Outcome following the treatment of congenital heart disease continues to improve and interest has focused on reducing morbidity as well as mortality. One important aspect of this is the psychological effect of congenital heart disease and its treatment on both children and their parents. This review addresses the extent and nature of the psychological morbidity associated with this disease, in particular the impact of the diagnosis on the management of patient and family. The etiology of the high level of psychosocial morbidity remains poorly understood. Data from the 1960s concerned with pre-school and school-age children is now of little value given the dramatic changes in the treatment of congenital heart disease, which is now predominantly in the neonatal period. While theory from developmental and pediatric psychology may be usefully applied to this area, further research is required to define effective psychological care for patients and their families.

A successful aortic coarctectomy performed beyond early infancy is followed, even in the long term, by persistence of left ventricular hypertrophy and by diastolic dysfunction, although systolic function is often increased. In this study we investigated whether earlier coarctectomy provides better preservation of left ventricular function. Experimental studies on the myocardial response to pressure overload show that neonates and young infants develop a functionally advantageous combination of myocytic hyperplasia (together with mild hypertrophy) and increased angiogenesis. Older patients, in contrast, generate myocytic hypertrophy in isolation, setting the scene for ventricular dysfunction. Cross-sectional echo-Doppler evaluation of left ventricular size, shape, mass and systolic and diastolic function was made in 13 patients a mean of 44±36 months (range 11 days-10 years) after successful coarctectomy in the first year of life. They were compared to 11 age, body surface area and gender-matched control subjects. In all patients, left ventricular mass normalized for body surface area was significantly greater than in the control group (66.2±12.3 vs 43±l2 p=0.0001). There was no correlation between left ventricular mass normalized for body surface area and age at operation, follow-up duration, degree of residual isthmic gradient, peak systolic wall stress, systolic blood pressure or left ventricular shape. No significant differences were noted between the two groups in regard to transverse diameter of the aortic arch, left ventricular afterload (meridional end-systolic wall stress), volume and shape (both in systole and diastole), systolic performance (fractional shortening and ejection fraction) and contractility (rate-corrected velocity of fiber shortening to meridional end-systolic wall stress relationship). Furthermore, no significant differences were found with respect to indices of mitral (including peak filling rate normalized to mitral stroke volume) and pulmonary venous flow, suggesting normal diastolic function. Repair of aortic coarctation in the first year of life promotes a more complete recovery of left ventricular function (particularly diastolic) than that reported after coarctectomy at older age, despite persistence of moderate ventricular hypertrophy.

The number of neonates who undergo intracardiac repair for congenital heart disease has increased in recent years. In considering appropriate surgical options in such circumstances, normal values for various cardiovascular dimensions are needed. We measured 19 parameters for the heart and great arteries (four valves, pulmonary tree, aortic arch, ascending and descending aorta, ventricular septum, left ventricular internal and posterior wall dimensions) using cross-sectional echocardiography in 55 premature and full-term infants (ranging in gestational age from 23 to 41 weeks, in body weight from 543 to 3966 g). The increase in each dimension correlated closely with body weight (r=0.62 to 0.87) and was found to follow a linear regression. The diameter of the isthmus was found to be smaller, and the size of mitral valve annulus larger, than previously published values.

Believing early repair to offer major benefits, we have repaired tetralogy of Fallot with pulmonary atresia in five infants aged less than six months. The ventricular septal defect was closed via a right atriotomy. The right ventriculotomy was 30±3% of the right ventricular length. The posterior wall of the right ventricular outflow tract was created by anastomosing directly the pulmonary trunk to the right ventriculotomy, or either by interposition of the left atrial appendage or an autologous pericardia! flap. The pathway was then roofed over with an equine pericardia! patch. All patients survived and are now doing well from 18 to 41 (31±11) months after the repair, although one patient required reoperation for relief of stenosis at the site of an anastomosis between the pulmonary arteries and the interposed left appendage. In the other patients, postoperative sequential echocardiography has shown no obstruction in the right ventricular outflow tract, nor significant pulmonary or tricuspid regurgitation. We conclude that primary repair in early infancy is an excellent option for surgical treatment of tetralogy of Fallot with pulmonary atresia.

In neonates with duct-dependent congenital heart defects, a reliable non-pharmacological, non-surgical method of maintaining flow through the arterial duct may simplify the management. Stent implantation into the arterial duct has been proposed as such a method. In order to evaluate the technical difficulties which may accompany clinical application, stent implantation into the arterial duct was attempted in 32 newborn lambs aged two to nine days (mean 4.2±1.4) weighing 2.4–7.3 kg (mean 4.6±1.0). Four different types of stent were implanted and the ease of implantation and outcomes were assessed. Stent implantation was technically successful in 28 of the 32 lambs (88%). Of these, 11 lambs died shortly after stent implantation with patent ducts, five lambs had early occlusion of the duct and 12 lambs were entered into a long-term study. The Gianturco-Roubin stent was too rigid and too long to enable successful implantation. The Palmaz-Schatz stent was poorly radio-opaque and was difficult to place accurately. When the aortic orifice was not completely covered, the duct occluded within a few days of implantation. The articulation gap also allowed duct tissue to prolapse into the middle of the stent. The Medinvent and Tower stents were simpler to position due to their superior radio-opacity. The Tower stent had an additional advantage in being a single strand stent which could be easily retrieved if misplaced.

The anatomic and pathophysiologic features of tricuspid valvar dysplasia are discussed in the light of two cases observed during fetal life which progressed with an unexpectedly favorable course. Some fetal echocardiographic features are proposed to explain this outcome. First, absence of associated cardiovascular abnormalities; second, clear separation between well represented right ventricular walls and the tricuspid valvar leaflets; third, normal proximal insertion of the tricuspid leaflets and, fourth, normal dimensions of the pulmonary trunk. We recommend karyotyping because of the possible association of this malformation with an aneuploid karyotype. We also describe transitory functional atresia of the pulmonary valve at birth, as far as we know for the first time, in a neonate that had normal forward flow of blood as seen on Doppler sampling in fetal life.

We report two female patients, one aged four years and the other a newborn, referred for evaluation of cyanosis with otherwise normal cardiovascular findings, who proved to have the right superior caval vein draining into the morphologically left atrium. In both patients, the diagnosis was made by color flow mapping. The older child underwent catheterization and subsequent successful surgical correction. A right superior caval vein draining into the left atrium, although very rare, should always be considered a diagnostic possibility in the presence of cyanosis and normal clinical findings. Color flow mapping is an excellent method with which to make the diagnosis.

We describe two patients, aged seven and 21 years, with unilateral atresia of a solitary pulmonary vein. Until now, to the best or our knowledge, this condition has been reported only twice. Although symptoms, diagnostic measures and treatment have been described in detail, clinical diagnosis is still difficult.

Aneurysmal dilation of the right atrium is described in a four-year-old boy who presented with cardiomegaly and symptoms of congestive heart failure. Echocardiography revealed the aneurysmal dilation of the right atrium, but a definite diagnosis was obtained only after surgical exploration. This had caused compression of the other cardiac chambers resulting in congestive heart failure. Surgical resection was successful and the patient was discharged on the eleventh postoperative day with good results.

Cerebrovascular accidents are a rare but feared complication of open heart surgery. We report a child with tricuspid atresia who suffered cerebral infarction after a Fontan procedure and discuss possible risk factors.

Complex congenital heart defects are accurately detectable antenatally using fetal echocardiography. In continuing pregnancies with fetal cardiac abnormalities, regular follow-up with advancing gestation is essential. This report documents the changes that occurred in the fetal cardiac anatomy in a fetus detected during antenatal scanning to have an atrioventricular septal defect with isomerism of the left atrial appendages.

We report a successful neonatal balloon aortic valvoplasty after prenatal diagnosis of critical aortic stenosis with severe left ventricular dysfunction. The infant was delivered by cesarean section at 34 weeks of gestation and had balloon valvoplasty via the right common carotid artery 67 minutes after birth. Left ventricular contraction improved immediately after the procedure. Scheduled balloon aortic valvoplasty immediately after birth subsequent to prenatal diagnosis can improve the outcome of critical aortic stenosis.

We report a case of interruption of the aortic arch associated with patency of the arterial duct, restrictive ventricular septal defect, obstruction of the left ventricular outflow due to a discrete subaortic shelf and severe pulmonary arterial hypertension. The patient survived to adulthood, when a complete angiographic diagnosis was made. The subaortic obstruction was treated by percutaneous balloon dilation performed through a right brachial arteriotomy. Correction of the interrupted arch was carried out surgically using a woven Dacron graft from the aortic arch to the descending thoracic aorta associated with ligation of the duct. Hemodynamic studies after one year revealed a marked decrease in pulmonary vascular resistance and a sustained reduction in the gradient across the left ventricular outflow tract. The case is of interest in the light of the unusual survival and the success of staged management of this complex situation.