Background: A growing number of adults with functional single ventricles are presenting as candidates for first-time and redo-Fontan operations. This study describes the clinical presentation and early operative results of adults who have undergone Fontan modifications.
Methods: Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses.
Results: Twenty-three Fontan operations (first-time [n = 8] and redo [n = 15]) were performed with no early or late deaths. No patient has required reoperation. One patient has been listed for orthotopic heart transplantation. The overall mean age is 23 years (18 to 41 years); mean follow-up, 30 months; median postoperative hospital stay, 8 days (4 to 34 days); and median duration of chest tube drainage, 4 days (2 to 12 days). The postoperative New York Heart Association (NYHA) functional class was improved in 22 of 23 patients. Eight first-time Fontan operations (7 of 8 nonfenestrated) were performed; lateral tunnel (n = 7) and extracardiac conduit (n = 1). Two patients had preoperative arrhythmias. New onset arrhythmias (ventricular tachycardia and sinus node dysfunction), requiring treatment, occurred in two patients. Fifteen redo-Fontan operations (all nonfenestrated) were performed; lateral tunnel (n = 5) and extracardiac conduit (n = 10). Fifteen patients had preoperative arrhythmias, thirteen of which had intraatrial reentry tachycardia (IART) and required antiarrhythmic medications. Concomitant intraoperative radiofrequency ablation (RFA) (n = 11) and cryoablation (n = 1) procedures were performed. In the immediate postoperative period, there was IART recurrence in five patients (post-RFA [n = 4] and postcryoablation [n = 1]). At latest follow-up, no patient is being treated with antiarrhythmic medications. Two patients had new onset atrial arrhythmias that required treatment.
Conclusions: The Fontan operation can be performed in adults with minimal morbidity and improved NYHA functional class. New onset arrhythmias requiring treatment are sources of perioperative morbidity. Complete arrhythmia resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in redo-Fontan patients. However, modification (intraoperative radiofrequency ablation-right atrial debulking) of the atrial tachycardia circuits in the redo-Fontan patients can result in complete resolution of preoperative atrial tachyarrhythmias at early follow-up.
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http://dx.doi.org/10.1016/j.athoracsur.2003.10.011 | DOI Listing |
J Cardiol Cases
January 2025
Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
Unlabelled: The incidence of invasive group A Streptococcus (iGAS) infection has been increasing across all age groups, including pediatric patients, and is associated with high mortality rates. Although iGAS infection leads to streptococcal toxic shock syndrome and necrotizing soft tissue infections, iGAS-associated infective endocarditis (IE) is rare. Here, we report a case of iGAS-associated IE, streptococcal toxic shock syndrome, and pyomyositis that occurred after the Fontan procedure in a 7-year-old patient.
View Article and Find Full Text PDFPediatr Cardiol
January 2025
Fetal Heart Program, Division of Cardiology. Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged with the delivery, postnatal resuscitation, and procedural care. A Cesarean section delivery is planned in an operating room with immediate access to a multifunctional procedural suite where the neonate can undergo rapid cardiac evaluation and initiation of interventional treatments which can have a positive, life-saving impact.
View Article and Find Full Text PDFOpen Heart
January 2025
Department of Cardiology, Rabin Medical Center, Petah Tikva, Israel.
Introduction: Data on the characteristics and outcomes of pregnancy and among patients with Fontan physiology are limited. We aimed to evaluate the immediate and long-term outcomes among these patients who were followed at our centre.
Methods: We included adult patients who had undergone Fontan surgery for congenital heart disease and were pregnant between 1994 and 2021.
Ann Pediatr Cardiol
December 2024
Postgraduate School of Public Health, Universitas Muhammadiyah Aceh, Banda Aceh, Indonesia.
Background: Children with one functional ventricle might receive the Fontan procedure as palliative management to prolong their lives. However, the heart remains functionally univentricular, which may result in disrupted absorption of nutrients, including Vitamin D. Individuals with Fontan circulation have limited physical activity and sun exposure, which might further contribute to Vitamin D deficiency.
View Article and Find Full Text PDFEur Heart J Case Rep
January 2025
Cardiac Electrophysiology, National Medical Center 'November 20th', ISSSTE, Av. Felix Cuevas #540, Col. Del Valle Del. Benito Juarez, C.P. 03100 Mexico City, Mexico.
Background: The 'univentricular' heart encompasses a variety of congenital cardiac defects characterized by a single functional ventricle and an underdeveloped ventricular chamber. Surgical intervention, typically in infancy or childhood, aims to regulate pulmonary blood flow volume. In adulthood, untreated patients may experience limitations in physical activity and elevated morbidity due to persistent cyanosis and arrhythmias, notably after the Fontan procedure.
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