Risk factors and early outcomes of repeat sternotomy in 1960 adults with congenital heart disease: A 30-year, single-center study

Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon?

The population of adults with congenital heart disease (ACHD) has increased dramatically over the past few decades, with many people who are now middle-aged and some in the geriatric age range. This improved longevity is leading to increased use of the medical system for both routine and episodic care, and caregivers need to be prepared to diagnose, follow up, and treat the older adult with congenital heart disease (CHD). The predictable natural progression of CHD entities and sequelae of previous interventions must now be treated in the setting of late complications, acquired cardiac disease, multiorgan effects of lifelong processes, and the unrelenting process of aging. Despite the advances in this field, death rates in the population from 20 to >70 years of age may be twice to 7 times higher for the ACHD population than for their peers.1 This American Heart Association (AHA) scientific statement will focus on the older adult (>40 years old) with CHD. It is meant to be complementary to the 2008 American College of Cardiology (ACC)/AHA guidelines for ACHD and orient the reader to the natural history, ramifications of childhood repair, and late initial diagnosis of CHD in the older adult. This population with CHD is unique and distinct from both the pediatric and young adult populations with CHD. Much of the information we provide is from scientific research combined with clinical experience from longitudinal care. We emphasize that this is the beginning of a discussion regarding this rapidly growing population, and continued research aimed at the progression of disease and complications reviewed here is necessary to advance the field of ACHD with the scientific rigor it deserves. ACHD encompass a broad range of presentations. There are people who are diagnosed for the first time in adulthood, as well as those with prior palliative repair …

Context: There are very few data about the unrepaired congenital heart disease (CHD) in adults. There are high chances of errors in diagnosis of CHD in adult population due to various reasons. If proper diagnosis is made, many of these CHDs are amenable to surgery leading to improved quality of life. Aims: Primary objective of the study was to know the detection rate of unrepaired CHD in adults. We have also reported common errors done while making the diagnosis of CHD in adults. Subjects and Methods: This was a retrospective observational study conducted from March 2020 to September 2021. Patients more than 18 years old who came for transthoracic echocardiogram (TTE) for any reason were included, and the detection rate of unrepaired CHD was calculated. Simultaneously, we have also checked for any errors in reports done outside our center and classified them based on their effect on management. Results: During the study period, 6675 TTEs were done in the study population, out of which 89 (1.33%) were detected with unrepaired CHD. Acyanotic CHD was detected in 81 (91%), whereas the rest 8 (9%) had cyanotic CHD. Forty-one patients had echocardiography report done outside, out of which 25 (61%) had discrepancies. Discrepancies were more in complex CHD. Conclusions: Cardiologists or echocardiographers should follow systematic approach and place special emphasis on identifying congenital heart diseases. Many potential lesions are amenable to surgery and near-complete correction leading to improved duration and quality of life.

Delayed Sternal Closure vs Emergency Sternal Reopening in Adults With Congenital Heart Disease

1. The problem - an overview.- 2. Pulmonary vascular disease in congenital heart disease in adults.- 3. The adolescent and adult with complex congenital heart disease: which patients should offer surgery?.- 4. Transoesophageal echocardiography in adolescents and adults with congenital heart disease.- 5. Magnetic resonance imaging.- 6. Interventional cardiac catheterization for primary or residual congenital cardiac lesions.- 7. Pulmonary atresia.- 8. The Fontan circulation.- 9. Non-invasive assessment of the Fontan circulation.- 10. Supraventricular arrhythmias in congenital heart disease.- 11. Ventricular arrhythmias after repair of congenital heart disease: who needs treatment?.- 12. Infective endocarditis: treatment and prophylaxis.- 13. Surgery for congenital heart disease in adults.- 14. Cardiac transplantation for congenital heart disease in adolescents and adults.- 15. Congenital heart disease in adolescents and adults: obstetric-gynaecologic counseling.- 16. Psychosocial aspects of congenital heart disease in adolescents and adults.

British Journal of Cardiac NursingVol. 6, No. 2 Policy UpdateAdult congenital heart disease: The nurse specialist’s roleSheena Vernon, Melanie Finch, Joy LyonSheena VernonSearch for more papers by this author, Melanie FinchSearch for more papers by this author, Joy LyonSearch for more papers by this authorSheena Vernon; Melanie Finch; Joy LyonPublished Online:27 Sep 2013https://doi.org/10.12968/bjca.2011.6.2.88AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References American College of Cardiology (2008) Guidelines for the Management of Adults with Congenital Heart Disease: executive summary. Circulation 118: 2395–451 Crossref, Google ScholarBritish Cardiac Society Working Party (2002) Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. 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Heart 85: 438–43 Crossref, Google Scholar FiguresReferencesRelatedDetailsCited byThe role of the nurse in adult congenital heart disease: past, present and futureMaggie Simpson, Emma Weingart, Jane Lewis, Toni Hardiman, Antonia Docherty, David Rogers, Anna Dinsdale23 June 2022 | British Journal of Cardiac Nursing, Vol. 17, No. 6Infective endocarditis in the case of a patient with transposition of the great arteriesHajar Habibi, Yaso Emmanuel, Natali Chung6 December 2018 | British Journal of Cardiac Nursing, Vol. 13, No. 12Use of 3D models of congenital heart disease as an education tool for cardiac nurses26 September 2016 | Congenital Heart Disease, Vol. 12, No. 1Emotional Support and Well-Being of Adults with Congenital Heart Disease (ACHD): Contributions of a Specialist Nursing ServiceOpen Journal of Nursing, Vol. 05, No. 12Pregnancy in a patient with coarctation of the aorta: a case studyHajar Habibi, Amit Bhan, Natali Chung13 November 2014 | British Journal of Cardiac Nursing, Vol. 9, No. 11Tetralogy of Fallot, late outcomes and quality of lifeHajar Habibi, Juliet Jaikumar17 January 2014 | British Journal of Cardiac Nursing, Vol. 8, No. 12Paediatric cardiac surgery changesJenny Tagney27 September 2013 | British Journal of Cardiac Nursing, Vol. 6, No. 3 1 February 2011Volume 6Issue 2ISSN (online): 2052-2207 Metrics History Published online 27 September 2013 Published in print 1 February 2011 Information© MA Healthcare LimitedPDF download

Heart failure admissions and poor subsequent outcomes in adults with congenital heart disease.

The Rhythmia™ system (Boston Scientific, Natick, MA, USA) facilitates the rapid acquisition of high-resolution electroanatomical and activation maps. However, there are limited data on its efficacy and safety in pediatric and adult congenital heart disease (CHD) patients. In a retrospective, observational cohort study, adult CHD and pediatric patients followed by pediatric cardiology underwent electrophysiologic study using the Rhythmia™ electroanatomic mapping system. Variables examined included the number of electroanatomical maps required, acquisition time, procedure time, fluoroscopy time, radiation dosage, and rate of recurrent arrhythmia. Twelve consecutive patients, including six male patients (50%), were included with an average age of 27.7 years (range: 11–64 years). Seven (58%) of these patients had a diagnosis of CHD [moderate complexity in two (17%) and great complexity in five patients (42%)] and 10 (83%) patients underwent ablation. A total of 37 high-density maps were created in 12 procedures, with a median of 8,140 mapping points, taking a median of 631 seconds. The median procedure time was 189.5 minutes. The median fluoroscopy time was 0.9 minutes, with eight (67%) patients receiving no fluoroscopy at all. Recurrence occurred in one patient (8%) over a median follow-up duration of 16 months (interquartile range: 12.8–17.3 months). No adverse periprocedural events were recorded. This study suggests the use of high-density electroanatomic mapping in adult CHD patients showed potential for rapid acquisition of highly detailed maps with minimal fluoroscopy time or risk of periprocedural events in the studied population.

The diagnosis of congenital heart disease de novo in adults is now uncommon. Increasing numbers of children with congenital heart disease are surviving to adulthood, leading to the development of...

Congenital heart disease (CHD) in low-and-middle income countries (LMIC) is often characterized by late presentation resulting from inadequate screening and healthcare access in these regions. Accurate estimates of the burden of CHD among school children are often lacking. The objective of this study was to determine the prevalence and distribution of CHD among school children in two communities (urban and semi-urban) in south western Nigeria. Using clinical assessment and portable echocardiography, 4107 school children aged 5 years to 16 years in Lagos, Nigeria, were selected using a multistage sampling procedure and screened for CHD. Diagnosis of CHD was made after echocardiography. Children identified with CHD were referred to a tertiary hospital for appropriate cardiac care. The 4,107 children screened had a mean age of 11.3 ± 2.7 years and 53.7% were females. Twenty seven children had echocardiography-confirmed CHD, representing a prevalence of CHD among school children in Lagos, Nigeria of 6.6 per 1000 children. Acyanotic CHD constituted 96.3% of detected cases. Two children diagnosed with CHD (Tetralogy of Fallot and severe pulmonary valve stenosis respectively) had successful intervention. The prevalence of previously undiagnosed CHD among school children in Lagos Nigeria is substantial and highlights gaps in the health care system and school health programs. Echocardiographic screening of school children provides an opportunity for missed early diagnosis and treatment of CHD and reduces the prevalence of first-diagnosed CHD in adulthood. Therefore, focused clinical examination of school children followed by echocardiography is a strategy that could bridge this diagnostic and treatment gap in CHD.

Introduction: With improved management, there are now more adults with congenital heart disease (ACHD) than children. However, long-term survival with moderate or severe ACHD remains limited, and there is relatively sparse literature on the intensity or quality of end-of-life care for these patients. The goal of this analysis is to examine the accuracy of administrative data for identifying patients who died with ACHD to facilitate study of care provided near the end of life. Methods: We created a list of ICD-9 and ICD-10 codes representing ACHD of moderate or great complexity. We performed a search for these codes in the electronic health record (EHR) of adults who received care 2010-2016 within our healthcare system. We used state death records to identify which of these patients died during the same timeframe. Manual EHR review was completed to evaluate performance of this search strategy. Identified patients were also compared to a list of patients seen in our ACHD clinic and known to have died during 2010-2016. Results: Using ICD data, 121 patients were identified, of which 66 actually had the moderate or greater complexity ACHD conditions by EHR review (positive predictive value, 0.55; 95% confidence interval 0.45, 0.63). EHR review confirmed 12 patients with Eisenmenger Syndrome, for which there is no specific ICD code. “Cyanosis+other” did not identify any of these, “VSD+other” (ventricular septal defect) identified 6, and there were 6 whose only ACHD code was VSD. Of the remaining 55 patients, 24 had ACHD not on the targeted list, largely due to coding error. In addition, despite being coded as having ACHD, 31 patients had no identified ACHD on EHR review. These misidentifications were attributed to coding error for 15 patients. Another 11 patients (35%) had acquired VSD due to myocardial infarction or endocarditis, for which there is no separate ICD code. Codes with the highest degree of error, incorrect more than 50% of the time, were those for congenitally corrected transposition, endocardial cushion defect, and hypoplastic left heart syndrome. The list of known deceased clinic patients included 21 with ACHD of interest. Only 1 of these was not identified by the ICD search, yielding a sensitivity for our list of ICD codes in this small sample of 0.95 (0.77,0.99). Conclusion: Use of administrative data to identify patients with ACHD of moderate or great complexity who have died had good sensitivity but suboptimal positive predictive value. Strategies to improve accuracy can be employed. Excluding patients who have codes for myocardial infarction or endocarditis in addition to VSD and using “VSD+other” as an additional proxy for Eisenmenger Syndrome are two examples. Administrative data is not ideal for identification of patients with ACHD of moderate or great complexity who have died, and manual EHR review is necessary to confirm these diagnoses.

Geriatric Congenital Heart Disease: Burden of Disease and Predictors of Mortality

Background Despite recent preventive strategies and advances in antimicrobial and surgical treatment the incidence and mortality rate of infective endocarditis (IE) remains high in adult Congenital Heart Disease (ACHD) patients. Purpose The aim of our retrospective study was to assess the clinical and microbiological characteristics, inhospital and one year mortality rates of ACHD patients admitted with IE in our tertiary referral hospital between 2010 and 2020. Methods Definitive diagnosis of IE were in agreement with modified Duke's criteria. Inhospital baseline data and up to one year follow-up data were collected from the hospital records. ACHD patients were classified based on their lesion severity (ESC guideline classification). Survival data and mortality predictors were analysed by Kaplan-Meier estimator and by uni- and multivariate model. Results 60 cases had been treated with IE. Mean age was 37±11.3 years (18–69), and most of them (76.6%, n=46) were men. ACHD complexity was as following: simple 66.7% (n=40), moderate 20% (n=12) and severe 13.3% (n=8). The most common pre-existing congenital cardiac abnormality was bicuspidal aortic valve seen in 52% (n=31) of cases. Sixty four percent (n=37) of patients had previous heart surgery (n=16, artificial heart valve surgery). Majority of patients had a left sided valve endocarditis (85%, n=51). IE related hospital stay was mean 28±9 days. Infection route was presumed in 30% of patients (n=18), they had an invasive procedure within 6 months preceding the IE diagnosis. Besides common bacterinemia caused by Staphylococcus (33%, n=20) and Streptococcus (25%, n=15), 18% (n=11) of patients had a negative blood culture. IE affected 36 patients with a native valve, and all 16 with an artificial valve. Seventy seven percent of patients required surgical intervention, immediate or urgent surgery in 22 and elective in 24 patients. Overall inhospital mortality was 13%, the majority 5 out of 8 patients having an immediate/emergent surgical intervention. IE was complicated by acute heart failure in 17 (28%) and by stroke in 4 (6.7%) patients. During one year follow-up 21 patients (38%) remained with chronic heart failure, and 3 more died. There was no significant difference in mortality between ACHD complexity groups, and no significant predictors of mortality were found. Conclusion The most common ACHD lesion affected by IE was bicuspid aortic valve. Two-third of patients required surgical intervention, those with immediate/emergent surgery having a 22% early mortality rate. ACHD complexity was not related to early or late outcome in this population. Funding Acknowledgement Type of funding sources: None.

Purpose of the study. Evaluation of the reasons for the untimely diagnosis of congenital heart disease (CHD) in adults and elderly patients. Material and methods. 30000 people over the age of 25 who underwent transthoracic and transesophageal echocardiography were examined. Results CHD for the first time were revealed in 417 of 30000 patients (1,39%) including 41 of 5346 patients over 60 years of age (0,77%). The most often CHD was atrial septal defect (ASD) – 179 patients, and ventricular septum defect (VSD) was significantly less diagnosed (48 cases). In 98 cases, an aortic valve bicuspidy was identified with hemodynamically significant aortic valve stenosis and / or insufficiency. Coarctation of the aorta was detected in 25 patients, in 16 cases it was combined with a bicuspid aortic valve. The diagnosis of Ebstein"s anomaly (AE) was established in 16 patients, patent ductus arteriosus (PDA) in 15 patients, isolated pulmonary artery stenosis - 12, congenitally corrected transposition of the great arteries (CCTGA) - 6, tetralogy of Fallot - 5, congenital aneurysm of the sinus of Valsalva (CASV) - 5, congenital aneurysm of the ascending part and the arch of the thoracic aorta - 4, Lutembacher syndrome - 2, congenital mitral insufficiency - 2, mesocardia - 2 cases. In patients with CHD over 60, secondary ASD prevailed (28 observations); less frequently were discovered VSD (4), CCTGA (3), CASV (4), PDA (1), AE (1). Conclusion Untimely detection and difficulties in primary diagnosis of CHD in adults and elderly patients can be due to established diagnostic stereotypes, when the results of clinical observation and instrumental examination were interpreted in favor of manifestations of coronary artery disease, arterial hypertension, chronic heart failure, acquired valvular heart disease, cardiomyopathies etc. In addition, overestimation of concomitant age-related structural and functional changes of the heart and improper medical monitoring may be important.

The aim of this study was to assess the age-standardized prevalence (prevalence) and age-standardized mortality (mortality) of congenital heart disease (CHD) by sex in Korean adults. Data were collected from the National Health Insurance Service in Korea from 2006 through 2016. The data consisted of main and secondary diagnoses related to CHD. We calculated the prevalence and mortality of CHD in adults with the direct method using the estimated Korean population in 2015 as the reference. The prevalence of CHD in Korean adults increased from 35.8 cases per 100,000 persons in 2006 to 65.6 cases in 2015. In 2015, the prevalence in the 20- to 44-year-old group, 45- to 64-year-old group, and the older than 65 years group was 54.6, 69.6, and 95.1 cases, respectively. Among women, the prevalence was 34.3 and 31.3 cases in men. The mortality of CHD in adults decreased from 3.061 persons per 100,000 persons in 2007 to 0.551 persons in 2015. The 5-year survival rate (SR) for people with CHD was 0.92 (95% confidence interval [CI] 0.91-0.93). The 5-year SR for the 20- to 44- and 45- to 64-year-old groups was more than 0.95; however, the 5-year SR of the older than 65 years group was only 0.73 (95% CI 0.72-0.74). The prevalence of CHD in Korean adults increased and the related mortality decreased during a decade. The 5-year SR of CHD for those over 65 years was lower than that for younger age groups.