Marfan Syndrome beyond Aortic Root-Phenotyping Using Cardiovascular Magnetic Resonance Imaging and Clinical Implications.

Marfan syndrome (MFS) is an inherited autosomal-dominant connective tissue disorder with multiorgan involvement including musculoskeletal, respiratory, cardiovascular, ocular, and skin manifestations. Life expectancy in patients with MFS is primarily determined by the degree of cardiovascular involvement. Aortic disease is the major cardiovascular manifestation of MFS.

Cardiovascular magnetic resonance for the evaluation of patients with cardiovascular disease: An overview of current indications, limitations, and procedures.

Cardiovascular disease (CVD) is the most common cause of morbidity/mortality worldwide. Early diagnosis is the key to improve CVD prognosis, and cardiovascular imaging plays a crucial role in this direction. Echocardiography is the most commonly used imaging modality.

Cardiovascular Magnetic Resonance Imaging Patterns in Rare Cardiovascular Diseases.

Rare cardiovascular diseases (RCDs) have low incidence but major clinical impact. RCDs' classification includes Class I-systemic circulation, Class II-pulmonary circulation, Class III-cardiomyopathies, Class IV-congenital cardiovascular diseases (CVD), Class V-cardiac tumors and CVD in malignancy, Class VI-cardiac arrhythmogenic disorders, Class VII-CVD in pregnancy, Class VIII-unclassified rare CVD. Cardiovascular Magnetic Resonance (CMR) is useful in the diagnosis/management of RCDs, as it performs angiography, function, perfusion, and tissue characterization in the same examination.

Long-Term Impact of Hospitalization for Kawasaki Disease on Health-Related Quality of Life.

Objective: To prospectively evaluate the long-term impact of Kawasaki disease (KD) hospitalization on health-related quality of life (HRQoL).

Methods: We merged the Outcomes Assessment Program and KD databases and queried for KD admissions between 1 month and 18 years of age. Patients with a diagnosis of community-acquired pneumonia were included as a comparison group.

The Deconditioning Effect of the COVID-19 Pandemic on Unaffected Healthy Children.

The COVID-19 pandemic has had devastating direct consequences on the health of affected patients. It has also had a significant impact on the ability of unaffected children to be physically active. We evaluated the effect of deconditioning from social distancing and school shutdowns implemented during the COVID-19 pandemic on the cardiovascular fitness of healthy unaffected children.

A Quality Initiative to Improve Appropriate Use of Initial Outpatient Echocardiography Among Pediatric Cardiologists.

Introduction: Appropriate use criteria (AUC) guide initial transthoracic echocardiogram (TTE) use in outpatient pediatrics. We sought to improve pediatric cardiologist TTE ordering appropriateness (mean AUC score) with a quality improvement initiative.

Methods: The outcome of interest was the prospective AUC score for all initial outpatient TTEs ordered between November 2016 and August 2017, categorized per the AUC: "appropriate" (score 7-9), "may be appropriate" (4-6), "rarely appropriate" (1-3).

mDixon ECG-gated 3-dimensional cardiovascular magnetic resonance angiography in patients with congenital cardiovascular disease.

Background: Cardiovascular magnetic resonance (CMR) angiography (CMRA) is an important non-invasive imaging tool for congenital heart disease (CHD) and aortopathy patients. The conventional 3D balanced steady-state free precession (bSSFP) sequence is often confounded by imaging artifacts. We sought to compare the respiratory navigated and electrocardiogram (ECG) gated modified Dixon (mDixon) CMRA sequence to conventional non-gated dynamic multi-phase contrast enhanced CMRA (CE-CMRA) and bSSFP across a variety of diagnoses.

Three-dimensional printing and virtual surgery for congenital heart procedural planning.

Complex unrepaired congenital heart disease requires extensive planning to determine the optimal procedural approach. Conventional noninvasive diagnostic imaging initially provides only two-dimensional (2D) representations of the complex, three-dimensional cardiovascular anatomy. With the expansion of 3D visualization techniques in imaging, a paradigm shift has occurred in complex congenital heart disease surgical planning using digital and 3D printed heart models.

Kawasaki Disease Substantially Impacts Health-Related Quality of Life.

Objective: To prospectively evaluate the acute impact of Kawasaki disease (KD) on health-related quality of life (HRQoL) and to assess deterioration in the HRQoL experienced by children with KD compared with other childhood diseases.

Study Design: We merged the Outcomes Assessment Program database obtained prospectively with the existing KD database and queried for KD admissions between 1 month and 13 years of age. HRQoL was evaluated with the parent-proxy Pediatric Quality of Life Inventory (PedsQL) 4.

Pediatric Appropriate Use Criteria for Outpatient Echocardiography: Practice Variations among Pediatric Cardiologists, Noncardiologist Subspecialists, and Primary Care Providers.

Background: The first pediatric appropriate use criteria (AUC) address the use of initial transthoracic echocardiography in outpatients by all ordering providers. The aim of this study was to appraise the performance of the AUC across pediatric cardiologists, noncardiologist subspecialists, and primary care providers (PCPs). A further aim was to describe the variations in ordering patterns of different groups of practitioners, which could serve as the basis for targeted quality improvement activities.

Inotropic agents improve the peripheral microcirculation of patients with end-stage chronic heart failure.

Background: Skeletal muscle microcirculation impairment in patients with chronic heart failure (CHF) seems to correlate with disease severity. We evaluated the microcirculation by near-infrared spectroscopy (NIRS) occlusion technique before and after inotropic infusion.

Methods: We evaluated 25 patients with stable CHF, 30 patients with end-stage CHF (ESCHF) receiving treatment with intermittent infusion of inotropic agents, and 12 healthy subjects.