Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

Consecutive percutaneous valve-in-valve replacement late after Ross procedure: A novel approach in an adult with congenital heart disease.

The emergence of transcatheter valve technology over the last decade has made significant impact on the treatment of patients with valvular heart disease. There has been increasing experience with both native and valve-in-valve indications with promising results. We present the case of a young woman with congenital heart disease who underwent the Ross procedure for bicuspid aortic valve endocarditis with subsequent reoperation and surgical aortic valve replacement for neo-aortic root dilation who experienced worsening symptoms related to both pulmonary and aortic valve dysfunction. She was successfully treated with percutaneous pulmonary and aortic valve replacement with excellent early term technical results and marked improvement in symptoms.

GUCH/ACHD: what is required of the newest field in medicine?

Cardiologists from the USA provide information from earlier experiences in this emerging specialty.

Aortic root dilatation in adults with surgically repaired tetralogy of fallot: a multicenter cross-sectional study.

BACKGROUND: Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS: A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS: Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

A sinus venosus atrial septal defect is diagnosed by echocardiography with an unusual bubble study.

A 68-year-old man underwent echocardiogram with agitated saline for a presumed diagnosis of primary pulmonary hypertension. Surprisingly, the bubbles from the agitated saline enter the left heart before filling the right side, leading to a diagnosis of Eisenmeger's syndrome from a sinus venosus atrial septal defect. Because of high right-sided pressure, the bubbles preferentially travel from the superior vena cava through the defect to the right superior pulmonary vein and left atrium, rather than the right side. This diagnosis was later confirmed on cardiac MRI.

Heart rate response during exercise and pregnancy outcome in women with congenital heart disease.

BACKGROUND: Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined. METHODS AND RESULTS: We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome. CONCLUSIONS: Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

Patients with bicuspid aortic valves may be associated with infra-hisian conduction disease requiring pacemakers.

BACKGROUND: Bicuspid aortic valves (BAVs) are associated with accelerated valvular dysfunction. Increasing rates of conduction system disease are seen in patients with calcific tricuspid aortic valves (TAVs). However, little is known regarding the extent of conduction disorders in BAV patients. We sought to determine the extent of infra-hisian conduction pathology among patients with BAVs undergoing EP studies. METHODS: We prospectively analyzed patients presenting to the EP laboratory from 2006 to 2017 at our institution. Thirty-three BAV patients had measured HV intervals. Each individual was matched by age and gender to two control patients. Clinical characteristics were collected and compared, and patients followed for outcomes. RESULTS: The BAV cohort had a mean age of 47.8 ± 17.2 years (range 19-76 years). Indications for referral to the EP lab in the BAV cohort included SVT ablation (n = 16), VT ablation (n = 10), and EP study for syncope, pre-syncope, or palpitations (n = 29). Patients with BAVs had a mean HV interval of 58.7 ms ± 18.6 ms, compared to a mean of 47.2 ms ± 9.6 ms for controls (p value = 0.0001). Over a 10-year follow-up period, 9 BAV patients (27%) went on to require permanent pacing compared to 6 patients (9%) in the control group (p value = 0.03). CONCLUSION: Compared to patients with TAVs presenting for EP evaluation, individuals with BAVs have longer HV intervals and a significantly increased requirement for pacemaker therapy over long-term follow-up. Closer monitoring of progressive conduction system disease in BAV patients may be warranted.

Prevalence of Mental Illness in Adolescents and Adults With Congenital Heart Disease from the Colorado Congenital Heart Defect Surveillance System.

The aim of this study was to estimate the prevalence of the full spectrum of mental illness in adolescents (aged 11 to 17) and adults (aged 18 to 64) with congenital heart defects (CHDs) in the population-level Colorado Congenital Heart Disease Surveillance System. Further we sought to investigate whether severity of the defect, frequency of recent cardiac procedures or underlying genetic disorders influence these estimates. The cohort included patients in clinical care for CHDs between January 1, 2011 and December 31, 2013, identified across multiple healthcare systems and insurance claims. Of 2,192 adolescents with CHDs, 20% were diagnosed with a mental illness with the most prevalent categories being developmental disorders (8%), anxiety disorders (6%), attention, conduct, behavior, impulse control disorders (6%), and mood disorders (5%). Of 6,924 adults with CHDs, 33% were diagnosed with a mental illness with the most prevalent categories being mood disorders (13%), anxiety disorders (13%), and substance-related disorders (6%). Greater lesion complexity was associated with a higher likelihood of anxiety and developmental disorders in both adolescents and adults. Adolescents and adults who had ≥2 cardiac procedures in the 3-year surveillance period had a 3- and 4.5-fold higher likelihood of a mental illness diagnosis, respectively, compared with those who had fewer than 2 cardiac procedures. Finally, patients with a genetic syndrome were more likely to have a mental illness diagnosis. In conclusion, mental illness is a prevalent co-morbidity in the adolescent and adult population with CHDs, thus comprehensive care should include mental health care.

Safety and outcomes of catheter ablation for atrial fibrillation in adults with congenital heart disease: A multicenter registry study.

BACKGROUND: An increasing number of adults with congenital heart disease (CHD) are undergoing catheter ablation for atrial fibrillation (AF). Data on ablation strategy and outcomes in CHD are limited. Rhythm control is often believed to be of greater importance among patients with complex CHD. OBJECTIVE: The purpose of this study was to examine the safety and efficacy of AF ablation in adult patients with CHD. METHODS: A multicenter retrospective analysis was performed of CHD patients undergoing AF ablation. Clinical data were collected, including AF and CHD type, procedural data, and outcomes. Patients were divided into 3 groups (simple, moderate, and severe) based on CHD complexity, as defined by the 2014 PACES/HRS (Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society) consensus statement. One-year procedural success was defined as freedom from recurrent AF, off antiarrhythmic drugs (complete) or off/on previously failed antiarrhythmic drugs (partial). RESULTS: Overall, 84 CHD patients (mean age 51.5 ± 12.1 years; 65.5% male; 45.2% with paroxysmal AF) undergoing AF ablation (51 simple, 22 moderate, 11 severe complexity) were included. Pulmonary vein isolation was performed in 80 (95.2%), of whom 30 (35.7%) underwent pulmonary vein isolation alone. Overall, complete and complete/partial freedom was achieved at 1 year in 53.1% and 71.6%, respectively, with no significant differences between those with simple, moderate, or severe complexity. There were no major complications and 7 minor complications, and 2 patients died during follow-up. CONCLUSION: There are dramatic differences in the degree of CHD complexity among patients referred for AF ablation. When performed at experienced centers, AF ablation is safe and effective even among patients with the most complex forms of CHD.

Perioperative electrophysiology study in patients with tetralogy of Fallot undergoing pulmonary valve replacement will identify those at high risk of subsequent ventricular tachycardia.

BACKGROUND: Ventricular tachyarrhythmias are the most common cause of death in patients with repaired tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. An electrophysiology study (EPS) has been proposed to risk stratify patients with TOF. OBJECTIVE: We sought to evaluate a perioperative EPS-guided approach to risk stratify patients with TOF undergoing pulmonary valve replacement (PVR) and guide concomitant cryoablation. METHODS: A prospective cohort study of patients with TOF undergoing an EPS at the time of PVR from 2006 to 2017 was conducted at 2 centers. Patients inducible at the time of pre-PVR had undergone concomitant cryoablation in addition to PVR. A repeat post-PVR EPS was performed in those initially inducible to guide implantable cardioverter-defibrillator (ICD) implantation. RESULTS: Of 70 patients who underwent a pre-PVR EPS, 34 (49%) had inducible sustained ventricular tachycardia (VT): 25 monomorphic VT and 9 polymorphic VT. Among patients undergoing cryoablation, 14 (45%) had inducible VT and underwent ICD implantation. During a mean follow-up period of 6.1 ± 3.2 years, 3 patients (21%) had appropriate ICD shocks for symptomatic VT. There was an average of 2.3 shocks (range 1-4 shocks), and the mean time to first shock post-device implantation was 3.6 years (range 2.9-4.3 years). Among patients with negative pre- or post-PVR EPS results, 2 had VT requiring radiofrequency ablation and/or subsequent ICD implantation. There were no arrhythmic deaths. CONCLUSION: A pre-PVR EPS identified patients with higher-risk TOF undergoing PVR. Despite empirical VT cryoablation at the time of PVR, a high percentage of patients remained inducible for VT. In this high-risk cohort, post-PVR EPS evaluation is important to identify patients at risk of VT despite cryoablation.

Racial differences are seen in blood pressure response to fosinopril in hypertensive children.

BACKGROUND: Few antihypertensive therapies have been systematically studied in children and dosages for many agents are either extrapolated from adult studies or obtained from small homogenous pediatric populations. It is well established that adult patients of different races show disparate response to angiotensin-converting enzyme (ACE) inhibitors, however no such studies have been performed in children. METHODS: Two hundred fifty three children ages 6-16 with hypertension or with high normal blood pressure with an associated medical condition requiring antihypertensive therapy were enrolled at 78 clinical sites in the US, Russia, and Israel in a double blind study to evaluate the efficacy of fosinopril compared to placebo. RESULTS: The racial composition of the cohort included 60.1% white (152/253), 20.6% black (52/253), 13.8% Hispanic (35/253), 2.0% Asian (5/253), 0.4% Native American (1/253), and 3.2% (8/253) children classified as other or of mixed race. After adjusting for baseline blood pressure and body surface area (BSA) there was no significant dose response seen in non-black patients. Non-blacks randomized to the low, medium, and high dosages of fosinopril all had a mean decrease of 12 mm Hg in their sequential systolic BP (SBP). Blacks, however, demonstrated a significant dose response to fosinopril; those who received the low dosage had a 5 mm Hg decrease in SBP, and those who received the high dosage had a mean 13 mm Hg decrease in SBP. CONCLUSIONS: Fosinopril was effective in treating hypertension, but black children required a higher dose per body weight in order to achieve adequate control. This suggests that black children treated with fosinopril for hypertension on average require higher doses to achieve adequate systolic blood pressure control that non-black children.

Five-year clinical and echocardiographic evaluation of the Das AngelWings atrial septal occluder.

BACKGROUND: The late outcome of patients treated with atrial septal occluder devices remains incompletely defined. The purpose of this study was to assess the late outcome (range 4-7 years postprocedure) of patients in whom the Das AngelWings septal occluder device was implanted in the atrial septum. We report the clinical and echocardiographic outcome, at an average of 5 years following the procedure, of patients treated with the Das AngelWings device used to close either a secundum atrial septal defect (ASD) or a patent foramen ovale (PFO). METHODS: Thirty-two patients underwent successful percutaneous closure of an atrial septal closure, patent foramen ovale, or fenestration in the lateral tunnel of their Fontan with the Das AngelWings device between June 1995 and March 1998 at Duke University Medical Center. Two of the 32 patients were lost to follow-up. The remaining 30 patients were divided into 3 groups based on indication for device implantation. Group 1 consisted of 14 patients with a secundum ASD and predominantly left-to-right atrial shunting. Group 2 consisted of 8 patients who had a PFO and who suffered a thromboembolic event. Group 3 (compassionate use) consisted of 10 patients with multiple comorbid medical problems with predominantly right-to-left shunting at the atrial level causing hypoxemia. Eight of the patients in group 3 were severely ill at the time of device implantation. The 2 remaining patients in group 3 underwent AngelWings implantation for closure of right-to-left shunting through a Fontan fenestration. Mean follow-up was 59 months. RESULTS: There was no device embolization. No patient in the ASD or PFO/stroke group had a clinical complication. By radiographic examination, 2 of 27 patients had evidence of fracture of the nitinol framework at 2-year follow-up. Residual shunting was present in 44% at 24 hours, 20% at 1 year, and 18.8% at 2 years by use of Doppler color flow imaging and/or microcavitation echocardiographic studies. Mild mitral regurgitation caused by the AngelWings device occurred in 1 patient. One patient in the compassionate use group had a subsequent neurologic event. Five of the 10 patients in the compassionate use group died of comorbid illnesses in follow-up, none directly related to device complications. CONCLUSION: The late clinical outcome of secundum ASD and PFO/stroke patients in this study demonstrates that Das AngelWings closure of the atrial septum is effective and safe. These data are encouraging with respect to the expanding use of other percutaneously implanted ASD occlusion devices, although late clinical safety and efficacy data are needed for the specific devices being implanted.

Pulmonic regurgitation and management challenges in the adult with tetralogy of fallot.

OPINION STATEMENT: Patients with tetralogy of Fallot (TOF) are living longer than ever because of advances in surgery in childhood since the 1950s. However, surgery in childhood is not a cure and remains only a palliative procedure because almost all patients will require further intervention throughout life. The most common intervention required in adulthood is pulmonary valve replacement (PVR) because of residual pulmonary regurgitation leading to right ventricular dilation and eventual dysfunction. The most appropriate timing for PVR remains difficult to determine and is based on many factors. Our practice is to weigh not only objective factors such as right ventricular size and function but also careful objective assessment of the patient's current quality of life and functional status.

Improving heart disease knowledge and research participation in adults with congenital heart disease (the Health, Education and Access Research Trial: HEART-ACHD).

OBJECTIVE: The objective of this prospective multi-center study was to evaluate heart disease knowledge within the adult congenital heart disease (ACHD) population, pilot an educational intervention and assess interest in research participation among new patients at ACHD clinics. BACKGROUND: Many adults with congenital heart disease lack knowledge about their heart condition that may contribute to undesirable outcomes. METHODS: Patients ≥18 years of age were recruited upon their first presentation to an ACHD clinic and underwent an educational intervention consisting of creation of a personal health information 'passport' and an introduction to web-based resources. Subjects were asked to complete initial and follow-up surveys documenting their perceived knowledge. RESULTS: Nine hundred twenty-two subjects were recruited from 12 ACHD centers, and 520 (57%) completed follow-up surveys. Patients who completed the follow-up survey were more likely to be women, have more education, and have mild heart disease. At follow-up, the ability of the subjects to name their heart condition improved (78% to 83%, p=0.002). Improvements were seen in mean Likert items regarding perceived knowledge of appropriate exercise (p<0.0001), symptoms of heart rhythm problems or endocarditis (p<0.0001), reasons for cardiac tests (p<0.007), and birth control options and pregnancy safety (p<0.0001). On follow-up, subjects reported a better understanding of medical research (p<0.01), and higher interest in research participation (p<0.003). CONCLUSION: This joint clinician-patient pilot program will help inform future efforts toward patient education and participation in research with a focus on standardization of protocols for life-long longitudinal follow-up and continued multi-center collaboration in the ACHD population.

Cryoablation of the slow atrioventricular nodal pathway via a transbaffle approach in a patient with the Mustard procedure for d-transposition of the great arteries.

The ablation of atrioventricular (AV) nodal reentrant tachycardia in patients with the Senning or Mustard procedure is quite challenging because these atrial baffle procedures isolate the AV node from systemic venous access. Cryoablation is commonly utilized for AV nodal slow pathway modification in patients with structurally normal hearts. The cryoablation technique offers the advantage of monitoring AV nodal conduction during lesions, with the ability to terminate a lesion prior to permanent injury to the AV node. This case describes the successful cryoablation of the slow AV nodal pathway via the transbaffle approach in a patient with d-transposition of the great arteries status post the Mustard procedure.

Antiphospholipid syndrome leading to myocardial infarction in a Fontan patient 17 years after Norwood palliation.

Appropriate regimens for anticoagulation in the single ventricle population remain controversial and are usually based on individual practitioners' preferences. We present the case of a patient with a history of hypoplastic left heart syndrome and Norwood palliation who presented at 17 years of age with an acute myocardial infarction and a new diagnosis of antiphospholipid syndrome.

Aortic pseudo aneurysm complicating coarctation stenting successfully treated with a PTFE balloon expandable covered stent.

Percutaneous stenting of both native and recurrent coarctation of the aorta has become an acceptable alternative to surgical repair in most centers throughout the world. Severe complications such as aortic rupture, dissection, and late pseudo aneurysm formation are rare but worrisome complications. In many countries, commercially available balloon expandable covered stents designed for intravascular use are used either for primary stenting, or in treating complications once identified. These endovascular stents, however, are not available in the United States. We report the use of a commercially available covered stent, which has been approved by the Food and Drug Administration for tracheal use, to exclude an aortic pseudo aneurysm that was identified late after stenting a native coarctation.