Failing with Cyanosis-Heart Failure in End-Stage Unrepaired or Partially Palliated Congenital Heart Disease.

Heart failure in cyanotic congenital heart disease (CHD) is diagnosed clinically rather than relying solely on ventricular function assessments. Patients with cyanosis often present with clinical features indicative of heart failure. Although myocardial injury and dysfunction likely contribute to cyanotic CHD, the primary concern is the reduced delivery of oxygen to tissues. Symptoms such as fatigue, lassitude, dyspnea, headaches, myalgias, and a cold sensation underscore inadequate tissue oxygen delivery, forming the basis for defining heart failure in cyanotic CHD. Thus, it is pertinent to delve into the components of oxygen delivery in this context.

Biventricular Assist Devices as a Bridge to Heart Transplantation Under the New Donor Heart Allocation System in the United States.

Biventricular assist devices (BiVADs) for pre-heart transplant care is rare. The outcomes of pretransplant BiVAD support after the 2018 heart transplant allocation policy change are entirely unknown at this time. The United Network of Organ Sharing database was retrospectively queried from October 2018 to June 2022 to identify patients supported to transplant with BiVADs. They were compared to patients listed as Status 2 for heart transplantation with an isolated VAD (uni-VAD). The primary outcome of interest was 1 year survival. Secondary outcomes included length of stay, posttransplant stroke, dialysis, and pacemaker implantation. The frequency of BiVAD use for heart transplantation has remained unchanged after the 2018 allocation policy change, making up approximately 2% of transplant recipients annually. Patients supported with BiVADs appeared to be similar to patients supported with uni-VADs. One year survival was similar between the groups (88.57% vs. 87.90%). Length of stay was longer and there was a trend toward higher frequencies of posttransplant dialysis use. Patients supported to transplant with BiVADs appear to have posttransplant outcomes comparable to patients commonly listed as Status 2 with an isolated VAD. Compared to past analyses, there is a suggestion of improved survival with the 2018 allocation policy change.

Serial cardiac biomarker assessment in adults with congenital heart disease hospitalized for decompensated heart failure☆.

Background: Biomarkers are increasingly part of assessing and managing heart failure (HF) in adults with congenital heart disease (CHD). Objectives: To understand the response of cardiac biomarkers with therapy for acute decompensated heart failure (ADHF) and the relationship to prognosis after discharge in adults with CHD. Design: A prospective, observational cohort study with serial blood biomarker measurements. Settings: Single-center study in the inpatient setting with outpatient follow-up. Participants: Adults (≥18 years old) with CHD admitted with ADHF between August 1, 2019, and March 1, 2020. Exposure: We measured body mass, Kansas City Cardiomyopathy Questionnaire (KCCQ-12) score, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and high-sensitivity C-reactive protein (hsCRP) at enrollment, discharge, and 1st clinic follow-up visit; soluble suppression of tumorigenicity 2 (sST2) was measured at the first two time points. Measures: Univariate regression assessed the association between changes in weight, biomarkers, and changes in KCCQ-12 scores, between enrollment and discharge (Δ Hospitalization ) and between discharge and 1st clinical follow-up visit (Δ Post-discharge ). Wilcoxon rank-sum tests assessed the association between change in biomarkers, KCCQ-12 scores, and the composite outcome of cardiovascular death or rehospitalization for ADHF. Results: A total of 26 patients were enrolled. The median age was 51.9 years [IQR: 38.8, 61.2], 13 (54.2%) were women, and median hospital stay was 6.5 days [IQR: 4.0, 15.0] with an associated weight loss of 2.8 kg [IQR -5.1, -1.7]. All three cardiac biomarkers decreased during hospitalization with diuresis while KCCQ-12 scores improved; a greater decrease in sST2 was associated with an improved KCCQ-12 symptom frequency (SF) subdomain score (p = 0.012), but otherwise, there was no significant relationship between biomarkers and KCCQ-12 change. Change in hsCRP and NT-proBNP after discharge was not associated with the composite outcome (n = 8, vs. n = 16 who did not experience the outcome; Δ Post-discharge hsCRP +5.1 vs. -1.0 mg/l, p = 0.061; NT-proBNP +785.0 vs. +130.0 pg/ml, p = 0.220). Conclusions: Serial biomarker measurements respond to acute diuresis in adults with CHD hospitalized for ADHF. These results should motivate further research into the use of biomarkers to inform HF therapy in adults with CHD.

Adult survivors of moderate and great complexity congenital heart disease undergoing general surgery procedures: How do they fare?

BACKGROUND: Patients with complex congenital heart disease (CHD) are now commonly surviving well into adulthood. We describe the clinical characteristics and outcomes for a cohort of adult patients with moderate and great complexity CHD undergoing general surgery procedures. METHODS: The electronic records of two tertiary centers were queried to identify adult patients with moderate and great complexity CHD who underwent a general surgery procedure between 2007 and 2017. RESULTS: 118 adult patients were included in the analysis. The mean age was 36 ± 17 years and 49.2% were male. The most common cardiac diagnoses were pulmonary valve anomaly (24.6%), tetralogy of Fallot (18.6%), coarctation of the aorta (15.3%) and common/single ventricle (10.2%). The most common general surgery procedures performed were cholecystectomy (23.7%), herniorrhaphy (23.7%) and colorectal resection (9.3%). In-hospital mortality and morbidity were 2.5% and 11.9%, respectively. CONCLUSION: Adults survivors of moderate and great complexity CHD undergoing common general surgery procedures in this study experienced excellent in-hospital outcomes.

Transition from parenteral prostacyclins to selexipag: safety and feasibility in selected patients.

There are limited data regarding the feasibility of transitioning from intravenous prostacyclins to selexipag in pulmonary arterial hypertension patients. We present a case series of successful transitions from intravenous prostacyclins to selexipag in the majority of carefully selected five stable pulmonary arterial hypertension patients using a standardized protocol in the outpatient setting.

Fontan-Associated Dyslipidemia.

Background Hypocholesterolemia is a marker of liver disease, and patients with a Fontan circulation may have hypocholesterolemia secondary to Fontan-associated liver disease or inflammation. We investigated circulating lipids in adults with a Fontan circulation and assessed the associations with clinical characteristics and adverse events. Methods and Results We enrolled 164 outpatients with a Fontan circulation, aged ≥18 years, in the Boston Adult Congenital Heart Disease Biobank and compared them with 81 healthy controls. The outcome was a combined outcome of nonelective cardiovascular hospitalization or death. Participants with a Fontan (median age, 30.3 [interquartile range, 22.8-34.3 years], 42% women) had lower total cholesterol (149.0±30.1 mg/dL versus 190.8±41.4 mg/dL, P<0.0001), low-density lipoprotein cholesterol (82.5±25.4 mg/dL versus 102.0±34.7 mg/dL, P<0.0001), and high-density lipoprotein cholesterol (42.8±12.2 mg/dL versus 64.1±16.9 mg/dL, P<0.0001) than controls. In those with a Fontan, high-density lipoprotein cholesterol was inversely correlated with body mass index (r=-0.30, P<0.0001), high-sensitivity C-reactive protein (r=-0.27, P=0.0006), and alanine aminotransferase (r=-0.18, P=0.02) but not with other liver disease markers. Lower high-density lipoprotein cholesterol was independently associated with greater hazard for the combined outcome adjusting for age, sex, body mass index, and functional class (hazard ratio [HR] per decrease of 10 mg/dL, 1.37; 95% CI, 1.04-1.81 [P=0.03]). This relationship was attenuated when log high-sensitivity C-reactive protein was added to the model (HR, 1.26; 95% CI, 0.95-1.67 [P=0.10]). Total cholesterol, low-density lipoprotein cholesterol, and triglycerides were not associated with the combined outcome. Conclusions The Fontan circulation is associated with decreased cholesterol levels, and lower high-density lipoprotein cholesterol is associated with adverse outcomes. This association may be driven by inflammation. Further studies are needed to understand the relationship between the severity of Fontan-associated liver disease and lipid metabolism.

Wireless Hemodynamic Monitoring in Patients with Heart Failure.

PURPOSE OF REVIEW: Wireless hemodynamic monitoring in heart failure patients allows for volume assessment without the need for physical exam. Data obtained from these devices is used to assist patient management and avoid heart failure hospitalizations. In this review, we outline the various devices, mechanisms they utilize, and effects on heart failure patients. RECENT FINDINGS: New applications of these devices to specific populations may expand the pool of patients that may benefit. In the COVID-19 pandemic with a growing emphasis on virtual visits, remote monitoring can add vital ancillary data. Wireless hemodynamic monitoring with a pulmonary artery pressure sensor is a highly effective and safe method to assess for worsening intracardiac pressures that may predict heart failure events, giving lead time that is valuable to keep patients optimized. Implantation of this device has been found to improve outcomes in heart failure patients regardless of preserved or reduced ejection fraction.

Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review.

Objective: Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time. Methods: PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years. Results: Four thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported. Conclusion: In Fontan patients, the best predictors of death and transplantation were a decline in peak VO2, heart rate variables and exercise oscillatory ventilation. Peak VO2 was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.

Clinical Utility of Cardiac Magnetic Resonance Imaging in Pericardial Diseases.

BACKGROUND: Pericardial diseases are relatively common in clinical practice and encountered in various clinical settings with consequent significant morbidity and mortality. However, the diagnosis as well as management can be complex and challenging, as the clinical presentation is usually non-specific. Therefore, there is an increasing role for Cardiac Magnetic Resonance Imaging (CMR) as an imaging tool to facilitate the diagnosis of pericardial diseases. CONCLUSION: Herein we describe conventional and unique CMR approaches to provide an increased non-invasive understanding of the pericardium in health and disease including a novel method to diagnose constrictive pericarditis via radio-frequency tissue tagging by defining unique visceralparietal adherence patterns easily learned by the cardiologist and radiologist.

Is there a role for diastolic function assessment in era of delayed enhancement cardiac magnetic resonance imaging?: a multimodality imaging study in patients with advanced ischemic cardiomyopathy.

UNLABELLED: Cardiac magnetic resonance (CMR) identifies important prognostic variables in ischemic cardiomyopathy (ICM) patients such as left ventricular (LV) volumes, LV ejection fraction (LVEF), peri-infarct zone, and myocardial scar burden (MSB). It is unknown whether Doppler-based diastolic dysfunction (DDF) retains its prognostic value in ICM patients, in the context of current imaging, medical, and device therapies. METHODS: Diastolic function was evaluated in ICM patients (LVEF ≤ 40% and ≥ 70% stenosis in ≥ 1 coronary artery) who underwent transthoracic echocardiogram and delayed hyperenhancement CMR studies within 7 days. The association of DDF with the combined end point was assessed after risk-adjustment using Cox proportional hazards models. RESULTS: A total of 360 patients with severe LV dysfunction (LVEF = 24 ± 9%) and extensive MSB (31 ± 17%) were evaluated; DDF was present in all patients (stage 1%-44%, stage 2%-25%, stage 3%-31%). There were 130 events (124 deaths and 6 heart transplants) over a median follow-up of 5.8 years (IQR, 3.7-7.4 years). On multivariable analysis, DDF > stage 1 (HR, 1.37; P = .007) was associated with the combined end-point, independent of clinical risk score (HR, 2.40; P < .0001), implantable cardioverter defibrillator implantation (HR, 0.60; P = .009), incomplete revascularization (HR, 1.32; P = .003), mitral regurgitation (HR, 3.37; P = .01), peri-infarct zone area (HR, 1.04; P = 0.02), and MSB (HR, 1.02; P = .01). DDF had incremental prognostic value for the combined end-point (model χ(2) increased from 89 to 95, P = .02). CONCLUSION: DDF is a powerful predictor of mortality in ICM patients with significant LV dysfunction, independent of clinical and CMR data. DDF assessment provides incremental value, improving risk stratification.

Appropriate test selection for single-photon emission computed tomography imaging: association with clinical risk, posttest management, and outcomes.

BACKGROUND: Appropriate use criteria (AUC) for stress single-photon emission computed tomography (SPECT) are only one step in appropriate use of imaging. Other steps include pretest clinical risk evaluation and optimal management responses. We sought to understand the link between AUC, risk evaluation, management, and outcome. METHODS: We used AUC to classify 1,199 consecutive patients (63.8 ± 12.5 years, 56% male) undergoing SPECT as inappropriate, uncertain, and appropriate. Framingham score for asymptomatic patients and Bethesda angina score for symptomatic patients were used to classify patients into high (≥5%/y), intermediate, and low (≤1%/y) risk. Subsequent patient management was defined as appropriate or inappropriate based on the concordance between management decisions and the SPECT result. Patients were followed up for a median of 4.8 years, and cause of death was obtained from the social security death registry. RESULTS: Overall, 62% of SPECTs were appropriate, 18% inappropriate, and 20% uncertain (only 5 were unclassified). Of 324 low-risk studies, 108 (33%) were inappropriate, compared with 94 (15%) of 621 intermediate-risk and 1 (1%) of 160 high-risk studies (P < .001). There were 79 events, with outcomes of inappropriate patients better than uncertain and appropriate patients. Management was appropriate in 986 (89%), and appropriateness of patient management was unrelated to AUC (P = .65). CONCLUSION: Pretest clinical risk evaluation may be helpful in appropriateness assessment because very few high-risk patients are inappropriate, but almost half of low-risk patients are inappropriate or uncertain. Appropriate patient management is independent of appropriateness of testing.

Impact of repeat myocardial revascularization on outcome in patients with silent ischemia after previous revascularization.

OBJECTIVES: This study sought to compare the survival of asymptomatic patients with previous revascularization and ischemia, who subsequently underwent repeat revascularization or medical therapy (MT). BACKGROUND: Coronary artery disease is progressive and recurring; thus, stress myocardial perfusion scintigraphy (MPS) is widely used to identify ischemia in patients with previous revascularization. METHODS: Of 6,750 patients with previous revascularization undergoing MPS between January 1, 2005, and December 31, 2007, we identified 769 patients (age 67.7 ± 9.5 years; 85% men) who had ischemia and were asymptomatic. A propensity score was developed to express the associations of revascularization. Patients were followed up over a median of 5.7 years (interquartile range: 4.7 to 6.4 years) for all-cause death. A Cox proportional hazards model was used to identify the association of revascularization with all-cause death, with and without adjustment for the propensity score. The model was repeated in propensity-matched groups undergoing MT versus revascularization. RESULTS: Among 769 patients, 115 (15%) underwent revascularization a median of 13 days (interquartile range: 6 to 31 days) after MPS. There were 142 deaths; mortality with MT and revascularization were 18.3% and 19.1% (p = 0.84). In a Cox proportional hazards model (chi-square test = 89.4) adjusting for baseline characteristics, type of previous revascularization, MPS data, and propensity scores, only age and hypercholesterolemia but not revascularization were associated with mortality. This result was confirmed in a propensity-matched group. CONCLUSIONS: Asymptomatic patients with previous revascularization and inducible ischemia on MPS realize no survival benefit from repeat revascularization. In this group of post-revascularization patients, an ischemia-based treatment strategy did not alter mortality.