ABSTRACT
Congenital heart disease (CHD) is present in 0.8-0.9% of live births. Prevalence of CHD is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. Most of the women with underlying CHD reach adulthood due to excellent surgical and interventional treatment options and most of them desire pregnancy. In Western countries, maternal heart disease is the major cause of mortality during pregnancy, thus risk estimation should be therefore individualized depending on the underlying CHD, functional class and treatment options. Pre-pregnancy counselling is mandatory in all women, especially in patients with high risk but even in patients with low risk, a plan for labor and delivery should be outlined in a heart pregnancy team. This review addresses the key element of pre-counselling, planning and successful disease management in patients with CHD during pregnancy.
ABSTRACT
BACKGROUND: Care for patients with Marfan syndrome (MFS) has improved substantially in recent decades. Increasing clinical knowledge and genetic analysis allow early diagnosis of the disease in childhood. Because of the earlier initiation to preventive and medical treatment, patients' life expectancy has risen. To ensure optimal care, pediatric patients require a safe follow-up regime, multidisciplinary care, and a safe transition to adult care. METHODS: We collected a sample of 149 pediatric Marfan patients, of whom 34 patients had already been transferred to adult care or who were currently transitioning. First, we evaluated clinical aspects of patients that manifest in childhood and are present in the transition process. Second, we analyzed the transition process itself. RESULTS: We found age-dependent manifestation of organ pathologies. Dilatation of the sinus of Valsalva showed a particularly high prevalence during the transition process and 62% of patients required medical treatment. Mean onset of aortic root dilatation was 9.9±5.8 years. Concerning systemic manifestation in MFS skin striae, wrist and thumb sign, and reduced elbow extension occurred significantly more often in patients who were transitioning than in younger children with MFS. All other clinical Marfan features showed an increased prevalence in patients who were transitioning compared with younger patients. In our cohort, transition was successful in 20 patients (58.9%), 12 patients (35.3%) are still in the transition process and 2 patients (5.9%) were lost to follow up. CONCLUSIONS: Marfan patients in the transition process are already under a chronic disease condition with a high onset of especially cardiovascular pathologies. Although early medical treatment in childhood is effective, the pathologies of the connective tissue require lifelong attention and influence life in many ways. The big challenge during transition is the double change of responsibility from the parents and pediatric doctor to the patient and adult doctor. Consequently, patients in transition process require special attention and close contact with the doctor and the family. A reevaluation by the supervising pediatric Marfan specialist of the successful transition to adult care is indispensable before the pediatric care of Marfan patients is completed.
ABSTRACT
With the hypothesis of low thromboembolic risk and higher late postoperative spontaneous closure, a new fenestration technique during extracardiac total cavopulmonary connection was attempted. From 2008 to 2009, 14 consecutive patients received an innominate vein-common atrium 5-mm Gore-Tex (W.L. Gore and Associates, Flagstaff, AZ) graft fenestration. Monitoring was performed by contrast bubble echocardiography at hospital discharge and up to 6 months postoperatively. The technique proved safe and reproducible, did not add to surgical difficulty or time, and provided reliable fenestration of up to at least 3 weeks, with a high rate of spontaneous closure during intermediate follow-up.
