Sexual Health and Well-Being in Adults With Congenital Heart Disease

As health care outcomes improve the priority for those living with adult congenital heart disease have changed to a more holistic focus on quality of life and well-being. Although health care has embraced this, there are still areas where there is a deficit in advice, allyship, and advocacy. One of these deficits is in the area of sexual health and well-being. A healthy sexual life has a myriad of physical and psychosocial benefits. However, individuals with adult congenital heart disease may have significant barriers to achieving well-being in this aspect of their lives. These barriers and their potential solutions are outlined in this paper.

T he lifestyle implications of living with a congenital heart lesion are routinely discussed in specialist adult congenital heart disease (ACHD) clinics. 1 There is, however, one lifestyle issue that is often overlooked: advice and support for a fulfilling sexual life.This is not just a deficit in ACHD care but is particularly important for young individuals with a complex, lifelong cardiac condition.[4] A reduction in mortality has also been reported. 5e clinical team, therefore, has an important role in minimizing the barriers patients experience in achieving sexual well-being.
All health care providers (HCPs) should have knowledge in sexual health, sexual orientation, and gender as it relates to health.There should be a high level of sophistication in the use of language and inclusivity.Sexual health is a complex interaction of health, beliefs, culture, values, and emotions.For some individuals with ACHD and HCPs, previous sexual trauma may also cloud this issue. 6The HCP should be mindful of this and recognize when patients need additional support beyond their own expertise.
The term sexual dysfunction is defined in this paper as the inability to experience sexual arousal or to achieve sexual satisfaction under appropriate circumstances.This dysfunction includes difficulties related to sexual desire, arousal, orgasm, and sexual pain.
The aim of this paper is to outline the psychological and physical barriers to sexual well-being for those living with ACHD.The paper will also signpost readers to resources that may help in the discussion and treatment of these challenges.(Table 1).The health care environment has usually framed health through a heteronormative lens, and LGBTQIAþ issues have been largely overlooked. 7The health care team needs to be mindful of this bias.

SEXUAL BEHAVIOR IN PEOPLE LIVING WITH ACHD SEXUAL HEALTH
LITERACY.Previous studies addressing sexual health in ACHD have focused on knowledge deficits. 8Despite different cultural beliefs and counseling practices around the world, these knowledge gaps are encountered internationally.
Most studies describe health literacy around pregnancy and contraception. 9Women with congenital heart disease (CHD) described their knowledge in these areas as 5 out of 10; they describe their knowledge around sexuality as a median of 3.5 out of 10. 10 Across multiple studies, 35 to 59% of women with CHD could not recall being counseled about these subjects. 11,12yond pregnancy and contraception even fewer studies exist.In Korea, ACHD patients scored lower on the Knowledge in Safe Sex Practice scale, especially those with complex cyanotic conditions. 13rstrom et al. identified that only 70% of adolescents with CHD could correctly identify whether sexual activity (SA) could worsen their heart Swan et al condition. 14Only 22 to 50% knew that SA was not a risk factor for endocarditis. 15,16XUAL BELIEFS AND PRACTICES.Data on SA, relationships, and safe sex practices is scarce and not uniform in its findings.Some studies suggest that people with ACHD exhibit similar sexual practices to their healthy peers.In an interview series, people living with CHD showed no evidence of avoiding SA and overall engaged in "normal" dating with the exception of those with physical limitations. 17Other studies endorsed these findings showing no difference in the likelihood of being sexually active, age of first intercourse or rates of sexually transmitted infections. 18This is consistent with data on adolescents A subset did, however, report anxiety and distress. 19ntrasting studies suggest avoidance and/or distress related to dating and sexual experiences.
However, once a relationship is established, it is often fulfilling.Other studies describe people with CHD having fewer romantic partnerships. 20,21But again, people with ACHD, once in a relationship, perceived this as more satisfying.Insecurities regarding body image and distress during sex are also reported. 22Fry et al. encountered lower rates of ever having had sexual intercourse among adolescents with CHD. 23id et al. also described this, but also reported that a high proportion were engaged in potentially risky sexual behaviors, including having multiple partners, inconsistent condom/contraceptive use, and/or concomitant use of alcohol and drugs. 24e mixed data surrounding sexual behaviors in Serious illness is known to negatively affect body confidence.Living with a chronic health condition and facing periodic health crisis can impact a sense of trust in one's body. 33Cardiac rehabilitation programs for people with CHD may go some way to rebuild body confidence and fitness.
MENTAL HEALTH DIFFICULTIES.5][36] Shared disadvantages with other minority groups including discrimination and ableism, disparities in income, education, employment, and underrepresentation in the media and politics. 37,38l of this considered, perhaps it is not surprising that the lifetime prevalence of depression, anxiety, and post-traumatic stress disorder for adults with CHD is 50%. 39These, in turn, can cause sexual dysfunction that can also be aggravated by psychotropic medications.When this is recognized, referral to psychological and psychosexual therapy can be very beneficial.Improved screening, recognition, and support for underlying mental health difficulties are vital to sexual health.
SEXUAL ABUSE RISK ASSESSMENT.It is important for HCPs to be aware that people with disabilities are at significantly increased risk of sexual violence and intimate partner violence.This increased risk is also associated with poorer health status and limited access to health care. 40

POSITIVE PSYCHOLOGICAL
HEALTH.Protective factors for psychological health include less parental overprotection, more affection during childhood, social acceptance 41 and disability pride. 37Many people who live with a disability report a high quality of life often in contrast to how they are perceived by others, known as the "disability paradox". 42Well-being is complex, and every person with CHD is unique, but promoting protective factors and mitigating risk factors is likely to optimize health.
Many people with CHD have positive adaptation such as having more meaningful relationships and increased resilience.This post-traumatic growth can be built by promoting empowerment and health literacy.It is essential that HCPs are skilled in confidently discussing sexual health with patients and create an environment conducive to them sharing their concerns.

PHYSICAL FACTORS AFFECTING SEXUAL FUNCTION
other single study demonstrated a 3-fold risk for erectile dysfunction in young adult CHD males on beta blocker and/or angiotensin receptor inhibitor therapy 47 but this finding has not been consistent in other studies.Endothelial dysfunction has also been implicated in sexual dysfunction.
Menstrual dysfunction affects 83% of young adult women with CHD although only 20% seek medical support. 48This includes menstrual cramping, irregular menses, and heavy menses.Delayed menarche is also common especially in those with complex CHD such as those with a Fontan circulation.There is a 3-fold risk of menorrhagia in women on anticoagulants, and this should be pro-actively discussed when prescribing these agents.
Swan et al course results in a modest but brief increase in myocardial oxygen demand. 49ough several studies look at the cardiovascular response of SA, these are limited by methodology.
Initial studies were performed in the laboratory setting. 50,51These studies suggested that SA had a high workload with peak heart rates of 140 to 180 beats/min and a mean increase in blood pressure of 80/50 mm Hg during orgasm.Subsequent studies of married heterosexual couples who were monitored in their own homes revealed less dramatic changes. 52,53 1 study, mean heart rate at the time of orgasm was 117 beats/min.A mean blood pressure of 162/89 mm Hg was estimated from equivalent levels of exercise, similar to values observed in other studies.
The risk of sudden cardiac death during SA is small with SA implicated in only 1.5% of cases.In an autopsy study, the annual incidence of sudden cardiac death during SA was 0.16 per 1,000 autopsies for women and 1.9 per 1,000 autopsies for men. 54   LGBTQIAþ (Gallup, CDC Vital Statistics, Williams Institute) over the past decade.As of 2020, 7% of the population and as high as 20% of Gen Z individuals (born between 1997 and 2002) identify as LGBTQIAþ with approximately 0.6% of the population identifying as transgender. 60These studies have a significant bias because they were conducted in western industrialized nations where there is growing social, cultural, and legal acceptance of LGBTQIAþ people.
In many societies, LGBTQIAþ individuals still experience marginalization, discrimination, persecution, and violence.
Despite recent advances, such as the 2020 AHA statement, there is still limited research into the health determinants of this population. 61This is despite the knowledge that, for example, in the United States alone over 200,000 LGBTQIAþ people, including over 14,000 transgender individuals, living with ACHD.
LGBTQIAþ individuals are also more likely to experience other challenges to their well-being including an increased risk of mental health conditions, an increased risk of suicide, higher rates of tobacco and substance abuse, higher rates of poverty, and higher risks of sexual violence. 62 LGBTQIAþ individuals with ACHD may also have other health conditions that they do not wish to disclose but may affect their cardiac disease.Human immunodeficiency virus infection and its treatment can be associated with cardiac complications.In some countries, the treatment for human immunodeficiency virus and other sexually transmitted diseases are provided outwith the usual health care settings, and therefore the ACHD HCP may be unaware of the potential for drug interactions or other complications.
Again, an open and safe environment will increase the opportunities for disclosure.Therefore, every effort should be made to facilitate gender-affirming care. 64rgical transition may involve chest/breast, genital, or facial reconstruction, as well as electrolysis or laser hair removal.The ACHD health care team can facilitate this in the same way as for other noncardiac surgical procedures.ISACHD will produce a subsequent paper to more fully explore the care of nonbinary and transgender individuals with CHD in conjunction with gender health physicians.
Swan et al

CULTURAL CONSIDERATIONS
When patients with CHD report a lack of knowledge on sexual intercourse, fertility, and pregnancy, this needs to be done in a culturally competent way.
Improving cultural competence among health care workers improves health care quality for culturally and ethnically diverse groups.This results in improved patient satisfaction, treatment adherence, and information seeking. 65In many cultures, discussing SA and menstruation are taboo.This can result in complications such as poor reproductive health, poor pregnancy outcomes, and anemia.In communities where sexual relations outside of heterosexual marriage are condemned, this inhibits open discussion and can lead to poor access to contraception and unplanned pregnancies.
One of the major cultural differences that needs to be recognized is the view of personal autonomy.In many cultures, there is a family-centered care model 66 where the head of the family oversees major health decisions.This lack of personal privacy and bodily autonomy adds complexity to these discussions.One study from Pakistan 67 revealed that a majority of parents and teachers thought that ageappropriate sexual education was against religious values, and while education on sexual abuse prevention was largely supported, education on pregnancy prevention was not encouraged.Lack of access to education for girls is also associated with poor reproductive female autonomy 68 and lower use of effective contraception.These issues, while affecting the general population may have an incrementally detrimental effect on those with CHD.
Health policymakers and HCPs should find solutions to address cross-cultural differences in sexual health.HCPs should assess the level of autonomy the patient has toward their reproductive health and gauge what concerns them.This level of autonomy will help HCPs take into confidence other people such as partners or even parents.If there is a hesitation by females to express their concerns to a male physician, they should be redirected to a female HCP if possible.

INDIVIDUALS WITH LEARNING DIFFICULTIES
Although people with CHD attain an average score on intelligence testing, there is growing evidence suggesting specific neurocognitive deficits including gross and fine motor skills, attention, visuospatial ability, speech and language, executive function, social cognition, and impulse control, which may impact sexual health and behavior.
Sexual health is an important determinant of quality of life, including for people with intellectual disabilities.However, these individuals, despite having similar needs for sex and intimacy, have lower levels of knowledge about sexual health. 69Disabled persons score lower on sexual knowledge of puberty, reproduction, and sexually transmitted infections.
Johoda et al. reported only 1% of patients with intellectual disability in their study had received sexual education. 70As a result, these individuals may participate in high-risk behaviors. 71, have an increased vulnerability to sexual abuse and engagement in inappropriate sexual behavior. 72In addition, individuals with intellectual disability may be unable to report a medical condition, which may appear as a problematic sexual behavior to others.Thus, recognition and acknowledgment of the importance of sexual health and education for both the individual and their parents/caregivers are required to empower individuals to develop their sexuality.A crucial issue to address during these conversations is, of course, the individual's capacity to consent. 73lidated tools exist for professionals to assess sexual attitudes, experiences, and needs. 72 ACHD. 21A reluctance to broach sexual health may result from attitudinal barriers and assumptions about sex and disability. 74The imbalance of power between patient and doctor can also inhibit communication.
The ACHD team has an opportunity to develop a model where psychological safety and trust are communicated through a compassionate approach to care.A named point of contact may improve ease of accessing care (Figure 2). 75Normalizing these discussions is essential.Some understanding of trauma informed care will also reduce the risks of difficult consultations or retraumatizing individuals who are vulnerable in this area (Table 3).Again, making assumptions about individuals' wishes may be detrimental.For many individuals, sexual well-being is not an important part of their lives and medical concerns.In conclusion, as the outcome for individuals with ACHD improves, the focus of care needs to move from reducing mortality to facilitating well-being in its fullest sense.Providing the resources for HCPs and those living with CHD to be able to discuss sexual well-being is another step toward a holistic approach to quality of life (Figure 3).
ACKNOWLEDGMENTS The authors thank those who consented to include their stories and experiences and they thank the Somerville Heart Foundation charity and Dr Liza Morton for facilitating this.The authors also thank Mr Michael Cumper, who we sadly lost last year, for the original idea for this paper.

FUNDING SUPPORT AND AUTHOR DISCLOSURES
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Female, 59 years old, Tetralogy of Fallot
As a young woman I struggled finding contracepƟon that worked for me…..so unlike my more carefree peers, I was preƩy choosy about who I spent Ɵme with.
Female, 59 years old, Tetralogy of Fallot For several months I was prescribed a beta blocker and there was no menƟon of possible reduced sexual response as a side effect.We hear a lot about erecƟle dysfuncƟon, but sexual response in women is overlooked.

Female, 59 years old, Tetralogy of Fallot
Sexual acƟvity with an ICD was very scary at first, and it made me feel old beyond my years.Even just finding a comfortable posiƟon has taken some experimentaƟon….it takes an understanding partner to make things work out…there was no discussion or informaƟon about sexual acƟvity when I had it implanted

FIGURE 1 HIGHLIGHTS
FIGURE 1 Lived Experience Quotes

2 0 2 3 : 1 0 0 7 1 6
Sexual Well-Being in ACHD Given the high prevalence of sexual dysfunction in individuals with CHD clinicians should proactively inquire about symptoms.The cardiac specialist's role will include a full cardiovascular assessment focusing on possible cardiac cause.Specific interventions include reviewing medication-for example, optimizing heart failure medication-and, if possible, changing medications known to be associated with sexual dysfunction.In women presenting with menorrhagia, the requirement and control of anticoagulation should be reviewed.In older patients with new erectile dysfunction, the possibility of CENTRAL ILLUSTRATION Challenges to Sexual Health and Well-being for Those Living With Adult Congenital Heart Disease Swan L, et al.JACC Adv.2023;2(10):100716.Swan et al J A C C : A D V A N C E S , V O L . 2 , N O . 1 0 , 2 0 2 3 Sexual Well-Being in ACHD D E C E M B E R 2 0 2 3 : 1 0 0 7 1 6 superimposed atherosclerotic disease should also not be forgotten.In the younger patient with a Fontan circulation, scrotal edema and hydroceles may be signs of Fontan failure.Sexual dysfunction may also be a sign of health deterioration and overall frailty, and in these circumstances, it may be warning of future adverse events.The importance of lifestyle modification is also key in optimizing sexual health.Normalizing body mass index, exercising, and reviewing blood pressure, blood glucose, and lipid profile should be part of this assessment.However, there should not be a barrier to patients being referred to urology, gynecology, and endocrine specialists.Comprehensive expert assessment is required before sexual dysfunction is attributed to the cardiac condition per se, and even then, patients should have access to the full range of treatment options.CARDIOVASCULAR RISKS OF SEXUAL ACTIVITY SA can be a cause of worry for individuals with heart disease.Several hemodynamic changes occur during SA, reaching a maximal effect upon orgasm.It has been determined that SA during the preorgasmic phase is equivalent to 2 to 3 metabolic equivalent of tasks (METs) of exertion, increasing to 3 to 4 METs during orgasm.This would be the equivalent of walking briskly on the flat or climbing stairs.With the data available, it can be concluded that sexual inter- Sexual Well-Being in ACHD thoughtful alliances with ACHD providers to avoid medical misadventures and to enable them to live healthy and fulfilling lives.Though LGBTQIAþ people are often grouped together, subgroups have distinct considerations when interfacing with congenital heart specialists.Surveys and studies demonstrate an increasing number of individuals who identify as Many individuals are reluctant to share their sexual orientation or gender identity with HCPs out of fear of misunderstanding, maltreatment, or simply out of wanting to avoid making the provider feel uncomfortable.Many LGBTQIAþ individuals are very adept at code switching to blend in with cis-gender heterosexual people.ACHD providers have a special opportunity, given the regularity of clinic visits and the longer-term therapeutic relationships, to intervene in specific ways that can help the LGBTQIAþ patient live a healthier life and promote their sexual satisfaction.There are simple steps HCPs can take to provide a safe environment for LGBTQIAþ patients.Avoiding assumptions when asking about romantic relationships, sexual behavior, and plans for having children and adopting a gender-neutral language can allow individuals to express a preference for their pronouns, discuss their gender identity, or disclose their sexual orientation.For example, asking an individual about a significant relationship removes the gender implications of asking someone if they have a boyfriend or girlfriend.Furthermore, some electronic health record interfaces enable patients to self-report their sexual orientation, gender identity, and preferred pronouns in nonthreatening interfaces.
Nonbinary and transgender individuals encounter many obstacles in obtaining compassionate and competent medical care including legal barriers to gender recognition.Individuals with ACHD have an added layer of complexity when seeking medical or surgical gender-affirming therapies.Transitioning is a very personal and individualized process that usually starts with social transition.Individuals may then seek medical transition, which involves administration of exogenous hormones or hormone-modifying agents.Transgender male patients seeking medical transition are administered testosterone.This does not appear to increase cardiovascular risk though long-term studies are limited.However, testosterone can interacts with several cardiac medications including anticoagulants, SGLT2 inhibitors, betablockers, and tolvaptan. 63Transgender female patients seeking medical transition are typically given estrogen and antiandrogen therapy.This often includes high-dose spironolactone or GnRH agonists.Estrogen therapies are associated with an increased risk of thrombosis, which may be challenging in those with ACHD.The risk of thrombosis can be reduced by using nonoral preparations.Despite concerns about increased risk of cardiovascular and thrombotic risks, suicide remains the leading cause of death in transgender individuals, which has been shown to decrease with gender-affirming hormone therapy.
Social and sexual education programs starting before adolescence and extending into young adulthood have proven beneficial in this population.HOW TO DO THINGS BETTER.Normalizing discussions about sexual health.Lack of medical training in sexuality contributes to sexual health being poorly addressed for many people with chronic health problems.In 1 study, individuals with ACHD scored physicians an average of 2/10 on being informed about their experience of sexuality in relation to their Sexual Well-Being in ACHD T r a n s i t i o n .Health care transition is the process of gaining independence with managing one's own medical care and integrates 2 objectives: the transfer of medical care from pediatric to adult health care professionals and the transfer of medical responsibility from parent to child.76Transition coincides with adolescence already a period marked by psychosocial concerns, sexual development, an increase in risk-taking behaviors, and disengagement with health care services.For these reasons, transition should be seen as an opportunity to engage in preventative health care including discussions centered on sexual and reproductive health.Although not the sole focus of a sexual health history, it is important that adolescents are given the information they need to make healthy choices regarding contraception.A comprehensive transition program empowers and equips adolescents with the tools to discuss scars, body image, and other components important to them in developing a healthy approach to sex and relationships.E d u c a t i o n a l r e s o u r c e s .In some circumstances, patients may feel uncomfortable about accessing information on sexual health directly from their ACHD provider.The use of a curated collection of websites and written material may be less challenging.As with pregnancy and contraception, ACHD services should be producing high-quality, inclusive, and cardiacspecific information.

E x p e
r t r e f e r r a l .As previously stated, the ACHD team often serves as the gateway to other specialty services including gynecologist, urologist, endocrine team, and psychology.Many barriers to sexual wellbeing are treatable, and all individuals, including those with ACHD, should not be denied a full assessment and comprehensive care plan.Not all referrals to 'experts' relate to health care professionals.The importance of utilizing peer-to-peer support, patient groups, and charities should not be underestimated.A d v o c a c y .There has been a tidal shift in sexual health policy over the last decade.Most important is

FIGURE 2
FIGURE 2 Practical Advice for Those Living With ACHD

J 3
A C C : A D V A N C E S , V O L . 2 , N O . 1 0 , 2 0 2 Sexual Well-Being in ACHD D E C E M B E R 2 0 2 3 : 1 0 0 7 1 6the linking of sexual and reproductive health rights (SRHR) to human rights.77It is now accepted that sexual health goes beyond pregnancy and contraception, which many ACHD guidelines have focused on, in favor of a more holistic definition as a "state of complete physical, mental, and social well-being in all matters relating to the reproductive system".SRHR is too often framed as a women's issue.Modern definitions acknowledge men's needs as well as the role men can and should play in supporting women's rights and access to health services.Contemporary definitions also highlight the needs of people of diverse gender identities and sexual orientations with an increasing number seeking care in ACHD centers worldwide.78ACHD programs are well positioned to develop policies and resources that help those living with CHD overcome barriers at the individual, interpersonal, community, and societal levels.Potential barriers include social exclusion, stigma, disability discrimination, and gender-based violence.The World Health Organization,79 United Nations Population Fund,80    and the Guttmacher-Lancet Commission on SRHR, all provide resources in this area.78I m p r o v i n g d a t a c o l l e c t i o n .There is a need for credible research into the sexual health and wellbeing needs of adults with CHD.ACHD community should be identifying research priorities that translate into meaningful improvements in the sexual and reproductive health, which is a right frequently under attack.

TABLE 1
Resources for Individuals With ACHD and Health Care Providers Namewith other chronic conditions.Moons et al. found that individuals with ACHD spent less time "worrying about [their] sex life" and "not enjoying having sex".
Risk factors include those with exercise 44Table 2 lists a more complete list of medications that may affect sexual function.On the other hand, optimal medical treatment may improve sexual function such as the use of sacubitril/valsartan in heart failure.

TABLE 2
Cardiac Drugs That May Be Associated With Sexual Dysfunction

Table 2
lists some of the currently available resources.

TABLE 3
First Line Assessment for the Cardiac Health Care Team FIGURE 3 Strategies to Improve Sexual Health and Well-beingFemale, 44, Complete Heart Block .I am in my mid 40s and I have never been asked about my sexual health and well-being by any of my healthcare providers Female, 44, Complete Heart Block My heart condiƟon has undoubtedly made me more cauƟous about trusƟng others and less carefree.I don't think this is enƟrely negaƟve, I deeply value those who fully accept me and believe this has led to more meaningful relaƟonships.Growing up I would have loved to have someone to talk to about body image.The open heart surgery scar…along with the 4 drain holes doesn't really do much for self esteem….In a child/teenager's mind, the scars are huge, but now as an adult I've learned to accept them and they no longer cause me any bother.