Tetralogy of Fallot is the most common cyanotic congenital heart disease. For decades, our institution has cared for humanitarian patients with late presentation of tetralogy of Fallot. They are characterized by severe right ventricular hypertrophy with consecutive diastolic dysfunction, increasing the risk of postoperative low cardiac output syndrome (LCOS). By right ventricular restrictive physiology, we hypothesized that patients receiving early postoperative beta-blockers (within 48 h after cardiopulmonary bypass) may have better diastolic function and cardiac output. This is a retrospective cohort study in a single-center tertiary pediatric intensive care unit. We included > 1-year-old humanitarian patients with a confirmed diagnosis of tetralogy of Fallot undergoing a complete surgical repair between 2005 and 2019. We measured demographic data, preoperative echocardiographic and cardiac catheterization measures, postoperative mean heart rate, vasoactive-inotropic scores, LCOS scores, length of stay, and mechanical ventilation duration. One hundred sixty-five patients met the inclusion criteria. Fifty-nine patients (36%) received early postoperative beta-blockers, associated with a lower mean heart rate, higher vasoactive-inotropic scores, and lower LCOS scores during the first 48 h following cardiopulmonary bypass. There was no significant difference in lengths of stay and ventilation. Conclusion: Early postoperative beta-blockers lower the prevalence of postoperative LCOS at the expense of a higher need for vasoactive drugs without any consequence on length of stay and ventilation duration. This approach may benefit the specific population of children undergoing a late complete repair of tetralogy of Fallot. What is Known: • Prevalence of low cardiac output syndrome is high following a late complete surgical repair of tetralogy of Fallot. What is New: • Early postoperative beta-blockade is associated with lower heart rate, prolonged relaxation time, and lower prevalence of low cardiac output syndrome. • Negative chronotropic agents like beta-blockers may benefit selected patients undergoing a late complete repair of tetralogy of Fallot, who are numerous in low-income countries.
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http://dx.doi.org/10.1007/s00431-024-05597-1 | DOI Listing |
Int J Cardiovasc Imaging
January 2025
University Medical Center Göttingen, Department of Cardiology and Pneumology, Georg-August University, Robert-Koch-Str. 40, 37099, Göttingen, Germany.
Background: Pulmonary stenosis (PS) is common in congenital heart disease and an integral finding in Tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) is more commonly found following surgery in repaired TOF. We aimed to evaluate the haemodynamic effects of PS and PR on cardiac physiology in a porcine model using cardiac magnetic resonance-based feature tracking (CMR-FT) deformation imaging.
View Article and Find Full Text PDFIran J Med Sci
December 2024
Neonatology Research Center, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Background: Inadequate pulmonary blood flow in tetralogy of Fallot (TOF) can lead to the development of major aortopulmonary collateral arteries (MAPCA), which interferes with surgical repair. The present study evaluated the features of MAPCAs among patients with TOF and their treatment approaches. Besides, perioperative parameters and mortality rates of our TOF patients with and without MAPCA were compared.
View Article and Find Full Text PDFAlthough the long-term outcomes of the surgical grafts are well defined and reported, the data regarding the mid-and long-term results of the balloon-expandable percutaneous valves in the native right ventricular outflow tract (RVOT) is limited. We retrospectively evaluated 42 patients who underwent PPVI (Sapien® XT valve) to native RVOT due to severe pulmonary regurgitation (PR) and/or moderate to severe pulmonary stenosis (PS) between August 2015 and November 2020. The median patient age at the time of PPVI was 13.
View Article and Find Full Text PDFMultimed Man Cardiothorac Surg
January 2025
Maria Fareri Children's Hospital, Westchester Medical Center, Valhalla, NY, USA.
An 8-week-old, 3.4-kg infant, who was diagnosed prenatally with tetralogy of Fallot and absent pulmonary valve syndrome, was intubated after birth and failed extubation due to severe tracheobronchomalacia. He was deemed inoperable prior to being transferred to our institution.
View Article and Find Full Text PDFAnn Pediatr Cardiol
December 2024
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.
Absent pulmonary valve syndrome, commonly linked with tetralogy of Fallot and ventricular septal defect, is a rare congenital condition. It is exceedingly rare to have an isolated absent pulmonary valve with an intact ventricular septum without cardiovascular shunt lesions, such as an atrial/ventricular septal defect or patent ductus arteriosus. This report presents a case of such rarity involving a young child with recurrent lower respiratory tract infections.
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