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.

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.

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.

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.

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.

Acquired cyanosis secondary to RVOT obstruction due to pacemaker lead prolapse and vegetation in a child with ASD.

Pacemaker implantation in the paediatric population is associated with significant perioperative complications. We report a child with atrial septal defect who developed cyanosis following pacemaker implantation. An 8-year-old male child presented to us with fever of 2 months, along with cyanosis and clubbing. He underwent endocardial transvenous pacemaker implantation for congenital complete heart block. His echocardiography revealed right atrial vegetation (1 × 1 cm), moderate tricuspid regurgitation, and a prolapsed lead loop extending into the right ventricular outflow tract and pulmonary valve causing severe obstruction of pulmonary valve. He was started on empirical treatment followed by sensitive antibiotics based on culture report; however due to recurrent ventricular tachycardia he was taken for emergency surgery. He underwent closure of atrial septal defect, removal of pulse generator, removal of endocardial leads/vegetation, and pulmonary valve repair. Postoperative echocardiography revealed mild tricuspid regurgitation and resolution of pulmonic stenosis. This case presents a rare complication of severe right ventricular outflow tract obstruction following pacemaker implantation. .

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.

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.

Fetal cardiac intervention and fetal cardiac surgery: where are we in 21st century?

Fetal cardiac intervention is an in-utero cardiac procedure done in fetuses with heart diseases like severe aortic stenosis with evolving hypoplastic left heart syndrome, hypoplastic left heart syndrome with an intact or restricted atrial septum, pulmonary atresia with an intact ventricular septum, fetal heart block obstructed total anomalous pulmonary venous return, pericardial collection. The successful biventricular repair can be done in postnatal life after aortic or pulmonary valvuloplasty. Fetal bypass is very challenging because of different physiology. Low prime volume with the high flow can be used to prevent an inflammatory response.

Calcified Amorphous Tumor of Left Ventricle: A Rare Cardiac Tumor.

Cardiac calcified amorphous tumor (CAT) is a rare, non-neoplastic, intra-cavity cardiac mass. Only a few cases have been described in the literature. A 46-year-old Indian female presented with decompensated heart failure. On echocardiography, 1.9 x 1.7 cm pedunculated mobile mass in the left ventricle attached to the intraventricular septum was seen. On cardiac magnetic resonance imaging (MRI), the lesion was isointense. Histopathology of the excised mass revealed fibrin deposition with eosinophilic amorphous material in the center with the periphery of the lesion showing calcification without any myxomatous tissue. A final diagnosis of CAT of the heart was established. CAT is composed of calcium deposits in the background of amorphous degenerating fibrinous material. It presents as a pedunculated mass in any chamber of the heart with a very high preponderance of distal embolization. Differentiation from calcified atrial myxoma, calcified thrombi, or other cardiac neoplasms is very difficult. Histopathological examination is the mainstay of diagnosis. Treatment is emergency excision to prevent distal embolization. CAT is a rare non-neoplastic tumor, which is mainly a tissue diagnosis after its resection.

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.

An unusual case report of mitral valve apparatus sparing left atrial appendage vegetation presenting as endogenous endophthalmitis.

BACKGROUND: Ocular manifestations of infective endocarditis are nonspecific and rare. Endophthalmitis, retinal artery occlusion, Roth spots and vitreal and retinal infiltrations can all be seen with infective endocarditis. Also, infective endocarditis involving the left atrial appendage with no involvement of the mitral valve apparatus is a rarity. CASE PRESENTATION: Here we report a case of infective endocarditis of the heart involving the left atrial appendage presenting with features of endogenous endophthalmitis which ultimately progressed to phthisis bulbi with subtle cardiac symptoms in a previous healthy young adult. CONCLUSION: Infective endocarditis involving the left chambers of the heart carries an inherent high risk of systemic embolization. Panophthalmitis which is considered to be the most severe form of endogenous endophthalmitis is a rare presenting feature. Although a definitive treatment algorithm is lacking, early surgery and parenteral antibiotics along with local antibiotic injections could help to save the vision.

Idiopathic right ventricular pseudoaneurysm presenting with ventricular tachycardia: a case report.

Right ventricular (RV) pseudoaneurysm is very rare and is seen after penetrating chest trauma, cardiac surgery, infective endocarditis, myocardial infarction, syphilis, endomyocardial biopsy, lead extraction. Idiopathic right ventricular pseudoaneurysm is even rarer. They have varied presentations depending on the etiology. Diagnosis is usually made by echocardiography. We present a case of 21-year-old male who presented to us with palpitations for 2 days and one episode of syncope and was diagnosed with monomorphic ventricular tachycardia (VT) and he was managed with electrical cardioversion during one of the episodes. Echocardiogram and CMR showed a larger right ventricular pseudoaneurysm at apex. Surgical excision was done uneventfully. The patient had no further VT episodes post-surgery. This case highlights the approach to diagnosis and management of RV pseudoaneurysm.

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.

Multimodality imaging of an interventricular septum hydatid cyst.

BACKGROUND: Cardiac hydatid over the interventricular septum is extremely rare. Echinococcus infests humans as an accidental host. Echocardiography usually clinches the diagnosis of cardiac hydatid. However, multimodality imaging including cardiac magnetic resonance (CMR) imaging, computed tomography (CT), and positron emission tomography (PET) helps in supporting the diagnosis and surgical planning. CASE PRESENTATION: We present a 29-year-old male who presented with dyspnea and was found to have cardiac hydatid on the interventricular septum on echocardiography. CT and CMR clinched the diagnosis. CT pulmonary angiography showed extensive pulmonary thromboembolization and cavitary consolidation in lungs. PET showed no active uptake in cardiac hydatid. Post-surgical enucleation of the cyst his hypotension worsened and succumbed. CONCLUSION: Cardiac hydatid has poor prognosis. Multimodality imaging helps in confirming the diagnosis and surgical planning.

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.