The infected right ventricular outflow tract in children and adults with congenital heart disease.

Abnormalities of the right ventricular outflow tract are common within the spectrum of congenital heart disease. Reconstruction of this outflow with or without the use of prosthetic material forms an integral part of many surgical procedures. Consequently, this part of the heart constitutes an important locus for infective endocarditis. Focused literature on infective endocarditis of the right ventricular outflow is sparse. This narrative review therefore attempts to collate the currently available data on a subject that is gaining importance because of the increasing numbers of surgical and catheter-based interventions on the right ventricular outflow. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-024-01748-z.

The modified Senning operation - surgical aspects.

The advent of the arterial switch operation for the treatment of transposition of great arteries (TGA) made the atrial switch operation largely redundant and its use in the developed world is now confined to the atrial component of the double-switch operation for congenitally corrected TGA. In resource-limited countries, however, it remains relevant as a treatment option for patients presenting late with transposition and a regressed left ventricle. The operation is intricate with many potential pitfalls, and this "how I do it" article describes the operative steps in detail to enable surgeons to learn this procedure and make it a part of their surgical armamentarium.

Valved conduits in the right ventricular outflow-the Achilles heel of congenital heart surgery!

Valved conduits play an important role in the reconstruction of the right ventricular outflow tract, but their limited longevity remains an unsolved problem for the congenital heart surgeon.

A systematic approach to epicardial echocardiography in pediatric cardiac surgery: An important but underutilized intraoperative tool.

Intraoperative echocardiography is an integral component of the peri-operative management of pediatric heart disease. It confirms the adequacy of surgery, identifies residual lesions, and can provide useful hemodynamic data. It, therefore, helps to decide on the need for revision of repair and guides the postoperative management strategy. Intraoperative echocardiography is done with the use of either an epicardial probe or a transesophageal probe. Epicardial echocardiography is a simple, useful modality and has the ability to perform imaging in cases where transesophageal echocardiography cannot be easily performed, for example, in low birth weight babies. We attempt to describe in detail the technique of epicardial echocardiography and the various views that we have found useful for a complete postsurgical evaluation. The limitations of the technique are also discussed in detail.

Double outlet of both ventricles: morphological, echocardiographic and surgical considerations.

OBJECTIVES: To describe the morphology, echocardiographic features and surgical management of the entity appropriately described as 'double outlet of both ventricles'. METHODS: Seven patients (5 males, age 0.5-7.5 months) were diagnosed to have a unique form of subarterial ventricular septal defect (VSD) and ventriculo-arterial connection, where a muscular outlet septum straddled the crest of the ventricular septum in a cruciate manner, such that both great arteries were equally committed to both ventricles. Diagnosis was established by echocardiography, with 6 patients submitted to surgical repair by means of intracardiac routing of the left ventricle to the aorta using 2 patches. RESULTS: Surgical repair was successful in all 6 patients in whom it was attempted. In addition, 1 patient underwent concomitant repair of aortic coarctation, and 2 had closure of multiple VSDs. We lost 1 patient to follow-up after diagnosis. Follow-up with a range from 3 months to 8 years in the remaining patients revealed all to be clinically well with satisfactory growth of both outflow tracts. CONCLUSIONS: We describe a series of patients with the ventriculo-arterial connection best described as 'double outlet of both ventricles'. Diagnosis is readily established by echocardiography. Good early and midterm results can be expected subsequent to surgical repair using 2 patches for interventricular septation.

Outcome of COVID-19-positive children with heart disease and grown-ups with congenital heart disease: A multicentric study from India.

BACKGROUND: Outcome data of children with heart disease who acquired COVID-19 infection are limited. AIMS: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. MATERIALS AND METHODS: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. RESULTS: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). CONCLUSIONS: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

IACTS guidelines: practice of cardiovascular and thoracic surgery in the COVID-19 era.

Patients undergoing cardiovascular and thoracic procedures are at an accentuated risk of higher morbidity and mortality, which are a consequence of the proliferative nature of the severe acute respiratory syndrome-corona virus 2 (SARS-CoV-2) on the lung vasculature, which in turn reflects as a cascading effect on the interdependent physiology of the cardiovascular and pulmonary organ systems. These are secondary to systemic inflammatory response syndrome and immunosuppressive responses to surgery and mechanical ventilation. Thus, the need to establish guidelines for the practice of cardiothoracic surgery which is safe for both the patient and the healthcare team presents as a priority, which is the mainstay of this article.

Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Through Limited Left Anterolateral Thoracotomy: An Alternative to Repeat Sternotomy.

We report two patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement through a limited left anterolateral thoracotomy. We describe the technique in detail. Both patients were at risk of cardiac injury during repeat sternotomy. This approach reliably avoids the risk of cardiac injury during repeat sternotomy and appears to be safe, simple, and reproducible.

Atypical Left Ventricular Myxoma: Unusual Echocardiographic and Histopathological Features.

Cardiac myxomas are the most common primary cardiac tumors and are typically attached to the interatrial septum. Left ventricular myxomas are exceedingly rare and presentation in children is all the more uncommon. We report a case of left ventricular myxoma with very atypical cystic appearance raising an initial suspicion of a hydatid cyst. Subsequently, cardiac magnetic resonance imaging was done, which ruled out the diagnosis of hydatid cyst. Complete surgical excision was done through transaortic and transmitral route. Histopathological examination revealed it to be a cardiac myxoma with vascular proliferation, which on echocardiography had appeared as a polycystic lesion. This is a very unusual histopathological presentation of cardiac myxoma.

Arterial Switch Operation With Neoaortic Valve Replacement in a 13-Year Old Patient With Transposition of Great Arteries With Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction-A Case Report.

The surgical management of d-transposition of great arteries (d-TGAs) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is ever evolving and still remains a challenge because of wide anatomic variability, age of presentation, surgical options available, and their variable long-term results in different series. We describe a patient with d-TGA, VSD, and LVOTO who presented to us at 13 years of age and underwent an arterial switch operation along with neoaortic valve replacement with a mechanical prosthesis. The postoperative course was uneventful, and at hospital discharge, the echocardiogram was satisfactory. We present the pros and cons of this hitherto undescribed treatment option.

Mid-Term Follow-Up of Neonatal Neochordal Reconstruction of Tricuspid Valve for Perinatal Chordal Rupture Causing Severe Tricuspid Valve Regurgitation.

BACKGROUND: Papillary muscle rupture in the perinatal period is a rare event that leads to severe mitral or tricuspid insufficiency due to a flail leaflet. Neonatal tricuspid chordal reconstruction for this condition is rarely reported. Early recognition and treatment have the potential to be lifesaving. We present our surgical experience with five such patients, along with their midterm follow-up. METHODS: Between August 2010 and November 2012, five neonates (aged 1-30 days) underwent surgery for severe atrioventricular valve regurgitation. All neonates had severe tricuspid regurgitation due to ruptured chordae. In addition, two had moderate mitral regurgitation; one due to ruptured chordae of the posterior mitral leaflet and the other due to prolapse of the anterior mitral leaflet. All underwent emergent surgery where the ruptured chordae to the anterior tricuspid leaflet were replaced with neochordae made with expanded polytetrafluoroethylene (ePTFE) suture. The mitral valve was repaired in two patients. RESULTS: All patients survived surgery without the need for postoperative mechanical circulatory assist. Predischarge echocardiograms showed good coaptation of tricuspid and mitral leaflets with minimal regurgitation in all. At follow-up between 75 months to 102 months, four patients had excellent outcomes with less than mild tricuspid regurgitation. One child with flail tricuspid and mitral leaflets developed progressive tricuspid and mitral regurgitation requiring surgical re-repair at 20 months following the initial surgery. CONCLUSION: Repair of chordal rupture of the tricuspid valve in neonates using ePTFE neo-chordae can provide acute salvage and gratifying midterm results in the management of this potentially fatal condition.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Giant congenital diverticulum of the right atrium.

Congenital diverticulum of heart is a rare entity, which may arise from the atria, atrial appendages, coronary sinus or the ventricles. A 3-year-old child presented with history of early fatigability for 6 months and recent upper respiratory tract infection. Chest X-ray and echocardiogram revealed marked right atrial enlargement. At surgery, a right atrial diverticulum was excised under cardiopulmonary bypass. Pathology revealed thickened endocardium with edema and myocardial fiber hypertrophy. Our experience with this rare congenital disease is presented along with a review of the literature.

Comparison between transcatheter closure and minimally invasive surgery for fossa ovalis atrial septal defect: a single institutional experience.

BACKGROUND: Although, conventional surgical closure of atrial septal defect (ASD) provides excellent results with very low mortality and morbidity, it leaves the scar of incision and postoperative pain. Newer treatment modalities like minimal invasive surgery and percutaneous closure are being increasingly used nowadays where available. AIM: To compare the patient population, success, safety, and efficacy of transcatheter closure of ASD (Group A) with that of minimally invasive surgery (Port Access) (Group B). METHODS: In this retrospective non-randomized study, a record of a total of 640 patients with diagnosis of ASD secundum between May 1997 and October 2006 were reviewed. A total of 470 out of 640 patients were selected for transcatheter closure (Group A) while 170 patients were taken for surgical closure by minimally invasive port access surgery (Group B). The safety and efficacy of two groups was evaluated on the basis of morbidity and mortality, duration of intensive care unit (ICU) stay, total duration of hospital stay, post-procedural complications, residual sequel at time of discharge, and residual flow across the ASD. RESULTS: Success rate in two groups was 97.1% and 99.4%, respectively and had no statistically significant difference. Similarly major complication rate also had no difference in statistical significance (1.8% and 2.9% for Group A and B, respectively). Group B patients had longer hospital stay. A small but significant number of patients were not found suitable for device closure. This number is likely to decrease as experience with technique increases. Port access surgery is currently not possible in small children (femoral artery diameter 35 mm) due to difficulty in cannulation. CONCLUSION: Percutaneous device closure of ASD can be offered as a treatment option in suitable patients. Port access is minimally invasive and an equally safe and effective alternative choice in ASDs with deficient rim in patient with appropriate age and weight.

Rosai-Dorfman Disease of Right Atrium Mimicking Myxoma.

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare multisystemic disorder that was first reported by Rosai and Dorfman in 1969. It is a distinct histioproliferative disorder due to overproduction of histiocytes, which accumulate in lymph nodes. The cardiac involvement of this disease is extremely rare, and until now, only 18 cases have been reported. We report the case of a 53-year-old woman with right atrial mass mimicking myxoma, which the histopathologic evaluation revealed to be Rosai-Dorfman disease of the right atrium.

Surgical Options for Uhl's Anomaly.

BACKGROUND: Uhl's anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies. PATIENTS AND METHODS: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty. RESULTS: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up. CONCLUSION: Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.

Aortico-left ventricular tunnel: echocardiographic recognition.

Aortico-left ventricular tunnel (ALVT) is a rare congenital malformation characterized by an abnormal communication between aorta and left ventricular cavity and the child presents with features of congestive heart failure in early infancy. We report the case of a 7-day-old baby who was diagnosed as a case of ALVT by echocardiography. The child was operated successfully and echocardiographic findings were confirmed.