No abstract

Multidisciplinary care involving specialists familiar with Marfan syndrome should be emphasized before, during, and after pregnancy with the involvement of Maternal Fetal Medicine, Genetics, Cardiology, Cardiothoracic Surgery, Anesthesia, and other specialties on a case-by-case basis. We review the important aspects of the evaluation and management of pregnant women with Marfan syndrome.

Hepatitis C infection in patients with congenital heart disease who underwent surgical palliation prior to 1992 is common, with prevalence higher than the general population. Our data emphasize the continued importance of screening the high-risk adult congenital heart disease patients.

The management of pediatric and adolescent patients with pure aortic valve regurgitation remains challenging and controversial (Christos et al., Eur J Cardiothorac Surg 17:125-133, 2000; Gersony and Sommerville, ACC Curr J Rev 31:97-98, 2000; Hasaniya et al., J Thorac Cardiovasc Surg 127:970-974, 2004; Sabet et al., Mayo Clin 74:14-26, 1999; Tweddell et al., J Thorac Cardiovasc Surg 129:551-558, 2005). We evaluated pediatric and young adult patients who underwent aortic valve replacement (AVR) primarily for aortic regurgitation in an effort to identify preoperative echocardiographic variables that are predictive of left ventricular (LV) recovery following AVR. Twenty-one patients with severe aortic valve regurgitation who underwent AVR were identified. Retrospective chart review for each patient was performed and transthoracic echocardiograms prior to and 6-months after AVR were analyzed. Improvement in LV size based on preoperative LV end-systolic dimension index when compared to 6-months post-AVR was observed in 68% of the patients. Patients with persistent dilation of their left ventricles had a greater preoperative LV end-systolic dimension index (p ≤ 0.05), a greater preoperative LV end-systolic dimension z-score (p ≤ 0.002), and a lower preoperative ejection fraction (EF) (p ≤ 0.001). A similar trend was present between the two cohorts in regards to LV end-diastolic parameters (LV end-diastolic dimension index and z-score), with patients with abnormal LV size at 6-month follow-up having larger preoperative dimensions. Increasing LV systolic dimensions and declining EF appear to be predictors of poor LV recovery following AVR in pediatric and young adult patients. LV end-systolic indices appear to be more predictive than LV end-diastolic indices. AVR should be performed prior to severe LV enlargement defined as an LV end-systolic dimension z-score >4.5.