Chronic pulmonary regurgitation following tetralogy of Fallot repair burdens the right ventricle. This study evaluated early outcomes of pulmonary valve reconstruction using right atrial tissue versus standard transannular patch repair.

A retrospective analysis of 412 tetralogy of Fallot patients (2014–2024) was conducted: Atrial Valve Group (n = 205) underwent valve reconstruction; No-Valve Group (n = 207) received standard repair. Patients were followed for 1 year with echocardiographic assessments. Outcomes included right ventricular insufficiency, ventilation duration, and ICU stay.

Atrial Valve Group had lower right ventricular insufficiency at 12 months (9.3% vs. 19.8%, p = 0.004, OR = 2.39, 95% CI: 1.32–4.33), shorter ventilation times (6.1 vs. 18.0 hours, p < 0.001, Cohen’s d = 3.54), and reduced ICU stays (3.0 vs. 5.7 days, p < 0.001, Cohen’s d = 1.87), despite longer CPB durations (47.1 vs. 40.5 minutes, p = 0.02).

Right atrial tissue reconstruction reduces early and intermediate-term right ventricular dysfunction post-tetralogy of Fallot repair. Long-term studies are needed.

Pulmonary regurgitation leading to right ventricular enlargement may occur after repaired tetralogy of Fallot (rTOF) or balloon dilation for pulmonary valve stenosis. Cardiac magnetic resonance imaging (CMR) guidelines to identify the timing of valve replacement in rTOF are not necessarily applicable to isolated pulmonary regurgitation. This study aims to compare deformation parameters of isolated pulmonary regurgitation and rTOF at comparable right ventricular volume loads.

Adopting a quantitative retrospective analytic framework, CMR was performed in 44 patients (0–30 years), 22 in each of the isolated pulmonary regurgitation and rTOF study arms, matched for age (±12 months), and Right ventricular end-diastolic volume z-score (±1). Right ventricular longitudinal strain/strain rate and circumferential strain/strain rate were measured. Comparisons between groups were analysed using two-tailed T-tests and one-way ANOVA.

Both groups showed predominance of longitudinal over circumferential strain. Circumferential strain was significantly greater in rTOF compared to isolated pulmonary regurgitation (–26.5% versus –22.3%, p < 0.05). Longitudinal strain did not differ between groups. The longitudinal:circumferential strain ratio was significantly lower in rTOF compared to isolated pulmonary regurgitation (1.24 versus 1.53, p = 0.05). Circumferential and longitudinal strain rates did not differ between groups.

The right ventricles in rTOF demonstrate greater reliance on circumferential strain in response to increased volumes. The decrease in longitudinal:circumferential strain ratio suggests rTOF right ventricles display a greater adaptive response to the volume load than isolated pulmonary regurgitation, highlighting the importance of the relative contributions of both circumferential and longitudinal strain in order to understand the mechanisms of right ventricular dysfunction in pulmonary regurgitation.

Progressive ventricular remodelling in children with repaired tetralogy of Fallot may or may not result in the need for pulmonary valve replacement. We aimed to model and compare the rates of right and left ventricular adaptation over time, as assessed by cardiac MRI after surgical repair of tetralogy of Fallot, in children who did or did not require pulmonary valve replacement later in adolescence.

Single-centre, retrospective cohort study from 2000 to 2020 including patients with tetralogy of Fallot who had complete surgical repair before 24 months.

From 214 patients included in this analysis, 142 (66.3%) had pulmonary valve replacement at a median age of 12 years (interquartile range 9–15.5) during follow-up. Assessing 323 cardiac MRI studies from 201 patients commencing from a median age of 9.4 years (interquartile range 5.9–12.3), the group that required pulmonary valve replacement later during the follow-up had a steeper time-related right ventricular dilation trajectory than non-pulmonary valve replacement patients: the increment in right ventricular end-diastolic volume index was 19.4 versus 2.8 ml/m2/log2year, P < 0.001; also, right ventricular end-systolic volume index incremented more quickly, at 11.9 versus 0.8 ml/m2/log2year, P < 0.001. Left ventricular end-diastolic volume index increased more quickly in patients who eventually had pulmonary valve replacement, at 7.2 versus 1.5 ml/m2/log2year, P = 0.005; the same occurred for indexed left ventricular end-systolic volume at 3.2 versus –0.4 ml/m2/log2year, P = 0.001.

Early right and left ventricular dilation over time are identifiable by cardiac MRI in patients destined to require pulmonary valve replacement following tetralogy of Fallot repair.

Tetralogy of Fallot is the most common cyanotic congenital malformation of the heart. The right ventricular outflow tract is of great interest in this setting, but most of the focus on this feature has been on the size of the so-called pulmonary valvar “annulus”. We aimed to characterise other aspects of the morphology of the pulmonary root in heart specimens with tetralogy of Fallot.

We reviewed archived hearts with tetralogy of Fallot from four registries. The pulmonary root was examined with specific attention to the number of sinuses, the number of leaflets, presence of any fusion of leaflets, and the direction of the zone of apposition between the leaflets. Cluster analyses were then conducted to see if the features permitted segregation into groups.

We examined a total of 155 hearts. The pulmonary valve had two leaflets in 62%, three leaflets in 34%, and one leaflet in 3%. Irrespective of leaflet morphology, most hearts had two sinuses. Cluster analysis permitted segregation into three groups, with the direction of the zone of apposition being the most important feature for segregation.

In two-thirds of our hearts with tetralogy of Fallot, the pulmonary valve had two leaflets. Most frequently there were three sinuses. In the setting of a valve with two sinuses, the zone of apposition between the leaflets pointing towards the aorta. Cluster analysis permitted statistically sound segregation of the heart and highlights the importance of delineating these features, specifically the leaflet and sinus morphology, with clinical imaging.

Right ventricular outflow tract stenting has emerged as a key palliative intervention for infants with tetralogy of Fallot who are not suitable candidates for complete repair. Although the Blalock-Taussig shunt remains the standard palliative approach for tetralogy of Fallot patients over one year of age, the potential of right ventricular outflow tract stenting in this older age group has not been widely explored. In this study, we present our experience with right ventricular outflow tract stenting in children beyond one year of age.

In this study, a total of 52 patients of tetralogy of Fallot who underwent palliative stenting of the right ventricular outflow tract from 2018 to 2022 were enrolled. Out of the 52 patients, 38 children were more than 1 year of age with a mean age of 4.82 ± 3.5 (1.5–13 years) and mean weight of 13.10 ± 7.0 (5.6–34) kgs. Most common indication was presence of unfavourable anatomy in 27 (71%) followed by presence of refractory spells in 14 (36.8%) patients. Stent embolisation was seen in one patient, while two patients developed features of pulmonary oedema and needed prolonged ventilatory support. There was no mortality seen in this study group.

Our study has shown that right ventricular outflow tract stenting in children even beyond one year of age is technically feasible, with good immediate outcomes, especially in those who present with any complication, thus reducing the perioperative morbidity and mortality.

Tetralogy of Fallot patients face an elevated risk of developing chylothorax and pleural effusions post-surgery. This patient group exhibits risk factors known to compromise the lymphatic system, such as elevated central venous pressure, pulmonary flow changes, and hypoxia. This study investigates the morphology and function of the lymphatic system in tetralogy of Fallot patients through lymphatic magnetic resonance imaging and near-infrared fluorescence imaging, respectively.

Post-repair tetralogy of Fallot patients aged 6–18 years were recruited, along with age and gender-matched controls. Magnetic resonance imaging was used to assess the morphology of the thoracic lymphatic vessels and the thoracic, while near-infrared fluorescence imaging was used to assess lymphatic activity utilising lymph rate, velocity, and pressure.

Nine patients and 10 controls were included. Echocardiography revealed that 2/3 of the patients had moderate-severe pulmonary regurgitation, while none displayed signs of elevated central venous pressure. Magnetic resonance imaging identified three patients with type 3 (out of 4 types) lymphatic abnormalities, while controls had none. The thoracic ducts showed severe (one patient) and moderate (one patient) tortuosity. Mean thoracic duct diameters were 3.3 mm ±1.1 in patients and 3.0 mm ± 0.8 in controls (p-value = 0.53). Near-infrared fluorescence imaging revealed no anomalous patterns.

Despite no presence of clinical lymphatic disease, 3/9 of the repaired tetralogy of Fallot patients exhibited lymphatic morphological abnormalities. The significance of these anomalies remains uncertain currently. Further research is needed to determine whether these lymphatic alterations in this patient cohort are a result of congenital malformations, haemodynamic shifts, or prenatal and early-life saturation levels.

Modified Blalock-Taussig shunt (BT shunt) is a palliative operation used for cyanotic heart diseases with decreased pulmonary blood supply. The definitive management of tetralogy of Fallot (TOF) is total corrective surgery, but these patients can be palliated with BT shunt. In the modern world, the BT shunt is getting out of favour in patients with TOF. In this article, we will share our 5-year experience at our institute, which also shows a decreasing trend.

It is a retrospective study. Files of all the patients admitted in our department from January 2019 to December 2023 were reviewed. Age, weight, hospital stay, inotropic support duration, mechanical ventilation duration, and outcomes were studied.

From January 2019 to December 2023, 173 patients underwent BT shunt for TOF. The mean age was 31 months, and the mean weight was 9.3 kg. The overall mortality for BT shunt was 15% after BT shunt. Hypercyanotic spell not controlled by medical management was the most common indication for BT shunt in our setup. Most of the patients with hypercyanotic spells were also candidates for total correction but due to the emergency, BT shunt was performed.

The role of BT shunt in patients with TOF is decreasing due to PDA/RVOT stenting, it is likely that the BT shunt in TOF will become a thing of the past in the future even in developing countries like ours.

We report the midterm results of our strategy utilizing transatrial-transpulmonary repair for tetralogy of Fallot at a single institution in a low-middle income country.

Medical records were retrospectively reviewed for 532 consecutive patients who underwent definitive repair of tetralogy of Fallot at our institution from 2010 to 2020.

The median age and weight of patients in the study patients were 11.6 months (interquartile range, 8.6–17.2 months) and 7.5 kg (interquartile range, 6.8-8.8 kg). The pulmonary valve annulus was preserved (no transannular patch) in 398 patients (75%) and a mini-transannular patch was utilized for 134 patients (25%). The overall survival was 98% at 1 year, and 97% at 10-years follow-up, respectively. Longer postoperative ventilation time was the only risk factor correlated to early death (p = 0.004; Odds Risk, 1.04; 95% confidence intervals, 1.01–1.07). Fourteen patients required pulmonary valve replacement (2.6%, 14/532), four required surgical resection to relieve right ventricular outflow tract obstruction (0.8%, 4/532), and freedom from reoperation of the right ventricular outflow tract was 87% at 10 years. The only risk factor for right ventricular outflow tract reoperation was a postoperative systolic pressure gradient through the right ventricular outflow tract of greater than 50 mmHg (p < 0.001; HR, 47; 95% confidence intervals, 9.1–244). In total, 94.6% (471/489) of the patients were asymptomatic at the latest follow-up without significant arrhythmia.

At our institution in an low-middle income country, the transatrial-transpulmonary repair for tetralogy of Fallot has excellent midterm results with few reoperations required. Close long-term follow-up is essential for patients who undergo repair with a mini-transannular patch and may eventually require pulmonary valve replacement.