Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children's hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease. Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school. Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, gen...

Cardiac mass and function were evaluated in 10 children with classical GH deficiency. Echocardiograms were performed at baseline, 3, 6, and 12 months after initiation of recombinant human (rh) GH therapy (0.3 mg/kg.wk). Before treatment, left ventricular (LV) mass indexed to body surface area (BSA) was low or low normal (<50 g/m(2)) in five children compared with reference control data. Height SD score (-3.2 +/- 0.9 vs. -1.8 +/- 1.3 yr; P < 0.01), growth velocity SD score (-2.7 +/- 1.6 vs. 5.8 +/- 3.1; P < 0.01), LV mass (36 +/- 9 vs. 60 +/- 30 g; P < 0.02), LV mass/BSA (51 +/- 12 vs. 72 +/- 11 g/m(2); P < 0.01), LV mass/height (36 +/- 9 vs. 54 +/- 15 g/m; P < 0.02), and LV mass/m(2.7) (36 +/- 12 vs. 45 +/- 8; P < 0.05) increased significantly with rhGH therapy. Pretreatment LV mass/BSA correlated inversely with fold increase in LV mass/BSA over the year (r = -0.83; P < 0.01). Load-dependent indices of diastolic performance were normal at baseline and did not change with rhGH therapy. Percentage increase of mean velocity of circumferential shortening, an index of systolic function, correlated with fold increase in LV mass/BSA (r = 0.88; P < 0.02) over the year of rhGH administration. LV mass can be lower than predicted for body size in some children with severe GH deficiency but is responsive to rhGH replacement. LV mass/BSA increases into the normal range during the first year of rhGH therapy. The rate of increase of LV mass is greater than the increase in BSA during rhGH treatment, suggesting that GH could also be a trophic factor for the heart.

Perhaps because it guards the inlet to the lesser circulation, the morphologically tricuspid valve has received less attention in terms of its anatomy than the well-explored mitral valve, which will receive attention in a subsequent review in this supplement.1 As we will show in our initial review, nonetheless, the approach to morphological analysis is the same for both valves, irrespective of whether the specific morphology is displayed in the autopsy room or the echocardiographic laboratory. It is essential that the valve be analysed so as to reveal the precise structure of each if its components – the so-called valvar complex.2 Equally important, in the current era, with the burgeoning use of three-dimensional displays that place the heart firmly within the context of the body, it is essential that the components of the valve be described as seen relative to the bodily axis,3 rather than following the present custom of describing the heart as though it is removed from the body and positioned on its own apex.