Generalized Arterial Calcification of Infancy Mimicking Coarctation of Aorta in a Neonate.

Generalized arterial calcification of infancy (GACI) is a rare genetic condition with varied clinical presentation. Consequently, diagnosis is frequently delayed or missed. GACI has a poor prognosis, with more than half of patients dying before the age of 6 months. Early diagnosis and treatment with bisphosphonates have been shown to improve survival in these patients. This is a case report of a newborn with respiratory distress who was initially diagnosed with coarctation of the aorta at echocardiography. Further imaging with CT revealed the aortic narrowing to be associated with GACI. Keywords: Genetic Defects, Congenital, Vascular, Calcification/Calculi, Aorta, Pulmonary Arteries, CT Angiography, Echocardiography, Pediatrics © RSNA, 2024.

Half-turned truncal switch operation for dextro-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction and an abnormal coronary pattern.

We describe a surgical technique for a half-turned truncal switch operation in a 5-year-old child with dextro-transposition of the great arteries (D-TGA), a ventricular septal defect, a left ventricular outflow tract obstruction and a complex coronary pattern. The benefit of the half-turned truncal switch is the creation of haemodynamically superior biventricular outflow tracts and the maximal use of an autologous pulmonary valve in the right ventricular outflow tract, thereby avoiding the right ventricular-pulmonary artery conduit.

A Rare Entity: Double Outlet of Both Ventricles.

Double outlet of both ventricles is an anomaly wherein both ventricles equally share the arterial trunks. A majority of the literature describes a variant of this disease with a muscular outlet septum which is perpendicular to the plane of the ventricular septum although a variant with a fibrous muscular septum can also be present. The condition may be associated with obstruction of either outflow tract, which may complicate repair. We report a child with double outlet of both ventricles and a fibrous outlet septum with unobstructed outflows. The child underwent a successful surgical repair using a two-patch technique.

Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.

Effects of on-table extubation on resource utilization and maternal anxiety in children undergoing congenital heart surgery in a low-resource environment.

Objective: To study the applicability of on-table extubation (OTE) protocol following congenital cardiac surgery in a low-resource setting and its impact on the length of intensive care unit (ICU) stay, hospital stay, hospitalization cost, parental anxiety, and nurse anxiety. Materials and Methods: In this prospective, nonrandomized, observational single-center study, we included all children above 1 year of age undergoing congenital cardiac surgery. We evaluated them for the feasibility of OTE using a prespecified protocol following separation from cardiopulmonary bypass. The data were prospectively collected on 60 children more than 1 year of age, belonging to the Risk Adjustment for Congenital Heart Surgery 1, 2, 3, and 4 groups and divided into two groups: those who underwent successful OTE and those who were ventilated for any duration postoperatively (30 children in each group). Duration of hospital stay, ICU stay, and total hospital cost were collected. Anxiety levels of the primary caregiver (nurse) in the ICU and the mother were assessed immediately after the arrival of the child in the ICU using the State Trait Anxiety Inventory (STAI). Results: Children who were extubated immediately following congenital cardiac surgery had significantly shorter ICU stay (median 20 [19, 22] h vs. 22 [20, 43] h [P < 0.05]). Patients extubated on table had a significant reduction in hospital cost {median Rs. 161,000 (138,330; 211,900), approximately USD 1970 (P < 0.05)} when compared to children who were ventilated postoperatively {median Rs. 201,422 (151,211; 211,900) , approximately USD 2464}. The anxiety level in mothers was significantly less when their child was extubated in the operating room (STAI 36.5 ± 5.4 vs. 47.4 ± 7.4, P < 0.001). However, for the same subset of patients, anxiety level was significantly higher in the ICU nurse (STAI 46.0 ± 5.6 vs. 37.8 ± 4.1, P < 0.05). Conclusion: OTE following congenital cardiac surgery is associated with a shorter duration of ICU stay and hospital stay. It also reduces the total hospital cost and the anxiety level in mothers of children undergoing congenital heart surgery. However, the primary bedside caregiver during the child's ICU stay had increased anxiety managing patients with OTE.

Complex complete transposition of great arteries and partial anomalous pulmonary venous connection with an intact interatrial septum-lessons learnt.

The association of pulmonary venous anomaly and a complex complete transposition of great arteries (d-TGA) is a rarity. Managing a combination of anomalies in a single setting is a surgical challenge. We report a very rare association of d-TGA with ventricular septal defect (VSD) and partial anomalous pulmonary venous connection (PAPVC) of the right pulmonary veins to the right atrium with an intact interatrial septum in a month-old infant. The child underwent a single-stage complete repair. Constant vigilance in the immediate postoperative period and early revision is mandatory for a good outcome.

Alternative Uses of the Ozaki Technique: Aortic Valved Conduit in a Bentall Operation and Right Ventricle-to-Pulmonary Artery Conduit.

The modified Ozaki technique within a conduit is reproducible and is an alternative to the use of homografts and commercially available conduits which may have special appeal in a low resource setting.

Clinical efficacy of levosimendan vs milrinone in preventing low cardiac output syndrome following pediatric cardiac surgery.

Background: Prophylactic milrinone is commonly used to prevent Low Cardiac Output Syndrome (LCOS) after pediatric cardiac surgery. This study compares the use of levosimendan with milrinone when used as the primary inotrope following pediatric cardiac surgery. Subjects and Methods: Forty infants undergoing corrective surgery for congenital heart disease were recruited during the study and randomized into two groups (group L and group M). During rewarming, a loading dose of levosimendan or milrinone was administered followed by a 24-hour infusion of the chosen inotrope. Echocardiographic variables were measured postoperatively. Statistical analysis was done with SPSS-20 computer package. Association between the variables was found by independent t test. P < 0.05 was considered statistically significant. Results: Mean age and weight of the patient in Group L was 8.55 ± 5.83 months and 6.05 ± 2.09 kgs, while that in group M was 6.85 ± 3.57 months and 5.26 ± 2.11 kgs. 4 patients (20%) treated with levosimendan had LCOS in comparison with 6 (30%) patients in those treated with milrinone. Echocardiographic parameters in both groups L and M were comparable (cardiac index 3.47 ± 0.76 vs 3.72 ± 1.05 L/min/m2, EF 66.10 ± 7.82% vs 59.34 ± 10.74%, stroke volume index 25.4 ± 6.3 vs 27.74 ± 10.35 mL/m2). The duration of ventilation, ICU stay and hospital stay were lesser in group L (12.75 ± 9.69, 35.95 ± 12.11, 119.10 ± 46.397 vs 23.60 ± 22.03, 51.20 ± 29.92, 140.20 ± 52.65 hours). Conclusions: The incidence of LCOS was lesser in those patients treated with levosimendan, when compared with those treated with milrinone. Cardiac index and stroke volume index were comparable between the two groups. Thus, levosimendan provides a non-inferior alternative to milrinone when used as the primary inotrope following pediatric cardiac surgery.

Extension of cardiopulmonary bypass outside the operating room as a short-term bridge to recovery "the poor man's ECMO".

This report describes the use of conventional cardiopulmonary bypass (CPB) circuit beyond the operating room to the critical care unit to provide cardiopulmonary support for patients requiring emergent resuscitation as well as short-term support for rest and recovery of the heart. We modified the CPB circuit and created a closed extra corporeal membrane oxygenation (ECMO) circuit using a conventional oxygenator excluding the venous reservoir. We believe that this technique provides a short-term mechanical support and also acts as a bridge to recovery, or a bridge to decision, in very limited resource settings.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Successful Surgical Management of a Rare Case of Mixed Type of Total Anomalous Pulmonary Venous Connection in a Very Low Birth Weight Preterm Child Using a Novel Surgical Technique.

We report a rare case of combined supra- and infracardiac type of total anomalous pulmonary venous connection (mixed-type TAPVC) in a 26-day-old low birth weight (1.9 kg) infant who was admitted with respiratory distress. The child underwent successful surgical repair using a novel surgical technique and is doing well at one year follow-up.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

Chasing the umbrella: delayed migration of ductal occluder device into the aorta.

Delayed migration of a ductal occluder device into the aorta after transcatheter closure of a patent ductus arteriosus is extremely rare. We present a case of delayed migration of a ductal occluder into the descending aorta 4 months after its deployment in an 11-year-old girl. Successful surgical removal of the device from the descending aorta and triple-ligation of the ductus arteriosus was performed via a left thoracotomy. The patient made an uneventful recovery.

Teratoma Arising from Hepato Duodenal Ligament in the Newborn with Transection of Portal Vein, Hepatic Artery and Common Bile Duct: A Surgical Challenge.

A 7-day-old neonate presented with a large intra-abdominal mass adherent to the hilum of the liver encasing the portal triad. During excision, the portal vein, hepatic artery, and common bile duct were injured. The repair was done promptly and needed massive blood transfusion. Histopathology revealed immature teratoma Grade III. Survival in neonate following total transection of portal triad is rare and has not been reported.

Outcomes and Short-Term Follow-Up in Complex Ross Operations in Pediatric Patients Undergoing Damus-Kaye-Stansel Takedown.

Review echocardiography and outcomes before and after Ross procedures, including patients undergoing biventricular conversion with Damus-Kaye-Stansel (DKS) takedown. A retrospective review was performed on 62 patients undergoing simple (control group) and complex Ross procedures, including 12 patients who underwent biventricular conversion with Ross operation and DKS takedown (complex). Echocardiography was reviewed preoperatively and at discharge and late follow-up. Kaplan-Meier estimates of patient survival and freedom from reintervention were obtained. In all, 62 patients had a median age of 4.5 years (interquartile range [IQR]: 1-12.5), weight of 16.4kg (IQR: 8-41), and follow-up of 3.8 years (IQR: 1.3-6). The complex DKS takedown group had 2 deaths, no neoaortic valve or root reinterventions, and 3 right ventricular outflow tract (VOT) reinterventions. There were no differences from the control group in left VOT or right VOT reinterventions. Neither group showed differences between pre- and late follow-up aortic root and ascending aorta dimensions, and no correlations were found among preoperative pulmonary valve (PV) size, late aortic regurgitation (AR), aortic root, or ascending aortic Z-scores. Aortic valve size increased from discharge to late follow-up for both groups (P ≤ .05); 90% of patients at late follow-up had mild or less AR with similar distributions in severity between complex and control groups. Severity of late AR showed no correlation with preoperative PV size and is independent of it. The Ross procedure has good short-term results in simple and complex patients and should be considered in those undergoing Ross operation with biventricular conversion and DKS takedown. Moreover, native PV size should not be a contraindication for Ross procedure.

Aortic root translocation (Nikaidoh procedure): Intermediate follow-up and impact of conduit type.

OBJECTIVE: Aortic root translocation is a promising surgical option for repair of transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. There are little data on the outcomes of this procedure, with no long-term follow-up available. We reviewed our experience with aortic root translocation and the impact of the type of right ventricular outflow tract reconstruction. METHODS: The demographic, procedural, and outcome data were obtained for 32 patients who underwent aortic root translocation from 1997 to 2013 at Boston Children's Hospital. Patients were grouped on the basis of right ventricular outflow tract reconstruction with a valved conduit or a nonvalved anastomosis of the pulmonary artery bifurcation to the right ventricular outflow tract with anterior patch augmentation (transannular patch). RESULTS: The median age was 7.5 months (16 days to 42 years). Twenty-six patients had valved conduits, and 6 patients had transannular patches. There were no significant differences between groups in baseline and operative characteristics. There was 1 early death (transannular patch group). There were no late deaths during a median follow-up of 20.8 months (1 month to 16.5 years). No patients developed late left ventricular outflow tract obstruction. Transcatheter reintervention was required in 14 patients, 9 with valved conduits (34.6%) and 1 with transannular patch (20%, P > .99). Six patients (19.4%) required reoperation, all with a valved conduit (P = .34). CONCLUSIONS: Aortic root translocation can be done with low early and late mortality. There was preserved aortic valve function and no left ventricular outflow tract obstruction at late follow-up. The use of a transannular patch had early outcomes comparable to valved conduits, with a trend for fewer late reoperations.

Transposition of the great arteries and sinus venosus defect with partially anomalous pulmonary venous return: physiological and anatomic considerations.

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.

Successful robot-assisted repair of congenital mitral valve regurgitation.

Congenital mitral valve regurgitation (CMR) is very uncommon in adults and is usually associated with other congenital malformations. Repair of the mitral valve remains the standard of care. Very limited reports are available on minimally invasive surgical approaches in treating CMR. This report represents the first case series of the successful application of robotics in correcting CMR and associated anomalies, including a partial atrioventricular canal defect.

Right ventricular unloading for heart failure related to Ebstein malformation.

BACKGROUND: Patients with Ebstein malformation (EM) and severe RV dilatation and dysfunction have increased operative risk. Early results with right ventricular unloading utilizing the bidirectional cavopulmonary shunt (BCPS) during repair of EM have been encouraging. We report our experience of the 1.5-ventricle repair strategy for this difficult group of patients. METHODS: Between July 1999 and January 2013, 62 patients with severe EM underwent BCPS at the time of tricuspid repair. Median age was 21.5 years (range, 9 months to 57 years), 51.6% were male, and 72.5% were children. Severe RV dilatation was present in all patients; severe RV dysfunction was present in 72.5% (n=45) and moderate to severe RV dysfunction in 22.5% (n=14). Mean RV systolic pressure was 32.7±0.7 mm Hg and mean PA pressure was 15.6±2.1 mm Hg. Mean preoperative left ventricular ejection fraction (LVEF) was 0.536±0.071; it was less than 40% in 10 patients (16.1%). New York Heart Association class III/IV heart failure was present in 43 patients (69.3%) preoperatively and 20 patients (32.2%) were initially referred for heart transplant evaluation. Prior EM surgery occurred in 35.4% (n=22; 8 prior valve repair, 8 prior valve replacement, Blalock-Taussig shunt in 4, atrial septal defect (ASD) closure in 2). RESULTS: Tricuspid repair was performed in 51.6% (n=32, 5 had re-repair). Bioprosthetic valve replacement was performed in 48.4% (n=30, 8 had rereplacement). The BCPS was a planned procedure in 53 patients (85.5%) because of RV dysfunction; BCPS was added after unsuccessful weaning from bypass in 7 (11.2%), and in the early postoperative period due to hemodynamic instability in 2. Concomitant procedures included ASD closure in 48.3%, maze in 38.7%, and mitral valve repair in 6.4%. Postoperative extracorporeal membrane oxygenation support was needed in 8 patients. Delayed chest closure was performed in 25.8%. Early mortality was 1.6% (n=1). Mean mechanical ventilation time was 69.7 hours. Mean intensive care unit and hospital stays were 5.4±3.5 and 10.7±3.5 days, respectively. Mean follow-up was 3.6±2.6 years (maximum, 12.8 years). Patients (n=10) with low preoperative LVEF (0.362±0.035) improved to 0.517±0.042 postoperatively (p=0.001). There was 1 late death in a patient with cystic fibrosis. Late reintervention was needed in 5 patients (8%). Late follow-up was available in 95% (n=59); all were acyanotic and 88% were in New York Heart Association functional class I/II. CONCLUSIONS: Concomitant BCPS is a useful adjunct in repair of advanced EM with severe RV dilatation and dysfunction. Operation can be performed with low early mortality. Intermediate-term survival and quality of life is good to excellent, and transplantation can be delayed or avoided in the majority.

Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle.

BACKGROUND: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. METHODS: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. RESULTS: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. CONCLUSIONS: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.