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Latest Advances in Tetralogy of Fallot

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: closed (30 April 2022) | Viewed by 4617

Special Issue Editors


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Guest Editor
Department of Pediatric Cardiology, Cardiac Surgery and Heart Lung Transplantation, Bambino Gesu Children’s Hospital and Research Institute, IRCCS, Rome, Italy
Interests: pulmonary valve replacement; tetralogy of Fallot; genetic abnormalities; QRS fragmentation; cardiopulmonary stress test in tetralogy of Fallot; congenital heart disease
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Guest Editor
Chief Pediatric Cardiac Surgery Unit, Department of Cardiac Surgery, Cardiology and Heart and Lung Transplant, Bambino Gesù Children Hospital, Rome, Italy
Interests: fontan circulation; neonatal repair of congenital heart disease; HLHS & single ventricle physiology and surgery; mechanical circulatory support; heart transplantation; adult congenital heart disease surgery
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Although the survival rate after Tetralogy of Fallot repair in infants has greatly increased, reaching over 95–97% with survival after 20 years of over 90%, the pulmonary regurgitation consequence of the right ventricular outflow tract reconstruction can lead to serious problems in the long-term follow-up of these patients. Indeed, even if the majority of cases are asymptomatic and those affected live a satisfactory life, the chronic volume overload can lead to right ventricle (RV) dilation, which in turn can determine biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Therefore, they need to undergo a further operation for the replacement of the pulmonary valve (PVR) in a certain moment of their life to avoid these adverse outcomes. PVR is not a risk-free procedure and the prosthetic valves currently available have a limited lifespan. Therefore, it would be desirable to perform PVR in the asymptomatic patient, just before the onset of ventricular dysfunction, if we could know at what threshold of RV dilation it arises. In addition, it is still not fully understood at what threshold of dysfunction, whether it is right or left, the percentage of having a fatal arrhythmia, significantly increases. Consequently, it continues to be complicated in asymptomatic repaired tetralogy of Fallot, to choose the right timing between a potentially fatal arrhythmia and/or a not-recoverable ventricular dysfunction and an early PVR with the increased risk related to repeated prosthetic valve replacement and endocarditis. Hence, this Special Issue will highlight recent advances in support of the assessment of biventricular function (echocardiographic and cardiac magnetic resonance strain) and the objective evaluation of the functional capacity (cardiopulmonary exercise testing).

Dr. Benedetta Leonardi
Dr. Lorenzo Galletti
Guest Editors

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Keywords

  • Right ventricular dysfunction
  • Left ventricular dysfunction
  • Cardiopulmonary exercise test
  • Pulmonary valve replacement

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Published Papers (3 papers)

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10 pages, 1083 KiB  
Communication
Relationship between Pulmonary Regurgitation and Ventriculo–Arterial Interactions in Patients with Post-Early Repair of Tetralogy of Fallot: Insights from Wave-Intensity Analysis
by Nikesh Arya, Silvia Schievano, Massimo Caputo, Andrew M. Taylor and Giovanni Biglino
J. Clin. Med. 2022, 11(20), 6186; https://doi.org/10.3390/jcm11206186 - 20 Oct 2022
Cited by 4 | Viewed by 1690
Abstract
This study aimed to investigate the effect of pulmonary regurgitation (PR) on left ventricular ventriculo–arterial (VA) coupling in patients with repaired tetralogy of Fallot (ToF). It was hypothesised that increasing PR severity results in a smaller forward compression wave (FCW) peak in the [...] Read more.
This study aimed to investigate the effect of pulmonary regurgitation (PR) on left ventricular ventriculo–arterial (VA) coupling in patients with repaired tetralogy of Fallot (ToF). It was hypothesised that increasing PR severity results in a smaller forward compression wave (FCW) peak in the aortic wave intensity, because of right-to-left ventricular interactions. The use of cardiovascular magnetic resonance (CMR)-derived wave-intensity analysis provided a non-invasive comparison between patients with varying PR degrees. A total of n = 201 patients were studied and both hemodynamic and wave-intensity data were compared. Wave-intensity peaks and areas of the forward compression and forward expansion waves were calculated as surrogates of ventricular function. Any extent of PR resulted in a significant reduction in the FCW peak. A correlation was found between aortic distensibility and the FCW peak, suggesting unfavourable (VA) coupling in patients that also present stiffer ascending aortas. Data suggest that VA coupling is affected by increased impedance. Full article
(This article belongs to the Special Issue Latest Advances in Tetralogy of Fallot)
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10 pages, 448 KiB  
Article
Syndromic and Non-Syndromic Patients with Repaired Tetralogy of Fallot: Does It Affect the Long-Term Outcome?
by Giulio Calcagni, Camilla Calvieri, Anwar Baban, Francesco Bianco, Rosaria Barracano, Massimo Caputo, Andrea Madrigali, Stefani Silva Kikina, Marco Alfonso Perrone, Maria Cristina Digilio, Marco Pozzi, Aurelio Secinaro, Berardo Sarubbi, Lorenzo Galletti, Maria Giulia Gagliardi, Andrea de Zorzi, Fabrizio Drago and Benedetta Leonardi
J. Clin. Med. 2022, 11(3), 850; https://doi.org/10.3390/jcm11030850 - 6 Feb 2022
Cited by 5 | Viewed by 2390
Abstract
Background: The impact of genetic syndromes on cardiac magnetic resonance imaging (cMRI) parameters, particularly on right and/or left ventricular dysfunction, associated with clinical parameters following the repair of Tetralogy of Fallot (rToF) is not well known. Therefore, this study aimed to assess the [...] Read more.
Background: The impact of genetic syndromes on cardiac magnetic resonance imaging (cMRI) parameters, particularly on right and/or left ventricular dysfunction, associated with clinical parameters following the repair of Tetralogy of Fallot (rToF) is not well known. Therefore, this study aimed to assess the differences in clinical, surgical, and cMRI data in syndromic and non-syndromic rToF patients. Methods: All syndromic rToF patients undergoing a cMRI without general anesthesia between 2010 and 2020 who were able to match with non-syndromic ones for birth date, sex, type of surgery, timing of cMRI, and BSA were selected. Demographic, clinical, surgical, MRI, ECG, and Holter ECG data were collected. Results: A total of one hundred and eight rToF patients equally subdivided into syndromic and non-syndromic, aged 18.7 ± 7.3 years, were studied. Del22q11.2 and Down syndrome (DS) were the most frequent syndromes (42.6% and 31.5%, respectively). Regarding the cMRI parameters considered, left ventricular (LV) dysfunction (LVEF < 50%) was more frequently found in syndromic patients (p = 0.040). In addition, they were older at repair (p = 0.002) but underwent earlier pulmonary valve replacement (PVR) (15.9 ± 5.6 vs. 19.5 ± 6.0 years, p = 0.049). On multivariate Cox regression analysis, adjusted for age at first repair, LV dysfunction remained significantly more associated with DS than del22q11.2 and non-syndromic patients (HR of 5.245; 95% CI 1.709–16.100, p = 0.004). There were only four episodes of non-sustained ventricular tachycardia in our cohort. Conclusions: Among the cMRI parameters commonly taken into consideration in rToF patients, LV dysfunction seemed to be the only one affected by the presence of a genetic syndrome. The percentage of patients performing PVR appears to be similar in both populations, although syndromic patients were older at repair and younger at PVR. Finally, the number of arrhythmic events in rToF patients seems to be low and unaffected by chromosomal abnormalities. Full article
(This article belongs to the Special Issue Latest Advances in Tetralogy of Fallot)
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6 pages, 571 KiB  
Opinion
Tricuspid Regurgitation in Patients with Tetralogy of Fallot
by Christopher DeZorzi, Anais Marenco and Anne Marie Valente
J. Clin. Med. 2023, 12(7), 2470; https://doi.org/10.3390/jcm12072470 - 24 Mar 2023
Viewed by 2421
Abstract
Tricuspid regurgitation in patients with repaired tetralogy of Fallot is an important finding with a wide spectrum of primary and secondary etiologies. Moderate or severe tricuspid regurgitation is associated with a greater incidence of atrial tachyarrhythmias in these patients. It remains uncertain which [...] Read more.
Tricuspid regurgitation in patients with repaired tetralogy of Fallot is an important finding with a wide spectrum of primary and secondary etiologies. Moderate or severe tricuspid regurgitation is associated with a greater incidence of atrial tachyarrhythmias in these patients. It remains uncertain which patients with repaired tetralogy of Fallot may benefit from a tricuspid valve intervention at the time of pulmonary valve replacement. Full article
(This article belongs to the Special Issue Latest Advances in Tetralogy of Fallot)
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