The Early “Unnatural” History Following Surgical Repair of Ventricular Septal Defects

Introduction Surgical outcomes for simple ventricular septaldefects (VSD) have been excellent in the past three decades. Forthis project, the timing of resolution of left-sided dilation and mitralregurgitation (MR) following VSD repair was assessed. Methods Echocardiographic data surrounding surgery of 42 consecutivechildren who underwent surgical patch repair of a VSD werereviewed. The echocardiograms were reviewed up to a mean of 12months post-operatively (range 9 - 14 months). Quantitative dataindexed to body surface area including left atrial (LA) volume, mitralvalve annulus diameter, and left ventricular end-diastolic dimension(LVEDD) was analyzed. Results The majority of our pre-surgical cohort had only trace(44%) or no MR (31%), with a small proportion having mild (16%)or moderate MR (9%). No patients had moderate or greater MR followingrepair at follow-up. The median mitral valve annular Z-scorewas 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6(range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p< 0.001) at follow-up. LV dilation was present in 81% of pre-operativepatients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9).There was significant improvement in qualitative assessment of LVenlargement (25%) with a median LVEDD Z-score of 0.5 (range:-2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram wasperformed at a mean of 5.7 days (range: 3 - 12 days) following surgery. Conclusions Normalization of LA, mitral valve annulus, and LV sizeoccurred within the first three months in the majority of patients, withsignificant changes occurring within the first post-operative weekfollowing surgical repair for VSD

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle–dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management. </jats:p

Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management

Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus. In more severe cases, however, the ductus arteriosus is absent, and the sole source of pulmonary blood flow is via major aortopulmonary collateral arteries (MAPCAs). The variability in the origin, size, number, and clinical course of these MAPCAs adds to the complexity of these patients. Currently, the goal of management is to establish pulmonary blood flow from the right ventricle (RV) with RV pressures that are ideally less than half of the systemic pressure to allow for closure of the ventricular septal defect. In the long term, patients with ToF-PA are at higher risk for reinterventions to address pulmonary arterial or RV-pulmonary artery conduit stenosis, progressive aortic root dilation and aortic insufficiency, and late mortality than those with less severe forms of ToF. </jats:p

Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children

Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes. </jats:p

The Early “Unnatural” History Following Surgical Repair of Ventricular Septal Defects

Introduction&#x0D; Surgical outcomes for simple ventricular septaldefects (VSD) have been excellent in the past three decades. Forthis project, the timing of resolution of left-sided dilation and mitralregurgitation (MR) following VSD repair was assessed.&#x0D; Methods&#x0D; Echocardiographic data surrounding surgery of 42 consecutivechildren who underwent surgical patch repair of a VSD werereviewed. The echocardiograms were reviewed up to a mean of 12months post-operatively (range 9 - 14 months). Quantitative dataindexed to body surface area including left atrial (LA) volume, mitralvalve annulus diameter, and left ventricular end-diastolic dimension(LVEDD) was analyzed.&#x0D; Results&#x0D; The majority of our pre-surgical cohort had only trace(44%) or no MR (31%), with a small proportion having mild (16%)or moderate MR (9%). No patients had moderate or greater MR followingrepair at follow-up. The median mitral valve annular Z-scorewas 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6(range: -1.7 to 2.4; p &lt; 0.001) at follow-up. LA dilation was present in70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p&lt; 0.001) at follow-up. LV dilation was present in 81% of pre-operativepatients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9).There was significant improvement in qualitative assessment of LVenlargement (25%) with a median LVEDD Z-score of 0.5 (range:-2.1 to 2.9; p &lt; 0.001) at follow-up. Discharge echocardiogram wasperformed at a mean of 5.7 days (range: 3 - 12 days) following surgery.&#x0D; Conclusions&#x0D; Normalization of LA, mitral valve annulus, and LV sizeoccurred within the first three months in the majority of patients, withsignificant changes occurring within the first post-operative weekfollowing surgical repair for VSD.</jats:p

Multisystem Inflammatory Syndrome in Children: Survey of Protocols for Early Hospital Evaluation and Management.

ObjectiveTo describe the similarities and differences in the evaluation and treatment of multisystem inflammatory syndrome in children (MIS-C) at hospitals in the US.Study designWe conducted a cross-sectional survey from June 16 to July 16, 2020, of US children's hospitals regarding protocols for management of patients with MIS-C. Elements included characteristics of the hospital, clinical definition of MIS-C, evaluation, treatment, and follow-up. We summarized key findings and compared results from centers in which &gt;5 patients had been treated vs those in which ≤5 patients had been treated.ResultsIn all, 40 centers of varying size and experience with MIS-C participated in this protocol survey. Overall, 21 of 40 centers required only 1&nbsp;day of fever for MIS-C to be considered. In the evaluation of patients, there was often a tiered approach. Intravenous immunoglobulin was the most widely recommended medication to treat MIS-C (98% of centers). Corticosteroids were listed in 93% of protocols primarily for moderate or severe cases. Aspirin was commonly recommended for mild cases, whereas heparin or low molecular weight heparin were to be used primarily in severe cases. In severe cases, anakinra and vasopressors frequently were recommended; 39 of 40 centers recommended follow-up with cardiology. There were similar findings between centers in which &gt;5 patients vs ≤5 patients had been managed. Supplemental materials containing hospital protocols are provided.ConclusionsThere are many similarities yet key differences between hospital protocols for MIS-C. These findings can help healthcare providers learn from others regarding options for managing MIS-C

Multisystem Inflammatory Syndrome in Children: Survey of Early Hospital Evaluation and Management

ABSTRACTBackgroundIn the absence of evidence-based therapies for Multisystem Inflammatory Syndrome in Children (MIS-C), we aimed to describe the similarities and differences in the evaluation and treatment of MIS-C at hospitals in the United States.MethodsWe conducted a cross-sectional survey from June 16 to July 16, 2020 of U.S. children’s hospitals regarding protocols for patients with MIS-C. Elements included hospital characteristics, clinical definition of MIS-C, evaluation, treatment, and follow-up. We summarized key findings and compared results from centers that had treated &gt;5 patients vs. those that had treated ≤5 patients.ResultsForty centers of varying size and experience with MIS-C participated. About half (21/40) of centers required only 1 day of fever for MIS-C to be considered. In the evaluation of patients, there was often a tiered approach. Intravenous immunoglobulin was the most widely used medication to treat MIS-C (98% of centers). Corticosteroids were listed in 93% of protocols for primarily the moderate or severe cases. Aspirin was commonly used including for mild cases, whereas heparin or low molecular weight heparin were used primarily in severe cases. In severe cases, anakinra and vasopressors were frequently recommended. Nearly all centers (39/40) recommended follow up with cardiology. There were similar findings between centers that had treated &gt;5 patients vs. those that had treated ≤5 patients. A supplement containing hospital protocols is provided.ConclusionThere are many similarities yet some key differences between hospital protocols for MIS-C. These findings can help healthcare providers learn from others regarding options for managing MIS-C patients.Article SummaryThis survey of U.S. hospitals highlights the interhospital similarities and differences in management of Multisystem Inflammatory Syndrome in Children.What’s Known on This SubjectMIS-C is a novel and life-threatening disease in children associated with COVID-19. Early cases were treated with immunomodulatory agents similar to current guidelines for Kawasaki disease. There are currently no evidence-based guidelines for treatment of MIS-C.What This Study AddsThis study describes the protocolized evaluation and treatment of children with MIS-C at 40 hospitals in the U.S. These findings can help other hospitals create protocols to care for these children at their centers.</jats:sec