Shafkat Anwar

Rapid resolution of cardiac rhabdomyomas following everolimus therapy

Cardiac rhabdomyoma is the most common primary cardiac tumour during childhood and is usually associated with tuberous sclerosis complex (TSC). These tumours are generally considered benign, and spontaneous regression occurs commonly. However, when the tumours cause significant symptoms, the current standard treatment is surgical resection. Everolimus is an mammalian target of rapamycin (mTOR) complex 1 inhibitor that has been successfully used to treat subependymal giant cell astrocytomas and renal angiomyolipomas associated with TSC. A few case reports have described the effectiveness of everolimus therapy in treating cardiac rhabdomyomas as well. We report a case of a newborn who had near complete resolution of multiple rhabdomyomas within a month of receiving everolimus therapy for non-cardiac masses. To the best of our knowledge, this is the fastest resolution of cardiac rhabdomyomas associated with everolimus therapy to date. Everolimus may be a promising alternative for high-risk surgical candidates with haemodynamically significant cardiac rhabdomyomas.

The Impact of the Right Ventricular Outflow Tract Patch on Right Ventricular Strain in Tetralogy of Fallot: A Comparison with Valvar Pulmonary Stenosis Utilizing Cardiac Magnetic Resonance

Abstract A non-contractile transannular patch (TAP) in the right ventricular outflow tract (RVOT) contributes to ventricular dysfunction after tetralogy of Fallot (TOF) repair. We compared regional right ventricular (RV) strain in repaired TOF with valvar pulmonary stenosis (VPS) after balloon valvuloplasty to investigate the effects of TAP. Retrospective review of 26 cardiac magnetic resonance studies of TOF (n = 13) and VPS (n = 13) subjects matched by degree and duration of pulmonary regurgitation (PR). Feature tracking strain analysis was performed. Student’s t tests, Pearson correlation, and linear regression were applied. RV ejection fraction (EF) was normal and similar between TOF and VPS (60 and 65%, respectively, p = 0.8). RV 4-chamber Lagrangian longitudinal strain (RV 4ch LS) was worse in both groups compared to normals but comparable to each other: −18.2 (95% CI −3.6 to −33) for TOF and −20.2 (95% CI −12.4 to −28) for VPS, p = 0.5. RVOT LS was worse than RV 4ch LS in TOF, p = 0.05, but not in VPS, p = 0.19. There were no significant differences in RVOT strain between groups, p = 0.18. RVOT strain and RV 4ch LS correlated positively with RV EF in VPS (r = 0.72, p = 0.003 and r = 0.55, p = 0.04). PR degree correlated negatively with RVOT LS for TOF and VPS. Longitudinal strain is diminished in VPS and TOF subjects with preserved RV EF. TAP could explain worse RVOT strain in TOF. Longitudinal studies are needed to ascertain if RV strain predicts worsening of RV EF.

Anomalous Origin of the Left Coronary Artery From the Noncoronary Cusp: Not a Benign Lesion

This case report describes two patients with a very rare condition who presented with pathologic symptoms. Anomalous origin of the left coronary artery from the noncoronary cusp has been described as a “benign” lesion by some authors in the past, although rare cases of morbidity/mortality are described in the literature. Both reported patients underwent surgical repair for the lesion and at this writing are asymptomatic at follow-up evaluation. These two patients presenting with pathologic symptoms and undergoing surgery afford novel descriptions. The authors believe these descriptions add to our knowledge of this rare disorder.

Pulmonary Atresia With Aortopulmonary and Coronary Artery Collaterals: Precise Depiction by Low-Dose Computed Tomography

![Figure][1] [![Graphic][3] ][3] A 6-year-old female with tetralogy of Fallot whose echocardiogram and cardiac catheterization showed pulmonary atresia, ventricular septal defect, aortopulmonary collaterals, and collaterals from the coronary arteries to the main pulmonary artery.

Comparison of two-dimensional strain analysis using vendor-independent and vendor-specific software in adult and pediatric patients:

Introduction Two-dimensional strain analysis is a powerful analysis modality, however, clinical utilization has been limited by variability between different analysis systems and operators. We compared strain in adults and children using vendor-specific and vendor-independent software to evaluate variability. Methods One hundred and ten subjects (50/110 pediatric, 80/110 normal left ventricular function) had echocardiograms with a General Electric ultrasound scanner between September 2010 and January 2012. Left ventricular longitudinal strain was derived with EchoPAC (General Electric, v10.8.1), a vendor-specific software, and Velocity Vector Imaging (Siemens, v3.5), which is vendor-independent. Three independent readers analyzed all the echocardiograms yielding 330 datasets. Results Mean left ventricular global longitudinal Lagrangian strain was −18.1 ± SD 4.4% for EchoPAC and −15.3 ± SD 4.1% for Velocity Vector Imaging. Velocity Vector Imaging yielded lower absolute global longitudinal Lagrangian strain by mean 2.9 (±SD 2.7, p < 0.0001), and lower regional longitudinal strain. These differences persisted in normal subjects versus those with cardiomyopathy. Longitudinal strain differences were slightly higher in the pediatric cohort. There was no significant difference in inter-observer longitudinal strain and a small difference in intra-observer strain between analysis systems. On repeat measurements, a significant change in global longitudinal Lagrangian strain occurred after the difference exceeded 3–5 strain points for EchoPAC and Velocity Vector Imaging, respectively. Conclusion Velocity Vector Imaging produces lower left ventricular longitudinal strain values versus EchoPAC for the same echo images. Both systems have similar inter-observer variability, Velocity Vector Imaging slightly higher intra-observer variability. A statistically significant change in global longitudinal Lagrangian strain occurs with changes >3–5 strain points on repeat measurements. Strain values between the systems are not interchangeable.

Pediatric Chest Pain: Findings on Exercise Stress Testing

Chest pain is a relatively frequent complaint in pediatrics, accounting for 6 in every 1000 emergency department (ED) or clinic visits. Among 2071 cardiology consultations in a tertiary care pediatric hospital over a 12-month period, chest pain accounted for 5.2% of consultations, 13% of all ED evaluations, and 19% of ED consultations for new patients. Chest pain is a symptom that can cause significant distress and have lifealtering effects: approximately 50% of children with chest pain report they have missed school because of this complaint, and 69% of adolescents with chest pain had restricted their own activity. Whereas chest pain has ominous connotations in adults, in children it is usually benign. From the literature, the proportion of chest pain attributable to a cardiac etiology is consistently low. In a recent review of all 406 patients evaluated for chest pain in a pediatric cardiology clinic over a 1-year period, only 1.2% were found to have a cardiac etiology. The authors of this study suggest that exercise testing should be eliminated as part of the evaluation of chest pain in children. Although children and adolescents with chest pain are frequently referred to pediatric cardiologists, there is as yet no standard protocol for evaluation. The specific etiologies to exclude via cardiac evaluation are previously undiagnosed structural abnormalities associated with ischemic pain, acquired myopericardial or coronary disease, and arryhthmias with palpitations and/or tachycardia described as pain by the child. Among children found to have significant coronary abnormalities, a history of exertional chest pain has been reported as an important identifying feature. A history of exertional chest pain or exercise-associated symptoms such as palpitations, lightheadedness, or syncope often leads to evaluation with exercise testing. Given the potential underlying cardiac conditions, the yield of positive diagnostic results might be expected to be higher in this group of patients but previous reports suggest that this is not the case. To evaluate this further, we reviewed the results of exercise testing in a consecutive series of children referred for evaluation of chest pain to a pediatric cardiology clinic. Methods

3D Printing Provides a Precise Approach in the Treatment of Tetralogy of Fallot, Pulmonary Atresia with Major Aortopulmonary Collateral Arteries

Patients with tetralogy of Fallot, pulmonary atresia, and multiple aortopulmonary collateral arteries (Tet PA MAPCAs) have a wide spectrum of anatomy and disease severity. Management of these patients can be challenging and often require multiple high-risk surgical and interventional catheterization procedures. These interventions are made challenging by complex anatomy that require the proceduralist to mentally reconstruct three-dimensional anatomic relationships from two-dimensional images. Three-dimensional (3D) printing is an emerging medical technology that provides added benefits in the management of patients with Tet PA MAPCAs. When used in combination with current diagnostic modalities and procedures, 3D printing provides a precise approach to the management of these challenging, high-risk patients. Specifically, 3D printing enables detailed surgical and interventional planning prior to the procedure, which may improve procedural outcomes, decrease complications, and reduce procedure-related radiation dose and contrast load.

Adult Congenital Heart Disease

Advances in medical and surgical care over the past few decades have dramatically improved the survival rate of the adult patients with congenital heart disease (ACHD). This population presents unique challenges for clinical management, often made difficult by limitations of standard 2-dimensional imaging techniques. Cardiac 3D printing is a powerful imaging application that offers several advantages to patient care in the ACHD population. 3D models enable in-depth understanding of complex anatomy, precise pre-surgical planning, and are useful tools for patient counseling, trainee education, and simulation. In the ACHD population, these distinct advantages add value to conventional imaging techniques and offer promise for improving clinical outcomes for a complex and growing population.

Feature tracking strain is similar to harmonic phase cardiac magnetic resonance in Fontan patients: a validation study

Background Feature tracking strain (FTS) is a new technique to evaluate myocardial deformation from routinely acquired cardiac magnetic resonance (CMR) cine images, however, it has not been validated in single ventricle patients. The purpose of this study is to validate FTS against myocardial tagged harmonic phase (HARP) images, considered the reference standard in non-invasive deformation analysis.

3D Printing is a Transformative Technology in Congenital Heart Disease

Summary Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further.