Congenital heart disease natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2], Keri Shafer, M.D. [3], Atif Mohammad, M.D.; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]
Overview
Congenital heart disease is the most common birth defect. With the advancement of surgical technique, there has been significant improvement in the prognosis of congenital heart disease patients.
Complications
AHA Scientific Statement: Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease
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. | ||
Prognosis
The prognosis of patients with congenital heart diseases has improved considerably over the few past decades due to advancements in surgical techniques. The prognosis post surgical correction depends on the following factors:
- Hemodynamic status
- Presence of residual defects
- Condition of myocardium
- Presence of cardiac electrical instability
In recent years there has been significant decrease in surgical mortality. There has been a marked improvement in hemodynamic function. The recent decrease in non-electrical complications post surgery has shifted the focus towards decreasing complications due to defects in conduction system (induced post surgery). Recent studies have shown that increased efforts are required to protect the conduction system more specifically, His bundle.[1]