Elisa A. Bradley

Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD.

Contemporary Hospitalization Rate Among Adults With Complex Congenital Heart Disease.

Background: Adults with congenital heart disease are hospitalized at increasing rates in the Western world. Identification of rates of and risk factors for hospitalization is essential for research and improving patient outcomes. Methods: We conducted a single-center retrospective analysis of patients with a primary diagnosis of tetralogy of Fallot (TOF), transposition of the great arteries (both d- and l-transposition of the great arteries [TGAs]), or single ventricle (SV). We investigated the rates of mortality, annual hospitalization, and patient-specific risk factors for unplanned cardiac hospitalization. Results: Adult patients with complex congenital heart disease are hospitalized at a rate four to eight times greater than the general US population (P < .001). In addition, there are significant differences between the rates of hospitalization in TOF and TGA (0.39 and 0.41 hospitalizations per patient-year, respectively) and SV (0.72 hospitalizations per patient-year). The majority of excess hospitalizations in the study group were due to cardiac disease (P < .001 for all three groups). Risk factors for unplanned cardiac hospitalization in TOF included pulmonary atresia, depressed left ventricular and right ventricular ejection fraction, and smoking; in TGA, they included Ebstein malformation, surgeries other than primary repair, noncardiac diagnoses, atrial arrhythmias, atrioventricular nodal block, left ventricular ejection fraction, and smoking; and in SV, they included atrial arrhythmias and cyanosis. Conclusions: Patients born with complex congenital heart disease are hospitalized far more frequently than the general US population, primarily for cardiac-related illness. Future research should focus on confirming the present findings and on identification of strategies to improve outcomes in this growing group of patients.

Major adverse maternal cardiovascular-related events in those with aortopathies. What should we expect?

Major adverse maternal cardiovascular-related events (MAMCRE) in aortopathy patients undergoing pregnancy are poorly defined. The aim was to assess for MAMCRE in pregnant patients with aortopathy or aortic enlargement in conotruncal defects (CTD), and determine if there are differences between groups. We conducted a single-center retrospective review of pregnant women (2000-2013) with hereditary vascular disease (HVD: BAV, COA), heritable fibrillinopathies (HF: MFS, EDS, LDS, FTAAS), and CTD with aortic dilatation (TOF, d-TGA, DORV). MAMCRE included: aortic dissection/surgery, therapeutic abortion, change in mode of delivery, and aortic growth > 0.5 cm within 1 year. We identified 73 patients/97 pregnancies (39/50 HVD, 15/20 HF, and 19/27 CTD). There were 14 MAMCRE (14%); 85% (n = 12) occurred in HV and HF patients and was associated with higher baseline cross-sectional-to-height (CSA/Ht) ratio (6.6 [Symbol: see text] 2.5 vs. 5.1 [Symbol: see text] 1.3, p = 0.005). There was more aortic surgery in the HF (vs. HV) (RR 3.9, p = 0.12). Only 2 MAMCRE (aortic growth) occurred in CTD. Overall and emergent C-section was higher than the general population (52% vs. 29%, p < 0.001 and 16% vs. 3%, p < 0.001) as was postpartum hemorrhage (PPH) (6% vs. 1.5%, p < 0.001). We describe the largest series of pregnant women with aortopathy and found a substantial incidence of MAMCRE, which was associated with higher pre-pregnancy CSA/Ht ratio. Rates of C-section and PPH were higher than the general population. Our data suggest that larger, multi-center studies are needed to define risks that predict MAMCRE/obstetric events in women with aortopathies, allowing optimal medical care during pregnancy.

First implantable hemodynamic monitoring device placement in single ventricle fontan anatomy

The Fontan anatomy leads to elevated central venous pressure along with chronic venous congestion and low cardiac output; this is felt to be responsible for deterioration of exercise tolerance and functional capacity over time. Real‐time hemodynamic evaluation of the Fontan anatomy has not been evaluated until now. Here, we report the technical aspects of the first two adult Fontan patients to undergo placement of an invasive hemodynamic monitor (IHM). We validate IHM readings with invasive pulmonary artery catheter derived hemodynamics in the Fontan and show successful home transmission of pulmonary artery hemodynamic tracings. This technology has the capacity to change current understanding of Fontan hemodynamics and treatment in patients with complex single‐ventricle anatomy.

Effectiveness of Early Invasive Therapy for Atrial Tachycardia in Adult Atrial-Baffle Survivors

Adults who underwent complex atrial baffling as children via Mustard or Senning procedures are at heightened risk for atrial arrhythmias. Antiarrhythmic therapies are typically ineffective in this population. Accordingly, our team of pediatric and adult electrophysiologists investigated the effectiveness of early invasive transbaffle-access techniques to perform early radiofrequency ablation at the source of these clinically significant arrhythmias. For this retrospective study, we selected 11 adult survivors of atrial baffling (mean age, 34 ± 9 yr) who underwent clinically indicated electrophysiologic study after no more than one trial of antiarrhythmic therapy. Using transbaffle-access techniques and 3-dimensional mapping of the venous atria, we found 12 inducible arrhythmias in 10 patients: intra-atrial reentrant tachycardia (n=6), atrioventricular nodal reentrant tachycardia (n=3), focal atrial tachycardia (n=2), and repetitive double firing of the atrioventricular node (n=1). Defining success as short- and midterm freedom from arrhythmia, we analyzed outcomes of radiofrequency ablation at 1 and 6 months. At 1 month, ablation was 100% successful. At 6 months, after 11 ablations in 9 patients, 5 patients had no clinical recurrence, 2 had improved arrhythmia control from minimal medical therapy, and 2 were to undergo repeat study for recurrent tachycardia. In the recurrence-free patients, arrhythmias during electrophysiology study matched the types found clinically before the study. To our knowledge, this is the largest one-year cohort of adult survivors of atrial baffling to have undergone study by a combined pediatric-adult electrophysiology team. We conclude that early invasive transbaffle access for ablating diverse atrial tachyarrhythmias was effective in these patients.

Mustard baffle obstruction and leak – How successful are percutaneous interventions in adults?

Atrial switch operations for D-Transposition of the great arteries (D-TGA) were performed until the late 20th century. These patients have substantial rates of re-operation, particularly for baffle related complications. This study sought to analyze the efficacy of percutaneous transcatheter intervention (PTI) for baffle leak and/or stenosis in adult atrial switch patients. Adult patients with a prior atrial switch operation who underwent heart catheterization (2002-2014) at a tertiary adult congenital heart disease referral center were retrospectively analyzed. In 58 adults (30 ± 8 years, 75% men, 14% New York Heart Association (NYHA) functional class ≥2) who underwent 79 catheterizations, PTI was attempted in 50 (baffle leak (n = 10, 20%), stenosis (n = 27, 54%), or both (n = 13, 26%)). PTI was successful in 45 and 5 were referred for surgery due to complex anatomy. A total of 40 bare metal stents, 18 covered stents, 16 occlusion devices, 2 angioplasties, and 1 endovascular graft were deployed. In isolated stenosis, there was improvement in NYHA functional class after PTI (8 vs. 0 patients were NYHA FC > 2, p = 0.004), which was matched by improvement in maximal oxygen consumption on exercise testing (VO2) (25.1 ± 5.4 mL/kg/min vs. 27.9 ± 9 mL/kg/min, p = 0.03). There were no procedure-related deaths or emergent surgeries in this cohort. This single-center cohort is the largest reported series of adult atrial switch operation patients who have undergone PTI for baffle stenosis and/or leak. We demonstrate that PTI with an expert multi-disciplinary team is a safe and effective alternative to surgery in adult patients with an atrial switch operation.

Management of Heart Failure in Adult Congenital Heart Disease

There are more than 1 million adults with congenital heart disease (ACHD) in the United States. Heart failure (HF) is the most common late cardiovascular complication. These patients are challenging to manage given their diverse presentation, anatomy, and complex hemodynamics. Examination of underlying anatomy is crucial because many require late transcatheter and surgical interventions after developing HF. Management of arrhythmia is equally important because this can modify HF symptoms. A multidisciplinary team with expertise in the care of ACHD-HF is critical.

Heart Failure in Women with Congenital Heart Disease

Heart failure remains the most common cause of morbidity and mortality in adults with congenital heart disease (CHD). Although gender-specific outcomes are not robust, it seems that women with CHD may be more affected by late heart failure (HF) than men. A specialized and experienced adult CHD team is required to care for these women as they age, including assessment for reversible causes of HF and in the management of pregnancy, labor, and delivery.

MUSTARD/SENNING OPERATION: IT IS NOT ALL “FLUTTER” ANYMORE

Complex inter-atrial baffling (Mustard/Senning operation) is a risk factor for late atrial tachyarrhythmias. We aim to characterize the source of arrhythmia, short-term RFA success, and medium-term freedom from clinically significant arrhythmia with a combined pediatric-adult EP team utilizing