Complex Adult Congenital Heart Disease Research Articles

Subcutaneous implantable cardioverter defibrillator in complex adult congenital heart disease. Results from the S-ICD “Monaldi Care” registry

BackgroundImplantable cardioverter defibrillators (ICD) are widely accepted therapy in congenital heart disease (CHD) patients at risk of life-threatening ventricular arrhythmias or sudden cardiac death (SCD). Initial experience with subcutaneous ICD (S-ICD) systems has shown a high efficacy. However, the use of S-ICD in complex ACHD (adult congenital heart disease) implies some specific considerations, as the safety for these patients in unknown and recommendations among physicians may vary widely. MethodsWe review the data and studied the indications for S-ICD in complex ACHD and discuss its usefulness in clinical practice. ResultsFrom a large cohort of 297 patients enrolled in the S-ICD “Monaldi care” registry, that encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 14 consecutive complex ACHD patients (aged 35.9 ± 16.7 years) who underwent S-ICD implant from February 2015 to June 2020. Mean follow-up was 23 ± 19.5 months. All the patients showed a good compliance to the device system with no complications (infections or skin erosions). Four patients (1 M/3F aged 38.2 ± 11.3) were listed for heart transplantation (HTX). Two of these underwent HTX, one died for refractory heart failure (HF) and one is still on the waiting list. ConclusionsThere might be a conceivable benefit from an extended use of the S-ICD in selected patients with CHD, especially in those with life-treating ventricular arrhythmias and complex anatomy or patients awaiting HTX. S-ICD appears to be a safe alternative to a transvenous system when a transvenous ICD cannot be implanted and pacing is not required.

Tuning and external validation of an adult congenital heart disease risk prediction model.

AimsAdequate risk prediction can optimize the clinical management in adult congenital heart disease (ACHD). We aimed to update and subsequently validate a previously developed ACHD risk prediction model.Methods and resultsA prediction model was developed in a prospective cohort study including 602 moderately or severely complex ACHD patients, enrolled as outpatients at a tertiary centre in the Netherlands (2011–2013). Multivariable Cox regression was used to develop a model for predicting the 1-year risks of death, heart failure (HF), or arrhythmia (primary endpoint). The Boston ACHD Biobank study, a prospectively enrolled cohort (n = 749) of outpatients who visited a referral centre in Boston (2012–2017), was used for external validation. The primary endpoint occurred in 153 (26%) and 191 (28%) patients in the derivation and validation cohorts over median follow-up of 5.6 and 2.3 years, respectively. The final model included 5 out of 14 pre-specified predictors with the following hazard ratios; New York Heart Association class ≥II: 1.92 [95% confidence interval (CI) 1.28–2.90], cardiac medication 2.52 (95% CI 1.72–3.69), ≥1 reintervention after initial repair: 1.56 (95% CI 1.09–2.22), body mass index: 1.04 (95% CI 1.01–1.07), log2 N-terminal pro B-type natriuretic peptide (pmol/L): 1.48 (95% CI 1.32–1.65). At external validation, the model showed good discrimination (C-statistic 0.79, 95% CI 0.74–0.83) and excellent calibration (calibration-in-the-large = −0.002; calibration slope = 0.99).ConclusionThese data support the validity and applicability of a parsimonious ACHD risk model based on five readily available clinical variables to accurately predict the 1-year risk of death, HF, or arrhythmia. This risk tool may help guide appropriate care for moderately or severely complex ACHD.

CT and MRI for Repaired Complex Adult Congenital Heart Diseases.

An increasing number of adult congenital heart disease (ACHD) patients continue to require life-long diagnostic imaging surveillance using cardiac CT and MRI. These patients typically exhibit a large spectrum of unique anatomical and functional changes resulting from either single- or multi-stage palliation and surgical correction. Radiologists involved in the diagnostic task of monitoring treatment effects and detecting potential complications should be familiar with common cardiac CT and MRI findings observed in patients with repaired complex ACHD. This review article highlights the contemporary role of CT and MRI in three commonly encountered repaired ACHD: repaired tetralogy of Fallot, transposition of the great arteries after arterial switch operation, and functional single ventricle after Fontan operation.

Reconstruction of right ventricular outflow tract stenosis and right ventricular failure after Ross procedure \u2013 comprehensive assessment of adult congenital heart disease with four-dimensional imaging: a case report

BackgroundRe-intervention after Ross procedure into the right ventricular outflow tract might be needed in patients in the long term. However, right ventricular outflow tract re-intervention indications are still unclear. Comprehensive assessment of total hemodynamics is needed.Case summaryA 42-year-old Japanese woman was referred to our hospital for moderately severe pulmonary regurgitation and severe tricuspid regurgitation after a Ross–Konno procedure. Thirteen years after surgery, she developed atrial fibrillation and atrial flutter and complained of dyspnea. Electrophysiological studies showed re-entry circuit around the low voltage area of the lateral wall on the right atrium. Four-dimensional flow magnetic resonance imaging revealed moderate pulmonary regurgitation, severe tricuspid regurgitation, and a dilated right ventricle. Flow energy loss in right ventricle calculated from four-dimensional flow magnetic resonance imaging was five times higher than in normal controls, suggesting an overload of the right-sided heart system. Her left ventricular ejection fraction was almost preserved. Moreover, the total left interventricular pressure difference, which shows diastolic function, revealed that her sucking force in left ventricle was preserved. After the comprehensive assessments, we performed right ventricular outflow tract reconstruction, tricuspid valve annuloplasty, and right-side Maze procedure. A permanent pacemaker with a single atrial lead was implanted 14 days postoperatively. She was discharged 27 days postoperatively. Echocardiography performed 3 months later showed that the size of the dilated right ventricle had significantly reduced.DiscussionA four-dimensional imaging tool can be useful in the decision of re-operation in patients with complex adult congenital heart disease. The optimal timing of surgery should be considered comprehensively.

Surgical outcomes in complex adult congenital heart disease: a brief review.

Life expectancy of patients with congenital heart disease (CHD) continues to increase dramatically over the last few decades, primarily due to significant medical and surgical advancements in the field. Adult congenital heart disease (ACHD) patients are now living longer but continue to face morbidity and reduced long-term survival when compared to the general population. We briefly describe the growth of ACHD with a focus on surgical procedures and outcomes in the more complex lesions including Tetralogy of Fallot, Ebsteins Anomaly of the tricuspid valve, Coarctation of the Aorta, Transposition of the Great Arteries and single ventricle lesions. The advancing role of mechanical circulatory support and cardiac transplantation in ACHD patients is also highlighted. Cardiac surgery in these patients continues to improve with low surgical mortality rate and outstanding long-term outcomes and efficacy.

155: Hospital admissions 2 to 9 months after delivery among women with adult congenital heart disease

Women with adult congenital heart disease (ACHD) are at increased risk of postpartum readmissions. However, longer term risk for hospitalizations is not well characterized. This analysis sought to characterize risk for hospital admissions 2 to 9 months after delivery hospitalization for women with ACHD. Delivery hospitalizations for women with ACHD (categorized into complex, non-complex, and unspecified ACHD) were abstracted from the 2010-2014 Nationwide Readmissions Database with follow up through 9 months after delivery hospitalization discharge. Multivariable log linear regression models were fit for the outcome of readmission from 2 to 9 months following delivery adjusting for type of ACHD, patient, obstetric, and other underlying cardiac factors. We identified 3,507 deliver hospitalizations occurring between January-March of 2010-2014 with an all-cause hospitalization rate of 7.9% in the first 9 months after delivery. 44% of readmissions occurred within 60 days after delivery hospitalization discharge with the majority (56%) occurring 2 to 9 months after discharge (Figure 1). The median time to readmission was 72 days. Among readmissions from 2 to 9 months after discharge, patients with complex ACHD were at 64% higher risk of readmission (RR 1.64, 95% CI 1.01, 2.67) while factors like congestive heart failure, arrhythmias, and obstetrical factors were not associated with elevated risk (Table 1). The majority of ACHD hospitalizations in the 9 months after delivery occur after 2 months postpartum. These findings support the importance of health care coverage and continuity for this high-risk population.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Ultrahigh-density mapping supplemented with global chamber activation identifies noncavotricuspid-dependent intra-atrial re-entry conduction isthmuses in adult congenital heart disease.

To evaluate the role of ultrahigh-density mapping for conduction isthmus (CI) characterization in adult congenital heart disease (ACHD). Catheter ablation remains suboptimal for ACHD with atypical intra-atrial reentrant tachycardias (IART) that can be challenging to define using existing mapping technology. An ultrahigh-density mapping system was selectively employed over a 1-year period for procedures involving noncavotricuspid isthmus-dependent-IART. A global activation histogram (GAH) was assessed for the ability to predict ablation targets. Procedural characteristics were compared to a group of matched controls. Twenty patients (mean age 43 ± 15, 70% male) underwent 20 procedures targeting 34 tachycardias during the study period. Diagnoses included single ventricle (8), tetralogy of Fallot (2), left heart obstruction (3), Ebstein's anomaly (2) atrial septal defect (2), Mustard operation (2), and Rasteilli operation (1). Prior catheter ablation/Maze operation had been performed in 12 (60%). The median time per map was 21 minutes (interquartile range [IQR], 16-32), representing 14 834 points per map (IQR 9499-43 191; P < .001 vs controls). Review of GAH maps showed lower trough values were associated with more favorable IART CI characteristics (P ≤ =.001 for all). Acute success was achieved in 19/20 (95%) procedures, with tachycardia termination during the first lesion in eight cases (P = .02 vs controls). There was one recurrence during 0.6 years follow-up. Ultrahigh-density mapping supplemented with the GAH tool was effective for CI identification in a cohort of complex ACHD patients. Catheter ablation was more efficient compared to controls, suggesting precise CI characterization using this technology.

Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population.

Patients with adult congenital heart disease (ACHD) have an increased risk of arrhythmic and sudden cardiac death. The subcutaneous implantable cardioverter defibrillator (S-ICD) provides a potentially safer alternative to transvenous ICDs in ACHD. Suitability for S-ICD depends on the surface electrocardiogram (ECG), which is often abnormal in ACHD patients. This study investigates the proportion of ACHD patients who meet the screening criteria for S-ICD implantation. A standard screening ECG was performed in 102 patients with complex ACHD (Tetralogy of Fallot, Fontan circulation, and transposition of the great arteries). This process was repeated postexercise for patients who also had an exercise test. Three quarters (75.4%) of ACHD patients meet screening criteria for an S-ICD with at least one suitable vector. The most common number of acceptable vectors in the eligible group was two (35% of total population). In only 12% of total population, all three vectors were suitable while only one vector was suitable in 28% of total population. The primary vector (equivalent of ECG lead III) was the most common suitable vector, found in 62% of participants who had appropriate sensing vectors. Twenty-five (24.5%) patients failed to meet the S-ICD screening criteria. Of these, 14 had repaired tetralogy of Fallot. A total of 92% of patients with a Fontan circulation met ECG screening criteria. Of those who had the protocol repeated following their cardiopulmonary exercise test (n=14), only one additional patient failed to meet the eligibility criteria. A quarter of ACHD patients do not meet the eligibility criteria for the S-ICD. However, more than 90% of patients with a Fontan circulation are suitable for an S-ICD.

Adverse effects of amiodarone therapy in adults with congenital heart disease.

Amiodarone is a highly effective antiarrhythmic therapy, however its toxicity profile often limits treatment. This is particularly relevant in adults with congenital heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD). A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling the clinical arrhythmia was assessed as complete, partial, or failed. Amiodarone was prescribed in 57 patients of 902 ACHD patients reviewed (6%), for a mean duration of 2.7±4.3years. Significant adverse effects occurred in 56%, most commonly thyroid dysfunction, with amiodarone-induced thyrotoxicosis (AIT) in 30% and amiodarone-induced hypothyroidism in 14%. AIT frequently led to arrhythmia exacerbation and occurred most in those with Fontan anatomy. Severe dermatological effects were seen in 7% and bradycardia requiring pacing in 5%. Interstitial lung disease, peripheral neuropathy and alopecia were observed in single cases. Amiodarone toxicity led to discontinuation of the drug in 42%. Amiodarone was highly effective when tolerated, however, achieving complete arrhythmia control in 63%, partial control in 35%, with failure to control in only one patient. Amiodarone therapy is effective in moderate to complex ACHD patients, but is frequently limited by adverse effects. ACHD patients seem especially vulnerable to thyroid dysfunction, with Fontan patients in particular at increased risk of AIT.

Analysis of surgical treatment of complex adult congenital heart disease: five years' outcomes from a single center

Analysis of surgical treatment of complex adult congenital heart disease: five years' outcomes from a single center

Adverse Effects of Amiodarone Therapy in Adults With Congenital Heart Disease

Objective Amiodarone is a highly effective antiarrhythmic therapy, however its toxicity profile often limits treatment. This is particularly relevant in adults with congenital heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD). Design A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling the clinical arrhythmia was assessed as complete, partial, or failed. Results Amiodarone was prescribed in 57 patients of 902 ACHD patients reviewed (6%), for a mean duration of 2.7 ± 4.3 years. Significant adverse effects occurred in 56%, most commonly thyroid dysfunction, with amiodarone-induced thyrotoxicosis (AIT) in 30% and amiodarone-induced hypothyroidism in 14%. AIT frequently led to arrhythmia exacerbation and occurred most in those with Fontan anatomy. Severe dermatological effects were seen in 7% and bradycardia requiring pacing in 5%. Interstitial lung disease, peripheral neuropathy and alopecia were observed in single cases. Amiodarone toxicity led to discontinuation of the drug in 42%. Amiodarone was highly effective when tolerated, however, achieving complete arrhythmia control in 63%, partial control in 35%, with failure to control in only one patient. Conclusions Amiodarone therapy is effective in moderate to complex ACHD patients, but is frequently limited by adverse effects. ACHD patients seem especially vulnerable to thyroid dysfunction, with Fontan patients in particular at increased risk of AIT.

Employment characteristics of a complex adult congenital heart disease cohort.

Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.

Trends in the Burden of Adult Congenital Heart Disease in US Emergency Departments.

We assessed trends in incidence, in-hospital mortality, and admission among patients with adult congenital heart disease (ACHD) presenting to the emergency department (ED) from 2006 to 2012. There is a considerable burden of ACHD in the US EDs. We used the 2006-2012 US Nationwide Emergency Department Sample. All ED visits with ACHD were identified using standard International Classification of Diseases, Ninth Edition, Clinical Modification codes. The number of patients presenting to the ED with simple (40.6%) as well as complex (37.6%) ACHD across 2006-2012 increased significantly. Also, there was a considerable increase in prevalence of traditional cardiovascular risk factors among ACHD patients, including hypertension, diabetes, smoking, obesity, and chronic kidney disease. Besides miscellaneous noncardiovascular conditions, nonspecific chest pain (15.9%) and respiratory disorders (15.0%) were the most common reasons for ED visits among patients with simple and complex ACHD, respectively. Although there was a trend toward decrease in admissions across 2006-2012 (Ptrend < 0.001), the proportion of patients with ACHD presenting to ED requiring admission remained substantial (63.4%). Finally, there was significant variation in admission trends across different geographic locations, hospital types, insurance status, and ED volume among ACHD patients presenting to the ED. There has been a progressive increase in number of ED visits among ACHD patients across 2006-2012 in the United States. Moreover, the cardiovascular risk-factor profile of ACHD patients has changed, adding to complexity in management. Current health care delivery to ACHD patients also shows significant geographical, hospital-based, and insurance status-based disparities.

Left and Right Parasternal Sensing for the S-ICD in Adult Congenital Heart Disease Patients and Normal Controls.

This study investigated the impact of a right parasternal sensing electrode position on the R- and T-wave amplitudes and the R:T ratio in three subcutaneous implantable cardioverter defibrillator (S-ICD) vectors in patients with adult congenital heart disease (ACHD) and normal controls. Conventional left parasternal sensing electrode position and right parasternal sensing electrode positions were used to collect 10-second electrograms, recorded through an 80-electrode body surface mapping technology (Prime ECG™ system, Heartscape Technologies Inc., now Verathon, Columbia, MD, USA). Recordings were made in the supine, prone, left lateral, right lateral, sitting, and standing positions in using both the standard electrode vector position and the right parasternal positions. Forty patients were recruited and 37 patients were used for analysis. Twenty-seven (73%) had complex ACHD; 10 patients had normal hearts and acted as controls. A total of 3,708 data points were analyzed. There were no significant differences in the R:T ratio when measured in ACHD patients in the right compared to the left parasternal lead position. In contrast, there were important differences in the magnitude of the R:T ratio when measured in control patients in the right compared to the left parasternal lead position; in the primary vector, the R:T ratio was greater in right than left by 2.99 (P = 0.0002; 95% confidence interval [CI]: 1.48-4.50) and in the secondary vector, the R:T ratio was smaller in the right than in the left by 0.77 (P = 0.004; 95% CI: -1.58-0.05). In selected patients, a right parasternal lead position may provide a useful alternative sensing configuration for the S-ICD.

Abstract 18744: Rising Costs of Adult Congenital Heart Disease Care: Does Gender Matter?

Introduction: The profile of congenital heart disease (CHD) has shifted, now with more adults than children who have survived. Few studies have provided a global assessment of adult congenital heart disease (ACHD) healthcare cost in the United States. Methods: Data from the National Inpatient Sample (2002-2012) utilizing diagnostic ICD-9 codes for moderate and complex ACHD were analyzed. Hospital discharges, total billed and reimbursed amounts, gender and age disparities were evaluated. Results: There was an overall increase in ACHD discharges (Moderate CHD: 4,742 vs. 6,545, Severe CHD: 807 vs. 1,115), billed and reimbursed dollar amounts (Billed: $542,703,961 vs. $1,506,945,042, +178%; reimbursed: $221,417,779 vs. $432,797,543, +95%). Women had more discharges in 2002 but not in 2012 (men: women, 2002: 6,503 vs. 7,805; 2012: 7,715 vs. 7,200) [Figure 1A]. Gender-based billed amounts followed a similar trend (2002: $262,918,357 vs. $279,785,604; 2012: $844,923,857 vs. $$662,021,185; p=0.006) as did total reimbursed (2002: $107,766,175 vs. $113,651,604; 2012: $243,183,638 vs. $189,613,905, p=0.008) [Figure 1B,C]. Healthcare expenditure increased across all age groups: this was most prominent in the &gt; 44 vs. 18-44 yr. group (Billed: $617,589,813 vs. $346,652,267, p&lt;0.001; reimbursed: $136,013,528 vs. $75,366,237, p&lt;0.001). Conclusions: We are the first to report a change in the rate of gender-based ACHD hospitalizations, whereby men now account for more hospitalizations in the U.S. As ACHD discharges, billed and reimbursed amounts continue to rise over the last decade, we are also the first to demonstrate increased expenditure in older (&gt; 44 yrs.) ACHD patients, a pattern that we predict will continue to grow and requires future investigation.

Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre.

Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. We included 6969 adult patients (age 29.9 ± 15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased. ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.

Abstract 19314: Impact of Body Mass Index on Clinical Outcomes in Complex Adult Congenital Heart Disease

Background: Obesity can adversely affect most organ systems and increases the risk of comorbidities likely to be of consequence for patients with complex adult congenital heart disease (ACHD). Conversely, several studies have demonstrated that low body mass index (BMI) is a risk factor for heart failure and adverse outcomes after cardiac surgery. However, there are currently no data regarding the impact of BMI in ACHD. Methods: We examined the charts of 87 randomly selected, complex ACHD patients whose first visit to our institution was at 18-22 years old. Patients were categorized according to BMI at initial visit: underweight (BMI &lt; 18.5 kg/m 2 ), normal (BMI 18.5 - 24.9 kg/m 2 ), overweight/obese (BMI ≥ 25 kg/m 2 ). Events occurring during follow-up were recorded. Data was censured on 1/1/2014. Cardiac events were defined as a composite of cardiac death, heart transplantation or admission for heart failure. Results: The cohort included patients with the following diagnoses: tetralogy of Fallot n=31, Mustard n=28, Fontan n=17, ccTGA n=9 and aortic coarctation n=2. The median (IQR) duration of follow-up was 8.7 (4.2 - 1.8) years. See table for distribution and outcomes by BMI category. Cardiac events occurred in 17/87 patients. After adjustment for age, sex, and underlying disease, the underweight group had increased risk of cardiac events (HR=12.9, 95% CI: 2.8-61.5, p &lt; 0.05). Kaplan-Meier curves demonstrate the poorer prognosis of underweight patients (Figure). Conclusions: Underweight was associated with increased risk of late cardiac events in ACHD patients. We were unable to demonstrate significant overweight/obesity impact.

Complex adult congenital heart disease is associated with impaired skeletal muscle function

Purpose: Complex congenital heart disease is often associatedwith impaired physical functioning, usually measured as peak oxygen uptake in an exercise test. Skeletal muscle function is, however, le ...

Abstract 19748: Predicting Outcome in Adult Congenital Heart Disease: Correlating the Seattle Heart Failure Model with peak VO2 from Cardiopulmonary Exercise Testing

Background : The adult congenital heart disease (ACHD) population continues to grow and has increased risk of mortality and morbidity, including heart failure. Currently, no model exists to identify high risk patients. Cardiopulmonary exercise testing (CPET) is used in risk stratification with peak VO2 (pVO2) as a surrogate marker for outcomes in ACHD. This measurement requires specific equipment and expertise, not widely available outside of tertiary centers. The Seattle Heart Failure Model (SHFM) is a validated prediction model that estimates total mortality in patients with heart failure by using commonly obtained clinical, laboratory, medication, and device variables. Hypothesis : The SHFM predicts CPET pVO2 in moderate and complex ACHD. Methods : We performed a retrospective IRB approved study of 106 individuals (&gt;18 years of age) with transposition of the great arteries (TGA), Ebstein anomaly, tetralogy of Fallot (TOF), double outlet right ventricle (DORV) and single ventricle (SV) with a clinically indicated CPET at the Massachusetts General Hospital (MA, USA) and Montefiore Medical Center (NY, USA) between 2008 and 2012. Clinical/laboratory data (within one year of CPET) were obtained for SHFM calculation. Results : Mean age was 35.6 years (range 18-64), 55.7% are female, average systemic ventricle ejection fraction was 56.3% (18-83%). Medication use: 9.4% of individuals on ACE-I/ARB, 34% beta-blockers, 23.6% diuretics. The mean SHFM 1 year survival was 93.9% (72.5-99.0%); mean pVO2 was 21.2 cc/kg/min (10.2-41.6). The SHFM 5-year model was able to predict an outcome of pVO2&lt;20 in the group as a whole with a C statistic of 0.69. In individuals with TGA (systemic right ventricle) and Ebstein, the predictive ability of the SHFM 5-year model had a C statistic of 0.86 and 0.87, respectively. Peak VO2 and SHFM predicted survival correlated strongly among TGA and Ebstein patients (r=0.65 and 0.57, p &lt; 0.03), but not among SV or TOF patients (r.= -0.02 and 0.12, p &gt; 0.4). Conclusion : The SHFM is able to offer an increased ability to discriminate among ACHD patients who have a pVO2 &lt; or &gt; 20cc/kg/min. This model is easily created using clinically obtained data. Targeted application of SHFM in the community may have predictive value and guide care in select populations.

Single ventricle, bicuspid aorta and interatrial wall aneurysm as a rare complex adult congenital heart disease: a case report

BackgroundSingle ventricle, bicuspid aortic valve and interatrial wall aneurysm in adulthood are a rare and unique case in medical literature. This presented case with congenital heart disease has never been treated surgically and clinical consequences seriously presented in adulthood.Case presentationA 27 year old man with complex congenital heart disease presented. At the age of six, the single ventricle was ultrasonographly diagnosed, but at age 27 clinical consequences started to be seriously present. We explored his history, clinical course, physical examination, laboratory findings, medical treatments and actual patient condition.ConclusionThe possibilities for surgical evaluation are presented.