Ari M. Cedars

Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease: An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS)

BACKGROUNDnAdults with congenital heart disease represent an expanding and unique population of patients with heart failure (HF) in whom the use of mechanical circulatory support (MCS) has not been characterized. We sought to describe overall use, patient characteristics, and outcomes of MCS in adult congenital heart disease (ACHD).nnnMETHODSnAll patients entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) between June 23, 2006, and December 31, 2015, were included. Patients with ACHD were identified using pre-operative data and stratified by ventricular morphology. Mortality was compared between ACHD and non-ACHD patients, and multivariate analysis was performed to identify predictors of death after device implantation.nnnRESULTSnOf 16,182 patients, 126 with ACHD stratified as follows: systemic morphologic left ventricle (n = 63), systemic morphologic right ventricle (n = 45), and single ventricle (n = 17). ACHD patients were younger (42 years ± 14 vs 56 years ± 13; p < 0.0001) and were more likely to undergo device implantation as bridge to transplant (45% vs 29%; p < 0.0001). A higher proportion of ACHD patients had biventricular assist device (BiVAD)/total artificial heart (TAH) support compared with non-ACHD patients (21% vs 7%; p < 0.0001). More ACHD patients on BiVAD/TAH support were INTERMACS profile 1 compared with patients on systemic left ventricular assist device (LVAD) support (35% vs 15%; p = 0.002). ACHD and non-ACHD patients with LVADs had similar survival; survival was worse for patients on BIVAD/TAH support. BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio 4.4; 95% confidence interval, 1.8-11.1; p = 0.001). For ACHD patients receiving MCS, ventricular morphology was not associated with mortality.nnnCONCLUSIONSnACHD patients with LVADs have survival similar to non-ACHD patients. Mortality is higher for patients requiring BiVAD/TAH support, potentially owing to higher INTERMACS profile. These outcomes suggest a promising role for LVAD support in ACHD patients as part of the armamentarium of therapies for advanced HF.

Adult Congenital Heart Disease Patients Experience Similar Symptoms of Disease Activity

Background—There is a lack of objective data on the symptoms characterizing disease activity among adults with congenital heart disease (ACHD). The purpose of this study was to elicit the most important symptoms from patients across the spectrum of ACHD and to examine whether reported symptoms were similar across the spectrum of ACHD as a foundation for creating a patient-reported outcome measure(s). Methods and Results—We constructed a 39-item survey using input from physicians specializing in ACHD to assess the symptoms patients associate with disease activity. Patients (n=124) prospectively completed this survey, and the results were analyzed based on underlying anatomy and disease complexity. A confirmatory cohort of patients (n=40) was then recruited prospectively to confirm the validity of the initial data. When grouped based on underlying anatomy, significant differences in disease-related symptom rankings were found for only 6 of 39 symptoms. Six symptoms were identified which were of particular significance to patients, regardless of underlying anatomy. Patients with anatomy of great complexity experienced greater overall symptom severity than those with anatomy of low or moderate complexity, attributable exclusively to higher ranking of 5 symptoms. The second patient cohort had symptom experiences similar to those of the initial cohort, differing in only 5 of 39 symptoms. Conclusions—This study identified 6 symptoms relevant to patients across the spectrum of ACHD and remarkable homogeneity of patient experience, suggesting that a single disease-specific patient-reported outcome can be created for quality and outcome assessments.

An Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) analysis of hospitalization, functional status, and mortality after mechanical circulatory support in adults with congenital heart disease

BACKGROUNDnAdult congenital heart disease (ACHD) prevalence is increasing worldwide, with advanced heart failure (HF) as a leading cause of death. Limited data are available on durable mechanical circulatory support (MCS) in ACHD patients.nnnMETHODSnACHD patients from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) database were identified and propensity matched with non-ACHD patients using risk factors from the INTERMACS Seventh Annual Report. We compared these groups for the primary outcome of post-MCS mortality. We also investigated adverse event rates, functional status, and health-related quality of life.nnnRESULTSnACHD (n = 128) and non-ACHD (n = 512) patients were appropriately matched by baseline characteristics. ACHD patients had a longer length of stay at MCS implant (24 vs 19 days, p = 0.006) but similar rates of post-MCS adverse events and hospitalization. There were similar improvements in functional status and health related quality of life post-MCS in both groups. ACHD patients had significantly higher mortality post-MCS exclusively during the first 5 months after implant (p = 0.003) and a lower probability of receiving a transplant (p = 0.003). Risk factors for early mortality were biventricular or total artificial heart device implant and age > 50 years.nnnCONCLUSIONSnACHD patients experience a higher early mortality after MCS but have similar adverse event rates and similar improvements in functional capacity and quality of life compared with non-ACHD patients. These data support expansion of MCS use in selected ACHD patients.

Comparison of Efficacy and Safety of Transcatheter Aortic Valve Implantation in Patients With Bicuspid Versus Tricuspid Aortic Valves

Bicuspid aortic valve (BAV) stenosis has been considered a contraindication to transcatheter aortic valve implantation (TAVI). The aim of this study is to compare the efficacy and safety of TAVI in patients with BAV with those with tricuspid aortic valve (TAV) using balloon-expandable and self-expanding transcatheter heart valves. This retrospective study included 823 consecutive patients with severe, symptomatic aortic valve stenosis undergoing TAVI in 2 institutions, Baylor Heart and Vascular Hospital (Dallas, TX) and The Heart Hospital Baylor Plano (Plano, TX), from January 2012 to February 2016. Efficacy was evaluated by postprocedural valve function as mean gradient, peak velocity, effective orifice area, and ≥moderate paravalvular leak. Safety end points included all-cause 30-day and 1-year mortality, immediate postprocedural mortality and 30-day cardiovascular mortality, procedural success, pacemaker implantation, and procedural complications. Of the 823 included patients, 735 had TAV and 77 had BAV. Baseline characteristics were similar between the 2 groups. Procedural success was high in both BAV and TAV (98.7% vs 99.1%, pu2009=u2009ns). There were no significant differences between groups in valve hemodynamics after TAVI, pacemaker implantation rate, or procedural complications. There were no differences regarding immediate postprocedural mortality (BAV vs TAV, 1.1% vs 0.8%, pu2009=u2009ns), nor 30-day cardiovascular mortality (3.4% vs 2.3%, pu2009=u2009ns). All-cause mortality at 30 days (3.4% vs 3.1%, pu2009=u2009ns) and 1-year (8.5% vs 10.5%) were similar. Patients with BAV showed similar procedural and clinical outcomes to patients with TAV. Therefore, TAVI appears to be a safe and effective procedure for patients with BAVs as well as those with TAVs.

Call for a Disease-Specific Patient-Reported Outcome Tool in Adult Congenital Heart Disease

Those who care for patients with adult congenital heart disease (ACHD) need a valid and sensitive measure to better quantify the effect of ACHD on patients’ health status, their symptoms, function, and quality of life (QOL). These insights are critical in evaluating the success of novel treatments, monitoring patients over time, and comparing treatment success across providers as a foundation for quality improvement.1 Given the unique and myriad manifestations of various forms of ACHD, generic QOL measures are likely inadequate and the time has come to create an ACHD-specific patient-reported outcome (PRO) tool.nnThe prevalence of ACHD is expanding rapidly in the developed world. As a result of medical and surgical advances over the last half century, patients who previously would have succumbed to their cardiac abnormality in early childhood now live well into adulthood.2,3 Currently, it is estimated that there are more adults living in the United States with congenital heart disease than there are children.4 Although medical and surgical advances have led to dramatic improvements in survival, these patients are not cured and live with various limitations and symptoms that require ongoing monitoring and treatment.nnThe growth of this unique population of patients with chronic heart disease creates a need for new research strategies to optimize care and improve outcomes.5 Like other patients with chronic heart disease, patients with ACHD have been demonstrated to have rapidly rising rates of hospitalization and medical resource utilization.6,7 Current data support the notion that appropriate disease-specific care improves both health outcomes and cost-effectiveness in populations with acquired heart disease.8–12 The recent demonstration that specialized ACHD care may result in more positive outcomes for ACHD patients suggests that these patients may benefit from novel disease-specific pharmacological and device therapies analogous to …

Characteristics of hospital admissions associated with implantable cardioverter defibrillator placement among adults with congenital heart disease

BACKGROUNDnCharacteristics of hospitalizations including healthcare utilization for adult patients with congenital heart disease (ACHD) at the time of implantable cardioverter defibrillator (ICD) placement has not been well studied.nnnMETHODSnWe analyzed data from the 2002-2014 United States National Inpatient Sample (NIS). ICD implantation, CHD, complications, and indications for admissions were determined based on diagnostic codes among adults. Propensity score matching was performed, based on age, sex and in-hospital mortality index with a 10:1 ratio between adults without CHD and those with CHD, to determine relative healthcare utilization attributable to CHD.nnnRESULTSnACHD accounted for 136,509u202f±u202f3488 admissions of which 1451u202f±u202f121 admissions (1.1u202f±u202f0.06%) were associated with an ICD placement. ICD placement occurred most frequently among patients with TOF, VSD, and transposition complexes usually in the context of a dysrhythmia. Compared to those without CHD, ACHD patients had higher adjusted total hospital charges (

Effects of eplerenone on markers of myocardial fibrosis, 6-minute walk distance, and quality of life in adults with tetralogy of Fallot and complete transposition of the great arteries

147,002u202f±u202f5516 vs

Coronary artery fistula between the left anterior descending artery and pulmonary artery

132,455u202f±u202f2182; pu202f<u202f0.001), length of stay (6.2u202f±u202f0.5 vs 5.2u202f±u202f0.1u202fdays; pu202f<u202f0.001), lower readmission score (5.5u202f±u202f0.5 vs 9.7u202f±u202f0.1; pu202f=u202f0.04) and a higher complication rate (13.4% vs 8.3%; pu202f<u202f0.001). Dysrhythmias were more frequently the primary diagnosis for admission in the ACHD cohort (63% vs 38%; pu202f<u202f0.001).nnnCONCLUSIONnCompared to a matched non-CHD population, ACHD patients had greater healthcare utilization and had more frequent complications. The reasons underlying this difference bear investigation to improve care quality.

Physical Activity-Related Drivers of Perceived Health Status in Adults With Congenital Heart Disease

ABSTRACT Ventricular dysfunction is common among patients with repaired cyanotic congenital heart disease. To date, no pharmacologic intervention has been demonstrated to be beneficial in this setting. To begin addressing this knowledge gap, we conducted a single-center prospective, randomized, open-label pilot study to investigate the effects of eplerenone on serologic markers of collagen turnover and inflammation, 6-minute walk distance, and quality of life in patients with tetralogy of Fallot (TOF) or transposition of the great arteries with a systemic right ventricle (transposition of the great arteries [TGA]). Patients were randomized to a 3-month drug-free period at the beginning of the treatment period or at the end. All patients received 12 months of eplerenone therapy during the treatment period. Twenty-six patients were enrolled in the trial; 17 completed the study protocol: 8 with TOF and 9 with TGV. Eplerenone had no effect on serum levels of procollagen 1 N-terminal peptide (PINP), procollagen 3 N-terminal peptide (PIIINP), or galectin-3 (G3). Similarly, eplerenone had no effect on 6-minute walk distance or quality of life. In conclusion, PINP and PIIINP levels are as high as or higher in patients with TOF and TGA than in patients with normal cardiac anatomy and heart failure, whereas G3 levels are lower. Eplerenone is well tolerated by adults born with congenital heart disease.

Clinical predictors of length of stay in adults with congenital heart disease

ABSTRACT Coronary artery fistula is a rare anomalous connection between a coronary artery and a cardiac chamber or major vessel. Here we present the case of a 36-year-old woman who presented with sharp, substernal chest pain, dyspnea, and dizziness. A fistula between her left anterior descending artery and pulmonary trunk was found. Herein, we describe our approach and review the epidemiology, pathophysiology, diagnosis, and treatment options.