Tools
Single Ventricle
Introduction
A functionally univentricular heart, or single ventricle, as defined by Jacobs and Anderson, refers to a condition where 1 ventricle cannot adequately support either systemic or pulmonary circulation. Various anomalies, including hypoplastic left heart syndrome (HLHS) and tricuspid atresia, fall under this classification. The International Pediatric and Congenital Cardiac Code provides standardized terminology for these conditions. The anomalous structure often leads to the mixing of oxygenated and deoxygenated blood through various mechanisms. The condition is typically attributed to genetic factors, though environmental influences can contribute to malformations.
With congenital cardiothoracic surgery advancements, children born with a single ventricle can now live for decades to reach adulthood and beyond. The surgically created univentricular anatomy and physiology enable an orphan ventricle to support systemic circulation as pulmonary blood flows passively in the lungs, a configuration known as the Fontan circulation.
Etiology
Register For Free And Read The Full Article
Search engine and full access to all medical articles- 10 free questions in your specialty
- Free CME/CE Activities
- Free daily question in your email
- Save favorite articles to your dashboard
- Emails offering discounts
Learn more about a Subscription to StatPearls Point-of-Care
Etiology
The formation of univentricular hearts is attributed to multiple factors, each producing a different pathological type, though the mechanisms of their development are not entirely understood. Malformation occurs during embryogenesis on days 30 to 56 of gestation.[1][2] Cardiac structural abnormalities are commonly associated with lateralization disorders, such as situs inversus totalis and heterotaxy.[3] In patients with primary ciliary dyskinesia, 12% have evidence of heterotaxy.[4]
Other genetic causes have also been identified, including Tbx5 and GATA4 anomalies. The inactivation of both genes has a direct influence on the formation of the ventricular septum.[5][6] Genetic malformations without known causes have been linked to defects in endocardial cushion formation and the influence of dynamic blood flow during development. Such malformations are often associated with extracardiac structural abnormalities, as observed in conditions like DiGeorge syndrome.[7]
Parental factors linked to univentricular heart formation include the following:
- Increasing parental age [8]
- Maternal conditions such as phenylketonuria, pregestational diabetes, febrile illnesses, and infections like influenza and rubella
- Use of anticonvulsants, ibuprofen, sulfasalazine, thalidomide, trimethoprim-sulfonamide, retinoids, marijuana, and organic solvents [9]
- Lithium intake
Lithium exposure increases the risk of cardiac malformations, particularly with high doses or exposure during the first trimester.[10][11] Pregnant patients should be closely monitored to balance maternal benefits and fetal risks.
Key univentricular variations and typical features include the following:
- HLHS: The left ventricle, mitral valve, aortic valve, and aorta are underdeveloped.
- Tricuspid atresia: The tricuspid valve fails to form, leading to an underdeveloped right ventricle.
- Ebstein anomaly: Abnormal growth of the tricuspid valve leaflets causes right ventricular atrialization. This anomaly is associated with various cardiac structural abnormalities, including pulmonary valve pathologies, septal defects, and electrical conduction lesions.
- Double outlet right ventricle (DORV): The aorta and the pulmonary artery exit from the right ventricle, leaving the left ventricle underdeveloped.
- Double inlet left ventricle (DILV): Both atria connect to the left ventricle, resulting in an underdeveloped right ventricle.
- Atrioventricular canal defect: An atrial or ventricular septal defect forms large enough to make a functionally single ventricle.
The development of univentricular hearts stems from a complex interplay of genetic, environmental, and embryologic factors. Understanding the diverse etiologies underlying univentricular heart formation is essential for early diagnosis and genetic counseling.
Epidemiology
The occurrence of congenital heart disease is approximately between 8 and 12 in 1,000 live births.[12][13] HLHS, the most common form of univentricular heart disease, is seen in 2.6 per 10,000 births, with a higher incidence in male infants.[14][15] Tricuspid atresia occurs in about 1 per 10,000 live births.[16][17] Ebstein anomaly is observed in about 0.5 per 10,000 live births with a sex predilection.[18] However, with maternal use of lithium, the likelihood of Ebstein anomaly can increase nearly 7 times.[19] Double outlet right ventricle occurs in 0.009 cases per 10,000 live births.[20] Double inlet left ventricle is reported in up to 0.01 per 10,000 live births.[21] Atrioventricular canal defect is found in 0.03 to 0.04 per 10,000 live births.[22]
Pathophysiology
With a single ventricle, mixed oxygenated blood circulates throughout the body. Depending on the structural anomaly, a patent ductus arteriosus (PDA), atrial septal defect, ventricular septal defect, or communication in the great arteries may be required to maintain pulmonary and systemic circulations. More information about the anatomical factors that improve survival for each type can be found in their respective StatPearls educational activities.
The Fontan procedure is a series of staged surgeries designed to manage blood flow in a univentricular heart and reduce ventricular strain. This technique ultimately redirects systemic venous blood to the pulmonary arteries without passing through the heart, creating a passive pulmonary circuit. In the short term, this approach can save lives with minimal impact on homeostasis. However, within 25 years, approximately half of all patients who undergo the Fontan procedure are expected to experience Fontan failure, characterized by circulatory dysfunction, reduced functional capacity [New York Heart Association (NYHA) class III or IV], takedown or conversion of the Fontan circuit, severe complications such as protein-losing enteropathy (PLE) and plastic bronchitis, the need for heart transplantation, or mortality.[23][24]
PLE is a severe and complex sequela of Fontan physiology that remains poorly understood. Development is believed to result from multiple factors, including chronically elevated systemic venous pressure, heightened mesenteric vascular resistance, reduced cardiac output, diastolic dysfunction, increased intestinal permeability, and abnormalities in the lymphatic system.[25]
History and Physical
A single ventricle may be identified as early as 18 to 24 weeks of gestation, although some major defects can be detected as early as 11 to 13 weeks.[26] Other structural abnormalities, such as mispositioning of the great arteries and reversal of blood flow in parts of the fetal cardiac system, may also be observed around this time. Ultrasound can detect extracardiac structural anomalies, aiding in diagnosis.[27][28]
Postnatal presentations vary based on underlying structural abnormalities. Signs such as a heart murmur, tachypnea, respiratory distress, cyanosis, or hypotension typically emerge when circulation and oxygenation become inadequate following closure of the ductus arteriosus.[29] Additional physical examination findings, including hepatomegaly or dysmorphic features, may suggest extracardiac involvement or syndromic associations.[30][31] A neonate may not show symptoms at birth or before discharge if circulation is sufficient at the time of examination. However, closure of the ductus arteriosus or changes in blood flow to vital organs may produce symptoms after discharge.
Evaluation
Prenatal diagnosis is made using routine ultrasound and fetal echocardiography, which can identify structural abnormalities or atypical blood flow patterns indicative of a single ventricle. Postnatal diagnosis relies primarily on echocardiography, the best modality for confirming a single ventricle. Additional diagnostic tools include electrocardiography, chest x-rays, and pulse oximetry, with physical examination findings providing further diagnostic clues.[32][33] Advanced imaging techniques, such as 3-dimensional ultrasound, spatiotemporal image correlation (STIC), and artificial intelligence-assisted methods, are emerging to improve diagnostic accuracy and support interprofessional planning.[34][35]
Treatment / Management
Management depends on the time of discovery, prognosis, and goals of care. Prognosis must be discussed in depth before proceeding with treatment, as an intervention may be futile in some instances. Home monitoring and comprehensive caregiver understanding of the disease have become integral to standard care, particularly to improve outcomes during the vulnerable period between surgical palliation stages.[36]
Medical management of univentricular heart syndrome focuses on addressing the ramifications of the underlying pathology. Supplemental oxygen may be used to alleviate hypoxemia. Correctable factors contributing to acid-base or metabolic disturbances should be addressed.[37] Inhaled nitric oxide may reduce pulmonary vascular resistance, improving oxygenation by enhancing pulmonary blood flow.[38] Inotropic agents may be used to support ventricular contraction in cases of cardiac strain, though catecholamines should be avoided due to the risk of arrhythmogenesis.[39] When collateral flow depends on a PDA, prostaglandin E1 may be administered to maintain ductal patency as a temporary measure until definitive interventions are performed. Nonsteroidal anti-inflammatory drugs should be avoided to prevent premature PDA closure. (A1)
The decision to render catheter-based management depends on the underlying etiology, timing of diagnosis, and prognosis. When identified in utero, catheter-based structural interventions or valvuloplasty can address anomalies early, potentially mitigating complications during development.[40][41][42] Many procedures may also be performed postnatally, although they have less influence on developmental progression. In conditions like Ebstein anomaly, associated pulmonary arteriovenous malformations may be treated using a transcatheter occlusion approach.[43](B2)
Surgical intervention may also correct associated anatomical anomalies with techniques tailored to specific conditions. The Fontan procedure, a widely used approach, directs blood to the lungs by relying on central venous pressure and intrathoracic pressure reduction.[44] Optimal pressure dynamics and low pulmonary vascular resistance are critical for maintaining effective anterograde circulation.[45] Perioperative management and advances in surgical techniques have improved early and midterm outcomes, with operative mortality now approaching 1% and 10-year transplantation-free survival rates nearing 90% in contemporary series.[46] While the Fontan procedure has shown consistent success, it should not be viewed as the sole option, as other surgical strategies may be more appropriate, depending on the individual case.
In patients with severe disease, palliative surgery is often the preferred approach over strictly comfort-focused measures.[47] Evidence remains inconclusive regarding the superiority of the Fontan procedure compared to other palliative options. Referral to tertiary care centers is recommended for specialized evaluation and management.[48] Cardiac transplantation may be considered, though it is associated with suboptimal outcomes due to comorbidities.[49][50] Transplantation may also become necessary despite prior surgical interventions.(B2)
Differential Diagnosis
Neonatal symptoms may appear immediately after birth or within a few days as the ductus arteriosus closes and collateral circulations diminish. The clinical presentation of congenital heart disease may be delayed, with symptoms such as cyanosis, tachypnea, or cardiovascular collapse appearing after 48 hours of life. Vigilant postnatal monitoring and screening before discharge are crucial. In infants with severe manifestations after delivery, the differential diagnoses should include sepsis, infantile respiratory distress syndrome, aortic or pulmonary disorders, and transposition of the great arteries.[51] Milder cases may not present until months or years later, detected only when growth is impaired. In such cases, infection, failure to thrive, and malnutrition should be investigated as potential causes.
Prognosis
HLHS is almost universally fatal without treatment. Recent population-based and institutional studies report 5- to 10-year survival rates after surgical intervention ranging from 55% to 65%, with some centers noting 10- and 20-year survival rates exceeding 90% in selected cohorts. However, these higher rates may reflect center-specific outcomes and patient selection. Survival to adolescence and young adulthood is increasingly common, with life expectancy beyond 18 years exceeding 90% among patients whose condition remains stable after the 1st year.[52][53][54][55] The American Heart Association recommends developmental evaluation for these patients, as neurodevelopmental issues may result from inadequate nutritional delivery.
Patients with tricuspid atresia generally have a favorable prognosis with intervention, with a 90% survival rate at 1 year and 80% at 10 years.[56][57] However, Ebstein anomaly has a high perinatal mortality, with up to 32% of live births expiring before discharge.[58] One-year and 10-year survival rates for Ebstein anomaly are 67% and 59%, respectively, with limited prognostic data available beyond 10 years.[59]
The prognosis for other causes of single ventricles varies by etiology. Nevertheless, more than half of the patients survive beyond 2 years, with an average lifespan of 30 to 40 years.[60]
After decades of successful single-heart palliative surgeries, specific sets of risk factors have been found to be associated with a bad prognosis. These risk factors include the following:
- Preoperative factors
- Intraoperative factors
- Postoperative factors
- Early:
- Fontan circulation pressure greater than 20 mm Hg
- Unsettled chest drainage lasting longer than 3 weeks [65]
- Ventricular filling pressure greater than 13 mm Hg
- Late:
- Early:
Notably, HLHS is not directly linked to increased mortality but is associated with a higher risk of late complications and Fontan failure.[71] Meanwhile, the absence of septal fenestrations and delayed intervention have been linked to an increased risk of PLE and plastic bronchitis.[72]
Complications
Single-ventricle heart syndromes are typically managed through direct surgical or interventional procedures, which can obscure the origins of some complications. The expected sequelae following correction of single-ventricle hearts include the following:
- Arrhythmias [73][74]
- Esophageal varices [75]
- Heart failure with thrombus formation and increased risk of bleeding from treatment [76][77]
- Greater risk of decompensation with anesthesia [78]
- Long-term cyanosis
- Restrictive and diffusion-limited lung disease [79][80]
- PLE [81][82]
- Recurrent laryngeal nerve injury [83]
- Reduced height and impaired somatic development [84]
- Renal dysfunction
- Systemic-to-pulmonary venous and systemic-to-pulmonary artery collaterals
Note that pulmonary disease in these patients may be partly attributable to subclinical plastic bronchitis and pulmonary embolism.[85] Such complications can contribute significantly to morbidity.
Recognition of the above sequelae underscores the importance of lifelong follow-up in patients with single-ventricle physiology. Multisystem involvement necessitates a holistic, interprofessional approach to care.
Consultations
Consultations with interventional specialists and thoracic surgeons should be made when the anomaly is discovered. Prompt referral enables timely intervention and ensures that patients and their families are fully informed about their options.
Deterrence and Patient Education
Intervention, whether medical or surgical, is not always the most appropriate or optimal choice. Detailed discussions about prognosis and potential outcomes should be held with family members. Clinicians should clearly explain that staged procedures may not be curative and that cardiac transplantation may be required in the future. If the condition is identified in utero, elective termination may be presented as an option during counseling. Additionally, living conditions should be addressed, especially for families residing at altitudes above 1,700 meters, as such environments may adversely affect long-term survival.[86]
Enhancing Healthcare Team Outcomes
The identification of a single ventricle can be made in utero or after birth. When made before birth, healthcare teams must report this finding and begin goal discussions with the mother as soon as possible. When found emergently after delivery, healthcare employees need to work as an interprofessional team to provide a quick resolution. Overall, general practitioners should understand the severe impact of single ventricles on patients and their families, as well as their suboptimal prognoses, to guide decision-making.
References
Dhanantwari P, Lee E, Krishnan A, Samtani R, Yamada S, Anderson S, Lockett E, Donofrio M, Shiota K, Leatherbury L, Lo CW. Human cardiac development in the first trimester: a high-resolution magnetic resonance imaging and episcopic fluorescence image capture atlas. Circulation. 2009 Jul 28:120(4):343-51. doi: 10.1161/CIRCULATIONAHA.108.796698. Epub [PubMed PMID: 19635979]
Sizarov A, Ya J, de Boer BA, Lamers WH, Christoffels VM, Moorman AF. Formation of the building plan of the human heart: morphogenesis, growth, and differentiation. Circulation. 2011 Mar 15:123(10):1125-35. doi: 10.1161/CIRCULATIONAHA.110.980607. Epub [PubMed PMID: 21403123]
Level 3 (low-level) evidenceKennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Molina PL, Robinson BV, Minnix SL, Olbrich H, Severin T, Ahrens P, Lange L, Morillas HN, Noone PG, Zariwala MA, Knowles MR. Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation. 2007 Jun 5:115(22):2814-21 [PubMed PMID: 17515466]
Level 2 (mid-level) evidenceShapiro AJ, Davis SD, Ferkol T, Dell SD, Rosenfeld M, Olivier KN, Sagel SD, Milla C, Zariwala MA, Wolf W, Carson JL, Hazucha MJ, Burns K, Robinson B, Knowles MR, Leigh MW, Genetic Disorders of Mucociliary Clearance Consortium. Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy. Chest. 2014 Nov:146(5):1176-1186. doi: 10.1378/chest.13-1704. Epub [PubMed PMID: 24577564]
Bruneau BG, Logan M, Davis N, Levi T, Tabin CJ, Seidman JG, Seidman CE. Chamber-specific cardiac expression of Tbx5 and heart defects in Holt-Oram syndrome. Developmental biology. 1999 Jul 1:211(1):100-8 [PubMed PMID: 10373308]
Level 3 (low-level) evidenceZeisberg EM, Ma Q, Juraszek AL, Moses K, Schwartz RJ, Izumo S, Pu WT. Morphogenesis of the right ventricle requires myocardial expression of Gata4. The Journal of clinical investigation. 2005 Jun:115(6):1522-31 [PubMed PMID: 15902305]
Level 3 (low-level) evidenceTowbin JA, Belmont J. Molecular determinants of left and right outflow tract obstruction. American journal of medical genetics. 2000 Winter:97(4):297-303 [PubMed PMID: 11376441]
Level 3 (low-level) evidenceMaterna-Kiryluk A, Wiśniewska K, Badura-Stronka M, Mejnartowicz J, Wieckowska B, Balcar-Boroń A, Czerwionka-Szaflarska M, Gajewska E, Godula-Stuglik U, Krawczyński M, Limon J, Rusin J, Sawulicka-Oleszczuk H, Szwalkiewicz-Warowicka E, Walczak M, Latos-Bieleńska A. Parental age as a risk factor for isolated congenital malformations in a Polish population. Paediatric and perinatal epidemiology. 2009 Jan:23(1):29-40. doi: 10.1111/j.1365-3016.2008.00979.x. Epub [PubMed PMID: 19228312]
Jenkins KJ, Correa A, Feinstein JA, Botto L, Britt AE, Daniels SR, Elixson M, Warnes CA, Webb CL, American Heart Association Council on Cardiovascular Disease in the Young. Noninherited risk factors and congenital cardiovascular defects: current knowledge: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007 Jun 12:115(23):2995-3014 [PubMed PMID: 17519397]
Patorno E, Huybrechts KF, Bateman BT, Cohen JM, Desai RJ, Mogun H, Cohen LS, Hernandez-Diaz S. Lithium Use in Pregnancy and the Risk of Cardiac Malformations. The New England journal of medicine. 2017 Jun 8:376(23):2245-2254. doi: 10.1056/NEJMoa1612222. Epub [PubMed PMID: 28591541]
Fornaro M, Maritan E, Ferranti R, Zaninotto L, Miola A, Anastasia A, Murru A, Solé E, Stubbs B, Carvalho AF, Serretti A, Vieta E, Fusar-Poli P, McGuire P, Young AH, Dazzan P, Vigod SN, Correll CU, Solmi M. Lithium Exposure During Pregnancy and the Postpartum Period: A Systematic Review and Meta-Analysis of Safety and Efficacy Outcomes. The American journal of psychiatry. 2020 Jan 1:177(1):76-92. doi: 10.1176/appi.ajp.2019.19030228. Epub 2019 Oct 18 [PubMed PMID: 31623458]
Level 1 (high-level) evidenceGBD 2017 Congenital Heart Disease Collaborators. Global, regional, and national burden of congenital heart disease, 1990-2017: a systematic analysis for the Global Burden of Disease Study 2017. The Lancet. Child & adolescent health. 2020 Mar:4(3):185-200. doi: 10.1016/S2352-4642(19)30402-X. Epub 2020 Jan 21 [PubMed PMID: 31978374]
Level 1 (high-level) evidenceZhao QM, Liu F, Wu L, Ma XJ, Niu C, Huang GY. Prevalence of Congenital Heart Disease at Live Birth in China. The Journal of pediatrics. 2019 Jan:204():53-58. doi: 10.1016/j.jpeds.2018.08.040. Epub 2018 Sep 27 [PubMed PMID: 30270157]
Moon-Grady AJ, Donofrio MT, Gelehrter S, Hornberger L, Kreeger J, Lee W, Michelfelder E, Morris SA, Peyvandi S, Pinto NM, Pruetz J, Sethi N, Simpson J, Srivastava S, Tian Z. Guidelines and Recommendations for Performance of the Fetal Echocardiogram: An Update from the American Society of Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography. 2023 Jul:36(7):679-723. doi: 10.1016/j.echo.2023.04.014. Epub 2023 May 24 [PubMed PMID: 37227365]
Wald RM, Mertens LL. Hypoplastic Left Heart Syndrome Across the Lifespan: Clinical Considerations for Care of the Fetus, Child, and Adult. The Canadian journal of cardiology. 2022 Jul:38(7):930-945. doi: 10.1016/j.cjca.2022.04.028. Epub 2022 May 12 [PubMed PMID: 35568266]
Hoffman JI, Kaplan S. The incidence of congenital heart disease. Journal of the American College of Cardiology. 2002 Jun 19:39(12):1890-900 [PubMed PMID: 12084585]
Rao PS. Tricuspid Atresia. Current treatment options in cardiovascular medicine. 2000 Dec:2(6):507-520 [PubMed PMID: 11096554]
Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations. Pediatric cardiology. 2003 May-Jun:24(3):195-221 [PubMed PMID: 12632215]
Diav-Citrin O, Shechtman S, Tahover E, Finkel-Pekarsky V, Arnon J, Kennedy D, Erebara A, Einarson A, Ornoy A. Pregnancy outcome following in utero exposure to lithium: a prospective, comparative, observational study. The American journal of psychiatry. 2014 Jul:171(7):785-94. doi: 10.1176/appi.ajp.2014.12111402. Epub [PubMed PMID: 24781368]
Level 2 (mid-level) evidenceDemir MT, Amasyall Y, Kopuz C, Aydln ME, Corumlu U. The double outlet right ventricle with additional cardiac malformations: an anatomic and echocardiographic study. Folia morphologica. 2009 May:68(2):104-8 [PubMed PMID: 19449298]
Level 3 (low-level) evidenceGidvani M, Ramin K, Gessford E, Aguilera M, Giacobbe L, Sivanandam S. Prenatal diagnosis and outcome of fetuses with double-inlet left ventricle. AJP reports. 2011 Dec:1(2):123-8. doi: 10.1055/s-0031-1293515. Epub 2011 Nov 7 [PubMed PMID: 23705101]
Level 3 (low-level) evidenceHoffman JI. Incidence of congenital heart disease: I. Postnatal incidence. Pediatric cardiology. 1995 May-Jun:16(3):103-13 [PubMed PMID: 7617503]
d'Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, Bullock A, Justo RN, Grigg LE, Sholler GF, Hope S, Radford DJ, Gentles TL, Celermajer DS, Winlaw DS. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014 Sep 9:130(11 Suppl 1):S32-8. doi: 10.1161/CIRCULATIONAHA.113.007764. Epub [PubMed PMID: 25200053]
Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O'Leary PW, Driscoll DJ, Cetta F. 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients. Journal of the American College of Cardiology. 2015 Oct 13:66(15):1700-10. doi: 10.1016/j.jacc.2015.07.065. Epub [PubMed PMID: 26449141]
Sanders E, Knecht K, Zakaria D. Protein-Losing Enteropathy in the Failing Fontan: Treatment With Angiotensin Receptor Neprilysin Inhibitor. JACC. Case reports. 2024 Jul 17:29(14):102395. doi: 10.1016/j.jaccas.2024.102395. Epub 2024 Jun 12 [PubMed PMID: 38973815]
Level 3 (low-level) evidenceMinnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH. Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus. Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology. 2020 May:55(5):637-644. doi: 10.1002/uog.21956. Epub [PubMed PMID: 31875326]
Berg C, Lachmann R, Kaiser C, Kozlowski P, Stressig R, Schneider M, Asfour B, Herberg U, Breuer J, Gembruch U, Geipel A. Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome. Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology. 2010 Feb:35(2):183-90. doi: 10.1002/uog.7499. Epub [PubMed PMID: 20101636]
Level 2 (mid-level) evidenceGalindo A, Comas C, Martínez JM, Gutiérrez-Larraya F, Carrera JM, Puerto B, Borrell A, Mortera C, de la Fuente P. Cardiac defects in chromosomally normal fetuses with increased nuchal translucency at 10-14 weeks of gestation. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians. 2003 Mar:13(3):163-70 [PubMed PMID: 12820838]
Wasserman MA, Shea E, Cassidy C, Fleishman C, France R, Parthiban A, Landeck BF 2nd. Recommendations for the Adult Cardiac Sonographer Performing Echocardiography to Screen for Critical Congenital Heart Disease in the Newborn: From the American Society of Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography. 2021 Mar:34(3):207-222. doi: 10.1016/j.echo.2020.12.005. Epub 2021 Jan 29 [PubMed PMID: 33518447]
Haxel CS, Johnson JN, Hintz S, Renno MS, Ruano R, Zyblewski SC, Glickstein J, Donofrio MT. Care of the Fetus With Congenital Cardiovascular Disease: From Diagnosis to Delivery. Pediatrics. 2022 Nov 1:150(Suppl 2):. pii: e2022056415C. doi: 10.1542/peds.2022-056415C. Epub [PubMed PMID: 36317976]
Freud LR, Seed M. Prenatal Diagnosis and Management of Single-Ventricle Heart Disease. The Canadian journal of cardiology. 2022 Jul:38(7):897-908. doi: 10.1016/j.cjca.2022.04.003. Epub 2022 Apr 13 [PubMed PMID: 35429589]
de-Wahl Granelli A, Wennergren M, Sandberg K, Mellander M, Bejlum C, Inganäs L, Eriksson M, Segerdahl N, Agren A, Ekman-Joelsson BM, Sunnegårdh J, Verdicchio M, Ostman-Smith I. Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ (Clinical research ed.). 2009 Jan 8:338():a3037. doi: 10.1136/bmj.a3037. Epub 2009 Jan 8 [PubMed PMID: 19131383]
Level 3 (low-level) evidenceMonaco MA, Liberman L, Starc TJ, Silver ES. Defining the electrocardiogram in the neonate with hypoplastic left heart syndrome. Pediatric cardiology. 2015 Jun:36(5):1014-8. doi: 10.1007/s00246-015-1112-x. Epub 2015 Jan 21 [PubMed PMID: 25605039]
Level 2 (mid-level) evidenceRo SS, Saini A, Morrow G, Ketchum D, Kreeger J, Michelfelder E. Utility of serial fetal echocardiograms in detecting in-utero changes for single-ventricle lesions: an 11-year experience. Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology. 2025 May:65(5):575-580. doi: 10.1002/uog.29206. Epub 2025 Mar 27 [PubMed PMID: 40150909]
Bravo-Valenzuela NJ, Giffoni MC, Nieblas CO, Werner H, Tonni G, Granese R, Gonçalves LF, Araujo Júnior E. Three-Dimensional Ultrasound for Physical and Virtual Fetal Heart Models: Current Status and Future Perspectives. Journal of clinical medicine. 2024 Dec 13:13(24):. doi: 10.3390/jcm13247605. Epub 2024 Dec 13 [PubMed PMID: 39768529]
Level 3 (low-level) evidenceRudd NA, Ghanayem NS, Hill GD, Lambert LM, Mussatto KA, Nieves JA, Robinson S, Shirali G, Steltzer MM, Uzark K, Pike NA, American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Lifelong Congenital Heart Disease and Heart Health in the Young; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Clinical Cardiology; and Council on Lifestyle and Cardiometabolic Health. Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association. Journal of the American Heart Association. 2020 Aug 18:9(16):e014548. doi: 10.1161/JAHA.119.014548. Epub 2020 Aug 11 [PubMed PMID: 32777961]
Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A, Cuneo BF, Huhta JC, Jonas RA, Krishnan A, Lacey S, Lee W, Michelfelder EC Sr, Rempel GR, Silverman NH, Spray TL, Strasburger JF, Tworetzky W, Rychik J, American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014 May 27:129(21):2183-242. doi: 10.1161/01.cir.0000437597.44550.5d. Epub 2014 Apr 24 [PubMed PMID: 24763516]
Atz AM, Munoz RA, Adatia I, Wessel DL. Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly. The American journal of cardiology. 2003 Apr 1:91(7):906-8 [PubMed PMID: 12667588]
Cai J, Su Z, Shi Z, Zhou Y, Xu Z, Xu Z, Yang Y. Nitric oxide and milrinone: combined effect on pulmonary circulation after Fontan-type procedure: a prospective, randomized study. The Annals of thoracic surgery. 2008 Sep:86(3):882-8; discussion 882-8. doi: 10.1016/j.athoracsur.2008.05.014. Epub [PubMed PMID: 18721577]
Level 1 (high-level) evidenceMäkikallio K, McElhinney DB, Levine JC, Marx GR, Colan SD, Marshall AC, Lock JE, Marcus EN, Tworetzky W. Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention. Circulation. 2006 Mar 21:113(11):1401-5 [PubMed PMID: 16534003]
Level 2 (mid-level) evidenceTworetzky W, Wilkins-Haug L, Jennings RW, van der Velde ME, Marshall AC, Marx GR, Colan SD, Benson CB, Lock JE, Perry SB. Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation. 2004 Oct 12:110(15):2125-31 [PubMed PMID: 15466631]
Level 3 (low-level) evidenceFreud LR, McElhinney DB, Marshall AC, Marx GR, Friedman KG, del Nido PJ, Emani SM, Lafranchi T, Silva V, Wilkins-Haug LE, Benson CB, Lock JE, Tworetzky W. Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients. Circulation. 2014 Aug 19:130(8):638-45. doi: 10.1161/CIRCULATIONAHA.114.009032. Epub 2014 Jul 22 [PubMed PMID: 25052401]
Fletcher SE, Cheatham JP, Bolam DL. Primary transcatheter treatment of congenital pulmonary arteriovenous malformation causing cyanosis of the newborn. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions. 2000 May:50(1):48-51 [PubMed PMID: 10816279]
Level 3 (low-level) evidenceShafer KM, Garcia JA, Babb TG, Fixler DE, Ayers CR, Levine BD. The importance of the muscle and ventilatory blood pumps during exercise in patients without a subpulmonary ventricle (Fontan operation). Journal of the American College of Cardiology. 2012 Nov 13:60(20):2115-21. doi: 10.1016/j.jacc.2012.08.970. Epub 2012 Oct 17 [PubMed PMID: 23083785]
Goldberg DJ, Dodds K, Rychik J. Rare problems associated with the Fontan circulation. Cardiology in the young. 2010 Dec:20 Suppl 3():113-9. doi: 10.1017/S1047951110001162. Epub [PubMed PMID: 21087567]
Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia TY, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, d'Udekem Y, American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation. 2019 Aug 6:140(6):e234-e284. doi: 10.1161/CIR.0000000000000696. Epub 2019 Jul 1 [PubMed PMID: 31256636]
Prsa M, Holly CD, Carnevale FA, Justino H, Rohlicek CV. Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics. 2010 Mar:125(3):e625-30. doi: 10.1542/peds.2009-1678. Epub 2010 Feb 15 [PubMed PMID: 20156891]
Uzark K, Zak V, Shrader P, McCrindle BW, Radojewski E, Varni JW, Daniels K, Handisides J, Hill KD, Lambert LM, Margossian R, Pemberton VL, Lai WW, Atz AM, Pediatric Heart Network Investigators. Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation. The Journal of pediatrics. 2016 Mar:170():166-72.e1. doi: 10.1016/j.jpeds.2015.11.016. Epub 2015 Dec 10 [PubMed PMID: 26685073]
Level 2 (mid-level) evidenceAlsoufi B, Deshpande S, McCracken C, Kogon B, Vincent R, Mahle W, Kanter K. Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2015 Nov:48(5):792-8; discussion 798-9. doi: 10.1093/ejcts/ezu547. Epub 2015 Jan 18 [PubMed PMID: 25602055]
Level 2 (mid-level) evidenceAlsoufi B, Mahle WT, Manlhiot C, Deshpande S, Kogon B, McCrindle BW, Kanter K. Outcomes of heart transplantation in children with hypoplastic left heart syndrome previously palliated with the Norwood procedure. The Journal of thoracic and cardiovascular surgery. 2016 Jan:151(1):167-74, 175.e1-2. doi: 10.1016/j.jtcvs.2015.09.081. Epub 2015 Sep 28 [PubMed PMID: 26520008]
Northway WH Jr, Rosan RC, Porter DY. Pulmonary disease following respirator therapy of hyaline-membrane disease. Bronchopulmonary dysplasia. The New England journal of medicine. 1967 Feb 16:276(7):357-68 [PubMed PMID: 5334613]
Level 2 (mid-level) evidenceZhu A, Meza JM, Prabhu NK, McCrary AW, Allareddy V, Turek JW, Andersen ND. Survival After Intervention for Single-Ventricle Heart Disease Over 15 Years at a Single Institution. The Annals of thoracic surgery. 2022 Dec:114(6):2303-2312. doi: 10.1016/j.athoracsur.2022.03.060. Epub 2022 Apr 14 [PubMed PMID: 35430225]
Erikssen G, Liestøl K, Aboulhosn J, Wik G, Holmstrøm H, Døhlen G, Gjesdal O, Birkeland S, Hoel TN, Saatvedt KJ, Seem E, Thaulow E, Estensen ME, Lindberg HL. Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990-2015. BMJ open. 2023 Jul 25:13(7):e069531. doi: 10.1136/bmjopen-2022-069531. Epub 2023 Jul 25 [PubMed PMID: 37491095]
Level 2 (mid-level) evidenceDalén M, Odermarsky M, Liuba P, Johansson Ramgren J, Synnergren M, Sunnegårdh J. Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study. Journal of the American Heart Association. 2024 Mar 19:13(6):e031722. doi: 10.1161/JAHA.123.031722. Epub 2024 Mar 18 [PubMed PMID: 38497454]
Nagase T, Fujita S, Harada T, Hosoda R, Okamoto K, Oda S, Nakano T. Mid-term outcomes of classical hypoplastic left heart syndrome after Fontan procedure. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2025 Mar 4:67(3):. pii: ezaf052. doi: 10.1093/ejcts/ezaf052. Epub [PubMed PMID: 40120080]
Alsoufi B, Schlosser B, Mori M, McCracken C, Slesnick T, Kogon B, Petit C, Sachdeva R, Kanter K. Influence of Morphology and Initial Surgical Strategy on Survival of Infants With Tricuspid Atresia. The Annals of thoracic surgery. 2015 Oct:100(4):1403-9; discussion 1409-10. doi: 10.1016/j.athoracsur.2015.05.037. Epub 2015 Jul 30 [PubMed PMID: 26233275]
Mair DD, Puga FJ, Danielson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. Journal of the American College of Cardiology. 2001 Mar 1:37(3):933-9 [PubMed PMID: 11693773]
Level 2 (mid-level) evidenceFreud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzky W. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study. Circulation. 2015 Aug 11:132(6):481-9. doi: 10.1161/CIRCULATIONAHA.115.015839. Epub 2015 Jun 9 [PubMed PMID: 26059011]
Level 2 (mid-level) evidenceCelermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebstein's anomaly: presentation and outcome from fetus to adult. Journal of the American College of Cardiology. 1994 Jan:23(1):170-6 [PubMed PMID: 8277076]
Level 2 (mid-level) evidenceYeh T Jr, Williams WG, McCrindle BW, Benson LN, Coles JG, Van Arsdell GS, Webb GG, Freedom RM. Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 1999 Aug:16(2):111-6 [PubMed PMID: 10485406]
Level 2 (mid-level) evidenceBurkhart HM, Dearani JA, Mair DD, Warnes CA, Rowland CC, Schaff HV, Puga FJ, Danielson GK. The modified Fontan procedure: early and late results in 132 adult patients. The Journal of thoracic and cardiovascular surgery. 2003 Jun:125(6):1252-9 [PubMed PMID: 12830041]
Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM, Nicolson SC, Spray TL. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? The Journal of thoracic and cardiovascular surgery. 2002 Feb:123(2):237-45 [PubMed PMID: 11828282]
Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Müller J, Derrick G, Giannakoulas G, Khambadkone S, Lammers AE, Picchio FM, Gatzoulis MA, Hager A. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. European heart journal. 2010 Dec:31(24):3073-83. doi: 10.1093/eurheartj/ehq356. Epub 2010 Oct 7 [PubMed PMID: 20929979]
Level 2 (mid-level) evidenceMitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S, Spray TL. Intermediate outcomes after the Fontan procedure in the current era. The Journal of thoracic and cardiovascular surgery. 2006 Jan:131(1):172-80 [PubMed PMID: 16399309]
Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, Landzberg MJ. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008 Jan 1:117(1):85-92 [PubMed PMID: 18071068]
Itkin M, Piccoli DA, Nadolski G, Rychik J, DeWitt A, Pinto E, Rome J, Dori Y. Protein-Losing Enteropathy in Patients With Congenital Heart Disease. Journal of the American College of Cardiology. 2017 Jun 20:69(24):2929-2937. doi: 10.1016/j.jacc.2017.04.023. Epub [PubMed PMID: 28619193]
Dori Y, Itkin M. Etiology and new treatment options for patients with plastic bronchitis. The Journal of thoracic and cardiovascular surgery. 2016 Aug:152(2):e49-50. doi: 10.1016/j.jtcvs.2016.05.008. Epub 2016 May 10 [PubMed PMID: 27423853]
Schilling C, Dalziel K, Nunn R, Du Plessis K, Shi WY, Celermajer D, Winlaw D, Weintraub RG, Grigg LE, Radford DJ, Bullock A, Gentles TL, Wheaton GR, Hornung T, Justo RN, d'Udekem Y. The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry. International journal of cardiology. 2016 Sep 15:219():14-9. doi: 10.1016/j.ijcard.2016.05.035. Epub 2016 May 14 [PubMed PMID: 27257850]
Carins TA, Shi WY, Iyengar AJ, Nisbet A, Forsdick V, Zannino D, Gentles T, Radford DJ, Justo R, Celermajer DS, Bullock A, Winlaw D, Wheaton G, Grigg L, d'Udekem Y. Long-term outcomes after first-onset arrhythmia in Fontan physiology. The Journal of thoracic and cardiovascular surgery. 2016 Nov:152(5):1355-1363.e1. doi: 10.1016/j.jtcvs.2016.07.073. Epub 2016 Aug 30 [PubMed PMID: 27751239]
Bulic A, Zimmerman FJ, Ceresnak SR, Shetty I, Motonaga KS, Freter A, Trela AV, Hanisch D, Russo L, Avasarala K, Dubin AM. Ventricular pacing in single ventricles-A bad combination. Heart rhythm. 2017 Jun:14(6):853-857. doi: 10.1016/j.hrthm.2017.03.035. Epub [PubMed PMID: 28528723]
Serfas JD, Thibault D, Andersen ND, Chiswell K, Jacobs JP, Jacobs ML, Krasuski RA, Lodge AJ, Turek JW, Hill KD. The Evolving Surgical Burden of Fontan Failure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. The Annals of thoracic surgery. 2021 Jul:112(1):179-187. doi: 10.1016/j.athoracsur.2020.05.174. Epub 2020 Aug 5 [PubMed PMID: 32763267]
Sharma VJ, Iyengar AJ, Zannino D, Gentles T, Justo R, Celermajer DS, Bullock A, Winlaw D, Wheaton G, Burchill L, Cordina R, d'Udekem Y. Protein-losing enteropathy and plastic bronchitis after the Fontan procedure. The Journal of thoracic and cardiovascular surgery. 2021 Jun:161(6):2158-2165.e4. doi: 10.1016/j.jtcvs.2020.07.107. Epub 2020 Aug 12 [PubMed PMID: 32928546]
Lasa JJ, Glatz AC, Daga A, Shah M. Prevalence of arrhythmias late after the Fontan operation. The American journal of cardiology. 2014 Apr 1:113(7):1184-8. doi: 10.1016/j.amjcard.2013.12.025. Epub 2014 Jan 14 [PubMed PMID: 24513470]
Level 2 (mid-level) evidenceDurongpisitkul K, Porter CJ, Cetta F, Offord KP, Slezak JM, Puga FJ, Schaff HV, Danielson GK, Driscoll DJ. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation. Circulation. 1998 Sep 15:98(11):1099-107 [PubMed PMID: 9736597]
Chin AJ, Whitehead KK, Watrous RL. Insights after 40 years of the fontan operation. World journal for pediatric & congenital heart surgery. 2010 Oct:1(3):328-43. doi: 10.1177/2150135110379623. Epub [PubMed PMID: 23804889]
Egbe AC, Connolly HM, Niaz T, Yogeswaran V, Taggart NW, Qureshi MY, Poterucha JT, Khan AR, Driscoll DJ. Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation. American heart journal. 2017 Jan:183():10-17. doi: 10.1016/j.ahj.2016.09.014. Epub 2016 Oct 4 [PubMed PMID: 27979032]
Mahnke CB, Boyle GJ, Janosky JE, Siewers RD, Pigula FA. Anticoagulation and incidence of late cerebrovascular accidents following the Fontan procedure. Pediatric cardiology. 2005 Jan-Feb:26(1):56-61 [PubMed PMID: 14994183]
Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery. Journal of cardiothoracic and vascular anesthesia. 2011 Apr:25(2):320-34. doi: 10.1053/j.jvca.2010.12.003. Epub [PubMed PMID: 21477759]
Level 1 (high-level) evidenceMatthews IL, Fredriksen PM, Bjørnstad PG, Thaulow E, Gronn M. Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiology in the young. 2006 Jun:16(3):261-7 [PubMed PMID: 16725065]
Level 2 (mid-level) evidenceIdorn L, Hanel B, Jensen AS, Juul K, Reimers JI, Nielsen KG, Søndergaard L. New insights into the aspects of pulmonary diffusing capacity in Fontan patients. Cardiology in the young. 2014 Apr:24(2):311-20. doi: 10.1017/S1047951113000358. Epub 2013 Apr 3 [PubMed PMID: 23552344]
Level 2 (mid-level) evidenceRychik J. Protein-losing enteropathy after Fontan operation. Congenital heart disease. 2007 Sep-Oct:2(5):288-300. doi: 10.1111/j.1747-0803.2007.00116.x. Epub [PubMed PMID: 18377444]
Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, Puga FJ, Danielson GK. Protein-losing enteropathy after the Fontan operation. The Journal of thoracic and cardiovascular surgery. 1996 Sep:112(3):672-80 [PubMed PMID: 8800155]
Ting J, Roy S, Navuluri S, Hanfland R, Mulcahy L, Yuksel S, Huang Z, Jiang ZY. Airway evaluation in children with single ventricle cardiac physiology. International journal of pediatric otorhinolaryngology. 2018 Aug:111():115-118. doi: 10.1016/j.ijporl.2018.06.004. Epub 2018 Jun 5 [PubMed PMID: 29958593]
Cohen MS, Zak V, Atz AM, Printz BF, Pinto N, Lambert L, Pemberton V, Li JS, Margossian R, Dunbar-Masterson C, McCrindle BW. Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome. American heart journal. 2010 Dec:160(6):1092-8, 1098.e1. doi: 10.1016/j.ahj.2010.07.039. Epub [PubMed PMID: 21146663]
Level 2 (mid-level) evidenceLiptzin DR, Di Maria MV, Younoszai A, Narkewicz MR, Kelly SL, Wolfe KR, Veress LA. Pulmonary Screening in Subjects after the Fontan Procedure. The Journal of pediatrics. 2018 Aug:199():140-143. doi: 10.1016/j.jpeds.2018.03.050. Epub 2018 May 7 [PubMed PMID: 29747936]
Johnson JT, Lindsay I, Day RW, Van Dorn CS, Hoffman J, Everitt MD, Yetman AT. Living at altitude adversely affects survival among patients with a Fontan procedure. Journal of the American College of Cardiology. 2013 Mar 26:61(12):1283-9. doi: 10.1016/j.jacc.2013.01.008. Epub 2013 Feb 13 [PubMed PMID: 23414794]
Level 2 (mid-level) evidence