Adult Congenital Heart Disease Centers Research Articles

Adult Congenital Heart Disease

Adults with congenital heart disease (CHD) represent an increasing population both because anomalies that might have remained undiagnosed in the past are now being diagnosed later in life on imaging and because significant therapeutic advances have resulted in survival to adulthood of patients with complex CHD. In this article, we discuss simple and common complex congenital anomalies that are encountered in general practice including their incidence, associations, and expected postoperative appearances. We will describe an approach to segmental anatomy and situs evaluation and details of some of the common vascular anomalies, simple shunts, and complex CHDs to refine the imaging strategies and diagnostic acumen of radiologists interested in CHD who may not be practicing in specialized adult CHD centers. Key imaging appearances on chest radiography, protocoling tips for answering clinically relevant questions on computed tomography and magnetic resonance imaging, and the specific imaging appearances and common complications related to long-standing CHD in the adult and complications of treatment are reviewed for each entity.

The value of stress perfusion cardiovascular magnetic resonance imaging for patients referred from the adult congenital heart disease clinic: 5-year experience at the Toronto General Hospital

Vasodilator stress perfusion cardiovascular magnetic resonance imaging is a clinically useful tool for detection of clinically significant myocardial ischaemia in adults. We report our 5-year retrospective experience with perfusion cardiovascular magnetic resonance in a large, quarternary adult congenital heart disease centre. We reviewed all cases of perfusion cardiovascular magnetic resonance in patients referred from the adult congenital heart disease service. Dipyridamole stress perfusion cardiovascular magnetic resonance was undertaken on commercially available 1.5 and 3 T cardiovascular magnetic resonance scanners. Late gadolinium enhancement imaging was performed 8-10 minutes after completion of the rest perfusion sequence. Navigator whole-heart coronary magnetic resonance angiography was also performed where feasible. RESULTS of stress cardiovascular magnetic resonance were correlated with complementary imaging studies, surgery, and clinical outcomes. Over 5 years, we performed 34 stress perfusion cardiovascular magnetic resonance examinations (11 positive). In all, 84% of patients had further investigations for ischaemia in addition to cardiovascular magnetic resonance. Within a subgroup of 19 patients who had definitive alternative assessment of their coronary arteries, stress perfusion cardiovascular magnetic resonance demonstrated a sensitivity of 82% and specificity of 100%. Of the 34 studies, two were false negatives, in which the aetiology of ischaemia was extrinsic arterial compression rather than intrinsic coronary luminal narrowing. Coronary abnormalities were identified in 71% of cases who had coronary magnetic resonance angiography. Stress perfusion cardiovascular magnetic resonance is a useful and accurate tool for investigation of myocardial ischaemia in an adult congenital heart disease population with suspected non-atherosclerotic coronary abnormalities.

Meeting the challenge: The evolving global landscape of adult congenital heart disease

BackgroundOnly limited information is available on the worldwide distribution and volume of adult congenital heart disease (ACHD) centers. We aimed to assess the centers using a bibliometric approach. Methods and resultsWe identified publications presenting original research in the field of ACHD between 1995 and 2011. A total of 94,119 articles were identified which underwent electronic filtering and manual review. Overall, a dramatic increase in ACHD publications was seen over the study period. This was accompanied by a matching increase in impact factors and an over-proportional rise in ACHD contributions relative to the general academic field. Research output correlated well with self-reported patient volume and the number of identified ACHD centers in Europe and North America was in agreement with published surveys, thus validating our methodology. We observed a steady increase in the number of publishing ACHD centers worldwide. The number of ACHD centers per 10-million population was highest for Europe (3.6), followed by North America (1.7), Oceania (1.5), South America (0.4), Asia (0.3) and Africa (0.1). In addition, we evaluated the relative research output between developed and emerging economies and provide an overview over the main areas of research in the ACHD field. ConclusionsGlobal interest in ACHD is increasing and this is reflected, both, in the number of publishing centers and the volume of research. Our data provides insights into the geographical and temporal distribution of ACHD research over the last 1 1/2 decades. These results could serve as benchmarks for international comparisons and guide efforts for improving ACHD infrastructure.

Perioperative Care of Adults with Congenital Heart Disease for Non-cardiac Surgery

In 2010 it was estimated that 1.2 million young adults in the United States have some form of congenital heart disease (CHD). Data from large adult congenital heart disease (ACHD) centers show that as many as 50 % of these patients are over 40 years of age. As this population ages, physicians are likely to encounter such patients in their practice. Risk stratification and an interdisciplinary approach are paramount in achieving safe outcomes. The assessment of the patient must consider the variable expression of CHD. As most lesions are “fixed but not cured” the altered physiology of the heart and the physiological challenges of surgical interventions must be considered. The 2008 AHA/ACC guidelines for the Management of Adults with CHD establish for the first time a streamlined approach to the care of these patients. This review will apply these guidelines to the ACHD patient undergoing non-cardiac surgery.

Usefulness of Progesterone-Only Components for Contraception in Patients With Congenital Heart Disease

The objective of the study is to report the contraceptive methods used by patients with congenital heart disease (CHD) before referral to a specific preconception clinic and evaluate safety and treatment adherence of the alternative contraception method, progesterone-only component (PC), offered. Contraceptive methods in the CHD population reported included estrogen-progesterone combined contraceptives (EPCC), despite the potential risk for thromboembolism. PC has been suggested as an alternative, but, no information on its use has been reported. Retrospective analysis was performed of all patients (n = 237) referred to the preconception clinic of an adult CHD center. Thirty-three percent of patients had used EPCC in the past; 3.8% had had thromboembolic events during its use. Current contraception consisted of barrier methods in 58% of patients, EPCC in 18%, and PC in 1.3%; 21.7% of patients were not using any contraception. PC was offered as an alternative in 146 patients; 73% of patients agreed to start the treatment. At a median follow-up of 1 year, 73% of patients who started PC maintained the treatment. Gynecologic side effects were reported in 25% of patients, with no cardiovascular effects. In conclusion, a significant proportion of patients with CHD were former users of EPCC, although some had formal contraindications, and the rate of PC use before referral to the preconception clinic was low. After being offered as an alternative treatment, the use of PC in its various forms was extensive, with no thrombogenic side effects and an acceptable rate of gynecologic side effects being reported.

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

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. Many adults with congenital heart disease lack knowledge about their heart condition that may contribute to undesirable outcomes. 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. 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). 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.

Lifetime Costs and Outcomes of Repair of Tetralogy of Fallot Compared to Natural Progression of the Disease: Great Ormond Street Hospital Cohort

BackgroundTetralogy of Fallot is a congenital heart disease that requires surgical repair without which survival through childhood is extremely rare. The aim of this paper is to use data from the mandatory follow-up of patients with Tetralogy of Fallot to model the health-related costs and outcomes over the first 55-years of life.MethodA decision analytical model was developed to establish costs and outcomes for patients up to 55 years after diagnosis and first repair of Tetralogy of Fallot compared to natural progression. Data from Adult Congenital Heart Disease (ACHD) centres that follow up Tetralogy of Fallot patients and Great Ormond Street Hospital (GOSH), London, United Kingdom (UK) medical records was used to establish the cost and effectiveness of current interventions. Data from a Czech cohort was used for the natural, no intervention condition.ResultsThe average cost per patient of a repair for Tetralogy of Fallot was £26,938 (SE = £4,140). The full life time cost per patient, with no discount rate, was £65,310 (95% CI £64,981–£65,729); £56,559 discounted (95% CI £56,159–£56,960). Patients with a repair had an average of 35 Quality Adjusted Life Years (QALYs) per patient over 55 years undiscounted and 20.16 QALYs discounted. If the disorder was left to take its natural course, patients on average had a total of 3 QALYs per patient with no discount rate and 2.30 QALYs discounted.ConclusionA model has been developed that provides an estimate of the value for money of an expensive repair of a congenital heart disease. The model could be used to test the cost-effectiveness of making amendments to the care pathway.

Aspects étiologiques des hémoptysies à propos de 81 cas

Pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) usually has a homogeneous pressure distribution. More rarely, complex CHD patients have segmental PAH. This is often post-surgically. The characteristics of these patients and their responsiveness to specific pulmonary vasodilator therapy have not been described.Seven adults with segmental PAH complicating CHD were treated at 3 specialized adult CHD centers between January 2006 and December 2010. Clinical characteristics, six minute walking distances (6MWD), laboratory tests and images were obtained from medical records and the responses to Bosentan, an endothelin-1 receptor antagonist, were assessed.All patients (mean age 32 (23–42) years, five females) had a primary diagnosis pulmonary atresia (PA), four with major aortopulmonary collateral arteries (MAPCAs). Four segmental PAH patients had a right pulmonary artery stenosis, two a left pulmonary artery stenosis and one a unilateral MAPCA stenosis. All patients were symptomatic (functional class II or III) and bosentan was started empirically. Bosentan treatment led to a significant improvement in functional class compared to baseline (1.7 ± 0.5 versus 2.4 ± 0.5; p < 0.01). Mean 6MWD (available in 6 patients) increased by 62 m (22–150 m) from 386 ± 135 to 448 ± 133 m (p = 0.03) after 12 months treatment. Most improvement was seen in patients with low baseline 6MWD. Higher baseline exercise heart rate was significantly associated with lesser improvement in 6MWD (r = − 0.91 p = 0.01). Laboratory results did not change after initiation of bosentan treatment.This small retrospective case series suggested a significant improvement of functional class and exercise capacity after bosentan treatment in patients with segmental PAH. These findings warrant a prospective study of the potential benefit of selective pulmonary vasodilator therapy in these complex patients. Therefore, we call on treating physicians to share similar cases.

Incidence of patent foramen ovale and migraine headache in adults with congenital heart disease with no known cardiac shunts

The purpose of this study was to understand why patients with adult congenital heart disease (CHD) but no obvious shunt have an increased frequency of migraine headaches (MH). CHD patients with no known cardiac shunts (CHD-NKS), based on their echocardiographic or angiographic procedures, were tested for a right-to-left shunt using agitated saline contrast transcranial Doppler (TCD). Medical records of 2,920 patients from the UCLA Adult CHD Center were screened to participate in a study to evaluate the prevalence of MH in adults with CHD; 182 patients (6.23%) had CHD-NKS; of these, 60 (30%) underwent a TCD; 23 (38%) tested positive and 37 (62%) tested negative for a right-to-left shunt (P = 0.01 compared with controls). The frequency of MH was 43% in CHD-NKS compared with 11% in controls (P < 0.0001). TCD demonstrated right-to-left shunting in approximately 2/3 of patients with pulmonary stenosis, the Marfan syndrome and congenitally corrected transposition of great vessels, 1/4 of patients with bicuspid aortic valve, 1/5 of patients with mitral valve prolapse and all patients with Ebstein's anomaly. Approximately half of these experienced MH. Patients who had MH did not show a higher frequency of right-to-left shunt when compared with patients without MH (P = 0.57). In conclusion, CHD patients with conditions usually not associated with a shunt have a higher than expected prevalence of PFO which permits intermittent right-to-left shunting undetected by standard non-contrast TTE and TEE; the increased prevalence of right-to-left shunting may partially explain the higher than expected frequency of migraines.

Congenital heart disease clinics — How to keep the adult patients on board

The steadily growing number of adults with congenital heart disease has led with some delay to an increase of specialized programs dedicated to the care of these patients. Still, in many countries the prevalence of congenital heart disease in adults is not known, nor the number of adults requiring specialized care. By extrapolating the results of epidemiological studies on the population in Europe and North America, only half or even less of all adults with moderate or complex congenital heart disease is currently under follow-up in specialized centers. Lapse of appropriate medical care in adults with congenital heart disease is associated with unnecessary morbidity and an increased likelihood of the need for urgent interventions. In order to improve medical care for adults with moderate or complex congenital heart disease, the number of patients with specialized follow-up in congenital cardiac clinics has to increase. One approach is to avoid lapses of care in patients already followed by a pediatric or adult congenital heart disease center. Therefore, congenital heart disease clinics should try to identify among their patients the ones at risk for lost to follow-up, and focus some of their efforts on measures to increase the awareness for life-long medical care among these patients. Characteristics of the adult congenital cardiac patient at high risk of inappropriate medical follow-up include having few interventions in early life despite a complex cardiac defect, being born in an earlier decade, receiving no specific follow-up recommendations, and having no education about defect- or surgery-related sequelae or residual lesions. Implementation of a structured transition program, teaching patients about their heart defect and the consequences of repair or palliation in earlier life, establishing a continuity of care within the congenital cardiac clinic, optimizing organization and communication between different care-givers, and encouraging patients to play an active role regarding their personal health care, are some of the recommendations on how we should try to avoid another generation of adult congenital heart disease patients from being lost to follow-up.

Hemodynamic Characteristics of Cyanotic Adults with Single-ventricle Physiology without Fontan Completion

The aim of the current study is to describe the long-term clinical and hemodynamic characteristics of adult patients with single-ventricle physiology who have not undergone the Fontan operation and consequently have remained cyanotic. Adult patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center with non-Fontan single-ventricle physiology who had undergone cardiac catheterization between 2005 and 2011 were included. Echocardiographic and cardiac catheterization data were reviewed. Mean estimated single ejection fraction was 56 ± 8%. Eight of 13 subjects had documented E/E' data with a mean of 6.44. Seven subjects had both A' and E' data documented, of which two subjects exhibited A' > E'. Mean ventricular end-diastolic pressure (MVEDP) was 15.77 ± 4.91 mm Hg, and was >12 mm Hg in eight of the 13 patients (62%). MVEDP was also analyzed by age, and in the single-ventricle patients was 13.55 ± 4.12 mm Hg in those <50 years of age, compared with 20.75 ± 1.89 mm Hg in those >50 years of age (P = .003). MVEDP prior to inhaled pulmonary vasodilator administration was 14.75 ± 5.5 mm Hg, compared to 15.00 ± 6.78 mm Hg in the postvasodilator group (P = .48). Subjects with end-diastolic pressure (EDP) <12 had a mean brain natriuretic peptide (BNP) of 108 ± 197 pg/mL, while subjects with EDP >12 had a mean BNP of 234.5 ± 127.36 pg/mL (P = .11). Cyanotic adult single-ventricle patients not palliated with Fontan completion have preserved single-ventricle systolic function but develop elevated ventricular filling pressure with increasing age. Only invasive hemodynamic measurements demonstrated elevated ventricular filling pressures, while traditional echo/Doppler criteria for diastolic dysfunction were not met. Aging with cyanotic single-ventricle physiology is associated with a greater degree of filling pressure elevations than in the general population. Single-ventricle patients with EDP >12 exhibited markedly elevated BNP compared to those with normal EDP.

Congenital Heart Defects in Adults : A Field Guide for Cardiologists

Advances in cardiology and cardiac surgery allow a large proportion of patients with congenital heart defects to survive into adulthood. These patients frequently develop complications characteristic of the defect or its treatment. Consequently, adult cardiologists participating in the care of these patients need a working knowledge of the more common defects. Occasionally, patients with congenital heart defects such as atrial septal defect, Ebstein anomaly or physiologically corrected transposition of the great arteries present for the first time in adulthood. More often patients previously treated in pediatric cardiology centers have transitioned to adult congenital heart disease centers for ongoing care. Some of the more important defects in this category are tetralogy of Fallot, transposition of the great arteries, functionally single ventricle defects, and coarctation. Through this field guide, we provide an overview of the anatomy of selected defects commonly seen in an adult congenital practice using pathology specimens and clinical imaging studies. In addition, we describe the physiology, clinical presentation to the adult cardiologist, possible complications, treatment options, and outcomes.

Response to Letters Regarding Article, “Comparison Between Transcatheter and Surgical Prosthetic Valve Implantation in Patients With Severe Aortic Stenosis and Reduced Left Ventricular Ejection Fraction”

We thank Subramanian et al and Ranucci et al for their insightful comments with regard to our study.1 In response to the comments of Ranucci et al, we provide the following statements. 1. The important variability in the 30-day mortality rates (8% to 33%) reported in previous studies is essentially related to the differences in the cutoff values used for the left ventricular ejection fraction (LVEF), but also, and more importantly, it is related to the differences in the baseline risk profile of the study populations. The fact that the mortality (12%) observed in our surgical aortic valve replacement (SAVR) series is higher than that (8% to 10%) of some previous series is essentially related to the worse-risk profile of the patients included in this series. 2. The assessment of SAVR operative risk was based on a comprehensive analysis of the factors included in the risk scores, and those (eg, frailty) not included in these scores, as well. All patients included in the transcatheter aortic valve implantation (TAVI) series had been refused for SAVR by at least 2 cardiac surgeons. 3. We agree that the presence of reduced LVEF should not be used as the sole argument to recommend TAVI rather than SAVR in patients with severe aortic stenosis. SAVR remains the standard of care in the vast majority of patients with reduced LVEF and high …

Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease

IntroductionPulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) usually has a homogeneous pressure distribution. More rarely, complex CHD patients have segmental PAH. This is often post-surgically. The characteristics of these patients and their responsiveness to specific pulmonary vasodilator therapy have not been described. MethodsSeven adults with segmental PAH complicating CHD were treated at 3 specialized adult CHD centers between January 2006 and December 2010. Clinical characteristics, six minute walking distances (6MWD), laboratory tests and images were obtained from medical records and the responses to Bosentan, an endothelin-1 receptor antagonist, were assessed. ResultsAll patients (mean age 32 (23–42) years, five females) had a primary diagnosis pulmonary atresia (PA), four with major aortopulmonary collateral arteries (MAPCAs). Four segmental PAH patients had a right pulmonary artery stenosis, two a left pulmonary artery stenosis and one a unilateral MAPCA stenosis. All patients were symptomatic (functional class II or III) and bosentan was started empirically. Bosentan treatment led to a significant improvement in functional class compared to baseline (1.7±0.5 versus 2.4±0.5; p<0.01). Mean 6MWD (available in 6 patients) increased by 62m (22–150m) from 386±135 to 448±133m (p=0.03) after 12months treatment. Most improvement was seen in patients with low baseline 6MWD. Higher baseline exercise heart rate was significantly associated with lesser improvement in 6MWD (r=−0.91 p=0.01). Laboratory results did not change after initiation of bosentan treatment. ConclusionThis small retrospective case series suggested a significant improvement of functional class and exercise capacity after bosentan treatment in patients with segmental PAH. These findings warrant a prospective study of the potential benefit of selective pulmonary vasodilator therapy in these complex patients. Therefore, we call on treating physicians to share similar cases.

Opinions of Physicians Regarding Problems and Tasks Involved in the Medical Care System for Patients with Adult Congenital Heart Disease in Japan

The aim of this study is to summarize the opinions of physicians regarding problems and tasks involved in the medical care system for patients with adult congenital heart disease (ACHD) in Japan. We conducted a semistructured interview with 30 subjects consisting of 13 pediatric cardiologists, 11 cardiovascular surgeons, and six cardiologists who were selected from among the randomly sampled medical facilities meeting each of the following institutional criteria: (1) facilities with ≥50 ACHD outpatients; (2) facilities with ACHD-specialized outpatient clinic; (3) facilities that are members of the Japanese Association of Chirdren's Hospitals and Related Facilities. The interview time was 27-91 minutes (mean, 70.0). The age of the subjects ranged from 36 to 62 years (mean, 46.7), and they had 0.5-34 years (mean, 16.2) of experience in treating congenital heart disease. From an analysis of interview details, the following four themes were extracted (in descending order of the number of comments): "(1) Who should treat ACHD" (comments by 29 subjects), "(2) Centralization of medical care" (comments by 29 subjects), "(3) What is the role of children's hospitals in ACHD?" (comments by 24 subjects), and "(4) Psychosocial problems" (comments by 24 subjects). Regional ACHD centers need to be established to promote centralization of patients, physicians, and educational function. This will provide higher quality medical service to more patients in the near future.

Monitoring the Patient with Transposition of the Great Arteries: Arterial Switch Versus Atrial Switch

Sufficient time has passed that adult congenital heart disease (ACHD) specialists now frequently encounter survivors born with complete transposition of the great arteries and palliated with an atrial or arterial switch procedure. To ensure the ongoing health of these patients, it is of paramount importance that their surgeries are understood and that physicians are aware of and remain vigilant for potential late complications. Adult survivors should be assessed annually in a regional ACHD center. Clinical assessment, electrocardiogram, and multimodality imaging are the mainstay of routine monitoring. Doppler echocardiography is the first-line imaging modality; other diagnostic tests are tailored to seek specific long-term complications. Clinicians, specialists in cardiovascular imaging, nurses and others involved in the delivery of care need special training and expertise. Care for these complex patients is best provided by multidisciplinary teams located in regional ACHD centers with access to adequate human and structural resources.

Assessment of Electronic Health Information System Use and Need in US Adult Congenital Heart Disease Centers

Efforts to improve care for adult congenital heart disease (ACHD) patients necessitates collection of accurate, detailed, longitudinal data. We sought to document what electronic health record systems are currently available at ACHD centers and to assess national interest in a uniform ACHD-focused system. Directors of ACHD centers in the United States were invited to complete an online questionnaire regarding current health information systems at their institution both for general cardiology and for ACHD. Topics that were surveyed included utility and perceived limitations of currently available systems. The survey also assessed the level of interest in an ACHD-specific system, and its optimal functions. Thirty-four centers responded, representing both pediatric and adult institutions that care for patients with ACHD. Of these, 80% reported using a variety of commercially supported electronic medical record products, whereas only 50% employed an ACHD-specific noncommercial database to supplement their institutional system. Comparison of the two systems revealed that most clinical activities are pursued through the institutional electronic medical record system. Research and tracking of clinical activities were the primary uses of ACHD-specific systems, which have several noted limitations. Strong interest in an integrated ACHD-specific system was found among responders. There is both an unmet need and a strong interest in an ACHD-oriented electronic health record that could facilitate research, outcome tracking, quality assurance, and inter-institutional collaboration, all functions that are lacking in electronic health systems currently in use.

Prevalence of Left Ventricular Systolic Dysfunction in Adults With Repaired Tetralogy of Fallot

Left ventricular (LV) systolic dysfunction has been observed in patients with repaired tetralogy of Fallot (TOF), although its clinical associations are unknown. Adults with repaired TOF were identified from 11 adult congenital heart disease centers. Clinical history was reviewed. Patients with pulmonary atresia were excluded. Echocardiograms were reanalyzed to estimate LV ejection fraction. LV function was defined as normal (LV ejection fraction ≥ 55%) or mildly (45% to 54%), moderately (35% to 44%), or severely (< 35%) decreased. Right ventricular (RV) and LV dimensions and Doppler parameters were remeasured. Function of all valves was qualitatively scored. Of 511 patients studied, LV systolic dysfunction was present in 107 (20.9%, 95% confidence interval 17.4 to 24.5). Specifically, 74 (14.4%) had mildly decreased and 33 (6.3%) had moderately to severely decreased systolic function. Presence of moderate to severe LV dysfunction was associated with male gender, LV enlargement, duration of shunt before repair, history of arrhythmia, QRS duration, implanted cardioverter-defibrillator, and moderate to severe RV dysfunction. Severity or duration of pulmonary regurgitation was not different. In conclusion, LV systolic dysfunction was found in 21% of adult patients with TOF and was associated with shunt duration, RV dysfunction, and arrhythmia.

Status and Future Needs of Regional Adult Congenital Heart Disease Centers in Japan - A Nationwide Survey -

Although the prevalence of adult congenital heart disease (ACHD) in Japan continues to rise, the number and geographic distribution of facilities potentially serving as regional ACHD centers remains unknown. We examined trends in ACHD care in Japan to identify needs and to determine potential regional responses to this growing patient population. A descriptive, cross-sectional, nationwide survey was conducted to assess the status and needs of cardiology specialists related to providing ACHD care. Questionnaires were mailed to 138 cardiology departments located in 8 geographical regions throughout Japan; respondents were asked to document the status and future direction of ACHD care for each facility. Of the 109 facilities that responded, approximately one-third currently treat or plan to treat all ACHD patients. Fourteen facilities (12.8%) fulfilled all criteria for becoming regional ACHD centers. Although each regional center was projected to serve a population of 9.1 million, in 2 regions, no centers possessed the necessary care structure. Our findings revealed a shortage of adult cardiologists dedicated to ACHD care. Moreover, basic as well as formal fellowship ACHD training was deemed necessary. In Japan, the number of potential regional ACHD centers has just reached international standards. However, based on the geographic gaps documented here, a strategy other than regional centralization might be required to deliver adequate ACHD care to rural areas.

Clinical Profile of the Adolescent/Adult Fontan Survivor

The study aims to describe the clinical profile of the adult Fontan survivor and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced. A descriptive, cross-sectional design was used. The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices. Fifty-four adolescent and adult patients with single ventricle congenital heart disease who have undergone the Fontan procedure participated in the study. The mean age was 26 ± 9 years with 52% female and 63% Caucasian. Demographic and clinical data were obtained by a standard intake form and retrospective chart reviews. The Congenital Heart Disease TNO/AZL Adult Quality Of Life questionnaire was completed to assess worries, symptoms, and the impact of cardiac surveillance. The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage >50 (66%), oxygen saturations >90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). Primary worries related to current health (83%), job/employment (69%), ability to work, (61%) and living independently (54%). The most bothersome symptoms were shortness of breath with ambulation (69%), dizziness (61%), and palpitations (61%). Fontan survivors experience residua and sequelae from multiple surgical procedures and the natural disease course. Our results support the need for ongoing assessment of both physical symptoms and psychosocial concerns, and suggest the need for multiple risk factor intervention strategies that improve physical and emotional health in Fontan survivors.