading to the false diagnosis of ostium secundum ASD.

Successful Surgical Management of a Rare Combination of Intrapericardial Teratoma and Thymoma in an Infant.

Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.

Right ventricular outflow tract obstruction caused by a right coronary cusp prolapse: An unusual finding.

Patients with large sub-pulmonic ventricular septal defect (VSD) present early as a results of their complications. Some present late, due to the restriction of VSD by the right coronary cusp (RCC) due to its prolapse. In this report, we present a rare case of sub-pulmonic VSD in a 33-year-old man who developed a sub-pulmonic stenosis due to the prolapse of the RCC into the right ventricular outflow tract.

Incidental diagnosis of a large aortopulmonary window with reversible pulmonary arterial hypertension in adult age and its surgical management.

Aortopulmonary window (APW) is a rare congenital cardiac condition. A large number of patients with a large APW usually die within 1 year of age. It is extremely rare to find cases of APW surviving till adult age and it is still rare to surgically treat such patients who are incidentally detected in adult age because such subsets of patients invariably have associated pulmonary vascular obstructive disease in advanced stage and thus there is therapeutic dilemma to surgically correct these patients. Although cases of uncorrected AP window presenting in adulthood have been reported but literature on surgically treated AP window in adult populations is limited. We describe case of APW in a 26-year-old male patient who was diagnosed incidentally while suspecting infective endocarditis and was subsequently surgically closed successfully with polytetrafluoroethylene patch after confirming reversibility of pulmonary arterial hypertension which is the key for successful outcome.

Dextrocardia, situs solitus, inlet ventricular septal defect (VSD), and iatrogenic tricuspid regurgitation (TR)-A surgical perspective.

Dextrocardia with situs solitus is a rare congenital anomaly, often associated with intra- or extracardiac malformations. A clear understanding of the spatial orientation of the chambers of the heart for planning surgical approach, is imperative. Access to the right side of the heart, especially for repair of the tricuspid valve can be challenging. We describe a case of dextrocardia, situs solitus, inlet ventricular septal defect (VSD) with iatrogenic tricuspid regurgitation (TR) in an adult patient, highlighting the surgical aspects essential for a successful outcome.

Delayed embolization of ductal occluder device into the aorta with near-complete obstruction of the aorta and critical cardiac decompensation.

Delayed embolization of ductal occluder device into the aorta after transcatheter closure of a patent ductus arteriosus (PDA) with almost complete obstruction of the aorta is extremely rare. Our patient had delayed migration of a ductal occluder device into the descending thoracic aorta (DTA) 6 months after its deployment. Because of critical biventricular dysfunction, urgent surgical removal of the device from the descending aorta was done via left posterolateral thoracotomy without using cardiopulmonary bypass. PDA was not closed because of doubt about the reversibility of pulmonary artery hypertension and severe right ventricular dysfunction so that it can act as an outlet to the decompensated right ventricle. The patient made an uneventful recovery. The patient was started on pulmonary vasodilators and is planned for right heart catheterization study after 2 months to check for the operability of PDA.

Pulmonary valve neocuspidisation with glutaraldehyde-treated autologous pericardium-A novel technique in pulmonary valve endocarditis.

Infective endocarditis (IE) is a serious condition leading to heart failure, persistent sepsis. The management of IE involving valve is mainly excision of the infected valve and replacement with a heart valve; which are also at the risk of prosthetic valve endocarditis. Hence repair of the valve with autologous pericardium is much more physiological. We had a 20-year-old male presented with features of heart failure and high-grade fever not responding to optimum medical management. Two-dimensional echocardiogram revealed vegetation on pulmonary valve cusps with the erosion of the left and right cusps. Neo cusps with autologous pericardium offered good hemodynamics with trivial regurgitation. The patient is doing well with normal pulmonary valve function 3 months after surgery. This technique is reliable, economic, and easily reproducible.

Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes.

OBJECTIVES: Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. METHODS: This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. RESULTS: There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 min vs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 h vs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). CONCLUSION: Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

Early surgical outcomes of a modified infarct exclusion technique in acute post-myocardial infarction ventricular septal rupture: a single-centre experience.

Introduction: Operative mortality in an acute post-myocardial infarction (AMI) ventricular septal rupture (VSR) is high. In addition to ventricular dysfunction, friable myocardium adds to the technical difficulty of the operation. In a modified infarct exclusion technique, the right ventricle is left undisturbed and the free edge of the pericardial patch is incorporated in the sutures while closing the left ventriculotomy. This simplifies the procedure and decreases the chances of right ventricular dysfunction, any residual defect, and bleeding. Study design: A retrospective analysis of patients with VSR following AMI operated in our institute from January 2018 to June 2021 was done. Results: Over the last 3 years, 16 patients with AMI VSR were treated with a modified infarct exclusion technique. Eight patients presented in cardiogenic shock preoperatively and were put on intra-aortic balloon pump support. All patients could be weaned successfully from the cardiopulmonary bypass, no patient had any residual defect, and none of the patients required re-exploration for bleeding. Postoperatively, 5 patients died within the first week and 2 more patients subsequently died due to intractable arrhythmias over the next 30 days. Conclusions: In our centre, the mortality following repair of VSR after AMI was 43%. The modified infarct exclusion technique is a good technique with less chances of postoperative re-exploration and residual defect.

Trileaflet pulmonary valve reconstruction with the Ozaki technique in an adult patient of tetralogy of Fallot with absent pulmonary valve.

Pulmonary valve interventions are one of the most common cardiac interventions that are being performed in patients with a wide variety of congenital heart diseases, more so in adults with congenital heart disease. Despite the introduction of various different reconstructive techniques and conduits, the ideal option still remains elusive. Lack of growth and re-operation for conduit replacement remains an Achilles heel in such conduits. So, surgeons have constantly tried to evolve surgical techniques that would obviate their use and allow age-related growth. The Ozaki procedure has proven to be technically reproducible and have excellent mid-term results in the aortic position in adults. Extending the same principle for reconstruction of the pulmonary valve can prove to be a reasonable alternative. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-022-01469-1.

Failure to wean off after a routine total anomalous pulmonary venous connection operation: An uncommon association.

A 6-month-old infant was operated on for supracardiac total anomalous pulmonary venous connection (TAPVC) with usual anatomy. The vertical vein was ligated. Weaning from bypass was attempted twice but was unsuccessful. Coronary sinus atresia was suspected and identified on the opening of the right atrium. A smooth postoperative course occurred after unroofing the coronary sinus. Coronary sinus atresia should be remembered as an uncommon association with TAPVC.

Surgical Outcomes of Post-myocardial Infarction Ventricular Septal Defect/Rupture: A Systematic Review and Meta-Analysis.

Ventricular septal defect (VSD) is a catastrophic acute myocardial infarction (MI) complication. Despite a significant reduction in the prevalence of post-MI VSD with the advancement of surgical techniques, it is still considered fatal with a high mortality rate. The trends in the clinical outcomes of patients with post-MI VSD show discretion due to the complexity of the disease. Therefore, the present analysis aimed to evaluate the surgical outcomes and associated risks in the patients of post-MI VSD. A thorough literature survey resulted in 40 studies of our interest. The pooled proportion of differential variables, including the incidence of cardiogenic shock, 30-day survival, and overall mortality, were estimated using Bayesian hierarchical models. The risk difference was estimated for the location of MI and VSD and mortality in patients with coronary artery bypass graft (CABG). In addition, the heterogeneity tests for inconsistency and publication biases using Egger's and Begg's tests were also estimated. The analysis revealed a significant risk difference of 0.23 and 0.27 for the anterior vs. posterior location of MI and VSD, respectively. Further, the pooled proportion of 30-day survival and mortality was found to be 54.43% (95% credible interval (CI): 52.88-55.98%) and 48.22% (95% CI: 4-12.3%), respectively. Moreover, the heterogeneity test revealed significant inconsistencies in all the datasets with an I2 index of >90% (p<0.0001). Lastly, the publication bias results suggested no evidence of asymmetry and small-study effects. Conclusively, the surgical management of post-MI VSD patients is considered beneficial; however, the outcomes signify its fatal behavior.

Early and Mid-Term Outcomes of Primary Repair After Atrioventricular Canal Defect: A Single-Center Eight-Year Experience.

BACKGROUND/AIM: Surgical repair techniques and management of patients with atrioventricular septal defect (AVSD) have progressed over the last few decades. Early and definitive interventions have become the choice of treatment for these patients. Based on this background, we aimed to review the early and mid-term outcomes of primary AVSD repair. METHODS: A total of 53 patients with a mean age of 3.45 ± 5.67 years underwent definitive repair for AVSD between January 2014 and June 2021. The clinical data including age, type of defect, associated co-anomalies, symptoms, pulmonary hypertension, etc. were collected and assessed retrospectively. Mitral regurgitation (MR) as a clinical outcome was assessed at 0, 1, 2, and 5 years. RESULTS: Among the recruited patients, 35 (66.1%) were male and 18 (33.9%) were female. Of 53 patients, repair for the complete defect was done in 38 (71.69%) patients, repair for intermediate/partial defect was done in 15 (23.1%) patients, and one patient underwent repair for incomplete type. Other associated co-anomalies were anterior mitral leaflet (12 (22.6%)), atrial and ventricular septal defect (VSD) (30 (56.6%)), and patent ductus arteriosus (PDA) (11 (20.8%)). Different procedures for surgical repair included patch closure, cleft repair, and polytetrafluoroethylene (PTFE) VSD closure. After repair, the mean follow-up period was 46.73 ± 27.37 months. Overall mortality was 3.78% (2/53), and two patients underwent reintervention due to symptomatic severe MR. CONCLUSIONS: A definitive and timely correction of AVSD shows satisfactory early and mid-term results.

Effectiveness and Safety of the Ozaki Procedure for Aortic Valve Disease in Pediatric Patients: A Systematic Review and Meta-Analysis.

The surgical treatment options for pediatric aortic valve disease are limited and have debatable long-term durability. In the current situation, the Ross procedure is considered in children for aortic valve disease(s). It is a complex surgical procedure with the risk of neo-aortic dilatation, converting a single valve disease into double valve disease, and associated with future re-interventions. Conversely, the Ozaki procedure has shown promising results in adults. Thus, the present study aimed to provide comparative evidence on the effectiveness and safety of the Ozaki versus Ross procedure for pediatric patients by performing a meta-analytic comparison of reporting outcomes. A total of 15 relevant articles were downloaded and among them, seven articles (one prospective study, five retrospective studies, and one case series) were used in the analysis. Primary outcomes such as physiological laminar flow pattern and hemodynamic parameters, and secondary outcomes such as hospital stays, adverse effects, mortality, and numbers of re-intervention(s) were measured in the meta-analysis. There were no significant differences in the age of patients between children who underwent the Ozaki procedure and those who underwent the Ross procedure at the time of surgeries. The Ozaki procedure is a good solution to an aortic problem(s) similar to the Ross procedure. Unlike the Ross procedure, the Ozaki procedure has restored a physiological laminar flow pattern in the short-term follow-up without the bi-valvular disease. Mean hospital stays (p = 0.048), mean follow-up (p = 0.02), adverse effects (p = 0.02), death, and numbers of re-intervention(s) of children who underwent the Ozaki procedure were fewer than those who underwent the Ross procedure. The time required for re-intervention(s) is higher for children who underwent the Ozaki procedure than those who underwent the Ross procedure. None of the procedures, including the Ozaki procedure for aortic valve disease(s), has significant effects on hemodynamic parameters and the frequent death rate of children after surgeries. Based on our analysis, we may suggest the Ozaki procedure for aortic valve disease surgery in children.

Anterior Mediastinal Benign Teratodermoid Tumour With Intraparenchymal Extension Through Cystobronchial Connection: A Rare Case Report.

Asymptomatic presentation is common in benign mature mediastinal tumours. Symptoms of the above diseases are sometimes life-threatening and can cause massive hemoptysis, recurrent pulmonary infection, hypoxia related to the pulmonary parenchymal hemorrhage, or pressure effect on or more of the major bronchi. A 16-year-old boy presented with frequent episodes of hemoptysis and recurrent fever unresponsive to antimicrobials. On investigation, it was found to be a benign mature mediastinal mass with cystobronchial connection to the right middle lobe. Resection of this mass resulted in the complete recovery of the individual. An anterior mediastinal benign teratodermoid tumour with intraparenchymal extension through cystobronchial connection is very rare. Surgical resection is challenging but offers the complete cure.