Determine whether weight gain velocity (g/day) 30 days after initiating feeds following cardiac surgery and other clinical outcomes improve in infants with single-ventricle physiology fed an exclusive human milk (EHM) diet with early fortification compared to non-protocolised “standard of care.”

This retrospective cohort study compares term infants with single-ventricle physiology who underwent neonatal surgical palliation. The retrospective control group (RCG) was fed according to non-protocolised standard of care at a single centre and was compared with infants in a previous protocolised multi-site randomised controlled trial assigned to either an EHM group or a control group (TCG). The primary outcome measure is weight gain velocity. Secondary outcomes include change in weight z-score, and incidence of feeding intolerance and necrotising enterocolitis.

We evaluated 45 surgically palliated neonates with single-ventricle physiology compared to the prior trial patients (EHM = 55, TCG = 52). Baseline demographics were similar between groups, except the RCG had fewer patients with hypoplastic left heart syndrome (51% vs. 77% vs. 84%, p = 0.0009). The RCG grew similarly to the TCG (7.5 g/day vs. 8.2 g/day), and both groups had significantly lower growth than the EHM group (12 g/day). Necrotising enterocolitis/suspected necrotising enterocolitis were similar in the RCG versus TCG but significantly higher in the RCG compared to the EHM group (20.5% vs. 3.6%, p = 0.033). Incidences of other morbidities were similar.

Neonates with single-ventricle physiology have improved short-term growth and decreased risk of necrotising enterocolitis or suspected necrotising enterocolitis when receiving an EHM diet after surgical palliation compared to non-protocolised feeding with bovine formula.

Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery.

We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital.

We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure.

Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

We evaluated the use of nesiritide in children with critical CHD, pulmonary congestion, and inadequate urine output despite undergoing conventional diuretic therapy.

We conducted a retrospective analysis of 11 patients with critical CHD, comprising 18 infusions, each of which occurred during separate hospitalisations. Haemodynamic parameters were assessed, and the stage of acute kidney injury was determined before and throughout the duration of therapy using a standardised definition of acute kidney injury – The Kidney Disease: Improving Global Outcomes criteria.

Children with critical CHD, pulmonary congestion, and inadequate urinary output despite undergoing diuretic therapy were included.

The use of nesiritide was associated with a significant decrease in the maximum and minimum heart rate values and with a trend towards a significant decrease in maximum systolic blood pressure and maximum and minimum central venous pressures. Urine output increased but was not significant. Serum creatinine levels decreased significantly during the course of therapy (−0.26 mg/dl [−0.50, 0.0], p=0.02), and the number of patients who experienced a decrease in the stage of acute kidney injury of 2 or more – where a change in the stage of acute kidney disease of 2 or more was possible, that is, baseline stage >1 – was highly significant (five of 12 patients, 42%, p<0.001).

Nesiritide had a favourable impact on haemodynamics, and its use was not associated with deterioration of renal function in patients with critical CHD.

A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice.

A web-based survey was distributed via e-mail to over 2000 paediatric cardiologists, intensivists, cardiothoracic surgeons, and cardiac advance practice nurses during three distribution periods. The results were analysed using McNemar’s test for paired data and Fisher’s exact test.

The response rate was 31.5% (69% cardiologists and 65% with >10 years of experience). Among respondents familiar with trial results, 74% reported current practice consistent with trial findings versus 48% before trial publication (p<0.001); 19% used angiotensin-converting enzyme inhibitor in this population “almost always” versus 36% in the past (p<0.001), and 72% reported a change in management or improved confidence in treatment decisions involving this therapy based on the trial results. Respondents familiar with trial results (78%) were marginally more likely to practise consistent with the trial results than those unfamiliar (74 versus 67%, p=0.16). Among all respondents, 28% reported less frequent use of angiotensin-converting enzyme inhibitor over the last 3 years.

Within 5 years of publication, the majority of respondents was familiar with the Infant Single Ventricle Trial results and reported less frequent use of angiotensin-converting enzyme inhibitor in single-ventricle infants; however, 28% reported not adjusting their clinical decisions based on the trial’s findings.