OC21 - Psychosocial functioning of parents of infants diagnosed with complex congenital heart defect.

Theme: Parenting/parenthood. INTRODUCTION: The diagnosis of complex CHD impacts the psychosocial status of parents and their functioning. PURPOSE: A critical evaluation of the literature concerning the psychosocial parental response to their infant's diagnosis of complex CHD. METHODS: Systematic review of 18 articles published after 2000 in PubMed and CINAHL. RESULTS: The impact of an infant's CHD on the family functioning is determined both by child's medical condition and family's psychosocial factors. The majority of parents experience intense loss and numerous stressors. Nurses and physicians need to be sensitive to the needs, thoughts and experiences of the parents when discussing treatment options. Families with poor social support networks may have the greatest need for professional interventions. CONCLUSIONS: Appropriate interventions assist the majority of parents to adapt to the diagnosis of complex CHD. However, there are a number of parents with an increased psychosocial risk associated with higher rates of emotional distress

Postcardiotomy Mechanical Circulatory Support in Two Infants with Williams’ Syndrome

Supravalvar aortic stenosis (SVAS) in patients with Williams’ syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use more efficient and less traumatic. We describe our experience of using the Levitronix CentriMag pump in two patients with Williams' syndrome who underwent surgical correction of supravalvular aortic stenosis

Postcardiotomy Mechanical Circulatory Support in Two Infants with Williams’ Syndrome

Supravalvar aortic stenosis (SVAS) in patients with Williams' syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use more efficient and less traumatic. We describe our experience of using the Levitronix CentriMag pump in two patients with Williams' syndrome who underwent surgical correction of supravalvular aortic stenosis

Diversity of congenital cardiac defects and skeletal deformities associated with the Holt–Oram syndrome

AbstractINTRODUCTIONThe Holt–Oram syndrome is a rare congenital disorder involving the skeletal and cardiovascular systems. It is characterized by upper limb deformities and cardiac malformations, atrial septal defects in particular.PRESENTATION OF CASEFour consecutive patients 1–15 years old with the Holt–Oram syndrome presented over a 10 year span for surgical treatment of their cardiac maladies. The spectrum of the heart defects and skeletal deformities encountered in these patients are described and discussed.DISCUSSIONThe Holt–Oram syndrome is an autosomal dominant condition; however absence of the morphological features of the trait in close family members is not rare. Although patients are known to predominately present with atrial septal defects, other cardiovascular anomalies, including rhythm abnormalities, are not uncommon. Skeletal disorders vary as well.CONCLUSIONCardiovascular disorders, skeletal malformations and familial expression of the Holt–Oram syndrome, vary widely