The optimal age at Fontan procedure and the ‘ticking clock’ theory: do we have an answer?

d’Udekem, Yves; Xu, Mary; Konstantinov, Igor E.

doi:10.1016/j.ejcts.2010.04.006

Cited by 9 publications

(5 citation statements)

References 7 publications

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“…Delaying the Fontan procedure has been proposed by believers in the "ticking clock theory": any Fontan circulation will eventually fail, and should thus be postponed to the last feasible stage. [9] However, it should be emphasized that contemporary Fontan surgical procedures have improved long-term prognosis [9], and several reports have shown that earlier Fontan surgery is associated with better outcomes. [10,11] Fontan completion in adulthood should therefore be considered the exception and can pose a challenge in treating ACHD patients in developing countries.…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.

Samaan,

Abdrabou,

Mostafa

et al. 2024

Preprint

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Background A major challenge facing adult congenital heart disease (ACHD) service in developing countries is the lack of adequate data on the disease burden and specific needs of this group. We aimed to describe ACHD patients in Egypt who are in need for further surgical correction, their various presentations, and the types of surgeries required. Methods The study included ACHD patients considered for further surgical correction after a heart team discussion at a tertiary cardiac center in Egypt. Data about age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, and the type of surgery decided were collected. Results Over one year, 103 cases (49.5% males, mean age 23 years) were referred to surgery out of 134 cases discussed at the heart team committee. One-third of the patients (34%) were cyanotic and the majority presented in NYHA functional class II (44.7%) and III (35.95%). More than half of the patients (56.3%) had a previous surgical or trans-catheter intervention with a median age of 4 years at the time of the first procedure. The most frequently encountered diagnoses were: Tetralogy of Fallot (ToF) (23.3%), atrial septal defect (18.4%), double outlet right ventricle (DORV) (11.6%), and transposition of great arteries (TGA) (7.8%). Surgical procedures that were required most were: Fontan procedure (20.3%), surgical ASD closure (18.4%), surgical pulmonary valve replacement (14.5%), total ToF repair (9.7%), subaortic membrane resection (6.7%) and tricuspid valve surgery (5.8%). Conclusions The group of ACHD patients in Egypt who require further surgical correction is heterogeneous with various presentations and needs. The required surgeries vary from simple to complex procedures. An efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this patient group.

show abstract

Section: Discussionmentioning

confidence: 99%

Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.

Samaan,

Abdrabou,

Mostafa

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

“…Furthermore, the debate about the optimal timing for Fontan completion renders them a lesser priority in many pediatric cardiac facilities. Delaying the Fontan procedure has been proposed by believers in the "ticking clock theory": any Fontan circulation will eventually fail, and should thus be postponed to the last feasible stage (9). However, it should be emphasized that contemporary Fontan surgical procedures have improved long-term prognosis (9), and several reports have shown that earlier Fontan surgery is associated with better outcomes (10,11).…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of adult patients referred to congenital heart surgery; a single center experience in Egypt.

Samaan,

Mostafa,

AbdElAziz

et al. 2024

Preprint

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Background A major challenge facing adult congenital heart disease (ACHD) service in developing countries is the lack of adequate data on the disease burden and specific needs of this group. We aimed to describe ACHD patients in Egypt who need further surgical correction, their various presentations, and the types of surgeries required. Methods The study included ACHD patients considered for further surgical correction after a heart team discussion at a tertiary cardiac center in Egypt. Data about age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, and the type of surgery decided were collected. Results Over one year, 103 cases (49.5% males, mean age 23 years) were referred to surgery out of 134 cases discussed at the heart team committee. One-third of the patients (34%) were cyanotic, and the majority presented in NYHA functional class II (44.7%) and III (35.95%). More than half of the patients (56.3%) had a previous surgical or trans-catheter intervention with a median age of 4 years at the time of the first procedure. The most frequently encountered diagnoses were: Tetralogy of Fallot (ToF) (23.3%), atrial septal defect (ASD) (18.4%), double outlet right ventricle (11.6%), and transposition of great arteries (7.8%). Surgical procedures that were required most were: Fontan procedure (20.3%), surgical ASD closure (18.4%), surgical pulmonary valve replacement (14.5%), total ToF repair (9.7%), subaortic membrane resection (6.7%) and tricuspid valve surgery (5.8%). Conclusion The group of ACHD patients in Egypt who require further surgical correction is heterogeneous, with various presentations and needs. The required surgeries vary from simple to complex. An efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this patient group.

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“…Proponents of early TCPC argue that a prolonged period of volume overload leads to adverse cardiac remodeling and reduced cardiac function 152 . Others argue that the Fontan circulation inherently leads to complications and that surgery should be delayed to reduce the amount of time in Fontan physiology 153 . Other factors to be considered are the techniques used; ILT allows TCPC at lower body weight than ECC since small-sized conduits (<18 mm) need to be avoided 152 , 154 .…”

Section: Remaining Challengesmentioning

confidence: 99%

State of the art of the Fontan strategy for treatment of univentricular heart disease

et al. 2018

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In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

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The optimal age at Fontan procedure and the ‘ticking clock’ theory: do we have an answer?

Cited by 9 publications

References 7 publications

Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.

Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.

Clinical profile of adult patients referred to congenital heart surgery; a single center experience in Egypt.

State of the art of the Fontan strategy for treatment of univentricular heart disease

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