Sudden death in adults with repaired coarctation of the aorta: A case for sex-based risk factors.

Background: Sudden cardiac death (SCD) is an important risk for adults with repaired coarctation of the aorta (rCoA). We aimed determine if there are clinical risk factors for SCD in adults with rCoA. Methods and results: SCD events and clinical data from all adults with rCoA at a tertiary care center (2007-2017) were evaluated. In 167 adults with rCoA (39 ± 11 years old, 75 (45%) female) SCD occurred in 8 (5%) (vs. age-matched adults 0.9%). Those with SCD demonstrated significant QTc prolongation (QTc: 479 ± 16 vs. 434 ± 30 msec, p < 0.001). Overall, adults with rCoA and a prolonged sex-normative QTc interval had a 12-fold increased risk of SCD (x2 (1) = 12.3, p < 0.001), with men sustaining SCD at younger ages (42 ± 13 years vs. women 60 ± 10 years, p < 0.05). Multiple logistic regression modeling demonstrated that prolonged QTc selectively advanced risk for SCD in men only (x2 QTc prolongation 8.46, p < 0.005 and x2 age 0.29, p = 0.587), whereas in women, age was associated with SCD risk (x2 QTc prolongation 2.84, p = 0.092 and x2 age 7.81, p = 0.005). Non-sustained ventricular tachycardia, ventricular dysfunction, and myocardial fibrosis did not significantly impact SCD risk. Conclusions: There is an unanticipated high burden of SCD in adults with rCoA, occurring in men at younger age than women, suspicious for primary electrophysiologic dysfunction. Future investigation of sex-specific SCD risk in rCoA is important to better understand this disease and its late phenotype.

Society for Cardiovascular Magnetic Resonance 2022 Cases of SCMR case series.

"Cases of SCMR" is a case series on the SCMR website (https://www.scmr.org) for the purpose of education. The cases reflect the clinical presentation, and the use of cardiovascular magnetic resonance (CMR) in the diagnosis and management of cardiovascular disease. The 2022 digital collection of cases are presented in this manuscript.

Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects.

BACKGROUND: To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications. METHODS: Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used. RESULTS: Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model. CONCLUSIONS: Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.

SARS-CoV-2 molecular diagnosis at airports to minimize travel-related COVID-19 spread.

Following the identification of SARS-CoV-2, screening for air travel helped mitigate spread, yet lessons learned from a case study of air travel within Canada display enhanced techniques to better identify infected individuals, informing future responsive screening. While international travel bans limit infectious spread beyond a country's borders, such measures are hardly sustainable economically and infrequently address domestic travel. Here, we describe a case study from Canada, where a diagnostic laboratory at point of travel conducted real-time PCR-based detection of SARS-CoV-2 in support of existing interventions, including clinical and epidemiological questionnaires, and temperature checks. All mining workers departing from a populated urban area flying to one of two sites (Site A and B) in a remote northern Canadian region, which we deemed "at-risk", because healthcare services are limited and vulnerable to epidemics. Data collected between June and November 2020 on 15,873 clinical samples, indicate that molecular diagnosis allowed for identification of 13 infected individuals, who would have otherwise been missed by using solely nonpharmaceutical interventions. Overall, no outbreaks, COVID-19-related or other, were detected at the point of travel up to December 2021 since the implementation of the laboratory, suggesting this screening process is an effective means to protect at-risk communities. The success of this study suggests a process more practical than travel bans or an unfocused screening of air travelers everywhere.

Operational and Ethical Considerations for a National Adult Congenital Heart Disease Database.

As more adults survive with congenital heart disease, the need to better understand the long-term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality-based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

The role of imaging in characterizing the cardiac natural history of Duchenne muscular dystrophy.

Duchene muscular dystrophy (DMD) is a rare but devastating disease resulting in progressive loss of ambulation, respiratory failure, DMD-associated cardiomyopathy (DMD-CM), and premature death. The use of corticosteroids and supportive respiratory care has improved outcomes, such that DMD-CM is now the leading cause of death. Historically, most programs have focused on skeletal myopathy with less attention to the cardiac phenotype. This omission is rather astonishing since patients with DMD possess an absolute genetic risk of developing cardiomyopathy. Unfortunately, heart failure signs and symptoms are vague due to skeletal muscle myopathy leading to limited ambulation. Traditional assessment of cardiac symptoms by the New York Heart Association American College of Cardiology/American Heart Association Staging (ACC/AHA) classification is of limited utility, even in advanced stages. Echocardiographic assessment can detect cardiac dysfunction late in the disease course, but this has proven to be a poor surrogate marker of early cardiovascular disease and an inadequate predictor of DMD-CM. Indeed, one explanation for the paucity of cardiac therapeutic trials for DMD-CM has been the lack of a suitable end-point. Improved outcomes require a better proactive treatment strategy; however, the barrier to treatment is the lack of a sensitive and specific tool to assess the efficacy of treatment. The use of cardiac imaging has evolved from echocardiography to cardiac magnetic resonance imaging to assess cardiac performance. The purpose of this article is to review the role of cardiac imaging in characterizing the cardiac natural history of DMD-CM, highlighting the prognostic implications and an outlook on how this field might evolve in the future.

Triage and management of the ACHD patient with COVID-19: A single center approach.

With the recent emergence of SARS-CoV-2 and COVID-19, healthcare facilities and personnel are expected to rapidly triage and care for patients with even the most complex medical conditions. Adults with congenital heart disease (ACHD) represent an often-intimidating group of complex cardiovascular disorders. Given that general internists and general cardiologists will often be asked to evaluate this group during the pandemic, we propose here an abbreviated triage algorithm that will assist in identifying the patient's overarching ACHD phenotype and baseline cardiac status. The strategy outlined allows for rapid triage and groups various anatomic CHD variants into overarching phenotypes, permitting care teams to quickly review key points in the management of moderate to severely complex ACHD patients.

Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach.

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

Double aortic arch presenting as neonatal respiratory failure in the delivery room.

Respiratory symptoms are prevalent in vascular ring anomalies, including double aortic arch, which is considered the most common type of vascular ring. However, respiratory failure owing to critical airway obstruction in the neonatal period is rare with few reported cases in the literature. We report a case of a newborn patient who required emergent intubation within the first few hours of life and was later discovered to have a double aortic arch.

Gradient-recalled echo sequences in direct shoulder MR arthrography for evaluating the labrum.

OBJECTIVE: The purpose of this study was to determine the utility of fat-suppressed gradient-recalled echo (GRE) compared with conventional spin echo T1-weighted (T1W) sequences in direct shoulder MR arthrography for evaluating labral tears. MATERIALS AND METHODS: Three musculoskeletal radiologists retrospectively reviewed MR arthrograms performed over a 12-month period for which surgical correlation was available. Of 180 serial arthrograms, 31 patients had surgery with a mean of 48 days following imaging. Paired coronal oblique and axial T1W or GRE sequences were analyzed by consensus for labral tear (coronal oblique two-dimensional multi-echo data image combination, 2D MEDIC; and axial three-dimensional double-echo steady-state, 3D DESS; Siemens MAGNETOM Sonata 1.5-T MR system). Interpretations were correlated with operative reports. RESULTS: Of 31 shoulders, 25 had labral tears at surgery. The GRE sequences depicted labral tears in 22, while T1W images depicted tears in 16 (sensitivity 88% versus 64%; p<0.05). Subdividing the labrum, GRE was significantly more sensitive for the posterior labrum (75% versus 25%; p<0.05) with a trend toward greater sensitivity at the anterior labrum (78% versus 56%; p=0.157) but not significantly different for the superior labrum (50% versus 57%; p>0.7). Specificities were somewhat lower for GRE. CONCLUSION: Thin section GRE sequences are more sensitive than T1W for the detection of anterior and posterior labral tears. As the specificity of GRE was lower, it should be considered as an adjunctive imaging sequence that may improve depiction of labral tears, particularly smaller tears, in routine MR arthrography protocols.

Ultrasound of the knee.

Ultrasound of the knee is best suited for the evaluation of tendons, fluid collections and effusion, synovitis, periarticular soft tissue masses, muscles, and the collateral ligaments. Advantages of sonographic evaluation include the lack of ionizing radiation, the noninvasive nature of the examination, focused evaluation correlated with the specific site of pain, multiplanar capability, and utility in patients for whom magnetic resonance imaging (MRI) is contraindicated. The ability to visualize the microanatomy of tendons, ligaments, nerves, and muscles is also an advantage over MRI. Moreover, ultrasound is generally readily accessible and of lower cost than MRI. Ultrasound is less suited for the evaluation of internal derangement. Certain structures that are exquisitely evaluated by MRI, including the menisci, cruciate ligaments, bone marrow, and articular cartilage, are suboptimally evaluated by sonography. This article reviews the structures and pathological processes for which ultrasound has been shown useful, emphasizing appropriate technique and normal anatomy, appearance of pathological processes, and advantages and limitations of sonographic evaluation.