Review of an institutional experience of 81 patients admitted to the Royal Liverpool Children's Hospital between January 1958 and December 1990 with a diagnosis of pulmonary atresia and intact ventricular septum revealed that successful definitive repair was achieved in about one-third of those who underwent surgical treatment. A three-stage protocol, consisting of a systemic-pulmonary anastomosis followed by right ventricular decompression on cardiopulmonary bypass and subsequent separation of the systemic and pulmonary circulations, facilitated a biventricular correction but rarely led to a successful Fontan procedure. Both functional class and probability of survival improved after each stage of management.

Tissue Doppler measurements of the right and left lateral ventricular walls were made before and after perforation of the pulmonary valve using radiofrequency energy in a patient with pulmonary atresia and intact ventricular septum. The ratio of peak tissue velocity during rapid ventricular filling to atrial contraction increased for both atrioventricular valves after perforation of the pulmonary valve, and the patient was able to be weaned off prostaglandins without further intervention. Such measurements made using tissue Doppler may aid in the management of patients with pulmonary atresia and intact ventricular septum by predicting improvements in right ventricular relaxation.

Patients with a functionally univentricular circulation represent a challenge for both surgeons and cardiologists. In this introductory paper, we aim to describe the variability in cardiac phenotype amongst these patients, and to review how function might relate to anatomy.

Objective: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. Methods: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. Results: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent–child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. Conclusions: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.