While guidelines recommend echocardiography for pregnant women with heart disease, there are limited data on its effect on clinical practice. In this study, we investigated pregnancy-associated echocardiographic changes and their impact on management.

This was a retrospective study of pregnant women with heart disease followed at an academic medical centre from 2016 to 2020. Data on maternal intrapartum and postpartum echocardiograms were collected and the impact on management analysed.

421 echocardiograms in 232 pregnancies were included in the study. The most common cardiac diagnosis was CHD (60.8% of pregnancies), followed by cardiomyopathy (9.9%). The frequency of baseline echocardiographic abnormalities varied by diagnosis, with abnormal right ventricular systolic pressure being the most common (15.0% of pregnancies in CHD and 23.1% of pregnancies with cardiomyopathy). 39.2% of the 189 follow-up echocardiograms had a significant change from the prior study, with the most common changes being declines in right ventricular function (4.2%) or left ventricular function (3.7%), and increases in right ventricular systolic pressure (5.3%) and aortic size (21.2%). 17.8% of echocardiograms resulted in a clinical management change, with the most common change being shorter interval follow-up.

Echocardiographic changes in pregnant women with heart disease are common, in particular increases in aortic size. Echocardiography results in changes in management in a small but significant proportion of patients. Further studies are needed to determine how other factors, including patient access and resource allocation, factor into the use of echocardiography during pregnancy.

Candida infections have become one of the most common causes of morbidity and mortality in paediatric ICUs, especially following complex surgeries, all over the world. Therefore, we conducted a 5-year analysis of Candida bloodstream infections in our tertiary paediatric cardiovascular surgery ICU.

One thousand nine hundred and thirty four children, 0–16-year-old, who underwent paediatric cardiovascular surgery between January 2016–June 2021 were enrolled in this retrospective study. Blood cultures obtained from 1056 patients, who needed mechanical ventilation and indwelling devices longer than 5 days and had the signs of infection according to Center for Disease Control criteria, were evaluated. The isolated pathogens were recorded. 137 with Candida bloodstream infections were reanalysed for their age, weight, cardiac pathologies, duration of mechanical ventilation, hospitalisation and antibiotic use.

One hundred and thirty-seven out of one thousand and fifty six patients (12.9%) had Candida growth in their blood cultures. C. albicans (n: 50, 36.5%), C. parapsilosis (n: 20, 14.6%), C. tropicalis (n: 8, 5.8%), C. glabrata (n: 5, 3.7%), and other non-albicans Candida species (n: 54, 39.4%) were isolated. The patients with Candida bloodstream infections had lower age, longer duration of mechanical ventilation, longer length of hospital stay and antibiotic use (p-values<0.05). They had cardiac pathologies as atrioventricular septal defect (18.9%), transposition of great arteries (17.6%), tetralogy of Fallot (12.4%), transposition of great arteries + double outlet right ventricle, or total anomalous pulmonary venous return + atrioventricular septal defect (37.9%), and others. The Candida bloodstream infections mortality was 11.6% (16/137).

The most common cause of Candida bloodstream infections in the last five years in our paediatric cardiovascular surgery ICU was non-albicans Candida species. Prolonged mechanical ventilation, hospitalisation and antibiotic use, low age, and weight were found as the main risk factors that raise the morbidity and mortality rates of Candida bloodstream infections.

The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology.

A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the “final” Residual Lesion Score.

The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the “final” Residual Lesion Score.

Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network’s Residual Lesion Score study.

To determine the safety and feasibility of over-expansion of right ventricle to pulmonary artery conduits during transcatheter pulmonary valve placement.

Transcatheter pulmonary valve placement is an alternative to surgical pulmonary valve replacement. Traditionally, it was thought to be unsafe to expand a conduit to >110% of its original size.

This retrospective cohort study from two centers includes patients with right ventricle to pulmonary artery conduits with attempted transcatheter pulmonary valve placement from 2010 to 2017. Demographic, procedural, echocardiographic and follow-up data, and complications were evaluated in control and overdilation (to >110% original conduit size) groups.

One hundred and seventy-two patients (51 overdilation and 121 control) had attempted transcatheter pulmonary valve placement (98% successful). The overdilation group was younger (11.2 versus 16.7 years, p < 0.001) with smaller conduits (15 versus 22 mm, p < 0.001); however, the final valve size was not significantly different (19.7 versus 20.2 mm, p = 0.2). Baseline peak echocardiographic gradient was no different (51.8 versus 55.6 mmHg, p = 0.3). Procedural complications were more frequent in overdilation (18%) than control (7%) groups (most successfully addressed during the procedure). One patient from each group required urgent surgical intervention, with no procedural mortality. Follow-up echocardiographic peak gradients were similar (24.1 versus 26 mmHg, p = 0.5).

Over-expansion of right ventricle to pulmonary artery conduits during transcatheter pulmonary valve placement can be performed successfully. Procedural complications are more frequent with conduit overdilation, but there was no difference in the rate of life-threatening complications. There was no difference in valve function at most recent follow-up, and no difference in rate of reintervention. The long-term outcomes of transcatheter pulmonary valve placement with conduit over-expansion requires further study.

This study aims to add proof to the safety profile of propranolol as first-line choice in treating infantile haemangiomas, in particular related to its cardiac side effects the main hindering reason for parents and physicians to start and comply with treatment.

This is a prospective observational and analytic study with a sample of 476 patients diagnosed with infantile haemangioma and treated with systemic propranolol during the time interval January 2011 to December 2021. We studied clinical propranolol adverse events experienced in hospital or outpatient and measured the impact of propranolol on blood pressure and heart rate.

This study showed that symptomatic adverse events caused by propranolol were mild and severe adverse events were rare. The most common clinical side effects were paleness, sweating, reduced feeding, and agitation. Only in 28 (5.9%) cases these symptoms were severe enough to review treatment, 1.8% had severe respiratory symptoms, 2.7% experienced hypoglycaemia, and 1.2% had heart-related symptoms. Mean blood pressure reduction with treatment was statistically significant only after achieving the maintenance dose 2 mg/kg body weight. Blood pressure under the 5th percentile was registered in 2.9% of cases, but only four patients had symptomatic hypotension. While heart rate reduction was noticed with the first dose, only two experienced symptomatic bradycardia.

We conclude that propranolol is not only an excellent drug in treating infantile haemangioma, but it has also a very safe profile, with mild side effects and very rare severe cardiac adverse events, easily overcome with treatment interruption.

The treatment of neonates with unrepairable heart valve dysfunction remains an unsolved problem because there are no growing heart valve replacements. Heart valve transplantation is a potential approach to deliver growing heart valve replacements. Therefore, we retrospectively analysed the semilunar valve function of orthotopic heart transplants during rejection episodes.

We included children who underwent orthotopic heart transplantation at our institution and experienced at least one episode of rejection between 1/1/2010 and 1/1/2020. Semilunar valve function was analysed using echocardiography at baseline, during rejection and approximately 3 months after rejection.

Included were a total of 31 episodes of rejection. All patients had either no (27) or trivial (4) aortic insufficiency prior to rejection. One patient developed mild aortic insufficiency during a rejection episode (P = 0.73), and all patients had either no (21) or trivial (7) aortic insufficiency at follow-up (P = 0.40). All patients had mild or less pulmonary insufficiency prior to rejection, which did not significantly change during (P = 0.40) or following rejection (P = 0.35). Similarly, compared to maximum pressure gradients across the valves at baseline, which were trivial, there was no appreciable change in the gradient across the aortic valve during (P = 0.50) or following rejection (P = 0.42), nor was there any meaningful change in the gradient across the pulmonary valve during (P = 0.55) or following rejection (P = 0.91).

This study demonstrated that there was no echocardiographic evidence of change in semilunar valve function during episodes of rejection in patient with heart transplants. These findings indicate that heart valve transplants require lower levels of immune suppression than orthotopic heart transplants and provide partial foundational evidence to justify future research that will determine whether heart valve transplantation may deliver growing heart valve replacements for children.

Surgery is considered as the first-line therapeutic strategy of partial anomalous pulmonary venous connection. The Warden technique has very good short-term results. The aim of this study is to evaluate the stability of these good results over a long period of follow-up.

We reviewed all patients who underwent a Warden procedure for partial anomalous pulmonary venous connection between 1997 and 2017 in our centre. A total of 73 patients were included. The median age was 14 years (5 months–72 years). Post-operative data were obtained through our hospital network. Late follow-up data were obtained through referrals, cardiologist letters and directly from the patient.

The mean length of follow-up was 8 years and the longest time was 22 years (range, 1–22). Twenty-five percent of our cohort had more than 10 years of follow-up. There were no cardio-vascular deaths. Eight (11.6%) patients suffered from post-operative rhythm disturbances, 5 (7.2%) of which were permanent. Two (2.9%) patients required a pacemaker implantation. At the end of the follow-up period, only one patient remained pacemaker dependent. There were no pulmonary vein obstructions. Two (2.9%) caval vein stenosis were detected, one at 7 months and the other at 7 years. These patients were treated by angioplasty alone. Five (6.8%) patients were lost to follow-up.

The good short-term results of the Warden procedure for right partial anomalous pulmonary venous connection appear to persist in the long term, with excellent freedom from pulmonary and caval stenosis in adolescence through to adulthood.

Kawasaki disease is a vasculitis that can lead to cardiac complications, including coronary artery disease and cardiogenic shock. Various scoring systems have been developed to determine those that will be refractory to routine intravenous immunoglobulin therapy or develop coronary artery disease. The objective of this study was to determine if the neutrophil–lymphocyte ratio could predict refractory disease and coronary artery lesions in patients with Kawasaki disease.

A systematic review of the literature was performed to identify manuscripts describing comparisons of neutrophil–lymphocyte ratio between those who had refractory disease and those who did not, and between those who developed coronary artery lesions and those who did not. Mean difference was compared between groups. Areas under the curve were utilised to determine the pooled area under the curve.

12 studies with 5593 patients were included in the final analyses of neutrophil–lymphocyte ratio for the prediction of refractory disease. Neutrophil–lymphocyte ratio before therapy was higher in refractory disease with a mean difference of 2.55 (p < 0.01) and pooled area under the curve of 0.724. Neutrophil–lymphocyte ratio after therapy was higher in refractory disease with a mean difference of 1.42 (p < 0.01) and pooled area under the curve for of 0.803. Five studies with 1690 patients were included in the final analyses of neutrophil–lymphocyte ratio for the prediction of coronary artery lesions. Neutrophil–lymphocyte ratio before therapy was higher in coronary artery lesions with a mean difference of 0.65 (p < 0.01).

The use of neutrophil–lymphocyte ratio may help physicians in the identification of patients at risk of refractory disease and coronary artery lesions in patients with Kawasaki disease.

Fontan baffle punctures and creation of Fontan fenestration for cardiac catheterisation procedures remain challenging especially due to the heavy calcification of prosthetic material and complex anatomy.

We sought to evaluate our experience using radiofrequency current via surgical electrocautery needle for Fontan baffle puncture to facilitate diagnostic, electrophysiology, and interventional procedures.

A retrospective chart review of all Fontan patients (pts) who underwent Fontan baffle puncture using radiofrequency energy via surgical electrocautery from three centres were performed from January 2011 to July 2021.

A total of 19 pts underwent 22 successful Fontan baffle puncture. The median age and weight were 17 (3–36 years) and 55 (14–88) kg, respectively. The procedural indications for Fontan fenestration creation included: diagnostic study (n = 1), atrial septostomy and stenting (n = 1), electrophysiology study and ablation procedures (n = 8), Fontan baffle stenting for Fontan failure including protein-losing enteropathy (n = 7), and occlusion of veno-venous collaterals (n = 2) for cyanosis. The type of Fontan baffles included: extra-cardiac conduits (n = 12), lateral tunnel (n = 5), classic atrio-pulmonary connection (n = 1), and intra-cardiac baffle (n = 1). A Fontan baffle puncture was initially attempted using traditional method in 6 pts and Baylis radiofrequency trans-septal system in 2 pts unsuccessfully. In all pts, Fontan baffle puncture using radiofrequency energy via electrocautery needle was successful. The radiofrequency energy utilised was (10–50 W) and required 1–5 attempts for 2–5 seconds. There were no vascular or neurological complications.

Radiofrequency current delivery using surgical electrocautery facilitates Fontan baffle puncture in patients with complex and calcified Fontan baffles for diagnostic, interventional, and electrophysiology procedures.