para>Not well studied
Three CDC pilot studies underway
Pregnancy Considerations
Contraindicated in cyanotic heart diseases
Cyanotic heart disease: <12% live birth rate if O2 saturation <85% (2)
Fetal cardiac echo: 16 to 18 weeks' gestation
Consultation with ACHD expert and MFM prior to pregnancy is recommended for all ACHD patients especially for those with right to left shunting, chronic anticoagulation, antihypertensives such as ACEI/ARBs; evaluation of Valsalva maneuvering, and heart response
Cesarean > vaginal, if cyanotic HF
Avoid estrogen due to VTE predilection (2)[C]; caution with progesterone due to fluid retention if HF development or potential (2,3)[C]
Breastfeeding is acceptable and encouraged as long as maternal medications are not excreted into breast milk.
PROGNOSIS
- Will vary on the type of ACHD
- Children reaching adulthood: mild: 98%; moderate: 95%; severe: 56%
- Combined survival = 84.1% (2)
- Steep rise in mortality >65 years of age (2)
- Comorbid conditions superimposed cause significant rise in morbidity and mortality.
COMPLICATIONS
Eisenmenger complex
REFERENCES
11 Webb G, Williams R. 32nd Bethesda Conference: care of the adult with congenital heart disease 1. J Am Coll Cardiol. 2001;37(5):1162-1165.22 Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and the Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(23):e143-e263.33 Bhatt AB, Foster E, Kuehl K, et al. Congenital heart disease in the older adult: a scientific statement from the American Heart Association. Circulation. 2015;131(21):1884-1931.44 Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31(23):2915-2957.55 Silversides CK, Marelli A, Beauchesne L, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: executive summary. Can J Cardiol. 2010;26(3):143-150.
CODES
ICD10
- Q24.9 Congenital malformation of heart, unspecified
- Q24.8 Other specified congenital malformations of heart
- Q21.3 Tetralogy of Fallot
- Q23.1 Congenital insufficiency of aortic valve
- Q21.0 Ventricular septal defect
- Q21.1 Atrial septal defect
ICD9
- 746.9 Unspecified congenital anomaly of heart
- 746.89 Other specified congenital anomalies of heart
- 745.2 Tetralogy of fallot
- 746.4 Congenital insufficiency of aortic valve
- 745.4 Ventricular septal defect
- 745.5 Ostium secundum type atrial septal defect
SNOMED
- congenital heart disease (disorder)
- Tetralogy of Fallot (disorder)
- bicuspid aortic valve (disorder)
- Atrial septal defect (disorder)
- Ventricular septal defect (disorder)
CLINICAL PEARLS
- Prompt referral and consult to ACHD facility if cardiac admission and/or surgery is warranted.
- Monitor for Eisenmenger syndrome and severe PAH.
- 2D/3D Doppler TTE is the first-line imaging modality in all ACHD.
- Forego routine CXR unless clinically warranted.
- Pulse oximetry and ECG obtained at each visit in conjunction with a thorough history and physical exam.