Sandbox adult CHD complications

Template:Adult CHD complications - 2017 Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: Arzu Kalayci, M.D. [2]

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease

Advantages and Disadvantages of Imaging Modalities for Detection of Liver Disease and Screening for HCC in Patients With Congenital Heart Disease(CHD)

	Advantages	Disadvantages	Additional Notes
Ultrasound	Inexpensive Widely available Highly sensitive for differentiating cystic and solid lesions No ionising radiation	Low sensitivity for detecting focal, solid liver lesions, particularly in the setting of diffuse disease Often unable to detect lesions <1 cm in size Low specs city High operator dependency	Use of contrast agents may improve characterization of hepatic tutors Useful for guiding liver parenchymal and some focal mass biopsies Elastography may overestimate degree of brosis and may not be useful for screening in CHD
CT	Best spatial resolution (submillimeter resolution)	Exposure to ionizing radiation dose Low sensitivity for detecting and characterizing lesions <1 cm in size Contrast contraindicated in renal failure Diffuse liver disease and fatty in ltration limit sensitivity for lesion detection	CT-guided liver mass biopsy useful in cases when ultrasound visualization is poor
MRI	High lesion-to-liver contrast High spatial resolution Better lesion detection and characterization than CT No ionising radiation Unenhanced MRI superior to unenhanced CT	Contrast relatively contraindicated in renal failure (eGFR <30 mL·min−1·1.73 m−2) High cost Long scan time Need for longer breath-holds Less widely available Unable to be used with many pacemakers and de brillators	Hepatobiliary contrast media useful in characterizing speci c liver tutors
CHD indicates congenital heart disease; CT, computed tomography; eGFR, estimated glomerular ltration rate; HCC, hepatocellular carcinoma; and MRI, magnetic resonance imaging.

Types of Heart Disease That May Be Associated With Liver Disease

Right-sided heart disease

Fontan physiology

TOF with residual pulmonary regurgitation

Complete transposition of the great arteries after atrial switch surgery

Pulmonary valve disease

Ebstein anomaly and other tricuspid valve disease

Eisenmenger syndrome

Pulmonary hypertension

Pericardial disease

Left-sided heart disease

Left ventricular out ow obstruction

Mitral valve disease

Ischemic and nonischemic cardiomyopathy

Cor triatriatum

TOF indicates tetralogy of Fallot.

Consideration for Liver Surveillance in Adults With Congenital Heart Disease

Physical examination for signs of liver disease. Signs can include an increased liver span consistent with hepatomegaly, splenomegaly, jaundice, right upper quadrant pain, or ascites.

Laboratory tests, including transaminases, GGT, alkaline phosphatase, bilirubin, albumin, total protein, INR, creatinine, and platelets every 1 to 2 y in patients with CHD at risk for liver disease, including all patients with Fontan circulation starting from 5 y after Fontan completion with frequency of testing increasing at 15 y after Fontan.

All patients who underwent heart surgery in or before 1992 should be screened for chronic hepatitis B and C infection.

All patients with evidence of liver disease should be vaccinated against hepatitis A and B. Those previously vaccinated against hepatitis B should have serologies checked because some patients exhibit waning immunity in adulthood.

Imaging of liver by ultrasound, MRI, or CT should be considered in patients with abnormal laboratory studies or signs of advanced liver disease.115 It is reasonable to perform baseline abdominal imaging in patients with Fontan physiology 5 y after Fontan completion regardless of the presence of other abnormal findings.

Liver biopsy may assist in staging hepatic brosis and diagnosing cirrhosis but is susceptible to sampling error. Liver biopsy remains important in the evaluation of nodules seen on hepatic imaging in patients with liver disease caused by CHD.

CHD indicates congenital heart disease; CT, computed tomography; GGT, γ-glutamyltransferase; INR, international normalized ratio; and MRI, magnetic resonance imaging.

Genetic Syndromes With Cardiac Disorders and Associated Endocrinopathies

Syndrome	CHD Association	Endocrine Associations	Recommendations
Alagille	TOF, peripheral PS	Short stature Osteoporosis	Tests: consider DEXA scan Treatment: calcium and vitamin D supplementation
DiGeorge (22q11.2 deletion)	Conotruncal abnormalities (TOF, IAA, DORV), AVC, VSD, PDA, PS, vascular ring	Hypothyroidism (25%) Hyperthyroidism (5%) Hypoparathyroidism (80%) Normal reproductive fitness	Tests: CBC, BMP, TSH, Ca, Mg, PTH, LFT, lipids, HbA1c annually Treatment: calcium and vitamin D supplementation
Down	AVC, TOF, VSD, PDA	DM (3- to 10-fold increased risk) Obesity Hypothyroidism (up to 25%) Hyperthyroidism (<5%) Hyperlipidemia Osteoporosis	Tests: lipids and TSH every 5 y FPG/HbA1c every 3 y if >45 y of age or sooner if risk factors are present DEXA scan every 2 year or if any risk factors are present
Kabuki	Coarctation of the aorta, ASD, VSD	Congenital hypothyroidism Growth hormone deficiency	Tests: consider TSH
Marfan	Dilated aortic root, mitral valve prolapse	Possible osteoporosis	Tests: consider DEXA scan
Noonan	Pulmonic stenosis, hypertrophic cardiomyopathy	Delayed puberty Reduced fertility in male patients Short stature	Tests: no specific endocrine screening
Turner	Bicuspid aortic valve, CoA, dilated aorta	Hypogonadism Hypothyroidism (24%) Hyperthyroidism (2.5%) Hyperlipidemia Impaired glucose tolerance and DM (50%) Osteopenia and osteoporosis Short stature	Tests: DEXA scan every 3–5 year; TSH, LFTs, lipids, OGTT/HbA1c annually Treatment: discuss risk/bene t of oestrogen replacement
Williams-Beuren	Supravalvular aortic stenosis, supravalvular PS, peripheral PS, coronary artery abnormalities, midaortic syndrome, renal artery stenosis	Impaired glucose tolerance and DM (75%); Osteopenia and osteoporosis (45%); Subclinical hypothyroidism (15%–30%); Hypercalcemia	Tests: BMP, Ca every 1–2 y; spot urine Ca/ Cr ratio annually; TSH every 3 year; OGTT/ HbA1c every 5 year; DEXA every 5 year Treatment: care with calcium supplementation given predisposition to hypercalcemia
AVC indicates atrioventricular canal; BMP, basic metabolic panel; CBC, complete blood count; CHD, congenital heart disease; CoA, coarctation of the aorta; Cr, creatinine; DEXA, dual-energy x-ray absorptiometry; DM, diabetes mellitus; DORV, double-outlet right ventricle; FPG, fasting plasma glucose; HbA1c, hemoglobin A1c; IAA, interrupted aortic arch; LFT, liver function test; OGTT, oral glucose tolerance test; PDA, patent ductus arteriosus; PS, pulmonic stenosis; PTH, parathyroid hormone; TOF, tetralogy of Fallot; TSH, thyroid-stimulating hormone; and VSD, ventricular septal defect.

Screening for Atherosclerotic Cardiovascular Risk Factors in Adults With Congenital Heart Disease

	Testing	Frequency
Diet and physical activity	NA	Yearly
Tobacco	NA	Yearly
Hypertension	Office blood pressure measurement and/or ambulatory/home blood pressure monitor	Yearly
Obesity	Weight, height, and BMI	Yearly
Dyslipidemia	Fasting lipid panel	Every 5 year
DM	Fasting plasma glucose, oral glucose tolerance test, or hemoglobin A1c	Every 3 y in adults ≥45 y of age or ≤45 y of age with BMI ≥25 kg/m2 and risk factors for DM
PAD	Ankle-brachial index	Insufficient evidence but can consider in patients with DM or an additional cardiovascular risk factor
BMI indicates body mass index; CHD, congenital heart disease; DM, diabetes mellitus; NA, not available; and PAD, peripheral artery disease.