Puberty challenges in ACHD and solutions Shabnam,Mohammadzadeh. MD Cardiologist Fellowship of ACHD IMAM Khomeini Hospital Complex

Prevalence as a glance Now expect 90% of CHD to reach adulthood Females accounted for 52 % of the CHD population in children and 57 % of the CHD population in adults

They are growing We are behind them

Puberty Hormonal change Emotional change Feature change

Simple classification in case of puberty Small ASD Small VSD Small PDA Repaired simple CHDs without significant residual disease Less than 2 times surgery TFTC Fontan Single V physiology Cyanotic heart disease Eisenmenger, severe PH Multiple surgical repair Complex cases Simple disease or repaired one

Becoming adult problems Menstural problem Psychological problems Menstrual problems Growth problems Reproductive issues

Heart problems due to puberty or vice Versa CHD Puberty 1.Growth retardation seems to occur in the majority of children with CHD needing repair, irrespective of the severity of CHD 2.Children with cyanosis and pulmonary hypertension show more severe growth impairment. 3.Decreased growth factor 4.Hormonal abnormality 1.All cardiac chambers growth

Growth problems Univentricular heart Before correction : significant growth retard Gain some growth after correction Final height behind normal TOF Almost normal growth after correction Weight gain Normal or increased after correction Shall we use growth hormone? Insulin resistant diabetes Changes in bone structure Enlarged or inflamed organs, especially the pancreas Sleep disorders Carpal tunnel syndrome Increased cranial pressure Bleeding Growth of internal organs leading to what is known as HGH Gut The appearance of moles on the skin High HGH levels could cause Gynomastia which means basically Breast growth in males

The most challenging cases 18 year old woman with HX of Fontan 14 year ago DOE FCII since 5 m ago Height:148 cm, w: 60 kg, O2 sat: 90%, Heart exam is unremarkable Drugs : warfarin Echo: Nl for Fontan Hgb: 8mg/dL, plt: 60000,INR: 1.2 Alfa 1 antitrypsin, pro, Alb: Nl Started menarche 1 year ago

Menarche problems Results. Overall, the age at menarche was slightly increased in women with CHD (13.3 vs years in the general population), mainly attributable to an increased prevalence of primary amenorrhea (n = 147; 9.2%). Other menstrual cycle disorders were documented: secondary amenorrhea (n = 181, 11.4%), polymenorrhea (n = 103, 6.5%), oligomenorrhea (n = 90, 5.6%), and menorrhagia (n = 117, 6.5%). The occurrence of these disorders also depended on the presence of cyanotic heart disease, surgical status, the number of surgical interventions, and the severity of CHD. Discussion. Menstrual cycle disturbances, in particular primary amenorrhea, were frequently observed in this population. Patients with complex (cyanotic) heart disease needing repeated surgical interventions prior to menarche are especially at risk Possible causes of DUB in general blood clotting disorders, renal and hepatic impairment, diabetes, enteric, rheumatic, cardiac and neurological diseases, thyroid disorders hyperandrogenic alterations

Possible Mechanisms Abnormal Gonado-Ovarian Axis Delayed growth and puberty Primary amenorrhea Dysfunctional uterine bleeding Anovulation 20-40% Venous HTN Anticoagulation Chronic Cyanosis Solution

Reproductive issues Significant number lack of contraception for unknown reason Start education at early adolescence Every type of corrected CHD was evaluated (both men and woman) Patients showed a delay in starting sexual activities compared with peers. Females with ConHD scored significantly worse compared with normative data on all scales of sexual functioning, Patients indicated to have been suboptimally informed about sexuality in early adolescence. Providing information to patients about sexuality, pregnancy, delivery and heredity should be improved, and given at young age 1.Pregnancy:one out of % using wrong contraception 3.28% of high risks not using contraception As young girls reach reproductive age, discussions regarding menstrual function, contraception and pregnancy should begin and, when sexually active, they should be referred to a gynecologist who specializes in adolescent gynecology.

Incidence of local infection Incidence of IE HBV,HCV, HIV Mental health and risky behaviors Tattooing and piercings Rates of endocarditis due to body modification among individuals with CHD are relatively unknown For the time being, in our own practice we strongly discourage all forms of body art and we recommend antibiotic prophylactic cover where individuals cannot be dissuaded, with strong advice for prompt treatment of any signs of subsequent infection. Case reports of IE after piercing and tattooing are increasing, particularly when piercing involves the tongue Currently no data are available on the incidence of IE after such procedures and the efficacy of antibiotics for prevention. Education of patients at risk of IE is paramount. They should be informed about the hazards of piercing and tattooing and these procedures should be discouraged not only in high-risk patients, but also in those with native valve disease. If undertaken, procedures should be performed under strictly sterile conditions, though antibiotic prophylaxis is not recommended

Drugs use is significantly lower More depression More anxiety Dysfunctional relationship memory disorders attention problems difficulty in planning, impaired intellectual and educational development. Overprotected

Drugs Psychological Problems and solutions More depression More anxiety Lower self esteem More depression More anxiety Lower self esteem Step 2 What How Step 1 Visit them as a full patient. Don’t look just at heart. Start Psycologist consult at time of diagnosis Blue girls feel more blue during puberty

Texas children hospital program. neurological visit 6 m later If neurological defect is present during surgery Any kind of heart surgery Non repaired First neuropsychology consult at 18 m

Take home message

Females accounted for 52 % of the CHD population in children and 57 % of the CHD population in adults