Peter Ermis

Clinical outcomes after ventricular assist device implantation in adults with complex congenital heart disease

BACKGROUND There are minimal data regarding ventricular assist device (VAD) implantation in adult patients with complex congenital heart disease (CHD). METHODS The medical records of 6 adult patients with complex CHD who underwent VAD implantation were retrospectively analyzed to characterize pre-operative clinical status, implantation techniques and challenges, post-operative management, and post-operative outcomes. RESULTS All patients had a systemic right ventricle, including 2 with single-ventricle physiology. The average age at VAD implantation was 41 years. The implanted VADs included 1 HeartMate XVE, 3 HeartMate II (Thoratec Corp, Pleasanton, CA), 1 Jarvik 2000 (Jarvik Heart, New York, NY), and 1 HeartWare HVAD (HeartWare Inc, Framingham, MA). In-hospital outcomes included no sub-pulmonic ventricular failure, no VAD thrombosis or mechanical failure, 30-day mortality in 1 patient, non-fatal sub-arachnoid hemorrhage in 1 patient, and sustained ventricular tachycardia requiring unsynchronized direct current cardioversion in 1 patient. Of the 5 patients who survived to discharge, 1 patient received 171 days of VAD support before cardiac transplantation and survived for 1,484 days after transplantation; 1 patient received 262 days of VAD support before out-of-hospital death of unknown etiology; 1 patient had received VAD support for 988 days as of December 1, 2012, while awaiting a transplant; and 2 patients who received VADs as destination therapy had received 577 and 493 days of VAD support, respectively, and were still alive as of December 1, 2012. CONCLUSIONS This case series characterizes important post-operative management challenges and clinical outcomes associated with VAD implantation as a bridge-to-transplant or as destination therapy in adult patients with complex CHD.

Left Ventricular Hemodynamic Changes and Clinical Outcomes after Transcatheter Atrial Septal Defect Closure in Adults

OBJECTIVES The objectives of this study are to assess current management algorithms for left ventricular (LV) hemodynamic and diastolic changes following atrial septal device occlusion in adult patients. BACKGROUND Percutaneous closure is now routine for atrial septal defects (ASDs). Previous studies show ventricular size normalization following percutaneous closure. Case reports have discussed the incidence of early LV dysfunction following ASD device placement with some recommending delay of closure or placement of a fenestrated device in patients with elevated LV pressures. METHOD All adult patients with an isolated secundum ASD who underwent percutaneous repair were included in this study. In addition to placement of the Amplatzer septal occluder, all patients had a pre and postprocedure transthoracic echocardiography performed measuring myocardial performance index (MPI). Left ventricular end diastolic pressure (LVEDP) was measured before and after balloon occlusion. RESULTS Nineteen patients (17 female and two male) were included in this study. Average age was 47.2 years (± 12.7 years). All defects were of clinical significance with average Qp : Qs = 2.0 (± 0.6). Balloon occlusion led to a significant (P < .01) increase in LVEDP (pre-LVEDP mean = 7.1 mm Hg, post-LVEDP mean = 15.3 mm Hg). There was no significant change in MPI. ASD device size displayed a modest correlation relative to the change in LVEDP (R = 0.42, P = .09). CONCLUSIONS Percutaneous ASD closure induces an increase in LVEDP. Despite this, all patients tolerated device closure without complication. It appears safe to close ASDs in these patients.

Electrophysiology procedures in adults with congenital heart disease.

BACKGROUND In adult congenital heart disease (CHD), arrhythmias contribute significantly to morbidity and mortality. Often, these adult patients are treated at a freestanding pediatric facility. Limited data exist looking at this cohort. METHODS A retrospective review was performed of all electrophysiology (EP) procedures performed in adults at our institution during a 5-year period from January 1, 2006 through December 31, 2010. RESULTS There were 99 cases performed in a total of 87 adults with CHD during this time period. The mean patient age was 27.1 years (18-51 years). The most common congenital cardiac diagnoses were: 27% with D-transposition of the great arteries (n = 27)-of which 85% (n = 23) have had a previous atrial switch procedure, 20% with tetralogy of Fallot (n = 20), and 16% with previous Rastelli repair (n = 16). Overall, 37 EP studies were performed, with the majority done in patients with complex CHD. There were 74 additional cases. These procedures consisted of: 38 pacemakers (51%), 26 implantable cardiac defibrillators (36%), six laser lead extractions (8%), two loop recorders (3%), and two pocket revisions (3%). During this 5-year period, there was one major complication (1%) and seven minor complications (7%). CONCLUSIONS The complex care of adults with CHD requiring EP procedures can be safely and effectively accomplished in a freestanding pediatric hospital with low complications, provided institutional support of an adult CHD program.

Cardiac resource utilization in adults at a freestanding children's hospital.

OBJECTIVE This article aims to give a comprehensive description of cardiac resource utilization in adults at a freestanding childrens hospital. DESIGN Retrospective chart review. SETTING Large, tertiary, freestanding childrens hospital. PATIENTS Adults (18 years of age and older) seen within the heart center or evaluated by heart center personnel from January 1, 2006 through December 31, 2010. RESULTS There were 2794 adults that utilized cardiology services during the study period. The mean patient age was 23.5 ± 8.4 years (18-70.5). The 1748 (62.6%) patients had a diagnosis of congenital heart disease. There were a total of 525 catheterization laboratory encounters and 104 cardiac surgical procedures performed on adult patients during the 5-year study period. The in-hospital mortality for all invasive encounters was 0%. The minor adverse event rate for all invasive encounters was 6.7% and 4.8%, respectively. Also, the overall major adverse event rates were 1.9% and 4.8%, respectively. There were 162 cardiology evaluations performed on adults in the emergency room. There were a total of 5489 adult cardiology clinic visits. The most common congenital heart disease diagnoses were: tetralogy of Fallot: 228 (13%), D-transposition of the great arteries: 208 (11.9%), single ventricle physiology: 187 (10.7%), atrial septal defect 128: (7.3%), and ventricular septal defect: 117 (6.7%). Overall, the complexity of congenital heart disease was: simple: 41%, moderate: 37%, and complex: 22%. CONCLUSIONS A significant and growing number of adult patients are currently being cared for at pediatric institutions. Excellent outcomes have been achieved at these centers with dedicated adult congenital heart disease services, consisting of both cardiologists and other faculty trained in adult medicine. Others with training in adult medicine, whether they be staff or contracted consulting services, are also required to help manage adult comorbidities.

LONG-TERM FOLLOW-UP IN ADULT SURVIVORS OF PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Background: Early childhood palliation for pulmonary atresia with intact ventricular septum (PA-IVS) can range from biventricular repair to single ventricle palliation. This study aims to evaluate the need for additional interventions long-term in a survivor cohort of adult PA-IVS patients following

USE OF NOVEL ORAL ANTICOAGULANTS IN ADULT FONTAN PATIENTS

Background: Thromboembolic complications are a leading cause of morbidity and mortality in patients with Fontan physiology. Current clinical practice for prevention and treatment of these complications involves the use of vitamin K antagonists and/or antiplatelet agents. Novel oral anticoagulants (

Pulmonary Valvular Stenosis in L-Transposition of the Great Arteries with Ventricular Septal Defect

A 39-year-old man presented with a fever of 102 °F. He had a history of viridans group streptococci (VGS) bacteremia, levo-transposition of the great vessels (also known as congenitally corrected transposition), large peri-membranous ventricular septal defect (VSD), and valvular pulmonary stenosis. Prior cardiac catheterization had shown his circulation to be well balanced, with a ratio of pulmonary blood flow to systemic blood flow of 1.4:1. Examination revealed nothing unusual except for a harsh systolic ejection murmur along the left sternal border. No emboli or immunologic phenomena were seen. The patients white blood cell count and cell count differential were normal, but his erythrocyte sedimentation rate and C-reactive protein level were elevated. During his initial 48 hours after presentation, 8 of 8 blood cultures grew VGS. We performed transesophageal echocardiography (TEE) to look for valvular vegetations and found neither intracardiac vegetations nor other evidence of endocarditis. Depicted are atrial–ventricular discordance (Fig. 1); ventriculo–arterial discordance (Figs. 2 and ​and3);3); short-axis view of the semilunar valves (Figs. 4 and ​and5);5); 3-dimensional (3D) imaging of the domed unicommissural pulmonic valve (Fig. 6); and a peak gradient of 80 mmHg, which suggests protected pulmonary arterial pressures (Fig. 7). Fig. 1 Transesophageal echocardiogram (0° midesophageal view) depicts a 4-chamber view. Note the usual arrangement of the atria, with atrioventricular discordance and the large inlet ventricular septal defect. Fig. 2 Transesophageal echocardiogram (60° midesophageal view) depicts atrioventricular and ventriculoarterial discordance between the left atrium, right ventricle, and aorta. Fig. 3 Transesophageal echocardiogram (60° midesophageal view) depicts atrioventricular and ventriculoarterial discordance between the right atrium, left ventricle, and pulmonary artery. Fig. 4 Transesophageal echocardiogram (140° midesophageal view) depicts short-axis view across the semilunar valves caudally. The aorta is anterior and leftward (L-transposition) to the pulmonary valve. Fig. 5 Transesophageal echocardiogram (140° midesophageal view) depicts short-axis view across the semilunar valves cranially. The pulmonary valve appears to be bicommissural, stenotic, thickened, and domed. Fig. 6 Three-dimensional transesophageal echocardiogram (140° midesophageal view) depicts short-axis view and level of pulmonary stenosis. The pulmonary valve is clearly unicommissural. Fig. 7 Continuous-wave Doppler echocardiogram across the pulmonary valve depicts an 80-mmHg gradient (4.5 m/s peak velocity) in the setting of systemic right ventricular and protected pulmonary arterial pressures. In Figure 1, the atrioventricular (AV) valves appear to be in the same plane, which raises the possibility of a partial AV canal with a large inlet VSD—rather than the usual apical displacement of the tricuspid valve (not shown) seen in structurally normal hearts and in congenitally corrected transposition. Also note that the AV valves follow their respective ventricles (Figs. 1–3), not their supplying atria. In comparing planar (Figs. 4 and ​and5)5) with 3D imaging (Fig. 6), note that the “bicommissural” valve is better defined with 3D imaging as unicommissural.

ADULT CONGENITAL HEART DISEASE ADMISSIONS TO PEDIATRIC HOSPITALS, 1999 TO 2010

Hospital admissions for adult congenital heart disease (ACHD) have increased by greater than two-fold within the past decade at adult facilities. Many of the most complex patients, however, continue to receive their care at childrens hospitals. We hypothesize that ACHD admissions to children&