The electrocardiogram may show the presence of arrhythmias, atrial or ventricular There is often P pulmonale due to right atrial dilatation The QRS axis usually shows left ventricular dominance Echocardiography should be used to document systematically and sequentially all the morphologic features previously discussed Residual lesions such as atrial septal defects and patent systemic shunts should be sought For those with a biventricular circulation, the presence and degree of tricuspid regurgitation, pulmonary regurgitation, and stenosis should be documented The size and zscore of the tricuspid valve should be documented For those with a Fontan circulation, careful assessment of the anastomoses is needed as well as assessment of left ventricular function, mitral regurgitation, and the presence of right atrial thrombus There should be low-velocity phasic flow in the inferior and superior venous pathways of the Fontan Imaging of the coronary artery origins and their size may suggest significant fistulous communications Cardiac catheterization may be required to assess the hemodynamics of the Fontan circuit Coronary arteriography is essential because stenoses and interruptions may play a significant part in the prognosis Assessment of the pulmonary arterial anatomy is important because the patient may have had a systemic-to-pulmonary arterial shunt in the past, with pulmonary arterial distortion In contrast to pulmonary atresia with tetralogy of Fallot, native stenoses or hypoplasia of the pulmonary arteries are relatively uncommon Baffle leaks and venovenous collateralization should be sought in those with a Fontan circulation Cardiac magnetic imaging and computer tomography may supplant catheterization as a means of noninvasive assessment of the range of morphologic lesions found in this condition They give excellent anatomic and, in the case of magnetic imaging, excellent functional information Nuclear imaging conveys important information about myocardial perfusion and ischemia, particularly for those patients with significant coronary artery lesions, although abnormal anatomy imposes technical challenges Implications in Adult Life Physiologic Many patients with this condition are reaching adulthood Their well-being in adult life depends on the cumulative effect of the consequences of the abnormal physiology and procedures throughout their life (be they cardiac and noncardiac), reasonable cardiac function, and avoidance of residual lesions.66 For those with biventricular or one-and-one-half ventricle repairs, residual lesions comprise pulmonary stenosis, regurgitation, and tricuspid regurgitation These can lead to atrial and ventricular arrhythmias, syncope, sudden death, reduced exercise tolerance, fatigability, and angina Increasingly, patients with significant pulmonary regurgitation and dilation of the RV are being managed with pulmonary valve replacement, either surgically or in the catheterization laboratory For those with a functionally univentricular repair, such as a total cavopulmonary connection or Fontan-type circulation, residual lesions comprise right atrial dilation, coronary arterial stenoses, high systemic venous pressures, and systemic-to-pulmonary arterial and venous collateralization These can lead to atrial and ventricular arrhythmias, sudden death, thromboembolism, right pulmonary venous occlusion, angina, left ventricular dysfunction, protein-losing enteropathy, hepatic dysfunction, and cyanosis For those with incompletely separated circulations, there is common mixing of systemic and pulmonary circulations leading to cyanosis, erythrocytosis, thromboembolism, fatigability, and arrhythmias (see Chapter 73) Contraception and Prepregnancy Prepregnancy counseling is essential Advice should be tailored to the individual with consideration of cardiac structure and function Patients with ongoing hypoxemia or a Fontan-type circulation should avoid the combined contraceptive pill Detailed advice is beyond the scope of this chapter but is thoroughly covered elsewhere (see Chapter 80).130 Biventricular Repair With Residual Pulmonary Stenosis or Regurgitation The increased hemodynamic load of pregnancy may precipitate right heart failure, atrial arrhythmias, or tricuspid regurgitation Balloon dilation can be performed during pregnancy, preferably after organogenesis, although it is better to treat before conception Functionally Univentricular Circulation There are increased risks of systemic venous congestion, deterioration in ventricular function, atrial arrhythmias, thromboembolism, and paradoxic embolization if there is a fenestration in the atrial baffle Successful pregnancy is possible, however, with meticulous cardiac and obstetric planning and supervision If anticoagulation is required, additional risks to the fetus are involved Mixed Circulations With Cyanosis There is an increased risk of maternal cardiovascular complications, prematurity, and fetal death, particularly when baseline maternal resting oxygen saturations are less than 85% All patients should have cardiologic counselling prior to conception, and follow-up by an adult congenital cardiologist and a high-risk obstetrician during pregnancy and the peripartum.130,131 Fetal echocardiography is recommended Employment Employment will depend on the original diagnosis and residual lesions There may not be specific stratification of risk for this particular lesion, and parallels from other lesions, such as pulmonary stenosis, tetralogy of Fallot, and patients with Fontan-type circulations, may have to be taken Employability will depend on exercise ability, avoidance of complications, and ongoing particularly neurologic morbidity Employment would need to be tailored to individual needs Detailed discussion of these topics is beyond the scope of this chapter, although they are thoroughly covered elsewhere.130