Impact of the Evolution of the Fontan Operation on Early and Late Mortality: A Single-Center Experience of 405 Patients Over 3 Decades

Ohuchi, Hideo; Kagisaki, Koji; Miyazaki, Aya; Kitano, Masataka; Yazaki, Satoshi; Sakaguchi, Heima; Ichikawa, Hajime; Yamada, Osamu; Yagihara, Toshikatsu

doi:10.1016/j.athoracsur.2011.05.055

Cited by 63 publications

(37 citation statements)

References 21 publications

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“…Management of the complications of the Fontan circulation and improvement of the long-term prognosis are becoming important issues in the field of pediatric and adult CHD. 17, 18 Cardiac complications include intractable atrial or ventricular arrhythmia, atrioventricular valve regurgitation, progressive heart failure, thromboembolism, pericardial effusion, and infectious endocarditis. 70,84-86 Extracardiac complications include pleural effusion, plastic bronchitis, progressive cyanosis because of pulmonary arteriovenous shunts or systemic venous shunt to the left heart, liver dysfunction, liver cirrhosis, hepatocellular carcinoma, 87 glucose intolerance and diabetes, 88 renal dysfunction, protein-losing enteropathy (PLE), 85,86 ascites, menstrual disorders and subfertility in female patients, 89 systemic edema, and systemic thromboembolism including cerebral infarction.…”

Section: Long-term Prognosis Of Heterotaxy Patientsmentioning

confidence: 99%

“…13, 14 The mid-to long-term prognosis of heterotaxy patients who undergo the Fontan procedure for single ventricular physiology is now an important issue in pediatric and adult CHD clinics. [15][16][17][18] Human Heterotaxy Syndrome sary to understand the underlying molecular and cellular mechanisms of determination of the left-right body axis during early embryogenesis. [8][9][10][11][12] This process initiates in the primitive node at E7.5 in mice and develops through the following pathways: 19,20 (1) breaking of symmetry as a result of the leftward nodal flow generated by rotational movement of primary cilia in the node; (2) transmission of asymmetric signals to the LPM; (3) asymmetric expression of Nodal and Lefty2, which encodes a feedback inhibitor of Nodal signaling, in the LPM; and (4) situs-specific morphogenesis mediated by asymmetric expression of Pitx2, which encodes a transcription factor and is regulated by Nodal signaling.…”

mentioning

confidence: 99%

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Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

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110

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Section: Long-term Prognosis Of Heterotaxy Patientsmentioning

confidence: 99%

mentioning

confidence: 99%

Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

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110

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“…During the early postoperative period, Fontan has the greatest risk for Fontan failure and associated complications 8 . The significant evolution of the Fontan operation and the better‐refined selection of patients have led to improvements in early mortality and morbidity rates 12 …”

Section: Discussionmentioning

confidence: 99%

11 Years of the Fontan operation at a single institution: Results from 87 consecutive patients

et al. 2020

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Fontan procedure is the preferred palliation for patients with single ventricles. Objectives To evaluate early morbidity and mortality after Fontan operation in 87 consecutive patients, between 2007 and 2017. Methods Early survival, duration of intensive care unit (ICU), and hospital stays were the main outcomes evaluated. Potential influencing factors evaluated included preoperative and intraoperative variables. Results Fontan procedure was performed at a median age of 4.2 years (range, 17 months‐26 years), and a median weight of 15.5 kg (range, 8‐72 kg). Extracardiac Fontan was the procedure of choice. The median cardiopulmonary bypass time was 122 minutes (range, 58‐550 minutes). The majority had a fenestration (75 out of 87). Postoperatively, the median duration of ICU stay and total hospital stay were (4, 1‐76 days) and (16, 1‐85 days), respectively. Fontan failure occurred in one patient (1%). Overall early survival was 94%, resulting in a mortality rate of 6%. Univariate analysis showed that heterotaxy (odds ratio [OR], 2.222; confidence interval [CI], 1.345‐6.250; P = .003) and decreased ventricular function (OR, 2.207; CI, 1.348‐6.061; P = .002) significantly decreased survival. The same analysis failed to identify any statistically significant risk factors for prolonged hospital and ICU stays. Conclusion Our reported mortality and morbidity rates compared favorably with the reported rates. Therefore, Fontan operation can be performed in a tertiary care center in the United Arab Emirates with favorable early postoperative outcomes.

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“…Since the pulmonary circulation is passive after Fontan surgery, loss of laminar flow significantly decreased the pulmonary circulation, thereby limiting its effectiveness. The original Fontan operation was associated with unacceptably high rates of morbidity and mortality [7], which encouraged further development and modification of the procedure. The modified Fontan that is most commonly performed uses an extracardiac conduit to channel blood from the IVC directly to the right PA.…”

Section: Currently Employed Vascular Conduits: a Limiting Factor In Tmentioning

confidence: 99%

Tissue-Engineered Vascular Grafts for Use in the Treatment of Congenital Heart Disease: From the Bench to the Clinic and Back Again

et al. 2012

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Since the first tissue-engineered vascular graft (TEVG) was implanted in a child over a decade ago, growth in the field of vascular tissue engineering has been driven by clinical demand for improved vascular prostheses with performance and durability similar to an autologous blood vessel. Great strides were made in pediatric congenital heart surgery using the classical tissue engineering paradigm, and cell seeding of scaffolds in vitro remained the cornerstone of neotissue formation. Our second-generation bone marrow cell-seeded TEVG diverged from tissue engineering dogma with a design that induces the recipient to regenerate vascular tissue in situ. New insights suggest that neovessel development is guided by cell signals derived from both seeded cells and host inflammatory cells that infiltrate the graft. The identification of these signals and the regulatory interactions that influence cell migration, phenotype and extracellular matrix deposition during TEVG remodeling are yielding a next-generation TEVG engineered to guide neotissue regeneration without the use of seeded cells. These developments represent steady progress towards our goal of an off-the-shelf tissue-engineered vascular conduit for pediatric congenital heart surgery.

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Impact of the Evolution of the Fontan Operation on Early and Late Mortality: A Single-Center Experience of 405 Patients Over 3 Decades

Cited by 63 publications

References 21 publications

Human Heterotaxy Syndrome

Human Heterotaxy Syndrome

11 Years of the Fontan operation at a single institution: Results from 87 consecutive patients

Tissue-Engineered Vascular Grafts for Use in the Treatment of Congenital Heart Disease: From the Bench to the Clinic and Back Again

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