**11. References**

176 Congenital Heart Disease – Selected Aspects

Freedom from systemic AV valve regurgitation and ventricular dysfunction was significantly higher after anatomic repair. The authors concluded that anatomic is superior to physiologic repair in patients with two adequately sized ventricles. However high risk groups such as those patients with RV dysfunction or the need for LV training warrant careful selection prior to undergoing anatomic repair. Taken together, these outcomes favor anatomic over classic /physiologic repair with careful preoperative assessment of TR for the

Patients with ccTGA require outpatient follow-up every 1-2 years by a pediatric or adult congenital cardiologist. Symptomatology, ventricular function, and valvar insufficiency should further guide frequency of follow-up. It is recommended an ECG be performed at each visit to monitor for AV block with periodic consideration of Holter monitor. Cardiopulmonary exercise testing is performed to assess overall function as well as response to medical or surgical therapy. RV function in the unrepaired or physiologically repaired ccTGA patient must be closely monitored with serial echocardiography even in asymptomatic patients (Bos et al., 2006). Cardiac MRI with cine data used to quantify RV volume, mass, and ejection fraction is the best modality to serially quantify RV function, and

Pregnancy in the ccTGA patient is generally well tolerated except in the presence of maternal NYHA class III-IV symptoms, moderate or severe AV valve regurgitation, or poor ventricular function (EF<40%). Evaluation of pregnancy outcome in 22 women with ccTGA revealed 50 live births in 60 total pregnancies (83%). However, the rate of miscarriage in the ccTGA mothers was higher than the general population (Connolly et al., 1999). A recent cohort of patients by Gelson and colleagues (2011) revealed high maternal and neonatal morbidity in women with systemic right ventricles with a significant number of babies born small for gestational age. Although cyanosis in women with ccTGA has been shown to be a risk factor for miscarriage, the women in the cohort of Gelson et al. were normally saturated (Gelson et al., 2011; Thierrien et al. 1999). The risk of congenital heart defects in the offspring

Patients for which heart transplantation may be considered are those with end-stage RV failure, significant LV dysfunction and pulmonary valve abnormalities precluding successful DS operation, or uncontrollable arrhythmia (Duncan & Mee, 2005). For patients undergoing surgical intervention, poor preoperative EF of the systemic ventricle has been

Although debate continues over efficacy and long-term follow-up of physiologic vs. anatomic repair for ccTGA, recent outcomes data favor anatomic correction in which systemic function is restored to the LV. Management considerations specific to this population of complicated patients include type and timing of surgical intervention to

shown to predict the eventual need for transplantation (Beauchesne et al., 2002).

purpose of risk stratification.

**9. Follow-up and special considerations** 

should be performed every 3-5 years.

of mothers with ccTGA has not been defined.

**10. Conclusions and special considerations** 

**9.1 Pregnancy** 

**9.2 Heart transplant** 


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**8** 

**Proximal Anomalous Connections** 

Pierre Aubry1, Xavier Halna du Fretay2, Patrick A. Calvert1,3, Patrick Dupouy4, Fabien Hyafil5, Jean-Pierre Laissy6 and Jean-Michel Juliard1

Isolated proximal **ANO**malous connections of **COR**onary arteries (ANOCOR) are not rare with an angiographic prevalence and tomographic prevalence of 0.5% and 1.3% respectively, in adult populations. The diagnosis of ANOCOR is sometimes fortuitous in adults undergoing a coronary angiography to detect a coronary artery disease (CAD). The absence of diagnosis in young people may have severe consequences with sudden death occurring frequently during intensive exertion. The prognosis depends mainly on the initial course of the ectopic coronary vessel. Preaortic course with intramural segment is recognized as high risk for sudden death in children or young adults. Management of ANOCOR with intramural course may be difficult in patients >35 years of age and with no evidence of myocardial ischemia. The mechanisms of life-threatening cardiac events are still not well understood even if anatomical risk factors are identified. Fortunately, most cases of ANOCOR are simply incidental anatomical findings. Multidetector computed tomography (CT) is recognized as the best imaging technique for identifying ANOCOR. Intravascular ultrasonography (IVUS) may help to quantify the severity of high-risk ANOCOR. In the ACC/AHA 2008 guidelines for the management of adults with congenital heart disease, surgical repair is the treatment of choice for high-risk ANOCOR (Warnes et al. 2008). However, this therapeutic management is based on little solid data with limited long-term follow-up. Percutaneous coronary intervention (PCI) has been proposed in some ANOCOR. The limited experience of most angiographers in detection of ANOCOR may explain non infrequent misdiagnoses with erroneous interpretations of ANOCOR. Large-scale prospective multicenter studies are needed to improve screening and imaging strategies and to better define the treatment of these potentially lethal congenital coronary abnormalities.

**1. Introduction** 

**of Coronary Arteries in Adults** 

*1Department of Cardiology, Bichat Hospital, Paris 2Department of Cardiology, Foch Hospital, Suresnes 3Department of Cardiology, Papworth Hospital* 

*4Department of Interventional Cardiology and* 

*Cardiovascular Imaging, Hôpital Privé d'Antony, Antony 5Department of Nuclear Medicine, Bichat Hospital, Paris 6 Department of Radiology, Bichat Hospital, Paris,* 

 *NHS Foundation Trust, Cambridge,* 

*1,2,4,5,6France 3United Kingdom* 

Congenitally Corrected Transposition of the Great Arteries (SLL). *Pacing and Clinical Electrophysiology*, Vol. 33, No. 1, (January 2010), pp. e4-7, ISSN 1540-8159.

