Challenges Posed by Adults With Repaired Congenital Heart Disease

Perloff, Joseph K.; Warnes, Carole A.

doi:10.1161/01.cir.103.21.2637

Cited by 184 publications

(116 citation statements)

References 48 publications

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“…[1][2][3][4] More than 85% of infants with congenital heart disease are now expected to reach adulthood. 5,6 For those with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, the 40-year survival rate is now at least 90%.…”

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confidence: 99%

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Fox

Devendra

Hart

et al. 2010

CCJM

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When 'blue babies' grow up: What you need to know about tetralogy of Fallot ■ AbstrActMost babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families. ■ KEY POINtsThe major long-term complication of tetralogy of Fallot repair is pulmonary valve insufficiency, which leads to right heart failure. Other problems include atrial and ventricular arrhythmias and sudden cardiac death.Surgical pulmonary valve replacement is the standard of care, but the optimal time to do this is unclear.Novel and experimental therapies include percutaneous pulmonary valve replacement and medical therapy with pulmonary arterial vasodilators.hildren born with tetralogy of Fallot and other congenital heart defects are living longer-long enough for new problems to arise, and, eventually, to present to your clinic. In primary care, the presentation of tetralogy of Fallot is still rare, but it is becoming more common.Congenital heart disease was once solely a pediatric specialty, but adults who have been treated for these conditions now outnumber children with congenital heart conditions. [1][2][3][4] More than 85% of infants with congenital heart disease are now expected to reach adulthood. 5,6 For those with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, the 40-year survival rate is now at least 90%. 5But these former "blue babies" eventually have serious problems. Most develop pulmonary valve insufficiency (regurgitation), which, over time, can result in right ventricular volume overload, enlargement, and dysfunction.7-10 These problems lead to arrhythmias, the most significant cause of illness and death in these patients.11-13 Ventricular and atrial arrhythmias occur in up to 35% of patients with tetralogy of Fallot, and over a follow-up period of up to 30 years the incidence of sudden cardiac death is 6%. 14 Furthermore, because many patients have no symptoms in early adulthood, they are often lost to follow-up, potentially missing the opportunity to have complications treated before they become irreversible. Recent data suggest that most patients who present with symptoms had stopped seeing a cardiologist about 10 years before.15 rEVIEW

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confidence: 99%

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Fox

Devendra

Hart

et al. 2010

CCJM

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“…The presence of these risk factors must be highlighted in the antenatal clinic so that the occurrence of cardiac symptoms (palpitations, chest pain, severe shortness of breath) will prompt early referral to the maternal medicine clinic for assessment and investigation. Women with pre-existing heart disease should be assessed preconceptually and risk factors for cardiac complications should be identified (prior cardiac event, baseline New York Heart Association class >II, cyanosis, left heart obstruction, reduced systemic left ventricular function and significant pulmonary regurgitation) 13,14 and used to guide the intensity of antenatal and postnatal care. The immediate postnatal period is particularly high risk for the development of pulmonary oedema and heart failure and management in critical care settings should be advocated in the presence of pulmonary arterial hypertension, severe obstructive valve lesions and impaired ventricular function.…”

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confidence: 99%

Heart disease – why is maternal mortality increasing?

Gelson

Gatzoulis

Steer

et al. 2009

BJOG

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Please cite this paper as: Gelson E, Gatzoulis M, Steer P, Johnson M. Heart disease -why is maternal mortality increasing ? BJOG 2009;116:609-611. The most recent triennial confidential enquiry confirmed that heart disease is the most common cause of maternal death in the UK. The maternal mortality rate associated with heart disease is 2.27 per 100,000 maternities double that reported in 1990. 1 The incidence of heart disease during pregnancy in the UK has remained constant at 0.9% over several decades. 2 However, the severity of heart disease and the risk it poses during pregnancy appear to be increasing in a wide range of healthcare settings. This commentary examines why mortality rates have risen and makes recommendations as to how this trend can be reversed.Ischaemic heart disease is now the most common cause of cardiac death in the pregnant population in the developed world. This is likely due to increased maternal age; smoking; the adoption of a sedentary lifestyle and poor diet leading to greater rates of obesity, diabetes and hypertension. 3-6 Acute myocardial infarction accounts for the majority of deaths and is most commonly due to coronary atherosclerosis, but coronary artery dissection and consequent occlusion are also relatively frequent. There is a high incidence of recognised risk factors for atherosclerosis in pregnant women suffering an acute myocardial infarction during pregnancy and the postpartum period, which should be targeted both for intervention and for identification. 6 Hyperlipidaemia, hypertension and diabetes should be optimally controlled preconception and smoking cessation and weight loss strongly encouraged. Women with multiple risk factors should be identified, alerting obstetricians to the possibility of ischaemic heart disease and lowering thresholds for the investigation of symptoms. When myocardial infarction is suspected or diagnosed, cardiac catheterisation with acute phase intervention (thrombolysis and/or coronary angioplasty) should not be withheld; these procedures are relatively safe in the pregnancy, 7 although larger studies are required to determine their specific risk to benefit ratios. Following the acute phase, secondary prevention (such as optimal blood pressure control, aspirin and statins [see below]) should be instituted. Many cardiac medications can be used without undue risk in pregnancy, for example beta blockers, digoxin, aspirin, diuretics and hydralazine. ACE inhibitors are known to have teratogenic effects in the first trimester and should therefore best be avoided during early pregnancy. 8 Exposure in the second and third trimesters can lead to marked fetal hypotension and decreased fetal renal blood flow. Where ACE inhibitors must be continued, the lowest possible dose should be used and amniotic fluid levels and fetal growth should be monitored carefully. Statins have been identified as potential teratogens on the basis of theoretical considerations. However, epidemiological data suggest this to be unfounded. Given the scarcity of available data,...

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“…This leads to poor health outcomes and impedes transfer to adult care. [23,24] Therefore, improving transition and transfer are critical to successful long-term disease management and survival. In many cases, in the clinical setting, providers often do not have the time or resources to address the educational and preparatory needs of transitioning adolescents.…”

Section: Introductionmentioning

confidence: 99%

Improving Transitions of Care for Young Adults With Congenital Heart Disease: Mobile App Development Using Formative Research (Preprint)

Lopez¹,

O’Connor²,

King³

et al. 2018

Preprint

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Abstract:Background: Congenital heart diseases (CHDs) are the most common type of birth defects. Improvements in CHD care have led to ~1.4 million survivors reaching adulthood. Thus, successful transition and transfer from pediatric to adult care is crucial. Unfortunately, <30% of adults with CHD successfully transition to adult care; this number is lower for minority and lower socioeconomic status (SES) populations. Few CHD programs exist to facilitate successful transition. Objective: To describe the development of a prototype mobile application (app) for CHD adolescents to facilitate transition.Methods: A literature search regarding best practices in transition medicine for CHD was conducted to inform app development. Formative research with a diverse group of CHD adolescents and their parents was conducted to determine gaps and needs for CHD transition to adult care. As part of the interview, surveys assessing transition readiness and CHD knowledge were completed. Two adolescent CHD expert panels were convened to inform educational content and app design.Results: Literature review revealed 113 articles, of which 38 were studies on transition programs and attitudes and three identified best practices in transition specific to CHD. Adolescents (n=402) participating in semi-structured interviews were 15-22 years old (Median age 16 years), female (42%), and racially/ethnically diverse (12.6% African American; 37.4% Latino. 36.4% received public insurance. Most adolescents (76.7%) had moderate or severe CHD complexity and reported minimal CHD understanding (79.2% aged 15-17 years and 61.5% aged 18-22 years). Average initial transition readiness score was 50.9/100, meaning that transition readiness training was recommended. A subset of participants (n=363) were asked about technology use: 94.5% reported having access to a smartphone. Interviews with parents revealed limited interactions with the pediatric cardiologist with transition-related topics: 79% reported no discussions regarding future family planning, and 55% reported the adolescent had not been screened for mental health concerns (depression, anxiety). Further, 66% reported not understanding how health care changes as adolescents become adults. Adolescents in the expert panel (n=6 total; two groups of n=3) expressed interest in a CHD-specific tailored app consisting of quick access to specific educational questions (e.g., "can I exercise"), a CHD story-blog forum, a mentorship platform, a question and answer space, and a transition checklist to facilitate transition. They expressed interest in using the app to schedule CHD clinic appointments and medication reminders. Based on this data, a prototype mobile application was created to assist in adolescent CHD transition.Conclusions: Formative research revealed that most adolescents with CHD had access to smartphones, were not prepared for transition to adult care, and were interested in an app to facilitate transition to adult CHD care. Understanding their needs, interests, and concerns will lead to the de...

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Challenges Posed by Adults With Repaired Congenital Heart Disease

Cited by 184 publications

References 48 publications

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Heart disease – why is maternal mortality increasing?

Improving Transitions of Care for Young Adults With Congenital Heart Disease: Mobile App Development Using Formative Research (Preprint)

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