Emma Payne^1,2, Thomas Wilson^2,3, Marjan Haghighi^1,4, Zoe McCallum^2,3, Yves d’Udekem⁵, Julian Ayer^1,4,*

Congenital Heart Disease, Vol.17, No.6, pp. 617-639, 2022, DOI:10.32604/chd.2022.024775 - 11 October 2022

Abstract Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies… More >

Eleni P. Asimacopoulos^*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

Congenital Heart Disease, Vol.17, No.5, pp. 579-590, 2022, DOI:10.32604/chd.2022.020334 - 06 September 2022

Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study… More >

Carter Biewen^1,*, Hitendra Patel², Olaf Reinhartz³, Ginny Gildengorin⁴, Natalie Cvijanovich⁵

Congenital Heart Disease, Vol.15, No.4, pp. 229-238, 2020, DOI:10.32604/CHD.2020.011671 - 07 September 2020

Abstract Purpose: To summarize Fontan procedure data from our institution, UCSF Benioff Children’s Hospital Oakland, in order to better understand outcomes of our surgical and post-operative care. Basic Procedures: This is a retrospective cross-sectional chart review summarizing characteristics and outcomes of patients who underwent the Fontan procedure at our institution between 2005 and 2016. Main Findings: Sixty-five Fontan procedures were performed at our institution between 2005 and 2016, all of which were extracardiac Fontan procedures performed by the same surgical team. At the time of Fontan procedure, the mean patient age was 56.4 ± 18.7 months and mean weight… More >

Rachel Wong¹, Mohammed Al‐Omary², David Baker², Neil Spratt^1,2, Andrew Boyle^1,2, Natasha Baker Cert¹, Peter Howe¹, Nicholas Collins²

Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763

Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐… More >

Uri Pollak^1,2,3,4,*, Tatyana Ruderman^5,*, Sharon Borik‐Chiger^5,6, David Mishaly^5,7, Alain Serraf^5,7, Amir Vardi^5,8

Congenital Heart Disease, Vol.14, No.6, pp. 968-977, 2019, DOI:10.1111/chd.12825

Abstract Objective: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.
Design: Single center retrospective cohort study.
Setting: Pediatric Cardiac Intensive Care Unit at a tertiary medical center.
Patients: Ninety‐nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.
Interventions: None.
Measurements and Main Results: Out of the four types of blood products, transfusion of platelets was found to… More >

Thomas G. Wilson^1,2, Yves d’Udekem^1,2,3, David S. Winlaw^4,5, Rachael L. Cordina^5,6, Julian Ayer^4,5, Thomas L. Gentles⁷, Robert G. Weintraub^1,2,8, Leeanne E. Grigg⁹, Michael Cheung^1,8, Timothy M. Cain¹⁰, Padma Rao¹⁰, Charlotte Verrall⁴, Karin Du Plessis¹, Kathryn Rice⁷, Ajay J. Iyengar^1,3

Congenital Heart Disease, Vol.14, No.3, pp. 454-463, 2019, DOI:10.1111/chd.12746

Abstract Background: Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross‐sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine‐based methods in estimating glo‐ merular filtration rate (GFR). Methods: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo ^99mTc‐DTPA clear‐ ance was performed. Various serum creatinine‐based equations were used to calcu‐ late estimated GFR (eGFR). Results: Mean mGFR was 108 ± 28 mL/min/1.73 m² in children and 92 ±… More >

Keri M. Shafer^1,2, Alexander R. Opotowsky^1,2, Jonathan Rhodes¹

Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
Results: Of the 42 cases, 27 had… More >

Mi Kyoung Song¹, Gi Beom Kim¹, Eun Jung Bae¹, Young Ah Lee¹, Hyun-Young Kim², Seung-Kee Min³, Jung Hee Kim⁴, Jae-Kyung Won⁵

Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625

Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence… More >

Laura S. Kverneland^1,2, Peter Kramer², Stanislav Ovroutski²

Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570

Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published… More >

Uri Pollak^1,2,3, David Mishaly^3,4, Gili Kenet^3,5, Amir Vardi^1,3

Congenital Heart Disease, Vol.13, No.1, pp. 16-25, 2018, DOI:10.1111/chd.12557

Abstract Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. The risk for HIT correlates with the cumulative dosage of heparin exposure. In Fontan patients, recurrent systemic anticoagulation, traditionally with heparin, is used to alleviate the thrombotic complications that may occur postoperatively when the venous pressure rises and the systemic venous flow into the pulmonary arteries becomes sluggish, putting them at increased risk. As a pressure gradient-dependent circulation, elevation in systemic venous pressure, most often by venous thrombosis, contributes to circuit failure. Therefore, when HIT complicates patients after the Fontan procedure, it is associated with More >