Fred H. Rodriguez

Outcomes of Heart Failure–Related Hospitalization in Adults with Congenital Heart Disease in the United States

BACKGROUND Heart failure (HF) accounts for >3 million hospital admissions annually in adults with acquired cardiovascular disease, but there are limited data on HF admissions in adults with congenital heart disease (ACHD). The purpose of this study was to test the hypotheses that HF admissions are common in ACHD and associated with significant morbidity and mortality. METHODS The 2007 Nationwide Inpatient Sample was used to assess national prevalence, morbidities, and risk factors for mortality during hospitalizations among ACHD with HF. RESULTS Of the 84,308 (95% CI 71,345-97,272) ACHD admissions in the United States in 2007, 17,193 (95% CI 14,157-20,229) had a diagnosis of HF (20%). ACHD with HF was associated with an increased risk of death compared to ACHD without HF (OR 3.3, 95% CI 2.6-4.1). On multivariable analysis independent risk factors for mortality included nonoperative intubation (OR 6.1, 95% CI 3.3-11.4), sepsis (OR 4.3, 95% CI 2.4-7.4), and acute myocardial infarction (OR 3.2, 95% CI 1.8-5.7). Cardiac defects associated with an increased risk of mortality included ventricular septal defects (VSDs) (OR 1.8, 95% CI 1.0-3.4). CONCLUSIONS In this large population-based study, HF-related hospitalizations were common in ACHD and associated with an increased risk of death compared to non-HF admissions. The risk of mortality is increased with the diagnoses of VSDs and the presence of specific comorbidities such as respiratory failure and sepsis.

Risk Factors for Major Adverse Events Late after Fontan Palliation

OBJECTIVE Risk factors for major adverse events late after Fontan palliation are unknown. Prior studies have suggested ventricular function and morphology as important risk factors. The aim of this study is to (1) characterize the late major adverse event profile in adult Fontan patients and (2) identify additional risk factors that may contribute to adverse outcomes. DESIGN AND SETTING A retrospective review of all adult patients >15 years post-Fontan seen at a tertiary academic center was conducted. Clinical, laboratory, cardiac data, and abdominal imaging were collected via chart review. Major adverse events (death, cardiac transplantation, or listing) were identified, and timing of events was plotted using Kaplan-Meier methods. Univariate and multivariate logistic regression was used to determine independent predictors of late-term events. RESULTS A total of 123 adult Fontan patients were identified (mean time post-Fontan 22.4 years [±4.4]). Major adverse events occurred in 19/123 patients (15%). In this 15-year survivor cohort, transplant-free survival rates were 94.6%, 82.9%, and 59.8% at 20, 25, and 30 years postoperation, respectively. Modes of death were Fontan failure with preserved function (4), congestive heart failure with decreased function (2), sudden death (2), thromboembolic event (1), post-Fontan conversion (2), and posttransplant (2). No differences in adverse outcomes were found based on morphology of the systemic ventricle, Fontan type, or systolic ventricular function. On the other hand, features of portal hypertension (OR 19.0, CI 4.7-77.3, P < .0001), presence of a pacemaker (OR 13.4, CI 2.6-69.8, P = .002), and systemic oxygen desaturation (OR 0.86, CI 0.75-0.98, P = .02) were risk factors for major adverse events in the multivariate analysis. CONCLUSIONS In adult Fontan patients surviving >15 years post-Fontan, portal hypertension, oxygen desaturation, and need for pacemaker were predictive of adverse events. Traditional measures may not predict late-term outcomes in adult survivors; further study of the livers role in late outcomes is warranted.

Outcomes of Hospitalization in Adults in the United States With Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect

Atrial septal defect, ventricular septal defect (VSD), and atrioventricular septal defect (AVSD) are among the most common congenital heart lesions, with most children surviving to adulthood. However, the clinical course of these patients is largely unknown, particularly pertaining to inpatient care. The purpose of this study was to assess hospitalizations for septal defects in adults with congenital heart disease (CHD) and risk factors associated with significant morbidity and mortality. The 2007 Nationwide Inpatient Sample was used to assess national prevalence of hospitalizations in adults with CHD with septal defects. Co-morbidities and risk factors for mortality were also determined. There were 84,308 adult CHD admissions in the United States in 2007. Fifty-four percent of adult CHD admissions had diagnoses of septal defects, with 48% having atrial septal defect, 7% having VSD, and 0.4% having AVSD. Overall in-hospital mortality was 2.1%. Common co-morbidities included arrhythmias (31%), heart failure (20%), and diabetes mellitus (18%). On multivariable analysis, independent risk factors for mortality included presence of VSD (odds ratio 3.1, 95% confidence interval [CI] 1.5 to 6.5), trisomy 21 (odds ratio 2.9, 95% CI 1.1 to 7.5), and pulmonary hypertension (odds ratio 1.5, 95% CI 1.0 to 2.4). In conclusion, this study of hospitalizations in adults with septal defects found that admissions are common and associated with significant co-morbidities. Overall mortality is low but is increased in patients with VSD. Cardiac and noncardiac co-morbidities are commonly encountered. Many noncardiac conditions, including trisomy 21 and the youngest and oldest groups, are associated with an increased risk of death.

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.

The Epidemiology of Heart Failure in Adults with Congenital Heart Disease

The impact of lifelong exposure to myocardial dysfunction in populations with congenital heart disease (CHD) is becoming increasingly recognized. Most children born with CHD now reach adulthood and the long-term sequelae of treatment are contributing to substantial comorbidity. The combination of structural changes present at birth with changes resulting from cardiac surgery can result in heart failure. This article reports on the current state of knowledge on the epidemiology of heart failure in this patient population.

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

BACKGROUND In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. METHODS A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse events (death or heart transplant) were examined using the Cox proportional hazard analysis. Correlations between the hemodynamic measurements and signs of end-organ dysfunction (MELD-XI, Child-Pugh, VAST score, estimated glomerular filtration rate [eGFR]) were analyzed. RESULTS Sixty post-Fontan patients (85% systemic left ventricle, 40% atriopulmonary Fontan, mean age of 28 years, and mean time since Fontan operation of 21.9 years) were included. At baseline, those with an event were statistically younger, had lower transcutaneous oxygen saturations, were more likely to have an atriopulmonary Fontan, and were more likely to have a pacemaker. Eighteen experienced a cardiovascularly significant event. Using univariate analysis to compare the event and nonevent groups, mean CI was 2.8 ± 0.9 vs. 2.4 ± 0.5 L/min/m2 (P = .004), and CVP was 18.6 ± 6.5 vs. 16.1 ± 4.3 mmHg (P = .03). However, the statistical significances did not persist in the multivariate model. Higher CVP and pulmonary capillary wedge pressure (PCWP) were associated with higher MELD-XI and Child-Pugh scores, and the VAST score was only associated with PCWP. CONCLUSIONS Symptomatic adult Fontan patients who experienced an event manifested with a higher CI and CVP, although the multivariate Cox proportional hazard analysis did not yield any significant associations. The presences of hepatic dysfunction and portal venous outflow obstruction were associated with a higher CVP and PCWP. Renal dysfunction was prevalent but no statistically significant association between the hemodynamic measurements was identified, although trends toward a higher CVP and transpulmonary gradient were identified.

Improving the quality of transition and transfer of care in young adults with congenital heart disease: Everitt et al.

The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease-specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients.

SERUM BIOMARKERS IN ADULT CONGENITAL HEART DISEASE

Adult congenital heart disease (ACHD) patients are often described as having a chronic heart failure (HF) syndrome. It is well known that acquired HF patients have serum biomarkers that may provide diagnostic and prognostic data. We sought to characterize these biomarkers in ACHD patients and

Atriopulmonary Fontan Spontaneous Echo Contrast Improved after Cardioversion

A 21-year-old woman with tricuspid atresia who had undergone an atriopulmonary Fontan operation at 3 years of age presented with intra-atrial reentrant tachycardia and tachycardia-induced cardiomyopathy. She had done well on no medications, and systolic function was normal on echocardiograms 3 months before this presentation. Since then, she had experienced exertional palpitations, increasing abdominal girth, loose bowel movements, vague abdominal pain, fatigue, and decreased appetite. Examination revealed a blood pressure of 120/70 mmHg, a regular tachycardic pulse, clear lungs, a pulse oximetry saturation of 97%, and a distended abdomen with ascites and hepatomegaly. Laboratory tests showed elevated alanine transaminase of 115 IU/L, brain natriuretic peptide of 556 pg/mL, an international normalized ratio of 1.4, negative stool antitrypsin (no protein-losing enteropathy), and normal renal function, lactate, and cardiac enzyme levels. An electrocardiogram showed flutter waves and 2:1 atrioventricular conduction (ventricular rate, 122 beats/min) (Fig. 1). A transthoracic echocardiogram revealed a dilated left ventricle with severely depressed systolic function, an ejection fraction of 0.25, and spontaneous echo contrast in the dilated inferior vena cava. A transesophageal echocardiogram showed no left atrial appendage thrombus; however, dense contrast was present in the dilated right atrium of the Fontan circuit (Fig. 2). Fig. 1 A 12-lead electrocardiogram shows intra-atrial reentrant tachycardia with 2:1 atrioventricular block; arrows point to flutter waves. Fig. 2 Transesophageal echocardiogram (bicaval view) shows 2:1 intra-atrial reentrant tachycardia with dense spontaneous echo contrast. We presumed that tachycardia-induced cardiomyopathy was exacerbating the patients Fontan circuit stasis, so we recommended terminating the tachycardia. Cardioversion yielded improvement (Fig. 3); however, contrast was still seen on cardiac magnetic resonance images (Figs. 4 and ​and5).5). The patient was placed on amiodarone, anticoagulation, and anticongestive therapy. An echocardiogram 4 months later showed improvement; the patient had mildly depressed systolic function with maintained sinus rhythm. Fig. 3 Transesophageal echocardiogram (bicaval view) after cardioversion to sinus rhythm shows improved but persistent spontaneous echo contrast. Fig. 4 Cardiac magnetic resonance image (axial view) shows atriopulmonary Fontan anatomy (right atrium-to-bilateral branch pulmonary artery anastomoses with visible contrast). Fig. 5 Cardiac magnetic resonance image (bicaval coronal view) shows the atriopulmonary Fontan circuit with swirling spontaneous echo contrast. IVC = inferior vena cava; LA = left atrium; RA = right atrium/atriopulmonary Fontan circuit to pulmonary arteries; ...

REPEAT PULMONARY VALVE REPLACEMENT: SIMILAR INTERMEDIATE-TERM OUTCOMES BETWEEN SURGICAL AND TRANSCATHETER PROCEDURES

There is no previous report comparing clinical or echocardiographic outcomes in patients with a history of prior surgical pulmonary valve replacement (SPVR) who undergo repeat SPVR or transcatheter pulmonary valve replacement (TPVR). We retrospectively identified patients who had previous SPVR and