Introduction : Preserved congenital heart specimens are an important component of training professionals working with children and adults with congenital heart disease. They are curated in few institutions worldwide and not freely accessible. This was a proof-of-concept project to explore the use of advanced cardiac imaging modalities (computed tomography [CT] and magnetic resonance imaging [MRI]) and virtual reality (VR) simulation to assess the feasibility and identify the best method of imaging curated cardiac pathology specimens. Methods : Seven specimens in glass jars with formalin, with varied anatomic lesions, from a curated collection were imaged using MRI and high-dose CT to compare the fidelity of models created via each modality. Three-dimensional (3D) models were created and loaded into a VR headset and viewed in virtual space. Two independent physicians performed a “virtual dissection” and scored the resultant models. Results : The highest fidelity and tissue characterization of more delicate structures was achieved with T2 spoiled gradient-echo sequences on MRI (median score of 4 out of 5). CT (median score of 3), while excellent for external anatomy, lost some fidelity with delicate internal anatomy, even at high-radiation doses. No specimens were damaged. Conclusions : We believe that in vitro heart specimens can be easily scanned with high fidelity at a relatively low cost, without causing damage, using high-dose CT and MRI. The ability to “walk through” different chambers of the heart makes the understanding of anatomy easy and intuitive. VR and 3D printing are technologies that could be easily adapted to digitize preserved heart specimens, making it globally accessible for teaching and training purposes.

Background : The aim of the study was to compare the effect of two different regimens of milrinone in pediatric patients with pulmonary artery hypertension (PAH) undergoing corrective procedure. Materials and Methods : This randomized prospective study included 100 pediatric patients undergoing corrective cardiac surgeries. Group E: Milrinone was started as infusion 0.5 μg/kg/min without a loading dose after induction of anesthesia and continued as infusion 0.5–0.75 μg/kg/min in the pediatric cardiac surgical intensive care unit (PSICU). Group L: Milrinone was started as a loading dose 50 μg/kg over 10 min before weaning from cardiopulmonary bypass (CPB) followed by infusion 0.5–0.75 μg/kg/min in the PSICU. We compared heart rate, mean arterial blood pressure, central venous pressure, cardiac index (CI), mean pulmonary arterial pressure (MPAP), serum lactate level, urine output, vasoactive inotropic score, mechanical ventilation duration, and intensive care unit (ICU)- and hospital length of stay between the groups. Results : There was an increase in mean arterial blood pressure, CI, and urine output in Group E compared to Group L (P < 0.05). MPAP, serum lactate level, and requirement of inotropes and vasopressors were lower in Group E compared to Group L (P < 0.05). Mechanical ventilation duration, ICU, and hospital length of stay were shorter in Group E than Group L (P < 0.05). Conclusions : Early use of milrinone in patients with PAH undergoing corrective cardiac surgeries improved CI and mean arterial pressure, decreased MPAP, improved urine output, decreased serum lactate level, and decreased requirement of inotropes and vasopressors after weaning from CPB compared to the milrinone bolus group.

Background : Congenital long QT syndrome (CLQTS) is a life-threatening ion channelopathy leading to syncope and sudden death. Early diagnosis during the prenatal period and timely intervention can prevent sudden cardiac death and catastrophic consequences of this genetic ion channelopathy. Fetal magnetocardiography and fetal electrocardiography (ECG) enable the measurement of fetal QT and JT intervals, but their inherently technically challenging and/or resource-intensiveness nature preclude their routine clinical application. On the other hand, the high-temporal resolution of M-mode echocardiography makes it a well-suited and widely available modality for the measurement of cardiac events. Aims and Objectives : We aimed to investigate the mechanical counterparts of the electrical QT and JT intervals on M-mode echocardiographic images of the tricuspid, mitral and aortic valves, and aortic wall. Methods : We performed a prospective study on consecutive children referred to the outpatient pediatric cardiology clinic at a tertiary children's hospital. We defined M-mode echocardiographic landmark points on tracings of tricuspid annular planar systolic excursion, mitral and aortic valves, and aortic wall with simultaneous electrocardiographic recording. We measured the mean±SD of the absolute time difference and RR-adjusted time difference in cases with non-coincident ECG events and echocardiographic landmarks. Results : Fifty healthy children were enrolled in the study. In 47 (94%) out of the 50 children, Q was coincident with the starting point of the tricuspid annular plane systolic excursion. In all children, the Q was coincident with the mid-point of the A-C line of the mitral valve. In 38 (76%) cases, there was a bump on the anterior wall of the aortic root immediately before the change in the slope of the aortic wall. This was coincident with the Q wave in 100% of cases. In all cases, the J point coincided with the point of acceleration of velocity on TAPSE. In all children, the J point coincided with the initial maximal opening of the aortic cusps. The end of the T wave occurred coincident with the peak of the tricuspid annular planar systolic excursion in 47 children (94%). In 48 children (96%), the end of the T wave coincided with the aortic cusps' closure point. Conclusions : Based on our findings, we propose to measure the averaged mechanical QT and JT intervals by using an angled M-mode tracing of the aortic and mitral valve in five consecutive beats in the parasternal long-axis view. This is the first study on mechanical QT and JT intervals in healthy children. The study opens the horizons into the in-utero diagnosis of congenital long QT syndrome by measuring fetal QT and JT intervals using the widely available M-mode echocardiography

Context and Background : The Cardiac Neurodevelopmental Outcome Collaborative has recommended using the Depression Anxiety Stress Scale (DASS) to evaluate for depression, anxiety, and stress in parents of children with congenital heart disease (CHD). There has not been a longitudinal study investigating its utility in these parents. Aims : The aim of this study was to determine the trend of depression, anxiety, and stress in parents of patients with CHD. Methods : Our center uses this self-reported survey at every visit between 6 and 36 months of age. This was a single-centered, retrospective study from January 1, 2018, to June 1, 2020. Statistical Analysis : Cox regression analysis was conducted using a composite end point of having an abnormal score in any of the three domains. Results : Two hundred and seventy-three mothers and 139 fathers were included in the study. For mothers, scores in each domain were elevated at 12 and 24 months. For fathers, scores in each domain were elevated at 6 months, followed by a decrease before peaking again, with depression increasing at 36 months and anxiety and stress increasing at 30 months. Increased length of stay for the index surgery was associated with an abnormal score for mothers (B = 0.02, P < 0.01) and fathers (B = 0.01, P = 0.04). Being in a relationship with the father (B = -0.8, P < 0.01) was associated with freedom of an abnormal score for mothers. Conclusions : Scores concerning for depression, anxiety, and stress peak at different points for parents. Length of stay for the index surgery and being in a relationship are important factors in the mental health of parents.

Background : A representational illustrated cardiac schema is useful for understanding and interpreting normal and abnormal fetal echocardiographic findings. Aim : Normal and abnormal fetal echocardiographic images can be better appreciated with the support of sectional schemas. Settings and Design : An attempt is made to include normal and abnormal variations in both grayscale and color images for easy understanding with the help of the schemas. Materials and Methods : The fetal cardiac sectional schemas are drawn by the author, using Microsoft Office Word drawing canvas. It is based on the best grayscale, color Doppler, power-angio, 3-dimensional, and high definition flow ultrasound images, supported by embryological and anatomic specimens in literature. Different sections are drawn in accordance with the planes suggested by Society for Maternal-Fetal Medicine, Society of Radiologists in Ultrasound, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American College of Radiology, and American Society of Echocardiography. Every effort has been meticulously pursued to match with the best ultrasound images with minor modifications for better clarity, understanding, and reproducibility. Results : The drawings include normal and most of the common cardiac anomalies depicting different sectional views starting from the abdomen and upward. Each drawing complies with the ultrasound images. Users with basic computer knowledge can easily modify these images using them as templates for reference, reporting, and publications. Conclusions : An attempt is made to represent the fetal echocardiographic images by simplified memorable sectional schemas. These schemas will facilitate a better understanding and interpretation of various normal and abnormal fetal echocardiographic images. Being electronically transmittable, these schemas can be used as templates for further modifications.

Background : Lung recruitment techniques are employed to help in improvement of pulmonary mechanics, facilitate early weaning, and shorten the duration of mechanical ventilation. We are reporting a novel lung recruitment technique employed in four children with left lung atelectasis, who underwent corrective surgery for congenital heart disease. Materials and Methods : From January 2020 to March 2021, four pediatric cardiac patients having left lung atelectasis, undergoing corrective surgery were subjected to lung recruitment technique and had elective endobronchial intubation and suctioning with chest physiotherapy in the form of vibration and percussion. This was done along with intermittent ventilation with 100% oxygen. Results : Successful recruitment of lung segments and clearance of atelectasis were confirmed by auscultation and chest X-ray in all four patients. All the cases were successfully weaned off the ventilator within 24–48 h. One patient had an opposite lung collapse after extubation, which was managed conservatively with chest physiotherapy. Another patient had bradycardia and desaturation during the procedure, which was improved after withdrawing the tube and instituting two lung ventilation with 100% oxygen. Conclusions : This novel lung recruitment technique helps in recruitment of collapsed lung segments and thus helps in early weaning and shortens the duration of mechanical ventilation.

Congenital portosystemic venous connections are rare vascular anomalies that connect the portal and systemic venous circulations. These vascular lesions can lead to complex and varied physiologic manifestations in single-ventricle patients as they progress through the various stages of palliation in the Fontan pathway. Specifically, these connections may be unmasked after a superior cavopulmonary anastomosis operation, but then “re-masked” after Fontan completion. We describe the complex physiology with an illustrative case report and present a novel method to unmask these pathologic connections after Fontan completion to facilitate transcatheter occlusion and resolve downstream symptoms.

Inappropriate sinus tachycardia (IST) is characterized by an inexplicably elevated sinus heart rate (HR) at rest, with an exaggerated response to physical activity. Its etiology is unknown, and IST is more common in the middle-aged population, being rare in children and adolescents. Although a favorable long-term prognosis has been described in a small series, tachycardia-induced cardiomyopathy has rarely been associated with IST. In this report, we describe an extremely rare case of IST detected in the first trimester of gestation, focusing on reviewing important features to improve the diagnosis of the different types of tachycardia by applying fetal cardiac ultrasonography. In the case reported, the diagnosis of IST was suspected in a fetus from a pregnant woman with celiac disease, who was referred at 9 weeks of gestation with fetal tachycardia. The fetus remained hemodynamically stable until term, and the female neonate was born unremarkably. Electrocardiography in the newborn showed sinus tachycardia (ST) with normal PR and QTc intervals. Possible causes of ST were excluded, thus confirming the fetal diagnosis. The patient presented with a fast HR at rest with an exaggerated increase with minimal physical activity accompanied by irritability. Beta-adrenergic blocker therapy was initiated, and the female neonate has progressed well.

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both the aorta and pulmonary artery arise completely or predominantly from the left ventricle. DOLV is a spectrum and can be classified depending on the position of the ventricular septal defect (VSD) relative to the great vessels, the relationship of the great vessels, and the presence or absence of pulmonary or aortic outflow obstruction. In the absence of tricuspid atresia or hypoplastic right ventricle, two ventricle repair is the preferred surgical treatment. We report a 31-day-old, 2.1 kg neonate with DOLV, subaortic VSD who underwent a successful arterial switch with VSD closure.

The presence of systemic and pulmonary venous anomalies in late presenting transposition of great arteries (TGA) may make standard atrial switch procedures difficult, necessitating modification in the surgical procedure. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein with TGA and regressed left ventricle, where a combination of Warden procedure and Mustard procedure was used.

Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.

Right-to-left shunt due to abnormal systemic venous drainage to the left heart is among the causes of hypoxemia following Fontan operation. There are conflicting data regarding the closure of the venovenous collaterals (VVCs) post-Fontan, showing decreased survival in older patients. In a child with visceral heterotaxy, we describe a rare fistula draining a right-sided hepatic vein into hepatic venous plexus and a right-sided pulmonary venous atrium. The patient presented with severe hypoxemia post-Fontan and underwent fistula occlusion with AMPLATZER™ Vascular Plug II, successfully improving hemodynamic status with resolution of the hypoxemia. Younger patients with cyanosis due to VVCs may benefit from percutaneous occlusion post-Fontan.

Myocardial infarction is extremely rare in children. However, chest pain suspected to be cardiac in origin by the parents is a very common presenting symptom in pediatric emergency services. In this case, an electrocardiogram is very important in the differential diagnosis of cardiogenic complaints and should be carefully considered.

What do we call a vascular structure that is in the left pneumopericardial space, drains systemic venous blood, and the total pulmonary venous return, into the right atrium, through a dilated coronary sinus (CS)? Can we preserve the CS drainage while correcting the total anomalous pulmonary venous connection?

Right ventricle-dependent coronary circulation coexisting with left main coronary atresia in the setting of pulmonary atresia-intact ventricular septum is rare. In the case described, the left coronary artery (LCA) origin from the aorta could not be found on conventional angiography or cardiac magnetic resonance imaging. During surgery, multiple LCA branches originating from the finger-like continuum of the primitive right ventricular sinusoidal network were observed. A Damus–Kaye–Stansel anastomosis and an aortopulmonary shunt operation were performed. Shunt takedown and a bidirectional Glenn anastomosis followed at 3 months of age. At 18 months follow-up, the child is thriving with stable hemodynamics and a saturation of 85%. Awareness about this rare coronary artery anomaly is necessary to prevent catastrophic consequences. The challenges, complications, and lessons learned while treating this rare variant are discussed.

Cor triatriatum dexter is an extremely rare congenital anomaly that is caused by the partitioning of the right atrium by a broad sheet of tissue due to the persistence of the right sinus venosus valve. We describe a rare case of prominent right sinus venosus valve (cor triatriatum dexter) with consequent right-to-left shunt across the patent foramen ovale in a newborn with significant cyanosis.

We report a case with prenatal diagnosis of transposition of great arteries (TGA) with L-posed aorta (SDL-TGA) which was confirmed by postnatal echocardiography. The anatomic findings were confirmed during the successful arterial switch operation (ASO). The technical challenges of ASO in the L-posed aorta are also described.

Spatiotemporal imaging correlation (STIC) technology has been employed to visualize the fetal heart for close to two decades, but the additional value of the technology remains debatable. The value of the technology in identifying the morphology of the cardiac valves is being recognized. We report a 21-week gestational age fetus with common arterial trunk where STIC imaging enabled us to identify a bicuspid arterial valve.