Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined.

We are reporting a case series of this rare CHD with its long-term outcome.

We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up.

Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis.

Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.

Review a single-centre experience with pulmonary artery sling repair and evaluate risk factors for re-intervention.

Patients with surgically repaired pulmonary artery sling at a single institution between 1996 and 2018 were retrospectively reviewed. A univariate Cox regression analysis was used to evaluate variables for association with freedom from re-intervention.

Eighteen patients had pulmonary artery sling repair. At operation, median age and weight were 6.9 months (interquartile range 4.1–18.1) and 9.5 kg (interquartile range 6.5–14.5), respectively. A median hospital length of stay was 12 days (interquartile range 5.8–55.3). Twelve patients (67%) had complete tracheal rings, of whom six (50%) underwent tracheoplasty (five concurrently with pulmonary artery sling repair). Airway re-intervention was required in five (83%) of the six patients who underwent tracheoplasty. One patient had intraoperative diagnosis and repair of pulmonary artery sling during unrelated lesion repair and required tracheoplasty 24 days post-operatively. One patient died 55 days after pulmonary artery sling repair and tracheoplasty following multiple arrests and re-interventions. Median post-operative follow-up for surviving patients was 6.3 years (interquartile range 11 months–13 years), at which time freedom from re-intervention was 61%. When controlling for patient and tracheal size, initial tracheoplasty was associated with decreased freedom from re-intervention (hazard ratio 21.9, 95% confidence interval 1.7–284.3, p = 0.018).

In patients with pulmonary artery sling, tracheoplasty is associated with decreased freedom from re-intervention. In select patients with pulmonary artery sling and complete tracheal rings, conservative management without tracheoplasty is feasible. Further study is necessary to delineate objective indications for tracheoplasty.

Identify diagnostic yield and frequency of echocardiograms for palpitation-related indications at outpatient paediatric cardiology clinics in relation to the 2014 ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE appropriate use criteria for Initial Transthoracic Echocardiography in Outpatient Paediatric Cardiology.

A single-centre, retrospective study of children presenting for evaluation of a chief complaint of palpitations to outpatient paediatric cardiology clinics from 2015 to 2017. Palpitations were defined as an unpleasant sensation of rapid, irregular, and/or forceful beating of the heart. Indications for echocardiogram in patients were retrospectively classified based on the appropriate use criteria as “appropriate,” “may be appropriate,” or “rarely appropriate.” The incidence of abnormal and incidental echocardiographic findings for each category was determined.

A total of 286 patients presented with palpitations, with 128 (52% female) meeting inclusion criteria. Exclusion criteria included patients with additional cardiac complaints, prior echocardiogram, or history of congenital heart disease. Echocardiograms were performed on 36 (28%) patients. The appropriate use criteria were retrospectively applied, and indications for their performance were classified as “appropriate” (n = 4), “may be appropriate” (n = 17), or “rarely appropriate” (n = 15). Minor echocardiographic abnormalities were present in 22% (n = 8) of echocardiograms obtained for all appropriate use criteria classifications. No moderate or severe echocardiographic abnormalities were found. Incidental findings were noted in eight echocardiograms.

Echocardiography in the evaluation of “rarely appropriate” and “may be appropriate” palpitation-related indications is of low diagnostic yield.

Prostaglandin E1 is used to maintain ductal patency in critical congenital heart disease (CHD). The standard starting dose of prostaglandin E1 is 0.05 µg/kg/minute. Lower doses are frequently used, but the efficacy and safety of a low-dose regimen of prostaglandin E1 has not been established.

We investigated neonates with critical CHD who were started on prostaglandin E1 at 0.01 µg/kg/minute. We reviewed 154 consecutive patients who were separated into three anatomical groups: obstruction to systemic circulation, obstruction to pulmonary circulation, and inadequate mixing (d-transposition of the great arteries). Treatment failure rates and two commonly reported side effects, respiratory depression and seizure, were studied.

A total of 26 patients (17%) required a dose increase in prostaglandin E1. Patients with pulmonary obstruction were more likely to require higher doses than patients with systemic obstruction (15/49, 31% versus 9/88, 10%, p = 0.003). Twenty-eight per cent of patients developed respiratory depression and 8% of patients needed mechanical ventilation. Prematurity (<37 week gestation) was the primary risk factor for respiratory depression. No patient required dose escalation or tracheal intubation while on transport. No patient had a seizure attributed to prostaglandin E1.

Prostaglandin E1 at an initial and maintenance dose of 0.01 µg/kg/minute was sufficient to maintain ductal patency in 83% of our cohort. The incidence of respiratory depression requiring mechanical ventilation was low and was mostly seen in premature infants. Starting low-dose prostaglandin E1 at 0.01 µg/kg/minute is a safe and effective therapy for critical CHD.

Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity.

We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8–68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient’s self-estimated exercise capacity with focus on differences between complex and non-complex defects.

Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho −0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho −0.11, p < 0.255).

The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD.

Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Cardiopulmonary exercise testing performance has been shown to be a predictor of morbidity, mortality, and quality of life in patients with Fontan physiology; however, the role of exercise performance along with other diagnostics is not fully understood. We evaluated the hypothesis that reduced exercise performance correlates with poorer quality of life in Fontan patients as they continue to age.

Chart review was performed on patients 12 years and older with Fontan who had completed cardiopulmonary exercise testing and age-appropriate quality of life surveys. Quality of life outcomes were analysed against exercise performance and other descriptive data.

For the younger cohort (n = 22), exercise performance predicted quality of life with different measures across domains and had a stronger correlation than echocardiographic parameters. For the older cohort (n = 34), exercise performance did not predict quality of life.

Objective exercise performance was a useful marker for general, physical, emotional, social, and school quality of life in a younger cohort but less helpful in older adults. This is perhaps due to older patients accommodating to their conditions over time. The role of exercise performance and objective data in predicting quality of life in patients with Fontan physiology is incompletely understood and additional prospective evaluation should be undertaken.

Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population.

Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients’ self-perception of their quality of life; cardiac and demographic parameters were collected.

Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters.

Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients’ quality of life.

Treatment of hypoplastic left heart syndrome varies across institutions. This study examined the impact of introducing a standardised programme.

This retrospective cohort study evaluated the effects of a comprehensive strategy on 1-year transplant-free survival with preserved ventricular and atrioventricular valve (AVV) function following a Norwood operation. This strategy included standardised operative and perioperative management and dedicated interstage monitoring. The post-implementation cohort (C2) was compared to historic controls (C1). Outcomes were assessed using logistic regression and Kaplan–Meier analysis.

The study included 105 patients, 76 in C1 and 29 in C2. Groups had similar baseline characteristics, including percentage with preserved ventricular (96% C1 versus 100% C2, p = 0.28) and AVV function (97% C1 versus 93% C2, p = 0.31). Perioperatively, C2 had higher indexed oxygen delivery (348 ± 67 ml/minute/m2 C1 versus 402 ± 102ml/minute/m2 C2, p = 0.015) and lower renal injury (47% C1 versus 3% C2, p = 0.004). The primary outcome was similar in both groups (49% C1 and 52% C2, p = 0.78), with comparable rates of death and transplantation (36% C1 versus 38% C2, p = 0.89) and ventricular (2% C1 versus 0% C2, p = 0.53) and AVV dysfunction (11% C1 versus 11% C2, p = 0.96) at 1-year. When accounting for cohort and 100-day freedom from hospitalisation, female gender (OR 3.7, p = 0.01) increased and ventricular dysfunction (OR 0.21, p = 0.02) and CPR (OR 0.11, p = 0.002) or ECMO use (OR 0.15, p = 001) decreased the likelihood of 1-year transplant-free survival.

Standardised perioperative management was not associated with improved 1-year transplant-free survival. Post-operative ventricular or AVV dysfunction was the strongest predictor of 1-year mortality.

There is limited data describing the characteristics of paediatric post-operative cardiac surgery patients who develop pneumothoraces after chest tube removal. Patient management after chest tube removal is not standardised across paediatric cardiac surgery programmes. The purposes of this study were to describe the frequency of pneumothorax after chest tube removal in paediatric post-operative cardiac surgical patients and to describe the patient and clinical characteristics of those patients who developed a clinically significant pneumothorax requiring intervention.

A single-institution retrospective descriptive study (1 January, 2010–31 December, 2018) was utilised to review 11,651 paediatric post-operative cardiac surgical patients from newborn to 18 years old.

Twenty-five patients were diagnosed with a pneumothorax by chest radiograph following chest tube removal (0.2%). Of these 25 patients, 15 (1.6%) had a clinically significant pneumothorax and 8 (53%) did not demonstrate a change in baseline clinical status or require an increase in supplemental oxygen, 14 (93%) required an intervention, 9 (60%) were <1 year of age, 4 (27%) had single-ventricle physiology, and 5 (33%) had other non-cardiac anomalies/genetic syndromes.

In our cohort of patients, we confirmed the incidence of pneumothorax after chest tube removal is low in paediatric post-operative cardiac surgery patients. This population does not always exhibit changes in clinical status despite having clinically significant pneumothoraces. We suggest the development of criteria, based on clinical characteristics, for patients who are at increased risk of developing a pneumothorax and would require a routine chest radiograph following chest tube removal.