With increasing numbers of children surviving complex pathologies to reach adulthood, the adult congenital heart disease population is ever expanding. Often such patients will have undergone multiple sternotomies before developing heart failure requiring advanced heart failure therapies. The late complication of heart failure is not uncommon and presents a therapeutic challenge due to the unusual nature of anatomy and physiology in these patients. Transplantation is often seen as the final staging procedure in adult congenital heart disease, particularly of the patient with single-ventricle physiology. This chapter presents the idiosyncratic issues surrounding organ transplantation in the adult congenital heart disease population.

Septostomy reduces right ventricular (RV) workload at the expense of hypoxemia in patients with advanced pulmonary hypertension (PH). A patent foramen ovale (PFO) may serve as a "natural" septostomy, but the incidence and impact of a PFO in PH remains uncertain. We prospectively examined echocardiograms in 404 PH patients referred for initial hemodynamic assessment. Patients included had saline bubble injection and if negative repeatinjection after Valsalva maneuver. Echocardiographic and hemodynamic data were examined. Survival was modeled using Kaplan-Meier method. Eisenmenger syndrome or known atrial shunts other than PFO were excluded: 292 patients met entry criteria. A PFO was identified in 16.8% of the entire cohort, 22.9% of pulmonary arterial hypertension (PAH) patients, and 8.6% of Dana Point group 2 PH patients. Right atrial to pulmonary capillary wedge pressure difference was lowest in the latter group (-7.9 +/- 7.1 vs 1.7 +/- 5.5 mm Hg for all others, p<0.01). Patients with a PFO were younger (53.9 vs 58.6 years, p = 0.02). A PFO was more often present with moderately or severely dilated (p = 0.01) or dysfunctional (p = 0.03) RVs. Six year survival was unchanged by PFO presence for all patients, including those with PAH. Proportional hazards analysis found only age and functional class independently predicted survival (p<0.01). A PFO is identified less often in Dana Point group 2 PH, likely due to inability of Valsalva maneuver to overcome right atrial to pulmonary capillary wedge pressure difference. In conclusion, the incidence of a PFO in the PH population increases with more dilated and dysfunctional RVs, suggesting that the PFO may be stretched open rather than congenital. The presence of a PFO does not impact survival in PH or PAH. (C) 2018 Elsevier Inc. All rights reserved.

OBJECTIVES:Pulmonary hypertension portends a poorer prognosis for blacks versus white populations, but the underlying reasons are poorly understood. We investigated associations of disease characteristics, insurance status, and race with clinical outcomes.STUDY DESIGN:Retrospective cohort study of patients presenting for initial pulmonary hypertension evaluation at 2 academic referral centers.METHODS:We recorded insurance status (Medicare, Medicaid, private, self-pay), echocardiographic, and hemodynamics data from 261 patients (79% whites, 17% blacks) with a new diagnosis of pulmonary hypertension. Subjects were followed for 2.3 years for survival. Adjustment for covariates was performed with Cox proportional hazards modeling.RESULTS:Compared with white patients, blacks were younger (50 ± 15 vs 53 ± 12 years; P = .04), with females representing a majority of patients in both groups (80% vs 66%; P = .08) and similar functional class distribution (class 2/3/4: 30%/52%/16% blacks vs 33%/48%/14% whites; P = .69). Blacks diagnosed with incident pulmonary hypertension were more frequently covered by Medicaid (12.5% vs 0.7%) and had less private insurance (50% vs 61%; P = .007) than whites. At presentation, blacks had more right ventricular dysfunction (P = .04), but similar mean pulmonary arterial pressure (46 vs 45 mm Hg, respectively; P = .66). After adjusting for age and functional class, blacks had greater mortality risk (hazard ratio [HR], 2.06; 95% confidence interval [CI], 1.18-3.44), which did not differ by race after additional adjustment for insurance status (HR, 1.74; 95% CI, 0.84-3.32; P =.13).CONCLUSIONS:In a large cohort of patients with incident pulmonary hypertension, black patients had poorer right-side heart function and survival rates than white patients. However, adjustment for insurance status in our cohort removed differences in survival by race.

Introduction: Septostomy is an accepted treatment in advanced pulmonary hypertension (PH), reducing right ventricular (RV) work-load at the expense of cyanosis. Patent foramen ovale (PFO), present ...

Background: Baseline right ventricular (RV) function is a major determinant of outcome in patients with pulmonary arterial hypertension (PAH). However, the impact of changes in echo-assessed RV fun...

Article, see p 101Congenital heart disease (CHD) is the most common of all congenital defects, affecting nearly 1% of live births. Creative operative and catheter-based interventions have facilitated survival to adulthood with nearly all defects, setting the stage for a rapidly growing population of adolescents and adults with CHD. Although the field of adult CHD (ACHD) remains in its infancy, it is important to acknowledge the tremendous achievements in the United States and abroad over the last decades. ACHD publications have increased almost exponentially (Figure). The first American College of Cardiology/American Heart Association joint guidelines on the diagnosis and management of ACHD were published in 20081 with a revision anticipated soon; subspecialty status in cardiology was granted by the American Board of Medical Specialties in 2012 with the first qualifying examination offered in the fall of 2015, and the first class of American Council for Graduate Medical Education–approved fellowship training begins July 2016. Multicenter and multinational research collaborations have formed, including the Alliance for Adult Research in Congenital Cardiology, as have patient/provider collaborative organizations such as the Adult Congenital Heart Association and the International Society of Adult Congenital Heart Disease. Funding for research has also improved, led by the US Department of Defense, the American Heart Association, and the National Heart, Lung, and Blood Institute.Figure. Trends in adult congenital heart disease (ACHD). PubMed cited articles directly relating to adults with CHD over 4-year intervals since 1980 ( blue ) and the estimated number of ACHD patients (from program survey by the Adult Congenital Heart Association and the International Society of Adult Congenital Heart Disease) seen at programs in the United States that self-report as providing specialized care to adults with congenital disease ( red ) during 4 different years since 2005.Despite tremendous progress, obstacles to the appropriate and sustained growth of …

We aimed to characterize the hemodynamic progression of aortic stenosis (AS) in a contemporary unselected cohort of patients with preserved left ventricular ejection fraction. Current guidelines recommend echocardiographic surveillance of hemodynamic progression. However, limited data exist on the expected rate of progression and whether clinical variables are associated with accelerated progression in contemporarily managed patients with AS. We conducted a retrospective analysis of patients presenting with AS and explored the trajectory of AS mean gradient over time using generalized estimating equations and fit a longitudinal linear regression model with adjustment for baseline clinical variables. A total of 1,558 patients (median age 72; interquartile range 65 to 79) having mild (n = 982), moderate (n = 363), or severe AS (n = 213) were included. In patients with mild AS at baseline (n = 983), 303 (31%) had progressed to moderate/severe AS/AVR within 5 years of the index echo. In patients with moderate AS, 159 of 363 (44%) had progressed to severe AS/AVR within 2 years of the index echo. The annual change in mean gradient was dependent on baseline AS severity. Average annual increases in mean gradient were 6.8% (95% confidence interval 6.0 to 7.6) and 7.1% (95% confidence interval 4.8 to 9.3) in patients with mild and moderate AS, respectively. In the subset of patients with mild AS at baseline, age (p = 0.0310) and gender (p = 0.0270) had significant interaction with change in mean gradient over time. In patients with moderate AS, age (p <0.0001), gender (p = 0.0346), renal dysfunction (p = 0.0036), and hyperlipidemia (p = 0.0010) demonstrated significant interaction with change in mean gradient over time. In conclusion, although average disease progression was slower than previously reported, a significant proportion of patients with mild and moderate AS progressed to higher grades within the currently recommended time windows for echocardiographic follow-up. (C) 2015 Elsevier Inc. All rights reserved.

Congenital Heart DiseaseVolume 10, Issue 5 p. 382-386 STATE OF THE ART ARTICLE Initial Experience of Left Ventricular Assist Device Support for Adult Patients with Transposition of the Great Vessels Jonathan N. Menachem MD, Jonathan N. Menachem MD Department of Internal Medicine, Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pa, USASearch for more papers by this authorAparna C. Swaminathan MD, Aparna C. Swaminathan MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorThomas M. Bashore MD, Thomas M. Bashore MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorCary C. Ward MD, Cary C. Ward MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorJoseph G. Rogers MD, Joseph G. Rogers MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorCarmelo A. Milano MD, Carmelo A. Milano MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorChetan B. Patel MD, Corresponding Author Chetan B. Patel MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USACorresponding Author: Chetan B. Patel, MD, Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, DUMC 3034, Durham NC 27710, USA. Tel: (+1) 919-681-3398; Fax: (+1) 919-668-7078; E-mail: [email protected]Search for more papers by this author Jonathan N. Menachem MD, Jonathan N. Menachem MD Department of Internal Medicine, Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pa, USASearch for more papers by this authorAparna C. Swaminathan MD, Aparna C. Swaminathan MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorThomas M. Bashore MD, Thomas M. Bashore MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorCary C. Ward MD, Cary C. Ward MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorJoseph G. Rogers MD, Joseph G. Rogers MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorCarmelo A. Milano MD, Carmelo A. Milano MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USASearch for more papers by this authorChetan B. Patel MD, Corresponding Author Chetan B. Patel MD Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USACorresponding Author: Chetan B. Patel, MD, Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, DUMC 3034, Durham NC 27710, USA. Tel: (+1) 919-681-3398; Fax: (+1) 919-668-7078; E-mail: [email protected]Search for more papers by this author First published: 27 April 2015 https://doi.org/10.1111/chd.12264Citations: 11Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL References 1Levin DL, Paul MH, Muster AJ, Newfeld EA, Waldman JD. d-Transposition of the great vessels in the neonate. A clinical diagnosis. Arch Intern Med. 1977; 137: 1421– 1425. 2Warnes CA. Transposition of the great arteries. Circulation. 2006; 114: 2699– 2709. 3Senning A. Surgical correction of transposition of the great vessels. Surgery. 1959; 45: 966– 980. 4Mustard WT. Successful two-stage correction of transposition of the great vessels. Surgery. 1964; 55: 469– 472. 5Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000; 36: 255– 261. 6Warnes CA, Somerville J. Transposition of the great arteries: late results in adolescents and adults after the Mustard procedure. Br Heart J. 1987; 58: 148– 155. 7Buch J, Wennevold A, Jacobsen JR, Hvid-Jacobsen K, Lauridsen P. Long-term follow-up of right ventricular function after Mustard operation for transposition of the great arteries. Scand J Thorac Cardiovasc Surg. 1988; 22: 197– 202. 8Joyce DL, Crow SS, John R, et al. Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries. J Heart Lung Transplant. 2010; 29: 1302– 1305. 9Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS, Hawker RE. Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart. 1998; 80: 322– 326. 10Wiklund L, Svensson S, Berggren H. Implantation of a left ventricular assist device, back-to-front, in an adolescent with a failing mustard procedure. J Thorac Cardiovasc Surg. 1999; 118: 755– 756. 11George RS, Birks EJ, Radley-Smith RC, Khaghani A, Yacoub M. Bridge to transplantation with a left ventricular assist device for systemic ventricular failure after Mustard procedure. Ann Thorac Surg. 2007; 83: 306– 308. Citing Literature Volume10, Issue5September/October 2015Pages 382-386 ReferencesRelatedInformation

BACKGROUND:Calcific aortic stenosis (AS) is the most common underlying pathology in patients undergoing heart valve surgery, with an expected increasing prevalence among the aging population.METHODS AND RESULTS:We identified the temporal trends in referral patterns, disease severity, and associated surgical risk among patients with AS between January 1, 1995 and December 31, 2012 at the Duke University Hospital. A total of 6103 patients had a finding of mild (n = 3303), moderate (n = 1648), or severe AS (n = 1152) in a native aortic valve. Overall presence of severe AS increased significantly over time (P = 0.009) with the most substantial increase occurring from 2010 and onward. Median age upon referral (P < 0.001) and attendant predicted surgical risk (P < 0.001) increased significantly in the observation period among patients with a finding of severe AS. Among patients with a finding of severe AS, the proportion of patients aged older than 80 years increased to 51.0% in the most recent time period (2010-2012) compared with 32.6% in the preceding time period (P < 0.001 for overall time trend). Similarly, the proportion of patients with a logistic EuroSCORE greater than 20% increased to 21.3% (2010-2012) from 12.1% (pre-2010).CONCLUSIONS:Among patients referred for echocardiography to a high-volume tertiary hospital center, a significant increase in the prevalence of severe AS was observed over time. This trend occurred in parallel with increasing age and predicted surgical risk at referral. Health-care resource planning should account for an increasing number of patients in need of high-risk aortic valve replacements in the near future.

Diabetes complicates management in a number of disease states and adversely impacts survival; how diabetes affects patients with pulmonary hypertension (PH) has not been well characterized. With insulin resistance having recently been demonstrated in PH, we sought to examine the impact of diabetes in these patients. Demographic characteristics, echo data, and invasive hemodynamic data were prospectively collected for 261 patients with PH referred for initial hemodynamic assessment. Diabetes was defined as documented insulin resistance or treatment with antidiabetic medications. Fifty-five patients (21%) had diabetes, and compared with nondiabetic patients, they were older (mean years ± SD, 61 ± 13 vs. 56 ± 16; [Formula: see text]), more likely to be black (29% vs. 14%; [Formula: see text]) and hypertensive (71% vs. 30%; [Formula: see text]), and had higher mean (±SD) serum creatinine levels (1.1 ± 0.5 vs. 1.0 ± 0.4; [Formula: see text]). Diabetic patients had similar World Health Organization functional class at presentation but were more likely to have pulmonary venous etiology of PH (24% vs. 10%; [Formula: see text]). Echo findings, including biventricular function, tricuspid regurgitation, and pressure estimates were similar. Invasive pulmonary pressures and cardiac output were similar, but right atrial pressure was appreciably higher (14 ± 8 mmHg vs. 10 ± 5 mmHg; [Formula: see text]). Despite similar management, survival was markedly worse and remained so after statistical adjustment. In summary, diabetic patients referred for assessment of PH were more likely to have pulmonary venous disease than nondiabetic patients with PH, with hemodynamics suggesting greater right-sided diastolic dysfunction. The markedly worse survival in these patients merits further study.

A 75-year old woman with a history of coronary disease status post 3-vessel coronary artery bypass grafting (CABG) 8 years ago and a repeat one-vessel CABG 2 years ago in the setting of aortic valve replacement with a #19 mm St. Jude bileaflet mechanical valve for severe aortic stenosis presented with two to three weeks of progressive dyspnea and increasing substernal chest discomfort. Echocardiography revealed a gradient to 31 mmHg across her aortic valve, increased from a baseline of 13 mmHg five months previously. Fluoroscopy revealed thrombosis of her mechanical aortic valve. She was not a candidate for surgery given her multiple comorbidities, and fibrinolysis was contraindicated given a recent subdural hematoma 1 year prior to presentation. She was treated with heparin and eptifibatide and subsequently demonstrated resolution of her aortic valve thrombosis. We report the first described successful use of eptifibatide in addition to unfractionated heparin for the management of subacute valve thrombosis in a patient at high risk for repeat surgery or fibrinolysis.

Adult congenital heart disease represents a growing population of patients. Many patients survive to adulthood and lead functional, productive lives. In fact, there are more adults living with congenital heart disease than pediatric patients. Many adult patients will have had prior surgical repair as children. However, some patients present in adulthood with a new diagnosis of congenital heart disease. Furthermore, there are a variety of complications associated with each individual congenital lesion and specific surgical repair procedure.

Supplemental Digital Content is available in the text.