The impact of severe obesity on image quality and ventricular function assessment in echocardiography and cardiac MRI.

This study sought to evaluate the impact of severe obesity on image quality and ventricular function assessment in cardiovascular magnetic resonance (MRI) and trans-thoracic echocardiography (TTE). We studied 100 consecutive patients who underwent clinically indicated cardiac MRI and TTE studies within 12 months between July 2017 and December 2020; 50 (28 females and 22 males; 54.5 ± 18.7 years) with normal body mass index (BMI) (18.5-25 kg/m2) and 50 (21 females and 29 males; 47.2 ± 13.3 years) with severe obesity (BMI ≥ 40 kg/m2). MRI and TTE image quality scores were compared within and across cohorts using a linear mixed model. Categorical left (LVF) and right (RVF) ventricular function were compared using Cohens Kappa statistic. Mean BMI for normal weight and obese cohorts were 22.2 ± 1.7 kg/m2 and 50.3 ± 5.9 kg/m2, respectively. Out of a possible 93 points, mean MRI image quality score was 91.5 ± 2.5 for patients with normal BMI, and 88.4 ± 5.5 for patients with severe obesity; least square (LS) mean difference 3.1, p = 0.460. TTE scores were 64.2 ± 13.6 for patients with normal BMI and 46.0 ± 12.9 for patients with severe obesity, LS mean difference 18.2, p < 0.001. Ventricular function agreement between modalities was worse in the obese cohort for both LVF (72% vs 80% agreement; kappa 0.53 vs 0.70, obese vs. normal BMI), and RVF (58% vs 72% agreement, kappa 0.18 vs 0.34, obese vs. normal BMI). Severe obesity had limited impact on cardiac MRI image quality, while obesity significantly degraded TTE image quality and ventricular function agreement with MRI.

The Role of Multimodality Imaging in the Evaluation of Heart Failure and Surgical Transplant Planning of Patients with Adult Congenital Heart Disease.

Cardiac imaging is pivotal in evaluating ventricular function, residual lesions, and long-term complications in patients with adult congenital heart disease (ACHD). Longitudinal imaging in ACHD is key for the timely identification of patients requiring evaluation for advanced therapies. The guidelines recommend routine imaging surveillance. In all patients undergoing evaluation with cardiac imaging, it is critical that studies are performed at centers with expertise and that the imaging protocols are tailored to the specific condition. The authors briefly highlight the utility and diagnostic yield of different modalities, review pertinent considerations for special populations, and focus on imaging for transplant planning.

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.

High-Risk Congenital Heart Disease in Pregnancy.

High-risk congenital heart disease (CHD) in pregnancy presents a complex clinical challenge. With improved medical care and increased survival rates, a growing population of adults with complex CHD are surviving to adulthood, including women of reproductive age. This chapter focuses on risk stratification and management of pregnant women with high-risk CHD, emphasizing the importance of considering both anatomical and physiological complexity. Maternal physiological changes, such as blood volume increase, cardiac output changes, and alterations in vascular resistance, can significantly impact high-risk CHD patients. Management of high-risk CHD in pregnancy necessitates a multidisciplinary approach and individualized care.

Early Experience and Lessons Learned Using Implanted Hemodynamic Monitoring in Patients With Fontan Circulation.

BACKGROUND: Data on the use of implanted hemodynamic monitoring (IHM) in patients with Fontan circulation are limited. This study reports our experience using the CardioMEMS HF system in adults with Fontan circulation. METHODS AND RESULTS: This single-center, retrospective study evaluated heart failure hospitalizations, procedural complications, and device-related complications in patients with Fontan circulation referred for IHM placement (2015-2022). The association of pulmonary artery pressure (by most recent catheterization and median IHM pressure within 30 days of placement) with both death and follow-up Model for End-Stage Liver Disease Excluding International Normalized Ratio score were evaluated. Of 18 patients referred for IHM placement, 17 were successful (median age, 30 [range 21-48] years, 6 women). Procedural complications (access site hematomas, pulmonary artery staining) occurred in 3 patients, without device-related procedural complications. In follow-up (median, 35 [range, 6-83] months), 1 patient developed a pulmonary embolism (possibly device-related). Heart failure hospitalizations/year were similar before and after IHM (median, 1 [interquartile range, 0-1.0] versus 0.6 [0-2.3]; P=0.268), though only 46% of heart failure hospitalizations had associated IHM transmissions. IHM pressures were associated with Model for End-Stage Liver Disease Excluding International Normalized Ratio scores (R2=0.588, P<0.001), though catheterization pressures were not (R2=0.140, P=0.139). The long-term mortality rate was 53% in this cohort. On unadjusted survival analysis, IHM pressures ≥18 mm Hg were associated with mortality (log rank P=0.041), which was not reproduced with catheterization pressures (log rank P=0.764). CONCLUSIONS: In patients with Fontan circulation, IHM did not reduce heart failure hospitalizations, though patient adherence to transmission was low. Device-related complications were low. IHM pressures may better represent real-life conditions compared with catheterization given associations with mortality and Model for End-Stage Liver Disease Excluding International Normalized Ratio score.

Novel uses for implanted haemodynamic monitoring in adults with subaortic right ventricles.

BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with subaortic right ventricles (sRVs), echocardiography is a poor screening tool for PH; implantable invasive haemodynamic monitoring (IHM) could be used for this purpose, but data are limited. The aim of this study is to report on novel uses of IHM in patients with sRV. METHODS: This retrospective study describes the uses of IHM, impact of IHM on heart failure hospitalisation (HFH) and device-related complications in adults with sRV from a single centre (2015-2022). RESULTS: IHM was placed in 18 patients with sRV (median age 43 (range 30-54) years, 8 female, 16 with D-TGA/AS, 2 with ccTGA); 16 had moderate or severe sRV systolic dysfunction, 13 had PH on catheterisation. IHM was used for (1) Medical therapy titration, (2) Medical management after ventricular assist device in patients with transplant-limiting PH and (3) Serial monitoring of pulmonary artery pressures without repeat catheterisations to help identify the optimal time for heart transplant referral. In follow-up (median 23 months), HFHs/year were similar to the year prior to IHM (median 0 (IQR 0-1.0) before vs 0 (0-0.8) after, p=0.984). Device migration occurred in one, without long-term sequelae. CONCLUSIONS: Uses of IHM in patients with sRV are described which may minimise the need for serial catheterisations in a population where PH is prevalent. HFHs were low overall but not impacted by IHM. One device-related complication occurred without long-term consequence.

Parametric mapping by cardiovascular magnetic resonance imaging in sudden cardiac arrest survivors.

Etiology of sudden cardiac arrest (SCA) is identified in less than 30% of survivors without coronary artery disease. We sought to assess the diagnostic role of myocardial parametric mapping using cardiovascular magnetic resonance (CMR) in identifying SCA etiology. Consecutive SCA survivors undergoing CMR with myocardial parametric mapping were included in the study. The determination if CMR was decisive or contributory in identifying SCA etiology was made if the diagnosis was unclear prior to CMR, and the discharge diagnosis was consistent with the CMR result. Parametric mapping was considered essential for establishing probable SCA etiology by CMR if the SCA cause could not have been determined without its utilization. If the CMR diagnosis could have been potentially based on the combination of cine and LGE imaging, parametric mapping was considered contributory. Of the 35 patients (mean age 46.9 ± 14.1 years; 57% males) included, SCA diagnosis was based on CMR in 23 (66%) patients. Of those, parametric mapping was essential for the diagnosis of myocarditis and tako-tsubo cardiomyopathy (11/48%) and contributed to the diagnosis in 10 (43%) additional cases. Inclusion of quantitative T1 and T2 parametric mapping in the SCA CMR protocol has the potential to increase diagnostic yield of CMR and further specify SCA etiology, especially myocarditis.

Patient Experience with Telemedicine in Adults with Congenital Heart Disease.

Introduction: The COVID-19 pandemic has resulted in increased use of telemedicine. There are limited data on patient experience with telemedicine in adults with congenital heart disease (ACHD). We hypothesized that due to their complex medical history, ACHD would prefer in-person clinic visits over telemedicine. Methods: We conducted a nurse-administered telephone survey based on Agency for Healthcare Research and Quality recommendations to assess patient experience after ACHD telemedicine visits in the early part of the pandemic from March 2020 to June 2020. Results: Of 216 ACHD who had telemedicine visits, 136 (63%) agreed to participate in the survey. Mean age was 45 ± 18 years, majority (65%) being video encounters. Most (98%) patients expressed that the telemedicine visit was successful in addressing their health care needs. Only 21 (15%) patients reported technical issues. Most patients (76%) preferred telemedicine given testing was provided separately, 25 (18%) preferred in-person clinic visits, and 8 (6%) had no preference. Of the 25 patients over 65 years, 19 (76%) would choose telemedicine over the in-person clinic, and only 1 patient reported technical difficulties. Conclusion: ACHD reported a positive experience with telemedicine. Technical limitations were infrequent even among the elderly. The majority of patients felt that their health care needs were addressed and would consider telemedicine over clinic visits. These data are important as payors move away from supporting telemedicine and hospitals restructure, with and without ongoing COVID-19 concerns.

Fulminant Myocarditis Following SARS-CoV-2 Infection: JACC Patient Care Pathways.

A 60-year-old woman with a past medical history of asthma presented with fulminant myocarditis 9 days after testing positive for SARS-CoV-2 and 16 days after developing symptoms consistent with COVID-19. Her hospital course was complicated by the need for veno-arterial extracorporeal membrane oxygenation, ventricular arrhythmias, and pseudomonas bacteremia. She ultimately recovered and was discharged to home with normal left ventricular systolic function. Thereafter, she developed symptomatic ventricular tachycardia, for which she received an implantable cardioverter-defibrillator and antiarrhythmic drug therapy.

Fulminant Myocarditis Following SARS-CoV-2 Infection: JACC Patient Care Pathways.

A 60-year-old woman with a past medical history of asthma presented with fulminant myocarditis 9 days after testing positive for SARS-CoV-2 and 16 days after developing symptoms consistent with COVID-19. Her hospital course was complicated by the need for veno-arterial extracorporeal membrane oxygenation, ventricular arrhythmias, and pseudomonas bacteremia. She ultimately recovered and was discharged to home with normal left ventricular systolic function. Thereafter, she developed symptomatic ventricular tachycardia, for which she received an implantable cardioverter-defibrillator and antiarrhythmic drug therapy.

Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis.

OBJECTIVES: While cardiac amyloidosis (CA) classically involves the left ventricle (LV), less is known about its impact on the right ventricle (RV) and pulmonary vasculature. We performed a retrospective analysis to identify the prevalence and types of pulmonary hypertension (PH) profiles in CA and to determine haemodynamic and cardiovascular magnetic resonance (CMR) predictors of major adverse cardiovascular events (MACE). METHODS: Patients with CA who underwent CMR and right heart catheterisation (RHC) within 1 year between 2010 and 2019 were included. Patients were assigned the following haemodynamic profiles based on RHC: no PH, precapillary PH, isolated postcapillary PH (IPCPH), or combined precapillary and postcapillary PH (CPCPH). The relationship between PH profile and MACE (death, heart failure hospitalisation) was assessed using survival analysis. CMR and RV parameters were correlated with MACE using Cox-regression analysis. RESULTS: A total of 52 patients were included (age 69±9 years, 85% men). RHC was performed during biopsy in 44 (85%) and for clinical indications in 8 (15%) patients. Rates of no PH, precapillary PH, IPCPH and CPCPH were 5 (10%), 3 (6%), 29 (55%) and 15 (29%), respectively. Haemodynamic PH profile did not correlate with risk of death (p=0.98) or MACE (p=0.67). Transpulmonary gradient (TPG) (HR 0.88, CI 0.80 to 0.97), RV, (HR 0.95, CI 0.92 to 0.98) and LV ejection fraction (HR 0.95, CI 0.92 to 0.98) were significantly associated with MACE. CONCLUSIONS: PH is highly prevalent in CA, even at the time of diagnosis. While IPCPH was most common, CPCPH is not infrequent. TPG and RV ejection fraction (RVEF) are prognostic markers in this population.

Maternal and fetal outcomes in pregnant women with pulmonary hypertension: The impact of left heart disease.

Background: Pulmonary hypertension (PH) due to left heart disease (World Health Organization (WHO) Group 2 PH) is the largest PH subgroup, however most reports of PH in pregnancy focus on patients with pulmonary arterial hypertension (WHO Group 1 PH). We evaluated pregnancy outcomes across WHO PH subgroups. Methods: We performed a retrospective single center cohort study of maternal and fetal outcomes in pregnant women with PH (2004-2018). Results: We analyzed outcomes of 70 pregnancies in 70 women with PH (30 ± 6 years-old), classified as WHO Group 1 PH (12 (17%)), Group 2 PH (45 (64%)), Group 3 PH (4 (6%)) and Group 5 PH (9 (13%)). Although no peripartum death occurred, 3 (4.3%) women with WHO Group 2 PH had late mortality (7 ± 4 months post- partum). Additionally, 33 major adverse cardiac events occurred in 26 (37%) women, preterm birth occurred in 32 (49%), and post-partum hemorrhage in 10 (14%), such that only 24 (37%) women completed a viable pregnancy free of an adverse cardiac, obstetric or fetal/neonatal event. Major adverse cardiac events were predominantly due to heart failure (24 (73%)), occurring only in WHO Groups 1 and 2 PH (3 (25%) women vs. 17 (38%), p = 0.07), and significantly associated with pre-eclampsia, left ventricular ejection fraction ≤45%, maternal diabetes, and systemic hypertension. Conclusions: WHO Group 2 PH carries similar risk for maternal cardiovascular events when compared to women with WHO Group 1 PH. Further studies evaluating maternal risk in this cohort are needed.

ACR Appropriateness Criteria® Suspected Acute Aortic Syndrome.

Acute aortic syndrome (AAS) includes the entities of acute aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer. AAS typically presents with sudden onset of severe, tearing, anterior, or interscapular back pain. Symptoms may be dominated by malperfusion syndrome, due to obstruction of the lumen of the aorta and/or a side branch when the intimal and medial layers are separated. Timely diagnosis of AAS is crucial to permit prompt management; for example, early mortality rates are reported to be 1% to 2% per hour after the onset of symptoms for untreated ascending aortic dissection. The appropriateness assigned to each imaging procedure was based on the ability to obtain key information that is used to plan open surgical, endovascular, or medical therapy. This includes, but is not limited to, confirming the presence of AAS; classification; characterization of entry and reentry sites; false lumen patency; and branch vessel compromise. Using this approach, CT, CTA, and MRA are all considered usually appropriate in the initial evaluation of AAS if those procedures include intravenous contrast administration. Ultrasound is also considered usually appropriate if the acquisition is via a transesophageal approach. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.