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Aortopathy including Marfan (MFS) and Loeys-Dietz syndrome (LDS) poses a high risk of aortic dissection, particularly during pregnancy and the puerperium. Current preventive measures of aortic root dilatation include medical therapy and prophylactic aortic root replacement. The Personalised External Aortic Root Support (PEARS) operation has been developed as an alternative surgical strategy to prevent aortic root dilatation and is now an established procedure with a good prognosis. However, outcomes in pregnant women are unknown. We present case series of nine successful pregnancies in seven women with aortopathy (6 MFS and 1 LDS) who underwent PEARS procedure prior to conception. At a mean follow-up of 4.3 years after delivery, there was no type A or B aortic dissections. Aortic dimensions remained stable, and no hypertensive disorders were observed. Although this is a small retrospective study, PEARS procedure may be a viable pre-conception surgical strategy for women with aortopathy, as an alternative to conventional aortic root surgery. Further studies are needed to conclude that PEARS could be a non-inferior or superior alternative to conventional aortic root surgery in these patients. Aortopathy poses a high risk of aortic dissection, particularly during pregnancy and the postpartum period. Here the authors report a retrospective case series of seven women with aortopathy who underwent PEARS, a surgical strategy to prevent aortic root dilatation, and a subsequent pregnancy.

Since its first description in 1971, the Fontan procedure and its modifications have led to a substantial improvement in the survival rates of patients with a variety of types of complex Congenital Heart Disease (CHD) characterised by the presence of a single, dominant ventricle. However, despite the significant improvement of the prognosis over the years, Fontan patients are still exposed to several cardiovascular and systemic complications. It is, therefore, important to fully understand the pitfalls hidden behind a Fontan anatomy and the potential predictors of ventricular failure. Cardiovascular imaging plays a key role in this context, allowing for the early identification of complications with important prognostic implications. Echocardiography remains the first-line imaging modality for serial evaluation of Fontan patients. However, there is a growing role of cardiovascular magnetic resonance and cardiac computed tomography from pre-operative assessment to longitudinal follow-up. The aim of this paper will be to provide a comprehensive overview of the role, strengths, and weaknesses of each imaging modality in the assessment of congenital cardiac conditions palliated with the Fontan procedure.

Tetralogy of Fallot (TOF) is the most common complex congenital heart disease with long-term survivors, demanding serial monitoring of the possible complications that can be encountered from the diagnosis to long-term follow-up. Cardiovascular imaging is key in the diagnosis and serial assessment of TOF patients, guiding patients’ management and providing prognostic information. Thorough knowledge of the pathophysiology and expected sequalae in TOF, as well as the advantages and limitations of different non-invasive imaging modalities that can be used for diagnosis and follow-up, is the key to ensuring optimal management of patients with TOF. The aim of this manuscript is to provide a comprehensive overview of the role of each modality and common protocols used in clinical practice in the assessment of TOF patients.

The complete transposition of the great arteries (C-TGA) is a congenital cardiac anomaly characterized by the reversal of the main arteries. Early detection and precise management are crucial for optimal outcomes. This review emphasizes the integral role of multimodal imaging, including fetal echocardiography, transthoracic echocardiography (TTE), cardiovascular magnetic resonance (CMR), and cardiac computed tomography (CCT) in the diagnosis, treatment planning, and long-term follow-up of C-TGA. Fetal echocardiography plays a pivotal role in prenatal detection, enabling early intervention strategies. Despite technological advances, the detection rate varies, highlighting the need for improved screening protocols. TTE remains the cornerstone for initial diagnosis, surgical preparation, and postoperative evaluation, providing essential information on cardiac anatomy, ventricular function, and the presence of associated defects. CMR and CCT offer additional value in C-TGA assessment. CMR, free from ionizing radiation, provides detailed anatomical and functional insights from fetal life into adulthood, becoming increasingly important in evaluating complex cardiac structures and post-surgical outcomes. CCT, with its high-resolution imaging, is indispensable in delineating coronary anatomy and vascular structures, particularly when CMR is contraindicated or inconclusive. This review advocates for a comprehensive imaging approach, integrating TTE, CMR, and CCT to enhance diagnostic accuracy, guide therapeutic interventions, and monitor postoperative conditions in C-TGA patients. Such a multimodal strategy is vital for advancing patient care and improving long-term prognoses in this complex congenital heart disease.

The aim of this study was to establish sex- and age-specific reference values for ramp cycle-ergometer and treadmill cardiopulmonary exercise testing (CPET) in patients with Tetralogy of Fallot (ToF). Despite successful surgical repair, residual pulmonary regurgitation remains common in repaired ToF (rToF), often leading to right or left ventricular dysfunction and reduced exercise capacity. CPET is a reliable tool for evaluating cardiopulmonary function. Although both treadmill and cycle ergometer protocols are used interchangeably, reference value ranges for each method in this population remain unclear. CPET data were collected from asymptomatic rToF patients who had undergone cardiac magnetic resonance imaging (CMR) and performed CPET on a treadmill or cycle ergometer between 2020 and 2024. Physical activity was assessed using the International Physical Activity Questionnaire (IPAQ). Among 290 patients, median age at CPET was 21.6 years (15.9-29.3), and median BMI was 22.5 kg/m (20.0-24.9). Nearly all participants (99%) were in NYHA class I. Physical activity was classified as sedentary in 29%, moderate in 61%, and high in 9%. Median VO₂ peak was 26.4 mL/min/kg (23.0-31.5), corresponding to 72.9% predicted (62.4-83.2). Median oxygen pulse at peak was 9.9 mL/beat (8.2-12.0), with 76.9% predicted (68.0-87.5). Median VE/VCO₂ slope at the respiratory compensation point was 29.0 (26.0-32.6), and median oxygen uptake efficiency slope (OUES) was 1,792.5 mL/min/log(L/min) (1,535.0-2,181.5). VO₂ peak and percent-predicted VO₂ were significantly higher with treadmill testing in both sexes (  < 0.05); oxygen pulse was higher only in females. VE/VCO₂ slope and OUES were unaffected by modality. When stratified by age (<18 vs. ≥18 years), treadmill-related differences in VO₂ peak and percent-predicted VO₂ remained significant in both sexes ≥18 years, and in females <18 years. Oxygen pulse was significantly higher with treadmill only in females ≥18 years. VE/VCO₂ slope and OUES remained unchanged across modalities and age groups. This study provides CPET values stratified by modality and sex in a large cohort of asymptomatic rToF patients, offering valuable reference data for clinical assessment. Future studies should validate pediatric normative CPET values through prospective, inclusive, statistically powered cohorts using standardized protocols and cross-center comparability.

Cardiovascular magnetic resonance (CMR) imaging offers a comprehensive, non-invasive, and radiation-free imaging modality, which provides a highly accurate and reproducible assessment of cardiac morphology and functions across a wide spectrum of cardiac conditions spanning from fetal to adult life. It minimises risks to the patient, particularly the risks associated with exposure to ionising radiation and the risk of complications from more invasive haemodynamic assessments. CMR utilises high spatial resolution and provides a detailed assessment of intracardiac and extracardiac anatomy, ventricular and valvular function, and flow haemodynamic and tissue characterisation, which aid in the diagnosis, and, hence, with the management of patients with cardiac disease. This article aims to discuss the role of CMR and the indications for its use throughout the different stages of life, from fetal to adult life.

Cardiopulmonary exercise testing (CPET) is recommended as part of routine care in people with congenital heart disease. A significant difference has been observed in many CPET parameters, depending on the ergometer and exercise protocol used. The aim of this study is to investigate such differences in Fontan patients. All Fontan patients (<40 years old, NYHA class I/I–II) underwent two consecutive CPETs on different ergometers (treadmill with ramped Bruce protocol versus cycle ergometer with ramp protocol) within less than 12 months. The exclusion criterion was the presence of significant clinical/anthropometric changes between the two tests. Anthropometric, surgical, clinical, electrocardiogram (ECG) and CPET data were collected. 47 subjects were enrolled (25 males, mean age 16.4 at first test). Peak heart rate (HR) tended to be higher on the treadmill (p = 0.05 as % of predicted, p = 0.062 in absolute value). Peak oxygen consumption (VO2) (mL/min, mL/kg/min, and % of predicted) was significantly higher on the treadmill (p < 0.01), as well the VO2 at the ventilatory anaerobic threshold (VAT) and the peak oxygen pulse. A different kinetics of the oxygen pulse wave was observed in the same patient comparing the two testing modalities. Maximal respiratory-exchange-ratio values (>1.1) were reached more frequently on the cycle ergometer (p < 0.001). The minute ventilation–carbon dioxide output slope (VE/VCO2 slope) was not different between the two tests (p = 0.400). Many parameters of CPET may differ depending on the ergometer used. These should be considered in clinical evaluation of Fontan patients and when exercise is to be prescribed.

Hypoplastic Left Heart Syndrome (HLHS) accounts for 2–3% of congenital heart diseases (CHDs). HLHS is characterized by reduced systemic blood flow due to hypoplastic left ventricle (LV) and underdeveloped left-sided cardiac structures. Without a series of staged interventional treatments, HLHS is often fatal, typically within the first hours or days of life. This manuscript aims to provide a comprehensive overview of the role of echocardiography, cardiovascular magnetic resonance (CMR), and cardiac computed tomography angiography (CCTA) in the optimal management of patients with HLHS. Specifically, it explores the contributions of various non-invasive imaging modalities to the diagnosis, planning of staged palliative interventions, interstage monitoring, and long-term follow-up of HLHS. Furthermore, the advantages and limitations of each imaging technique will be highlighted to aid in clinical decision-making; however, it is important to note that, at present, no universal guidelines exist, and imaging strategies remain largely dependent on individual centre expertise and protocols.

We present the case of a 31-year-old female with Fontan circulation who developed signs of protein-losing enteropathy 10 days after second COVID-19 vaccination. After standard investigations for identification of potential triggers for protein-losing enteropathy, we concluded that coronavirus disease 2019 (COVID-19) booster vaccination could have been the most probable underlying trigger. Prompt investigation of new symptoms post-vaccination in high-risk patients is necessary.