Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables.

Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively.

We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively.

An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

To evaluate the impact of intravenous ketorolac on renal function and haematologic values in patients less than six months old following cardiothoracic surgery.

Ketorolac is a potent nonsteroidal anti-inflammatory drug indicated for short term management of moderate to severe post-operative pain. Little data is available related to its safety in infants less than six months of age.

This was a retrospective, case-control chart review of 19 patients aged less than six months of age with biventricular circulations who received intravenous ketorolac following cardiothoracic surgery. They were compared with 19 age-matched control patients. Those with functionally univentricular anatomy were excluded due to their higher risk for renal impairment following surgery. Student’s t-test was used to compare the incidence of renal impairment and haematologic complications between the groups, as well as the number of analgesic doses administered. Charts were reviewed for number of blood transfusions.

Patients receiving intravenous ketorolac had no statistically significant changes in pre-operative versus post-treatment renal function or haematologic effects compared to the control group. No statistically significant differences were detected for number of post-operative blood transfusions or additional analgesic administration between groups.

Intravenous ketorolac appears to be safe when used in infants less than six months of age with biventricular circulations following cardiothoracic surgery. Ketorolac as used in these patients does not decrease the use of standard analgesic therapy.

While the usefulness of percutaneous endoscopic gastrostomy is clearly established in the nutritional support of children with neurodisability, the role in substituting for prolonged nasogastric feeding in children with congenitally malformed hearts is a relatively recent development. There are no previously published experiences of the perceptions of parents or those providing care following the insertion of percutaneous endoscopic gastrostomy in such children.

Descriptive qualitative survey of parental perceptions using a semi-structured questionnaire.

We obtained completed 27 point semi-structured questionnaires from 38 providers of care for children with congenitally malformed hearts. Time taken to feed their children reduced significantly after the percutaneous endoscopic gastrostomy, from 30 to 60 minutes previously to 15 minutes subsequently. The frequency of feeding also reduced significantly, from 6 times a day to 4 to 5 times a day. Those providing care perceived significant reductions in pre-procedural symptoms, the ease of administering medications, and noted an enhanced level of happiness in their children. Of those providing care, 97% were highly satisfied with the procedure, with 15 parents (40%) wishing that the operation was done earlier, while the remainder considered it had been done at the appropriate time.

Those caring for children with congenitally malformed hearts perceive significant improvements in the symptoms, wellbeing, and ease of administering medication for their children after percutaneous endoscopic gastrostomy. Of the group, 97% regarded the procedure as the appropriate means of assisting nutritional support.

Dexmedetomidine is an α2-adrenergic agonist that causes sleep-like sedation and mild analgesia without narcosis or respiratory depression, and has relative cardiovascular stability. Due to these properties, it may be an effective agent for prolonged use in the sedation of patients in the paediatric cardiothoracic intensive care unit. We reviewed our experience with the drug to detail its safety and efficacy.

We conducted a retrospective chart review of all patients who received dexmedetomidine over a six month period in a dedicated paediatric cardiothoracic intensive care unit. Patients were identified from pharmacy records showing administration of drugs. We collected demographic data, information relating to doses of dexmedetomidine, physiologic parameters, and clinical outcomes.

We identified 54 patients who received the drug. The median age of recipients was 6 months, with a range from 1 day to 16 years. The mean duration of administration was 37.3 hours, with a range from 2 to 177 hours. The mean duration of continuation of administration after extubation was 16.7 hours, with a range from zero to 112.5 hours. Physiologically, there were no clinically significant changes in mean arterial pressure, heart rate, respiratory rate, or saturations of oxygen before, during, or after utilization of the drug. Use of dexmedetomidine significantly reduced the need to administrate narcotics, and scores using the COMFORT system were not different between patients who received dexmedetomidine and those who did not.

In this limited and retrospective review, dexmedetomidine was found to be safe and efficacious. Its use as a sedative agent for extended periods of time in critically-ill children deserves investigation in a prospective and controlled manner.

To identify the causes of death and main cardiovascular complications in adolescents and adults with congenitally malformed hearts.

Retrospective review of 102 necropsy reports from a tertiary centre obtained over a period of 19 years.

The diagnosis, the operated or non-operated state of the main defect, the cause of death, and main complications were related to the age and gender. Other clinically relevant conditions, and identifiable sequels of previous diseases, were also noted.

The ages ranged from 15 to 69 years, with a mean of 31.1 and a median of 28 years, with no difference detected according to the gender. Of the patients, two-thirds had been submitted to at least one cardiac surgery. The mean age of death was significantly higher in non-operated patients (p = 0.003). The most prevalent cause of death in the whole group was related to recent surgery, found in one-third. From them, two-fifths corresponded to reoperations. Among the others, cardiac failure was the main terminal cause in another third, and the second cause was pulmonary thromboembolism in just over one-fifth, presenting a significant association with histopathological signs of pulmonary hypertension (p = 0.011). Infection was the cause of death in 7.8% of the patients, all previously operated. Acute infective endocarditis was present or was the indication for the recent surgery in one-tenth of the patients, this cohort having a mean age of 27.8 years. There was a statistically significant association between the occurrence of endocarditis and defects causing low pulmonary blood flow (p = 0.043).

Data derived from necropsies of adults with congenital heart defects can help the multidisciplinary team refine both their diagnosis and treatment.