Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease.

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

Higher Cost of Hospitalizations for Non-cardiac Diagnoses in Adults with Congenital Heart Disease.

Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD.

Hepatocellular carcinoma in the adult Fontan patient.

In this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.

Complex Congenital Heart Disease and Congenital Uterine Anomalies Impacting Pregnancy Outcomes.

Complex congenital heart disease is often accompanied by extracardiac manifestations; , the significance of genitourinary involvement remains unclear. We present 3 patients with palliated complex congenital heart disease and with pregnancies complicated by premature delivery who were found to have congenital uterine anomalies that may have contributed to their obstetrical complications.

Feasibility and Findings of Including Self-Identified Adult Congenital Heart Disease Patients in the INVESTED Trial.

Background: Adult congenital heart disease (ACHD) patients have significant morbidity and rise in cardiac admissions. Their outcome with high-dose influenza vaccination is unknown in comparison to those without ACHD. Objectives: The purpose of this study was to compare all-cause mortality or cardiopulmonary hospitalizations in self-identified ACHD versus non-ACHD patients receiving high- or low-dose influenza vaccination within the INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure trial. Methods: We prospectively included ACHD patients in the INVESTED (INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure) trial. The primary endpoint was all-cause death or hospitalization for cardiovascular or pulmonary causes. Results: Of the 272 ACHD patients, 132 were randomly assigned to receive high-dose trivalent and 140 to standard-dose quadrivalent influenza vaccine. Compared to the non-ACHD cohort (n = 4,988), ACHD patients were more likely to be younger, women, smokers, have atrial fibrillation, and have a qualifying event of heart failure. The primary outcome was 49.8 events versus 42.8 events per 100 person-years (adjusted HR: 1.17; 95% CI: 0.95-1.45; P = 0.144) in the ACHD group and non-ACHD group, respectively. The interaction between ACHD status and randomized treatment effect was not significant for the primary outcome (P = 0.858). Vaccine-related adverse events were similar in both groups. Conclusions: Patients who self-identify as being ACHD had similar primary outcome of all-cause death or hospitalization for cardiovascular or pulmonary causes compared to non-ACHD cohort. High-dose influenza vaccination was similar to standard-dose influenza vaccination on the primary outcome in patients who self-identify as ACHD.

Unicommissural Unicuspid Aortic Valve, Ascending Aortic Aneurysm, and Bovine Arch: A Rare Case Report.

A 24-year-old man presented with rapidly progressive dyspnea due to mixed aortic stenosis and insufficiency. Unicommissural unicuspid aortic valve, ascending aortic aneurysm, and a bovine arch were identified on computed tomography angiography. Uncomplicated surgical mechanical valve replacement and ascending aortic graft placement improved his symptoms. Aortopathy is common in unicuspid valve patients.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Melody Transcatheter Pulmonary Valve Placement in a HeartMate II Patient.

Transcatheter pulmonary valve placement is emerging as a standard therapy for dysfunctional right ventricular outflow tract conduits. The Melody transcatheter pulmonary valve is indicated for use in the management of pediatric and adult patients with right ventricular outflow tract conduits measuring at least 16 mm in diameter. This is the first reported case of placement in a patient with a left ventricular assist device. We outline the preprocedural evaluation process, the procedural methods, and the outcomes of a successful implantation in a complex patient. With a team-based approach including thoughtful preprocedural evaluation, and close monitoring, successful deployment of a transcatheter pulmonary valve is possible in complex patients in the setting of mechanical circulatory support.

Acute Coronary Syndrome and Ischemic Heart Disease in Pregnancy: Data From the EURObservational Research Programme-European Society of Cardiology Registry of Pregnancy and Cardiac Disease.

Background The prevalence of ischemic heart disease (IHD) in women of child-bearing age is rising. Data on pregnancies however are scarce. The objective is to describe the pregnancy outcomes in these women. Methods and Results The European Society of Cardiology-EURObservational Research Programme ROPAC (Registry of Pregnancy and Cardiac Disease) is a prospective registry in which data on pregnancies in women with heart disease were collected from 138 centers in 53 countries. Pregnant women with preexistent and pregnancy-onset IHD were included. Primary end point were maternal cardiac events. Secondary end points were obstetric and fetal complications. There were 117 women with IHD, of which 104 had preexisting IHD. Median age was 35.5 years and 17.1% of women were smoking. There was no maternal mortality, heart failure occurred in 5 pregnancies (4.8%). Of the 104 women with preexisting IHD, 11 women suffered from acute coronary syndrome during pregnancy. ST-segment‒elevation myocardial infarction were more common than non‒ST-segment‒elevation myocardial infarction, and atherosclerosis was the most common etiology. Women who had undergone revascularization before pregnancy did not have less events than women who had not. There were 13 women with pregnancy-onset IHD, in whom non‒ST-segment‒elevation myocardial infarction was the most common. Smoking during pregnancy was associated with acute coronary syndrome. Caesarean section was the primary mode of delivery (55.8% in preexisting IHD, 84.6% in pregnancy-onset IHD) and there were high rates of preterm births (20.2% and 38.5%, respectively). Conclusions Women with IHD tolerate pregnancy relatively well, however there is a high rate of ischemic events and these women should therefore be considered moderate- to high-risk. Ongoing cigarette smoking is associated with acute coronary syndrome during pregnancy.

Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

BACKGROUND: Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels. METHODS: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status. RESULTS: There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana. CONCLUSIONS: The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.

Long-term Outcomes of the Arterial Switch Operation for d-Transposition of the Great Arteries.

Dextrotransposition of the great arteries (d-TGA) is a relatively rare form of complex childhood congenital heart disease, which occurs in approximately 0.2 in 1000 live births (Long et al, 2010). The most common palliative procedure for this anatomy has become the arterial switch operation (ASO). We will review in this paper the evidence that is currently available regarding the clinical management following the ASO. Individuals with d-TGA who undergo ASO at a young age thus far have excellent long-term outcomes. Long-term complications for the ASO should be monitored for and patients should have routine follow-up with specialists in adult congenital heart disease.

Long Term Consequences of the Fontan Procedure and How to Manage Them.

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.

Atrial Fibrillation in Patients with Congenital Heart Disease.

Advances in surgical techniques have led to the survival of most patients with congenital heart disease (CHD) up to their adulthood. During their lifetime, many of them develop atrial tachyarrhythmias due to atrial dilatation and scarring from surgical procedures. More complex defects and palliative repairs are linked to a higher incidence and earlier occurrence of arrhythmias. Atrial fibrillation (AF) is common in patients who have atrial septal defects repaired after age 55 and in patients with tetralogy of Fallot repaired after age 45. Patients with dextrotransposition of the great arteries who undergo Mustard or Senning atrial switch procedures have an increased risk of atrial flutter due to atrial baffle suture lines. Patients with Ebstein's anomaly are also prone to supraventricular tachycardias caused by accessory bypass tracts. Patients with a single ventricle who undergo Fontan palliation are at risk of developing persistent or permanent AF due to extreme atrial enlargement and hypertrophy. In addition, obtaining vascular access to the pulmonary venous atrium can present unique challenges during radiofrequency ablation for patients with a Fontan palliation. Patients with cyanotic CHD who develop AF have substantial morbidity because of limited hemodynamic reserve and a high viscosity state. Amiodarone is an effective therapy for patients with arrhythmias from CHD, but its use carries long-term risks for toxicity. Dofetilide and sotalol have good short-term effectiveness and are reasonable alternatives to amiodarone. Pulmonary vein isolation is associated with better outcomes in patients taking antiarrhythmic medications. Anticoagulants are challenging to prescribe for patients with CHD because of a lack of data that can be extrapolated to this patient population. Surgical ablation is the gold standard for invasive rhythm control in patients with CHD and should be considered at the time of surgical repair or revision of congenital heart defects. When possible, patients with complex CHD should be referred for care to an adult congenital heart disease center of excellence.